The Unfavorable Result in Plastic Surgery_ Avoidance and Treatment (2018, Thieme)

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716
necessary to improve function and appearance or manage
unfavorable results, it is important to individualize proce­
dures based on extensive evaluation while understanding
the limitations of each technique for the specific patient.
Several residual deformities can be corrected with revision
procedures, but multiple revisions in a short period of time
can result in additional scarring with little chance for fur­
ther improvement and should be discouraged.
Conclusion
Patients with clefts of the lip and/or palate will require sev­
eral procedures and intervention from birth to adulthood
to achieve complete habilitation. Superior results can be
achieved with appropriate and extensive evaluation, short-
and long-term planning with optimal timing for each pro­
cedure, and close cooperation among various members of
the cleft team. When additional surgical procedures are
Fig. 40.32 (a–d) A 42-year-old who presented with residual cleft nasal deformity and airway obstruction after three previous corrective
procedures. (e–g) Postoperative result 1 year after nose and lip revision. She has improvement of breathing and olfaction and no upper
respiratory infections for longer than a year (as opposed to the constant infections she experienced before surgery).
a
b
c
d
e
f
g
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719
Chapter 40 Cleft Lip and Cleft Palate
Discussion by Richard E. Kirschner and Oksana A. Jackson
Avoiding unfavorable results in cleft lip and palate care
requires thoughtful preoperative planning, careful
operative technique, and longitudinal team care into
adulthood, including timely secondary interventions
when appropriate. Some complications are unexpected
and unavoidable, such as acute traumatic dehiscence
after cleft lip repair, but many untoward postoperative
events can be avoided by recognizing patients at risk
for unfavorable outcomes and modifying protocols ac­
cordingly.
Patient-, surgeon-, and environment-related fac­
tors can all affect outcomes after primary cleft lip and
palate repair, and only some of these may be control­
lable. Thoughtful preoperative assessment of each pa­
tient’s unique cleft anatomy, medical comorbidities, and
wound-healing ability, as well as the caregivers’ ability
to understand and comply with postoperative care, is
the first critical step. In some cases, preoperative modi­
fications can minimize risk of complications and result
in improved aesthetic outcomes, as in the case of preop­
erative treatments for complete clefts of the lip. In oth­
ers, modifications in surgical protocol and/or technique
may be necessary; for example, choosing to stage the
repair of extremely wide clefts can diminish the risk of
dehiscence or fistula formation. Surgical technique, me­
ticulous tissue handling, choice of suture materials, and
postoperative management are all of critical importance
in optimizing outcomes.
Unfavorable aesthetic and speech results after cleft
lip and cleft palate repair, as well as oronasal fistulas,
can significantly impact the quality of life for patients
and their families. Complications after surgery can
condemn patients to repeated operations that can have
deleterious effects on facial growth as well as speech,
language, and psychosocial development. Optimal out­
comes in cleft surgery can best be achieved by expe­
rienced surgeons following established protocols and
supported by teams of medical specialists in a multi­
disciplinary care setting.
Avoiding Unfavorable Results and
Complications in Cleft Lip Repair
Our protocol for managing a cleft lip and our preferred
surgical techniques for cleft lip repair are detailed here,
followed by several approaches to correction of re­
sidual lip deformities. The goal of definitive lip repair
is to approach normal and symmetrical nasolabial ap­
pearance and function. With careful planning and me­
ticulous surgical technique, the need for major surgical
revision should be rare. Minor revisions still may be
necessary, such as trimming mucosal excess from the
lateral lip element or secondary nasal revision, despite
the performance of tip rhinoplasty at the time of pri­
mary lip repair.
Cleft lip repair is most often performed between 3
and 6 months of age. For complete clefts of the lip, we
advocate preoperative treatment of the cleft to improve
the position of the maxillary segments, reduce cleft
width, and improve nasal form. Nasoalveolar molding
is preferred; however, lip adhesion with placement of
nasal conformers can often accomplish the same ana­
tomic changes, and lip taping alone can lead to signifi­
cant improvements. These techniques reduce tension of
the lip closure, thus minimizing the risk of dehiscence
from tension alone; improve nasal tip contour; and re­
sult in better healing and scar formation. This is particu­
larly relevant for bilateral complete cleft lip, for which
these treatments can radically reposition the displaced
central premaxillary segment and restore the effaced
columella. Asymmetrical bilateral cleft lip is a unique
challenge, particularly if one side is complete. In this
circumstance, staged repair with unilateral lip adhesion
on the complete side can ultimately accomplish greater
symmetry of the lip and nose.
My technique (R.K.) for repair by a modified Mil­
lard’s rotation-advancement with primary rhinoplasty
is described here. Careful measurement and incisional
design are of critical importance; a lip that is too long or
too short or otherwise unbalanced is most often the re­
sult of an intraoperative error in design. Although post­
operative scar contracture can result in minor elevation
of the Cupid’s bow despite proper surgical design, a lip
that is too short on the table will always remain so post­
operatively.
Markings are made of the key anatomic landmarks
at the vermilion–cutaneous junction and include the
following:
•• Nadir of the Cupid’s bow
•• Peak of the Cupid’s bow on the noncleft side
•• Peak of the Cupid’s bow on the cleft side at an equal
distance as the peak of the Cupid’s bow on the cleft
side
In a very short lip, additional length may be gained by
slightly narrowing the Cupid’s bow. The shape of the
philtral column is marked on the noncleft side, and the
rotation incision is drawn to mirror this shape, curving
across the upper philtrum at the columellar–lip junc­

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
720
tion. The lateral margin of the C-flap is then marked;
the C-flap will be used to lengthen the columella on the
cleft side and to reconstruct the medial nasal sill.
The lateral lip incisions are performed as follows:
  1. The length of the lip on the noncleft side from the
alar base to the Cupid’s bow peak is measured and
transposed to the cleft side to determine and mark
the position of Cupid’s bow peak on the lateral lip
element at the vermilion–cutaneous junction.
  2. A second mark is placed at the crest of the white
roll to create an equilateral triangular white roll
flap. This modification adds a small amount of ad­
ditional lip length and also breaks up the scar as it
crosses the vermilion–cutaneous juction, thereby
avoiding flattening the lip at the white roll and ef­
facing the normal vermilion–cutaneous interface.
  3. The advancement flap is drawn as a slightly
curved line extending superiorly from the superior
base of the white roll flap.
  4. Transverse incisions across the superior aspect
of the lateral lip element should be minimized or
avoided altogether.
  5. A triangular flap is created on the lateral lip seg­
ment of the excess vermilion to be inset into a
backcut at the red line on the medial lip element.
This modification achieves several goals:
•• Evens the width of the vermilion across the
repair
•• Creates a continuous and symmetrical red line
•• Prevents a whistle deformity at the site of the
repair
  6. Mucosal incisions mirror the rotation and ad­
vancement incisions on the cutaneous surface.
  7. An incision in the upper buccal sulcus of the
lateral lip element is made and used to dissect the
entire alar–lip complex from the maxilla. This is
critical to allow symmetrical repositioning of the
displaced alar base on the cleft side.
  8. After the incisions are made, the orbicularis
muscle is dissected from the medial and lateral lip
elements.
  9. A synchronous tip rhinoplasty is performed by
dissecting between the medial crura and over the
alar domes to free the skin and subcutaneous fat
from the underlying cartilage.
10. Transnasal mattress sutures are then used to
symmetrize the medial crura and to unite and
symmetrize the alar domes.
11. The mucosa is closed with interrupted 5–0
chromic sutures, and the muscle is repaired with
interrupted 5–0 polyglactin horizontal mattress
sutures to construct a philtral ridge on the cleft
side.
12. The vermilion is repaired and the vermilion flap
inset with 6–0 chromic. Absorbable buried deep
dermal sutures and a skin adhesive obviate the
need for suture removal and prevent suture marks
on the skin (Fig. 40D.1).
For the skin design, I (O.J.) choose between an anatomic
subunit approach as described by David Fisher1 and a
modified Mohler repair2 depending on the cleft config­
uration and the discrepancy between philtral column
a
b
c
Fig. 40D.1 (a) Markings for a unilateral cleft lip repair. (b) Completed incisions.
(c) The dissected orbicularis. (d) Final repair.
d

721
Chapter 40 Cleft Lip and Cleft Palate
heights. When the difference between the cleft and
noncleft philtral columns exceeds 2.5 mm, the modi­
fied Mohler approach is performed:
  1. Markings begin by dotting a central line down the
tip of the nose, which ends at the center of the
columella in the lip–columellar crease.
  2. In the lip-columellar crease, points are placed at
the top of the cleft and noncleft philtral columns,
then additional marks are added, bisecting the
distance between the center point and the points
at the top of the columns on each side; these rep­
resent the base of the columellar triangle.
  3. A point is then placed on the columella about
1 mm above the center point, which represents
the top of this triangle.
  4. Additional key anatomic landmarks are marked
similar to those described previously in the
vermilion–cutaneous junction:
•• Nadir of the Cupid’s bow
•• Peak of the Cupid’s bow on the noncleft side
•• Peak of the Cupid’s bow on the cleft side at an
equal distance as the peak of Cupid’s bow on the
cleft side
  5. The noncleft philtral column is identified and
marked along its most prominent ridge, which
typically forms a curved line because of distortion
by the orbicularis oris muscle.
  6. The center of the philtrum is marked in a parallel
curvilinear fashion.
  7. The rotation incision is then drawn to parallel to
these lines at its base. About midway up the lip, it
curves back to allow it to join the same side limb
of the columellar triangle as a straight line, and
overall forming a gentle S-curve. Thus when this
incision is made and rotated inferiorly, additional
length is gained by displacement of the columellar
triangle as well as straightening of the S-curve.
  8. The C-flap is then marked.
  9. Markings on the lateral lip are very similar to
those described previously, including use of a
white roll triangle from the lateral lip to be inset
into a backcut at the vermilion–cutaneous junc­
tion on the medial lip segment.
10. Use of a vermilion triangle to even the height of
the dry vermilion, handling of the muscle, and tip
rhinoplasty approach are similar to those de­
scribed previously.
11. Distal septal repositioning is also performed by
elevating the distal septum off its displaced at­
tachment to the noncleft side of the anterior nasal
spine and shifting it centrally. A nasal conformer
is used for 1 month postoperatively to retain the
septal and nasal dome position.
12. The skin is closed with a few 7–0 Vicryl sutures in
the deep dermis, followed by cutaneous sutures of
7–0 Prolene, which are removed 5 days postop­
eratively in the office and replaced by Steri-Strips
(Fig. 40D.2).
Atraumatic tissue handling and meticulous suture
placement help to optimize scar formation. Still, a
near-perfect on-the-table repair may subsequently
heal with a hypertrophic scar that can result in short­
ening and tightness of the lip and an unfavorable out­
come. Our postoperative protocol includes postopera­
tive nasal stenting for 1 month, as well as aggressive
postoperative scar management with twice daily scar
massage and topical silicone treatments. Close follow-
up is important, and worsening or persistent hypertro­
phy of the scar can be treated early and usually success­
fully with corticosteroid injections.
Management of Secondary Lip
Deformities
A wide variety of secondary lip deformities occur and
can be treated with a range of techniques. It is impor­
tant to note, however, that optimal outcomes after sec­
ondary lip repair require precision in diagnosis and a
complete understanding of the anatomy and the cause
of the secondary deformity.
Fig. 40D.2 (a) Intraoperative markings for a modified Mohler unilateral cleft lip repair. (b) Immediate postoperative appearance
after lip repair and tip rhinoplasty.
a
b

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
722
In many cases, best results can be achieved by tak­
ing down the primary repair and completely repairing
the lip. Careful consideration of timing for revision is
important and can impact ultimate results. Repeated
interventions in early childhood can negatively affect
facial growth. Noncompliance with postoperative care
because of age, activity level, or lack of understanding
can undermine results. These considerations also need
to be balanced by the negative psychosocial effects on
the child and family of unacceptable scarring or naso­
labial appearance.
Vermilion Whistle Deformity
The vermilion whistle deformity is characterized by
deficiency of the red lip creating a visible gap with gen­
tle lip closure at the cleft site unilaterally or centrally
bilaterally. It can be treated by a variety of techniques.
As noted previously, the best approach may often be
complete takedown and repair. When camouflage is
elected, autologous fat grafting can be used to fill the
site successfully; however, scarred tissues and a tight
mucosal envelope can limit the volume to be injected
and the amount of fat that survives where it is most
needed. A revision technique using a combination of
local tissue rearrangement with dermal–fat grafting is
preferred when the deformity is significant:
1. A dermal–fat graft is typically harvested from the
previous iliac bone harvest site to avoid an addi­
tional scar.
2. An ellipse is scored and deepithelialized, and full-
thickness dermis and fat is harvested.
3. Typically, a V-Y approach is used for elevation of the
mucosa, hinged at the free vermilion border. The
incisions are not extended beyond the vermilion
free border to keep them concealed.
4. Once the flap is raised, dissection is performed in
the subcutaneous space just beyond the entire area
of deficiency, including the vermilion–cutaneous
junction area.
5. A tunnel is developed laterally to accommodate the
lateral portions of the graft.
6. The graft is shaped with maximal fat thickness
centrally. The lateral limbs contain dermis only and
are advanced through premade subcutaneous tun­
nels and secured with mattress sutures tied on the
mucosal surface close to the oral commissures to
hold the graft in place.
7. The dermal surface of the graft is then sutured to
the muscular surface with fine absorbable sutures.
This orientation is important because the muscular
surface should provide excellent vascularization of
the dermis and it allows trimming of the fat before
closure for a more precise result.
8. The flap is then replaced and closed as a Y to
provide sufficient tissue to correct the surface defi­
ciency (Fig. 40D.3).
A shallow upper buccal sulcus can result from muco­
sal scarring or deficiency and can tether the lip and
interfere with dental care and oral hygiene. This can
Fig. 40D.3 (a) A central whistle deformity in a patient with a repaired bilateral cleft lip. (b) Design of the V-Y advancement incision
hinged on the free vermilion border. (c) The V-flap is elevated and the graft secured in place with the dermal surface of the graft
sutured to the underlying orbicularis muscle. (d) Immediate postoperative appearance after closure of the incisions.
a
b
c
d

723
Chapter 40 Cleft Lip and Cleft Palate
be treated with local advancement of the lateral buc­
cal mucosa. A premaxillary-based semicircular flap
centered over the area of greatest severity is designed
with incisions that continue laterally just above the up­
per buccal sulcus on both sides past the deformity. The
flap is raised in the supraperisosteal plane, and addi­
tional supraperiosteal dissection continues superiorly
along the maxilla. The flap is then sutured to the peri­
osteum and closure is accomplished by advancing the
lateral buccal mucosa equally on both sides and sewing
it to the border of this flap. In the midline the buccal
mucosa is closed side to side, lengthening the central
lip on its mucosal surface and creating a deeper upper
buccal space.
Avoiding Unfavorable Results and
Complications in Cleft Palate Repair
Unfavorable results after cleft palate repair include oro­
nasal fistula formation and velopharyngeal dysfunc­
tion. The former results from inadequate healing of the
repair, usually the result of excessive tension or poor
tissue quality. Pedicle injury can result from introaop­
ertive laceration or from excessive tension on the ves­
sels during closure. This may lead to the devastating
complication of flap necrosis and the formation of a
large anterior fistula.
Fistula formation can be avoided by meticulous
dissection, gentle tissue handling, careful suturing, and
eliminating tension at closure. Traumatic dehiscence of
the repair is uncommon and should be treated with im­
mediate repair.
The occurrence of velopharyngeal dysfunction af­
ter cleft palate repair, however, can be linked to sur­
gical technique or to intrinsic patient factors such as
cleft anatomy, pharyngeal dimensions, or neuromuscu­
lar function. The choice and execution of palatoplasty
technique can significantly affect speech outcomes, but
many patient factors are not controllable.
Our preferred palatoplasty technique is the Furlow
double-opposing z-plasty with liberal use of lateral re­
laxing incisions to reduce tension on the closure; de­
tails of the technique have been previously reported.3–5
Our protocol is to proceed with palate repair at 9 to 12
months of age in most patients. Many authors have re­
ported improved speech outcomes using this technique
as well as very low rates of postoperative oronasal fis­
tula formation.3,4,6–13 The opposing z-plasty design has
several advantages6:
•• Few overlapping suture lines, minimizing the risk of
postoperative oronasal fistula formation
•• Palatal lengthening accomplished by the geometric
tissue rearrangement
•• Decreased potential for longitudinal scar contracture
during healing, reducing the risk of velar shortening
•• Improved muscle viability and reduced muscle
scarring because of dissection on only one surface of
the muscle and absence of suture placement within
the muscle
Even with relaxing incisions, some clefts may be dif­
ficult to repair without tension, such as the U-shaped
palatal cleft characteristic of Pierre Robin sequence or
the wide bilateral complete cleft. Recognizing the chal­
lenging palate repair preoperatively and modifying the
timing or technique is critical to avoiding fistula forma­
tion. In these patients, width of the defect, configura­
tion of the palatal vault, and hypoplasia of the palatal
shelves may contribute to a deformity that cannot eas­
ily be repaired. Delaying palate repair to 18 years of age
can be beneficial, because narrowing of the maxillary
arch and transverse growth of the palatal shelves often
occurs with time, thereby narrowing cleft width.
Successful uncomplicated repair also can be ac­
complished with a staged approach to palate repair
by performing (1) an early adhesion of the soft pal­
ate followed by (2) a delayed repair of the hard palate
and soft palate musculature after some postoperative
narrowing of the cleft has occurred. Another approach,
either planned in advance or added intraoperatively
when excessive tension is encountered unexpectedly
during closure, is to incorporate one or two buccal
myomucosal flaps as described by Mann and Fisher.14
It cannot be stressed enough that in the challenging
wide palatal cleft, a thoughtful conservative approach
is advocated.
It is often noted that with each subsequent attempt
at oronasal fistula repair, the liklihood of success­
ful healing diminishes multifold. The impediments to
proper healing after repair (i.e., scarring, stiffness of the
tissues, and reduced tissue vascularity) increase with
each attempt. We agree that more extensive procedures
to bring healthy tissue without tension to the area, pref­
erably in multiple layers, are the key to successful clo­
sure. A small central hard palate fistula will likely not
heal with local undermining and direct closure. Wide
elevation of the palatal mucoperisoteum with relax­
ing incisions to accomplish tension-free nasal and oral
closure is recommended. In addition, we advocate the
placement of acellular dermal matrices between the two
layers to support the repair. Even if partial dehiscence
occurs and the matrix becomes exposed, complete heal­
ing typically occurs by tissue ingrowth and remucosal­
ization over the matrix. Several reports document im­
proved rates of success with this technique.15,16
Healthy local tissues from the buccal mucosa or
tongue should be used without hesitation when needed.

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
724
Finally, the contribution of local trauma to the repair as
a result of feeding or tongue thrusting should not be un­
derestimated. Protecting the repair with a prefabricated
acrylic splint is a useful adjunct to surgery.
Our protocol for treating velopharyngeal dysfunc­
tion after cleft palate repair begins with comprehensive
assessment of the patient’s velopharyngeal anatomy and
function. Previous surgical procedures, severity of velo­
pharyngeal dysfunction, and imaging of the velopharyn­
geal mechanism all factor into surgical planning. Imag­
ing should be performed with nasopharyngoscopy and/
or multiview videofluoroscopy during speech to assess
the degree, pattern, and level of velopharyngeal closure.
Our philosophy is to first restore the normal palatal
anatomy and optimize palatal function, then proceed
with pharyngoplasty as necessary. Thus patients pre­
senting with previous straight-line repair with or with­
out incomplete intravelar veloplasty are first treated by
revision to the Furlow palatoplasty.
The midline scar is divided, and soft palatal flaps
are raised in the standard fashion. The result is appro­
priate muscle repositioning in addition to greater pala­
tal length. This technique has been successful in many
cases, particularly if the velopharyngeal gap is 5 mm or
less.17–19 If the velopharyngeal gap is small (< 5 mm), the
levator sling is appropriately reconstructed, and good
palatal motion is present, palatal lengthening with bi­
lateral buccinator myomucosal flaps overlapped at the
hard palate–soft palate junction can restore a normal
mechanism without significantly altering pharyngeal
anatomy. This technique is described in further detail by
Hens et al.20 and Mann et al.21 Finally, larger velopharyn­
geal gaps are treated with pharyngoplasty procedures,
the selection of which is based on the pattern of closure.
Coronal closure patterns with poor lateral pharyn­
geal wall motion are best treated by sphincter pharyngo­
plasty, whereas circular or sagittal patterns are best ad­
dressed by superiorly based posterior pharyngeal flaps.
Pharyngoplasty outcomes are optimized by placement
of the sphincter or pharyngeal flap precisely at the level
of attempted velopharyngeal closure as determined by
preoperative imaging. Evidence of significant tonsillar
or adenoid hypertrophy should be treated with removal
several months before VPI surgery to reduce the risk of
obstructive sleep apnea and to clear the surgical field
of lymphatic tissues, thus allowing for proper pharyn­
goplasty placement. This stepwise approach can suc­
cessfully restore velopharyngeal function and normalize
speech without undue additional complications.
Conclusion
Long-term experience with cleft surgery has taught us
of the critical importance of the initial repair in achiev­
ing optimal aesthetic and functional results. Unfavor­
able outcomes after primary cleft lip or cleft palate
repair can have long-standing deleterious effects on
patients’ quality of life. Secondary cleft deformities are
exceedingly challenging to correct, and too often their
treatment produces outcomes that remain suboptimal.
Careful planning and meticulous surgical technique
are essential to success. Anticipating a challenging case
and proceeding thoughtfully and conservatively is of
utmost importance to avoid unfavorable results.
References
1. Fisher DM. Unilateral cleft lip repair: an anatomical
subunit approximation technique. Plast Reconstr Surg
2005;116(1):61–71
2. Mohler LR. Unilateral cleft lip repair. Plast Reconstr
Surg 1987;80(4):511–517
3. Kirschner RE, Wang P, Jawad AF, et al. Cleft-palate re­
pair by modified Furlow double-opposing Z-plasty:
the Children’s Hospital of Philadelphia experience.
Plast Reconstr Surg 1999;104(7):1998–2010, discus­
sion 2011–2014
4. LaRossa D, Jackson OH, Kirschner RE, et al. The Chil­
dren’s Hospital of Philadelphia modification of the
Furlow double-opposing z-palatoplasty: long-term
speech and growth results. Clin Plast Surg 2004;31(2):
243–249
5. Randall P, LaRossa D, Solomon M, Cohen M. Experi­
ence with the Furlow double-reversing Z-plasty for
cleft palate repair. Plast Reconstr Surg 1986;77(4):
569–576
6. Furlow LT Jr. Cleft palate repair by double opposing
Z-plasty. Plast Reconstr Surg 1986;78(6):724–738
7. Jackson O, Stransky CA, Jawad AF, et al. The Children’s
Hospital of Philadelphia modification of the Furlow
double-opposing Z-palatoplasty: 30-year experience
and long-term speech outcomes. Plast Reconstr Surg
2013;132(3):613–622
8. Yu CC, Chen PK, Chen YR. Comparison of speech re­
sults after Furlow palatoplasty and von Langenbeck
palatoplasty in incomplete cleft of the secondary pal­
ate. Chang Gung Med J 2001;24(10):628–632
9. Dong Y, Dong F, Zhang X, et al. An effect comparison
between Furlow double opposing Z-plasty and two-
flap palatoplasty on velopharyngeal closure. Int J Oral
Maxillofac Surg 2012;41(5):604–611
10. Horswell BB, Castiglione CL, Poole AE, Assael LA. The
double-reversing Z-plasty in primary palatoplasty:
operative experience and early results. J Oral Maxil­
lofac Surg 1993;51(2):145–149, discussion 149–150
11. McWilliams BJ, Randall P, LaRossa D, et al. Speech
characteristics associated with the Furlow palato­
plasty as compared with other surgical techniques.
Plast Reconstr Surg 1996;98(4):610–619, discussion
620–621
12. Khosla RK, Mabry K, Castiglione CL. Clinical outcomes
of the Furlow Z-plasty for primary cleft palate repair.
Cleft Palate Craniofac J 2008;45(5):501–510

725
Chapter 40 Cleft Lip and Cleft Palate
13. Williams WN, Seagle MB, Pegoraro-Krook MI, et al.
Prospective clinical trial comparing outcome mea­
sures between Furlow and von Langenbeck Palato­
plasties for UCLP. Ann Plast Surg 2011;66(2):154–163
14. Mann RJ, Fisher DM. Bilateral buccal flaps with double
opposing Z-plasty for wider palatal clefts. Plast Recon­
str Surg 1997;100(5):1139–1143, discussion 1144–
1145
15. Kirschner RE, Cabiling DS, Slemp AE, Siddiqi F, LaRos­
sa DD, Losee JE. Repair of oronasal fistulae with acel­
lular dermal matrices. Plast Reconstr Surg 2006;
118(6):1431–1440
16. Aldekhayel SA, Sinno H, Gilardino MS. Acellular der­
mal matrix in cleft palate repair: an evidence-based
review. Plast Reconstr Surg 2012;130(1):177–182
17. Hudson DA, Grobbelaar AO, Fernandes DB, Lentin R.
Treatment of velopharyngeal incompetence by the
Furlow Z-plasty. Ann Plast Surg 1995;34(1):23–26
18. Chen PK, Wu JT, Chen YR, Noordhoff MS. Correction
of secondary velopharyngeal insufficiency in cleft
palate patients with the Furlow palatoplasty. Plast
Reconstr Surg 1994;94(7):933–941, discussion 942–
943
19. Noorchashm N, Dudas JR, Ford M, et al. Conversion
Furlow palatoplasty: salvage of speech after straight-
line palatoplasty and “incomplete intravelar veloplas­
ty”. Ann Plast Surg 2006;56(5):505–510
20. Hens G, Sell D, Pinkstone M, et al. Palate lengthening
by buccinator myomucosal flaps for velopharyngeal
insufficiency. Cleft Palate Craniofac J 2013;50(5):e84–
e91
21. Mann RJ, Neaman KC, Armstrong SD, Ebner B, Ba­
jnrauh R, Naum S. The double-opposing buccal flap
procedure for palatal lengthening. Plast Reconstr
Surg 2011;127(6):2413–2418

726

Craniosynostosis
Peter J. Taub and Joseph E. Losee
CHAPTER
41
Normal skull growth and potential neurologic function
may be affected by the presence of continued pressure in
any one area of the cranium or by the presence of one or
more pathologically fused cranial sutures. Posterior defor­
mational plagiocephaly, most often secondary to a supine
sleeping position, will generally resolve with positional
changes, physiotherapy, or helmet therapy and is only
rarely a surgical condition. The presence of a pathologi­
cally fused suture will produce deformity of the skull and
is frequently an indication for operative release. The fused
sutures determine the phenotype of the craniofacial defor­
mity. According to Virchow’s Law, bone growth can no lon­
ger occur across (perpendicular to) the affected suture and
therefore only occurs along it (parallel)1 (Fig. 41.1).
Craniosynostosis can be classified into simple (single su­
ture) versus complex (multiple sutures) and nonsyndromic
versus syndromic subtypes. Assessment of an infant with
an abnormal head shape includes a detailed medical his­
tory and physical examination to rule out a positional cause
and identify any complicating sequelae. The medical his­
tory should identify any history of skull irregularities, as­
sociated syndromes, family history of calvarial deformities,
and symptoms of intracranial hypertension (headache or
vomiting, developmental changes, irritability, or oculomo­
tor paresis). Often, parents note an abnormal head shape
at the time of delivery or one that becomes more apparent
later in infancy. Patients with irritability, feeding difficul­
ties, failure to thrive, headache, developmental delays, visual
changes, marked turricephaly, supraorbital recession, bulg­
ing or “tight” fontanels, abnormal distended scalp veins, or
a lack of circumferential skull growth may have an increase
in intracranial pressure.
For patients with head shape abnormalities, a careful
physical examination should be able to identify the cause.
Positional deformation presents with a classic parallelo­
gram appearance when looking from above. Skull deforma­
tion caused by premature fusion produces characteristic
findings, including asymmetry (supraorbital retrusion, tow­
ering or turricephaly, frontal or occipital bossing), inability
to palpate the anterior fontanelle (which normally remains
open until 12 to 18 months of age), perisutural ridging, and
signs of intracranial hypertension (papilledema). Studies
have shown that the presence of intracranial hypertension
is dependent on the number of affected sutures, ranging
from approximately 14% for single-suture synostosis to ap­
proximately 47% in multisuture synostosis.2,3 Suture release
in craniosynostosis is often offered to correct the craniofa­
cial deformity and to address potential increased intracra­
nial pressure that in some patients has resulted in blindness
and developmental delay.
Fig. 41.1 Virchow’s law: Bone growth can no longer occur across
(perpendicular to) a fused suture and therefore only occurs along
it (parallel).

727
Chapter 41 Craniosynostosis
ipsilateral frontal bossing in the absence of palpable lambdoid
suture ridge, and no mastoid bossing as is seen with lamb­
doid suture fusion (Fig. 41.2). Posterior plagiocephaly caused
by lambdoid suture synostosis is rare; the majority of posterior
plagiocephaly is secondary to positional molding. Understand­
ing the phenotypic differences between lambdoid synostosis
and posterior deformational plagiocephaly is critical for mak­
ing the appropriate diagnosis and designing the proper course
of treatment. Head circumferences, cranial indices, and anthro­
pometric measurements should also be noted.
Imaging studies may be necessary to confirm the diag­
nosis and rule out any associated intracranial abnormalities.
For younger patients, ultrasonography has been used as a
screening tool to visualize the sutures through the open fon­
tanelles. Although not as sensitive as computed tomography
(CT), it avoids the risk of radiation exposure. CT remains the
most sensitive modality to assess bony sutural fusion and
may suggest elevated intracranial pressure, as noted by areas
of erosion of the inner calvarial table (“copper-beaten” ap­
pearance) (Fig. 41.3). Pathognomonic for unicoronal synos­
tosis, elevation of the ipsilateral orbit can be seen secondary
to superior displacement of the greater wing of the sphenoid,
also known as the “harlequin” deformity. Modern CT proto­
cols attempt to minimize the radiation dose to the infant.4
Both CT and magnetic resonance imaging (MRI) are helpful
in evaluating the underlying brain for structural or functional
abnormalities, including hydrocephalus, holoprosencephaly,
cortical dysplasias, and Chiari malformations. The latter are
structural defects in the part of the brain that controls bal­
ance (cerebellum). Without sufficient space, the cerebellum
and parts of the brain stem may sit too low, causing pressure
and blocking the flow of cerebrospinal fluid. This is important
to identify preoperatively, because intraoperative maneuvers
may worsen the condition.
The specific calvarial shape can indicate which sutures
are involved. Children with sagittal synostosis present with
a narrow, elongated skull (dolichocephaly describes long-
headedness; scaphocephaly describes a boat-shaped head).
Depending on the region of greatest fusion of the sagittal su­
ture, the child may manifest frontal or occipital bossing or a
combination of both. Some children will also demonstrate a
“towering” skull, also known as turricephaly. Metopic synos­
tosis is marked by a variable degree of phenotypic severity,
ranging from mild ridging to the formation of a triangular
head (trigonocephaly) or prominent “keel” forehead with
or without hypotelorism. Although an endocranial ridge is
not commonly seen in patients with metopic synostosis, an
endocranial notch can be observed on axial CT images and
is virtually diagnostic of pathologic suture fusion. Patients
with unicoronal synostosis present with anterior plagio­
cephaly, whereas those with bilateral coronal involvement
demonstrate brachycephaly. Phenotypic features of anterior
plagiocephaly include ipsilateral perisutural ridging, fore­
head flattening, and orbital recession, coupled with contra­
lateral compensatory frontal bossing. Facial deformities are
also common, including nasal root displacement toward the
ipsilateral side, anterior displacement of the ipsilateral ear,
and chin deviation toward the contralateral side, resulting in
a classic C-shaped craniofacial deformity—concave contralat­
eral to the fused suture. Children with lambdoid synostosis
characteristically have a trapezoid-shaped head (seen from
the vertex view) in association with posterior displacement
of the ipsilateral ear, contralateral occipital bossing, ipsilat­
eral mastoid bossing, and ridging of the affected lambdoid
suture.
In contrast, posterior deformational plagiocephaly is
marked by a parallelogram-shaped head when viewed
from the vertex, anterior displacement of the ipsilateral ear,
Fig. 41.2 Clinical lambdoid synostosis.

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
728
lay, psychosocial dysfunction). Patients with multisutural
synostoses present with increased severity of physical and
neurologic symptoms; therefore surgical intervention is
even more important. The optimal surgical age has been
debated, because the techniques of surgery are variable.
Although the literature is inconclusive regarding the ap­
propriate timing for correction of craniosynostosis, most
craniofacial surgeons operate within the first year of age,
taking into consideration the number and pattern of su­
ture fusion, evidence of intracranial hypertension, and the
surgical technique chosen. Minimally invasive techniques,
which rely on less extensive craniotomies and external
postoperative molding, are generally performed at an ear­
lier age than are traditional open techniques.
The technical portions of the procedures are compli­
cated and have multiple risks. Avoidance of these risks is
important to produce improved results. This begins with
carefully evaluating potential patients preoperatively and
optimizing their physical condition before surgery. In prep­
aration for surgery, measures to ensure stable blood vol­
umes should be undertaken. These include obtaining a base­
line hemoglobin, blood type and crossmatch, and potential
directed blood donation from a matched family member.
Some surgeons use preoperative erythropoietin to mini­
mize the need for transfusion.5 Surgery is performed under
general anesthesia, which should be provided by a fellow­
ship-trained pediatric anesthesiologist. Patients should be
monitored postoperatively in an intensive care unit under
the direction of pediatric critical care–trained physicians.
Surgical Considerations
A broad range of surgical options has been proposed for
the surgical treatment of craniosynostosis. The goal of each
is to remove the affected suture and either immediately
remodel the remaining calvarial vault or provide postop­
erative guidance (distractors, springs, helmets) to allow
this to occur. For most types of craniosynostosis repairs,
patients are placed in the supine position to facilitate ex­
posure. However, if the posterior vault is being addressed,
as in a lambdoidal synostosis or some sagittal synostoses,
the patient may be placed in the prone or sphinx position.
In the prone position, care must be taken to ensure proper
positioning with adequate protection of the eyes. Injury
to the globe is devastating and should be a “never event.”
Before the procedure begins, lubrication should be placed
over the corneas; some surgeons approximate the eyelids
with either adhesive tapes or formal tarsorrhaphy sutures
that can be removed at the conclusion of the procedure. If
the patient must be in the prone position, the face can be
supported with a foam pillow or horseshoe headrest, with
the eyes uncompressed within the opening of the support.
Venous air embolism (VAE) is the introduction of air or
gas from the operative field into either the arterial or venous
vasculature and is a potentially fatal complication of neuro­
surgical procedures.6 Previous studies have suggested that
the rate of VAE may be as high as 80% during such proce­
dures. This may be lower for endoscopic techniques.7 Some
Avoiding Unfavorable
Results and Complications in
Craniosynostosis
Summary Box
Common Complications in Synostosis
Correction
Intraoperative
• Bleeding
• Venous air embolism
• Ocular injury and vision loss
• Dural and brain injury, cerebrospinal fluid leak
• Death
Postoperative
• Infection
• Growing skull defects
• Persistent calvarial defect
• Hardware-related complication
• Restenosis, relapse, growth restriction, and recur-
rence of deformity
Preoperative Considerations
Surgical intervention is indicated in craniosynostosis to
correct the craniofacial deformity and potentially treat or
prevent functional disabilities believed to be secondary to
intracranial hypertension (blindness, developmental de­
Fig. 41.3 Copper-beaten appearance of the inner table of the skull.

729
Chapter 41 Craniosynostosis
the dura along the proposed craniotomy path. Once the bone
cuts have been made, the bone flap should be removed care­
fully to avoid additional trauma to the underlying dura from
sharp edges.
The most severe bleeding can occur if there is injury to
the sagittal sinus. The sagittal sinus is most at risk when the
craniotome passes across the midline. For this reason, any
cuts traversing the sagittal sinus are performed last. In the
event of significant bleeding, the bone flap can then be re­
moved relatively quickly to address the blood loss. With the
remaining bone cuts already performed, the bone flap can
be removed relatively quickly and any bleeding controlled.
Injury to the sagittal sinus more posterior in the skull is
more worrisome.
The earliest approaches to treating craniosynostosis in­
cluded simple suturectomy, allowing the brain to direct skull
growth through the remaining open sutures. Inadequate cor­
rection prompted more extensive procedures of calvarial
remodeling, and these have yielded improved results. These
current techniques include wide scalp dissection, more re­
mote calvarial osteotomies, and skull remodeling that is
individually tailored to each cranial vault deformity.12 For
sagittal synostosis, the surgical approaches range from mini­
mal removal of the involved suture (usually combined with
postoperative use of springs, distractors, or helmets) to ex­
tensive total calvarectomy and reconfiguration. Simple syn­
ostectomy, or simple strip craniectomy, when combined
with postoperative springs, distractors, or helmet therapy,
can be safe and well tolerated, providing adequate cosmetic
results in select patients with mild deformities.
Disadvantages include its lack of specifically address­
ing the severe compensatory changes in skull shape that re­
sult from the fused suture. It also leaves a large unprotected
area over the vertex of the skull, an area with a high rate
of restenosis and renewed growth restriction. The midvault
“expanded strip craniectomy” provides a more immediate
reconstruction of skull contour by shortening the anteropos­
terior dimension, expanding the biparietal dimension, and
partially addressing the frontal and occipital prominences.
The “Pi reconstruction,” a version of a midvault expanded
strip craniectomy, is used for older infants (6 to 12 months
of age) with scaphocephaly caused by sagittal synostosis
(Fig. 41.4). Bone is removed lateral to the sagittal suture
over the lateral parietal areas and molded, leaving two bony
defects in the design of the Greek letter Pi (π). Cranial bone
overlying the sagittal sinus is left intact to minimize bleed­
ing. Axial compression is used to shorten and widen the
skull, and absorbable plates are placed to hold the position.
For coronal and metopic synostosis, the goal is to correct
the frontal and orbital asymmetries. In the former, there is
asymmetry in the shape of the orbits and forehead. In the
latter, the typical deformity involves a midline ridge with re­
trusion of the supraorbital bar (“bandeau”) on either side of
the forehead. The most common technique is fronto-orbital
advancement (FOA). With an FOA, dissection continues within
the orbits, freeing the periorbita off the upper half of the or­
bit. Caution must be taken to avoid injury to the underlying
globes. To expose the roof of the orbits or anterior cranial
believe that real-time monitoring, to identify the presence
of a venous air embolism and allow for immediate interven­
tion, mitigates the risk. Despite the introduction of newer
diagnostic techniques, use of a precordial Doppler remains
a standard modality. Although transesophageal echocardiog­
raphy is the most sensitive method of detection,8,9 it is more
costly and invasive. When properly placed between the third
and sixth intercostal spaces along the right parasternal bor­
der, a precordial Doppler is potentially capable of identify­
ing VAE at an air infusion rate as low as 0.015 mL/kg/min
and consistently at a rate of 0.021 mL/kg/min.10,11 VAE may
be graded by severity: change in Doppler tones (grade I),
change in Doppler tones and decrease in end-tidal carbon
dioxide (grade II), or decrease in systolic blood pressure by
20% from baseline (grade III).
The calvarium is often approached via a standard
“stealth” (zigzag) coronal incision, providing for adequate
exposure while minimizing any postoperative scar. Periop­
erative antibiotics and steroids may be administered before
the procedure. The incision is infiltrated with 1:200,000 epi­
nephrine in 0.5% lidocaine to minimize bleeding along the
incision site. The frontal and temporal regions are dissected
in the subgaleal plane, and care is taken to preserve the peri­
osteum on the surface of the bone, which helps minimize
blood loss and may be used to stabilize the bony segments.
Bleeding can occur at several points in the procedure.
Incisions into the scalp can produce significant bleeding in
the infant. This bleeding can be controlled with metal clips
and cautery. Bleeding can also occur with elevation of the
coronal flap. This is usually greater with subperiosteal dis­
section rather than supraperiosteal dissection. With either,
punctate bleeding should be controlled with pinpoint cau­
tery or bone wax. The latter should be used judiciously, be­
cause any wax left behind can serve as a nidus for infection.
To “turn down” or reflect the coronal flap, the supraor­
bital vessels must be released from their attachments to the
frontal bone. Sometimes, the vessels exit the bone through a
notch; in older children they exit through a foramen. If the
surgeon is not careful of these vessels, unnecessary bleeding
can occur. When the vessels may exit through a foramen, a
2-mm osteotome should be used to convert the foramen to
a notch by removing the inferior portion with the bony fora­
men. The vessels within the underlying periorbita can then
be carefully dissected away from the bone.
Performing the craniectomy involves making several
small access holes and joining them with a side-cutting cra­
niotome with a protective footplate. The access holes are
made with a high-speed burr. Injury may occur if the burr
is used too aggressively to enter the calvarial vault. The burr
is often used to traverse the outer table, diploic space, and a
portion of the inner table. As the remaining bone becomes
thin enough, the burr can be put aside and communication
to the epidural space made with a fine curette. Once a small
opening is made with the curette, a Kerrison rongeur can
be used to widen the opening further. The Kerrison has a
protective footplate to minimize dural injury. Once the
opening is large enough, a small elevator, such as a No. 4
Penfield, can be used to dissect beneath the bone and above

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
730
When replaced, its lateral aspects and midline are fixed to
the calvarial vault. This is performed for both unilateral and
bilateral cases. To avoid relapse or a persistent deformity,
overcorrecting the expansion of the affected side by 5 to
10% while also providing a convex shape at the lateral bor­
der is commonly necessary for a satisfactory reconstruction.
The frontal bone flap is then attached to the supraorbital bar,
taking care to match it to the previously overcorrected (5 to
10%) orbital bandeau on the affected side.
For lambdoid synostosis, a variety of surgical ap­
proaches, aiming to release the affected suture(s) and
normalize the posterior calvarial vault contour, have been
described. Options include simple synostectomy, unilateral
remodeling of the affected occipital region, and bilateral oc­
cipital reconstruction with or without the use of an occipital
bandeau. Most lambdoid surgical candidates have significant
parietal and frontal compensatory changes in addition to
their occipital deformation; therefore they are best served
by a more extended calvarectomy and remodeling. The re­
construction is similarly maintained with intervening bone
grafts and resorbable hardware. Titanium was used early on
but fell out of favor when it was noted that the plates trans­
located endocranially to the internal surface of the skull with
calvarial growth. Resorbable plates made of polylactic acid
provide temporary support across the osteotomy sites and
dissolve within 12 to 24 months. After placement of the re­
configured supraorbital bar, the frontal bone is remodeled
using the remaining portions of bone and fixed with absorb­
able plates or suture. Closure is performed in a routine fash­
ion, with some surgeons placing subgaleal drains.
To address concerns regarding incision length, operative
blood loss, and length of stay associated with open cranio­
facial procedures, minimally invasive techniques have been
proposed. These techniques include endoscopic sutural re­
lease,13 spring-assisted cranioplasty14 (Fig. 41.5), and dis­
traction osteogenesis15 (Fig. 41.6). Endoscopic release has
been proposed to minimize the intraoperative risks by lim­
base, a bifrontal craniotomy is marked and cut with an osteo­
tome. Typically 1 to 2 centimeters of the superior orbital rim
is left for support. The bone flap is placed aside and replaced
in a more corrected position at the end of the procedure
The supraorbital bar or bandeau is removed with care­
ful osteotomies through the lateral orbit at the frontozy­
gomatic suture, the superior orbit along the roof, and the
nasion just above the nasofrontal suture. Care is taken to
protect the underlying dura and brain as well as the orbital
contents with adequate exposure and judicious use of re­
tractors. This is a relatively low-risk maneuver but should
similarly be performed with caution. The eyes are perhaps
most at risk when cutting the orbital bandeau. Here, suffi­
ciently wide malleable retractors should be placed between
the globes and the saw blade or osteotome. The saw should
not be run unless there is good protection of the underlying
structures and the blade only advanced through the bone as
far as needed. As the blade moves either medially or later­
ally, the retractors should follow to avoid inadvertent injury.
The sphenoid wing is cut and the coronal sutures are
opened to the level of the skull base to prevent continued
growth restriction and resultant postoperative hollowing
of the pterional regions. The bandeau is then reconfigured
and fixed with resorbable sutures or plates and screws to
maintain the newly contoured shape. Reconfiguration of
the supraorbital bar might require osteotomies, both in the
midline to flatten the forehead and laterally to normalize
the lateral supraorbital angle. The goal is expansion on the
affected side as well as recession on the contralateral side.
Fig. 41.4 Extended strip craniectomy for sagittal synostosis.
Fig. 41.5 Spring-assisted cranioplasty. (This image provided cour­
tesy of Lisa David, MD.)

731
Chapter 41 Craniosynostosis
Intraoperative Complications
Blood Loss, Venous Air Embolism, and
Death
Intraoperative blood loss and transfusion requirements,
leading to hemodynamic instability, constitute great dan­
gers to the patient and should never be underestimated.
Based on outcome data on open craniosynostosis surgery,
estimated blood loss spans 25 to 500% of estimated blood
volume (EBV), transfusion requirement ranges from 25
to 500% of EBV, complication rates are between 6.8 and
23.3%, and mortality rates center around 0 to 1.1%. Delayed
bleeding in the postoperative period is rare but potentially
life-threatening. Initial hemodynamic support followed by
reoperation is often warranted in these instances. Common
sites of bleeding should be examined, both extracranial and
intracranial. The latter requires removal of the plates and/
or sutures and taking down the reconstruction.
Management of a patient with suspected or docu­
mented VAE is important. The goal is to prevent further
air entry, reduce the volume of air introduced, and provide
hemodynamic support. The exposed dura should be cov­
ered with a saline-soaked dressing and the patient placed
in a left lateral decubitus position in an attempt to keep the
air in the right atrium.17 Administration of 100% oxygen
will reduce the volume of the embolus by eliminating ni­
trogen.18 Optimizing fluid volume and providing inotropic
support if necessary to maintain adequate systemic arte­
rial pressure will also help keep the patient hemodynami­
cally stable.19 More invasive techniques include aspiration
of air from the right atrium, which has an up to 60% success
rate.20 Hyperbaric oxygen therapy is beneficial, because it
causes compression of existing air bubbles by establishing
a high diffusion gradient to speed dissolution of bubbles
and by improving oxygenation in the ischemic tissues.21
Anticoagulation therapy with heparin in patients with air
embolism decreases the severity of the disease if they are
treated with heparin before air embolization occurs.22 Be­
cause steroids do not have any effect on cytotoxic brain
edema, which occurs in patients with air embolism, ste­
roid use for this condition is controversial.23 Interestingly,
prophylactic lidocaine is effective in reducing the gas em­
bolism effect on the brain; it has decreased brain edema in
experimental animals.
Ocular Injury and Vision Loss
Injury to the globe or surrounding structures can occur
during removal of the orbital bandeau. Fortunately, this is
an uncommon occurrence. It is best avoided with thorough
subperiosteal dissection of the orbit and careful protection
of the intraorbital contents when making the osteotomies.
If injury is discovered, prompt ophthalmology consultation
is warranted.
iting the amount of bleeding from larger scalp incisions
and bone flap elevation. The technique uses one or more
small incisions, minimal dissection, and removal of the af­
fected suture, possibly with adjunctive osteotomies into
the surrounding bone. The technique does not attempt to
completely correct the craniofacial deformity at the time of
surgery but rather remove the offending sutures and allow
remodeling during a period of postoperative brain growth,
with or without external molding, or distractive force.
The use of springs works in a similar fashion; the affected
suture is removed and one or more internal springs are
placed across the open osteotomy to guide remodeling of
the skull (see Fig. 41.5). Of note, a second, smaller procedure
is required to remove the springs. Similarly, distraction os­
teogenesis has been used to impart an internal force across
the ostectomy site (see Fig. 41.6). New bone is laid down in
the distracted osteotomy gap.
Managing Unfavorable
Results and Complications in
Craniosynostosis
Postoperative mortality rates are low after surgery for cra­
niosynostosis and continue to decline with technological
advancements and experience. However, notable compli­
cations can occur both intraoperatively and postopera­
tively. Hospital length of stay is typically between 4 and
7 days, with longer admissions for complex synostoses.
­Reoperation rates range from 7.2 to 23.3% and are even
higher for complex synostoses.16
Fig. 41.6 Distraction osteogenesis.

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
732
­occurs secondary to a dura injury or attenuation that post­
operatively results in a leptomeningeal cyst that enlarges
with time and prevents bony healing, resulting in an en­
larging or growing skull defect. This unusual postopera­
tive complication requires secondary surgery that includes
neurosurgical correction of the meningeal defect and cra­
nioplasty.
Scalp Alopecia
With continued cranial vault growth after correction of syn­
ostosis, the coronal scar is placed under continual tension
that can lead to scalp scar alopecia. This is especially true
for the typical linear coronal access incision, as illustrated
in Fig. 41.8. The advantage of a zigzag triangular pattern
coronal incision is that the scar is better camouflaged and
the tension redirected to minimize the risk of coronal scar
scalp alopecia. Our experience is that once skull growth is
completed, the scar can be easily revised by advancement
flaps and temporal transposition flaps to redirect the scar.
Persistent Calvarial Defect
Gaps left at the time of surgery in an infant younger than
18 months of age generally fill in over time because of
the osteogenic potential of the dura. This does not occur
with older children and adults. In older infants, cranial de­
fects should be reconstructed at the time of surgery with
split cranial bone grafts or other techniques such as “bone
mush” or demineralized bone matrix and bilaminate mesh
cranioplasty.26,27 Despite these efforts, persistent calvarial
defects can occur. Depending on the size of the defect and
Dural or Brain Injury and Cerebrospinal
Fluid Leak
Injury to the dura and underlying brain tissue occurs most
commonly when performing the craniotomy (Fig. 41.7).
Unrecognized or poorly repaired dural injury can lead to a
persistent cerebrospinal fluid (CSF) leak. This is manifested
by increased fluid within a drain or beneath the scalp. If
severe, it may require reoperation to identify the site of the
leak and repair it either directly or with a replacement ma­
terial.
Postoperative Complications
Infection
Arguably the most difficult to avoid, infectious complica­
tions can be minimized in several ways. The patient should
receive appropriate intravenous antibiotics before the start
of the procedure. The surgical site can be well irrigated dur­
ing and at the conclusion of the procedure with an anti­
biotic solution. A superficial drain can be left beneath the
scalp to evacuate any residual fluid, which may later be­
come infected. Finally, the time of the procedure should be
minimized, because the rate of infection has been shown to
increase with the time of the surgery. The rates of infection
for primary craniofacial surgery are very low but increase
with repeat surgeries.24 When a serious postoperative in­
fection occurs, open débridement and irrigation is often
performed and repeated as needed. Some authors have re­
ported placing continuous irrigation systems; however, no
conclusive data is available.
Growing Skull Defects
A very rare complication of craniofacial surgery is a grow­
ing skull defect. Similar to growing skull fractures that oc­
cur after trauma, a growing skull defect represents an en­
larging bony defect after craniofacial surgery.25 This likely
Fig. 41.7 Dural injury after craniotomy.
Fig. 41.8 Coronal scalp scar alopecia with continued normal cranial
vault expansion after craniosynostosis correction in infancy. The scar
would be better camouflaged and the tension redirected if the initial
coronal incision had been broken into a zigzag pattern.

733
Chapter 41 Craniosynostosis
the time of primary surgery is advisable. Secondary sur­
gery may be advisable when patients desire improvement
in appearance. Minor improvements may be performed
the activity of the child, this may require secondary cranio­
plasty with placement of a bone graft or bone substitute.
Stem cell therapy has yet to be introduced into the clinical
mainstream but has been shown to be promising experi­
mentally.28
Hardware-Related Complications
The use of resorbable hardware is well accepted today. It is
not uncommon for the polylactic or polyglycolic constructs
to remain in place for 12 to 18 months before eventual
resorption. In rare individuals, dissolution of the mate­
rial may lead to the formation of a sterile abscess at one
or more sites of prior hardware placement. These may be
addressed by simple exposure and superficial débridement
if they are problematic.
The use of distractors or springs carries the potential
of hardware malposition or failure, requiring premature re­
moval and possible injury to surrounding structures. Wound
infection, scalp injury, and dural injury with CSF leak have
been reported. In all cases, secondary surgery for removal
of the device is required.
Restenosis, Relapse, Growth Restriction,
and Recurrence of Deformity
Despite an adequate on-table result, postoperative reste­
nosis, relapse, and growth restriction may contribute to
a less-than-adequate long-term appearance.29 Because of
the variability of the outcome, patients should be followed
yearly from birth through adulthood to assess the results of
surgical intervention (Figs. 41.9 and 41.10). In an attempt
to minimize inadequate correction and recurrence of the
deformity, some degree of overcorrection of the defect at
a
b
Fig. 41.9 This infant underwent endoscopic correction of metopic synostosis with an unsatisfactory outcome. (a) Before surgery.
(b) One year after surgery. Trigonocephaly remains and the patient required a formal open approach.
Fig. 41.10 Two decades after correction of nonsyndromic bicoronal
craniosynostosis. A contour discontinuity is notable at the origin of
the temporalis muscle and the frontal bone; the decreased bifron­
tal width and the temporal muscle prominence interfere with the
temporal arms of his glasses.

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
734
Conclusion
Repair of a patient with craniosynostosis is challenging and
potentially life-threatening. However, long-term outcomes
demonstrate that craniofacial surgery can be performed
safely if notable steps are taken to avoid the common pitfalls.
with contouring or only cranioplasty. At times, simple cra­
nioplasty is inadequate, and the need for repeat FOA and
cranial vault procedures are necessary. Of note, in cases of
severe deformity, initial complete correction may not be
feasible given soft tissue limitations and a staged recon­
struction may be required.
References
1. Virchow R. Ueber den Cretinismus, namentlich in Fran­
ken, und uber pathologische Schadelformen. Verh Phys
Med Ges (Wurzburg) 1851;2:230–271
2. Renier D, Sainte-Rose C, Marchac D, Hirsch JF. Intracranial
pressure in craniostenosis. J Neurosurg 1982;57(3):370–
377
3. Thompson DN, Harkness W, Jones B, Gonsalez S, Andar U,
Hayward R. Subdural intracranial pressure monitoring in
craniosynostosis: its role in surgical management. Childs
Nerv Syst 1995;11(5):269–275
4. Graves JM, Kanal KM, Vavilala MS, Applegate KE, Jar­
vik JG, Rivara FP. Hospital-level factors associated with
use of pediatric radiation dose-reduction protocols for
head CT: results from a national survey. J Am Coll Radiol
2014;11(7):717–724.e1
5. Vega RA, Lyon C, Kierce JF, Tye GW, Ritter AM, Rhodes JL.
Minimizing transfusion requirements for children under­
going craniosynostosis repair: the CHoR protocol. J Neu­
rosurg Pediatr 2014;14(2):190–195
6. Buckland RW, Manners JM. Venous air embolism during
neurosurgery. A comparison of various methods of detec­
tion in man. Anaesthesia 1976;31(5):633–643
7. Tobias JD, Johnson JO, Jimenez DF, Barone CM, McBride DS
Jr. Venous air embolism during endoscopic strip craniec­
tomy for repair of craniosynostosis in infants. Anesthesi­
ology 2001;95(2):340–342
8. Cucchiara RF, Nugent M, Seward JB, Messick JM. Air em­
bolism in upright neurosurgical patients: detection and
localization by two-dimensional transesophageal echo­
cardiography. Anesthesiology 1984;60(4):353–355
9. Domaingue CM. Anaesthesia for neurosurgery in the sit­
ting position: a practical approach. Anaesth Intensive
Care 2005;33(3):323–331
10. Gildenberg PL, O’Brien RP, Britt WJ, Frost EA. The efficacy
of Doppler monitoring for the detection of venous air em­
bolism. J Neurosurg 1981;54(1):75–78
11. Schubert A, Deogaonkar A, Drummond JC. Precordial
Doppler probe placement for optimal detection of ve­
nous air embolism during craniotomy. Anesth Analg
2006;102(5):1543–1547
12. Whitaker LA, Munro IR, Salyer KE, Jackson IT, Ortiz-Mon­
asterio F, Marchac D. Combined report of problems and
complications in 793 craniofacial operations. Plast Recon­
str Surg 1979;64(2):198–203
13. Jimenez DF, Barone CM, Cartwright CC, Baker L. Early
management of craniosynostosis using endoscopic-as­
sisted strip craniectomies and cranial orthotic molding
therapy. Pediatrics 2002;110(1 Pt 1):97–104
14. Lauritzen CG, Davis C, Ivarsson A, Sanger C, Hewitt TD. The
evolving role of springs in craniofacial surgery: the first 100
clinical cases. Plast Reconstr Surg 2008;121(2):545–554
15. Akai T, Iizuka H, Kawakami S. Treatment of craniosyn­
ostosis by distraction osteogenesis. Pediatr Neurosurg
2006;42(5):288–292
16. Jeong JH, Song JY, Kwon GY, et al. The results and com­
plications of cranial bone reconstruction in patients with
craniosynostosis. J Craniofac Surg 2013;24(4):1162–1167
17. Durant TM, Long J, Oppenheimer MJ. Pulmonary (venous)
air embolism. Am Heart J 1947;33(3):269–281
18. Sibai AN, Baraka A, Moudawar A. Hazards of nitrous ox­
ide administration in presence of venous air embolism.
Middle East J Anaesthesiol 1996;13(6):565–571
19. Archer DP, Pash MP, MacRae ME. Successful management
of venous air embolism with inotropic support. Can J An­
aesth 2001;48(2):204–208
20. Colley PS, Artru AA. Bunegin-Albin catheter improves air
retrieval and resuscitation from lethal venous air embo­
lism in upright dogs. Anesth Analg 1989;68(3):298–301
21. Blanc P, Boussuges A, Henriette K, Sainty JM, Deleflie
M. Iatrogenic cerebral air embolism: importance of
an early hyperbaric oxygenation. Intensive Care Med
2002;28(5):559–563
22. Ryu KH, Hindman BJ, Reasoner DK, Dexter F. Heparin re­
duces neurological impairment after cerebral arterial air
embolism in the rabbit. Stroke 1996;27(2):303–309, dis­
cussion 310
23. Dutka AJ, Mink RB, Pearson RR, Hollenbeck JM. Effects of
treatment with dexamethasone on recovery from experi­
mental cerebral arterial gas embolism. Undersea Biomed
Res 1992;19:131–141
24. Fearon JA, Yu J, Bartlett SP, Munro IR, Chir B, Whitaker
L. Infections in craniofacial surgery: a combined report
of 567 procedures from two centers. Plast Reconstr Surg
1997;100(4):862–868
25. Havlik RJ, Sutton LN, Bartlett SP. Growing skull frac­
tures and their craniofacial equivalents. J Craniofac Surg
1995;6(2):103–110, discussion 111–112
26. Greene AK, Mulliken JB, Proctor MR, Rogers GF. Pediatric
cranioplasty using particulate calvarial bone graft. Plast
Reconstr Surg 2008;122(2):563–571
27. Losee JE, Karmacharya J, Gannon FH, et al. Reconstruction
of the immature craniofacial skeleton with a carbonated
calcium phosphate bone cement: interaction with biore­
sorbable mesh. J Craniofac Surg 2003;14(1):117–124
28. Taub PJ, Yau J, Spangler M, Mason JM, Lucas PA. Bioengi­
neering of calvaria with adult stem cells. Plast Reconstr
Surg 2009;123(4):1178–1185
29. Taylor JA, Paliga JT, Wes AM, et al. A critical evaluation
of long-term aesthetic outcomes of fronto-orbital ad­
vancement and cranial vault remodeling in nonsyn­
dromic unicoronal craniosynostosis. Plast Reconstr Surg
2015;135(1):220–231

735
Chapter 41 Craniosynostosis
Discussion by David J. David
The task of dealing with unfavorable results emanat­
ing from the management of craniosynostosis in a
textbook setting that is meant to inform the concerned
reader, but not provide an in-depth analysis, is a diffi­
cult one. The approach to concisely outline the pathol­
ogy, pathogenesis, classification, and clinical findings
in patients with craniosynostosis is important to make
sense of this wide-ranging topic and point the more
specialized reader to the much wider and complex is­
sues involved in the management of the syndromes.
At a time when there is much focus on surgical tech­
niques, the fundamentals of the pathogenesis of these
conditions must be put first. Virchow’s mechanical the­
ory of the production of the deformities is very impor­
tant.1 The forces transmitted to the cranial base by the
dural attachments, as well as the massive influence of
the rapidly growing brain in the first year of life, are fur­
ther considerations.2 I, like many of my colleagues, stress
the facility with which the calvarial bones can regener­
ate from the dura in the first years of life, thus setting
the scene for a logical discussion about problems related
to managing the craniosynostoses.
With the advent of intensive genetic research cou­
pled with long-term clinical follow-up for larger cohorts
of patients, the boundaries between these subtypes have
become less distinct. Frontal plagiocephaly from unicor­
onal synostosis may be syndromal, and the suspicion
is raised by the association of deafness.3 Similarly, the
occurrence of repeated episodes of raised intracranial
pressure, in some cases of sagittal synostosis, may point
to the identification of a genetic anomaly.4
The concise segment on the differential diagnosis of
posterior plagiocephaly is very important and is a sig­
nificant aid in differentiating the common but benign
condition of deformational plagiocephaly from true
lambdoid synostosis. Historically this confusion resulted
in much unnecessary surgery, which in itself is a compli­
cation of management of craniosynostosis.5
Modern imaging has made a huge difference to the
management of these conditions. Not only are the surgi­
cal interventions better planned, but understanding the
postoperative progress of the patient through growth
and development has become possible. Computed to­
mography (CT) and magnetic resonance imaging (MRI)
are vital tools, especially in the care of the syndromes
(Apert’s, Crouzon’s, and others), because the diseases are
never “cured.”
In recent years this author and team have explored
the frequency of diagnosis of craniosynostosis at the
20-week ultrasound examination and have shown that
many of the patients, particularly with scaphoceph­
aly, who suffer maternofetal trauma at birth, had de­
monstrable evidence of the deformity on prenatal ul­
trasound, and other recent publications have endorsed
these findings.6,7
Management Protocols
Designing management protocols for the wide-ranging
spectrum of craniosynostosis conditions is a difficult
task. The chapter encapsulated the essence of proper
preoperative assessment for the whole range of anom­
alies, but quite appropriately confines the bulk of the
section to those pertaining to the simpler conditions,
leaving aside the complexities of the severe syndromes.
For the lesser deformities there is remarkable una­
nimity in approach, despite a plethora of minor and per­
sonalized variations in timing of surgery and technique.
This is because the basic principles are not contested.
I consider the following to constitute these basic prin­
ciples:
•• The brain grows rapidly in the first year of life, and
the brain growth curve is steep.
•• The synostotic process starts at various times and
has variable severity.
•• Virchow’s rule1 applies.
•• Moss’s theory2 about the cranial base applies.
•• If the cranial base is distorted, the face will be
distorted.
•• Infants grow calvarial bone from dura aggressively
in the early years of life.
•• Early and adequate surgery gives the best chance
of normalizing growth and development of the
craniofacial skeleton.
Fundamental to avoiding unfavorable results and com­
plications in the perioperative period is the presence
of an experienced, well-drilled, multidisciplinary team.
In the early days of establishing such a team, I found it
helpful to write down all of the perioperative steps and
circulate the document to the relevant staff.
The Problem of Blood Loss
The emphasis on intraoperative blood loss is significant.
Not only is it easy to lose blood in dramatic events, such
as damaging the sagittal sinus, but protracted opera­
tions with slow and persistent blood loss can be very
destructive, especially when washout coagulopathy oc­
curs. The surgical and anesthetic team need to be expe­
rienced and in harmony to prevent what I believe to be
the most common, and serious, complication and the
one most likely to lead to patient mortality.
There are many variations to techniques for rais­
ing the coronal scalp flap, exposing the calvarium, and

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
736
gaining access to the pathology to be corrected. Of all
the problems that might occur during this phase, blood
loss is the most significant for various reasons. I have
never had an operative death in 40 years of craniofa­
cial practice but have been involved in the assessment
of cases where it has occurred. Consistently the prob­
lem has been that the treating team has tried to avoid
blood transfusion and has been unprepared for massive
hemorrhage when it has occurred in infants during the
first year of life. My strategy is to transfuse all patients
being operated for “simple” craniosynostosis and start
the blood early in the operative procedure and not as a
“catch-up” procedure.
This leads the discussion to the issues surrounding
minimally invasive surgery, because one of the reasons
for this approach is reduction of blood loss and avoid­
ance of blood transfusion. The addition of minimally in­
vasive techniques to previously inadequate techniques
of suturectomy may only work in mild deformities and
may have greater risks. When recently auditing our se­
ries of sagittal synostosis patients, referred for second
opinions, the major complications came from attempts
at minimally invasive surgery that culminated in major
hemorrhage, dural tears, refusion, and lack of bone re­
generation.
Treatment of Sagittal Synostosis
The preferred technique for the treatment of sagit­
tal synostosis at the Australian Craniofacial Unit is to
sun-ray the frontal bone, barrel-staving the midsec­
tion, to widen the skull after removing the fused su­
ture widely and across the normal sutures in front and
back. When present, the posterior “bullet” is sun-rayed
and its inferior attachment weakened to allow the seg­
ment to move forward. For the best results we take the
barrel staves down to the cranial base. Frontal fixation
is achieved with four 30-g stainless steel wires. Wide
gaps are left for spontaneous regeneration and a firm
bandage is applied for 4 days. Using the cephalic index
as an outcome measure, the results with this technique
have improved markedly since its inception almost 15
years ago.
Coronal, Metopic, and Unicoronal
Synostoses
Although cranio-orbital deformities are often corrected
with treatment for coronal and metopic synostoses,
there are multiple variations in technique. However,
the principles of doing surgery early enough to use
brain growth and being radical enough to prevent re­
fusing of the bone are well expressed and widely held.
When considering unfavorable results, both metopic
synostosis and unicoronal synostosis are prone to their
own peculiar problems related to the unique pathology
and pathogenesis of each condition. In patients with
unicoronal synostosis the cranial base is curved to the
affected side, producing a three-dimensional distortion
of the facial skeleton, because the cranial base forms a
template for the face. Therefore cutting the sphenoid
wing and advancement of the cranial base as described
is very important. If uncorrected, or inadequately cor­
rected, this distortion increases with growth and be­
comes increasingly difficult to correct at maturity.8 This
unfavorable result may not be fully manifest for many
years.
Metopic synostosis is a somewhat enigmatic condi­
tion. At its most severe, the goal, as stated, is to normal­
ize the fronto-orbital region. With lesser manifestations
of the condition it is sometimes much more difficult to
make a decision regarding surgical intervention, because
the “cosmetic“ result may not be very dramatic, and
there is often some recurrent distortion in frontotem­
poral region as growth proceeds. In addition, seemingly
unrelated to severity is the occurrence of some form of
developmental delay in 20 to 30% of patients that be­
comes manifest between 2 and 9 years of age. The rela­
tionship of this outcome to the disease process and to
the surgery is yet to be clarified.
Lambdoid Synostosis
Because of the relative rarity of lambdoid synostosis
(1 to 2% of craniosynostosis cases), the cumulative
experience in the craniofacial surgical community is rel­
atively rare. Apart from the parietal and frontal compen­
satory changes mentioned, the inferior displacement of
the mastoid region and the curving of the cranial base
can result in severe craniofacial deformity. In an even
smaller group of patients the synostosis can be bilateral
and extend into the posterior part of the sagittal suture,
producing the “Mercedes Benz” sign.9 The surgery may
need to be more radical and include a range of surgical
maneuvers to reshape the skull fragments.
Other Complications
There is no particular reference to the craniofacial syn­
dromes that constitute the complex end of the spectrum
of this disease process. It becomes important, however,
for the reader to understand the long-term potential
complications that can occur in conditions such as Ap­
ert’s, Crouzon’s, and Pfeiffer’s syndromes. The intraop­
erative and postoperative complications that are listed
are more likely to occur and are likely to be more severe
in the craniosynostosis syndromes.
Air Embolism
In 40 years’ experience at the Australian Craniofacial
Unit, I have not experienced a single case of clinical air

737
Chapter 41 Craniosynostosis
embolism; I know it does occur, but it has not presented
as a clinical problem in my practice. These variations in
experience show the value of interunit communication
and point the way to meaningful clinical research.
Intracranial Pressure
Fortunately injury to the eyes and dural breaches in­
volving the brain are rare in the simple craniosynos­
toses, especially for experienced teams. It is in the
syndromal cases where the raised intracranial pressure
results in spikes of bone from the inner table of the cal­
varium penetrating the dura that cerebrospinal fluid
leaks and brain damage are more likely.
Infection
There is, of course, a continuum of influences from
pathology through the operative phase that affect the
postoperative and long-term outcomes with respect to
complications. Infection is a much more prevalent and
serious matter in the syndromes, sometimes resulting
in meningitis and osteomyelitis presenting many years
later. Key factors in this causation are the dead space
behind the advanced frontal bone, connection of the
extradural space with the nasal cavity, and especially
the presence of a tracheostomy where there is an in­
fected passage from the trachea to the cranial cavity.10
There is a relationship between infection and time of
surgery and repeat operations, again underlining the
need for experience.
Calvarial Defects
The problems with persistent calvarial defects, and in­
deed the more complex problem of the growing fracture,
relate in the latter to trauma to the dura and arachnoid.
They are iatrogenic and more likely to occur in syndro­
mal cases11 (Fig. 41D.1). The gaps left at surgery in the
younger age groups will regrow bone from the dura, and
in our experience this can be the case in patients up to
3 years of age, but it is less likely to do so as time passes.
Thus there is a lot to understand, namely that early op­
eration allows calvarial regeneration, regeneration in the
area of the fused suture is to be discouraged, and dam­
age to dura and underlying meninges may discourage
bone regeneration altogether.
New Technologies
The relative value of the new technologies of endoscopy,
springs, and distraction in craniosynostosis surgery il­
luminates the need for deeper analysis of the uses and
results from surgery involving these techniques. I have
a
b
Fig. 41D.2 (a) Postoperatively after frontofacial advancement for Crouzon’s syndrome. (b) Frontal bone osteomyelitis present­
ing 2 years later.
Fig. 41D.1 A growing fracture in a patient with Apert’s syn­
drome. The condition is more common in the syndromes
because of the thinness of the dura and the ease of potential
damage during surgery.

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
738
found that some of the worst complications have come
from the use of relatively untried techniques.
Regarding the problems of new technologies, I draw
attention to the potential limitations of tools and prod­
ucts that are purported to enhance results, because
those same tools and products may cause even bigger
problems. Two examples of patients referred to my care
for second opinions illustrate this point: (1) when sheets
of polylactic or polyglycolic material are used to fill in
across suturectomized calvarium in infants, bone regen­
eration is hindered and dense scar forms; (2) the use of
springs near the venous sinuses has caused torrential
bleeding both at insertion and removal.
The need for long-term follow-up cannot be empha­
sized enough (Fig. 41D.2). The history and development
of the diseases associated with craniosynostosis have
variable outcomes, some of which are related to the ad­
equacy of the initial treatment, and some of which are
the result of the disease process itself.
Conclusion
Advances both clinically and in the laboratory have
shown that what was once considered simple synos­
tosis may in fact be syndromal, and that limiting fol­
low-up to the short term may produce findings that are
unsafe. Modern standards increasingly demand longi­
tudinal studies linked to specific outcome measures.
Recording of complications and unfavorable results is a
vital part of this clinical scientific process.
References
1. Virchow R. Ueber den Cretinismus, namentlich in
Franken, und uber pathologische Schadelformen.
Verh Phys Med Ges (Wurzburg) 1851;2:230–271
2. Moss ML. The pathogenesis of premature cranial syn­
ostosis in man. Acta Anat (Basel) 1959;37:351–370
3. Doherty ES, Lacbawan F, Hadley DW, et al. Muenke
syndrome (FGFR3-related craniosynostosis): expan­
sion of the phenotype and review of the literature.
Am J Med Genet A 2007;143A(24):3204–3215
4. Flapper WJ, David DJ. Sagittal synostosis: a review
of 213 consecutive cases. The 16th Congress of In­
ternational Society of Craniofacial Surgery (ISCFS),
14–18th September 2015, Tokyo Bay, Japan
5.
Menard R, David D. Occipital plagiocephaly – Experi­
ence with 207 patients. Proceedings of the Seventh
International Congress of the International Society of
Craniofacial Surgery Monduzzi Editore, Bologna Italy,
Ed. Linton A Whitaker: Medimond S R L; 1997:127-130
6. Anderson PJ, McLean NR, David DJ. Craniosynostosis
and childbirth. Eur J Plast Surg 2005;28(2):94–98
7. Swanson J, Oppenheimer A, Al-Mufarrej F, et al. Ma­
ternofetal Trauma in Craniosynostosis. Plast Reconstr
Surg 2015;136(2):214e–222e
8. Anderson PJ, David DJ. Late results after unicoro­
nal craniosynostosis correction. J Craniofac Surg
2005;16(1):37–44
9. Moore MH, Abbott AH, Netherway DJ, Menard R,
Hanieh A. Bilambdoid and posterior sagittal synos­
tosis: the Mercedes Benz syndrome. J Craniofac Surg
1998;9(5):417–422
10. David DJ, Cooter RD. Craniofacial infection in 10
years of transcranial surgery. Plast Reconstr Surg
1987;80(2):213–225
11. Yamamoto M, Moore MH, Hanieh A. Growing skull
fracture after cranial vault reshaping in infancy. J Cra­
niofac Surg 1998;9(1):73–75

739
Summary Box
Hemifacial Microsomia
•• Erroneous distraction vector
•• Incomplete consolidation period
•• Lack of satisfactory
overcorrection criteria
•• Hypertrophic scar
•• Loose device
•• Device deformation breakage
•• Unstable device
•• Tooth follicle or damaged teeth
•• Premature consolidation
•• Lack of treatment protocol
•• Lack of preorthodontic and
postorthodontic protocol
Major Craniofacial Clefts
•• Hypertrophy or widening of
scars
•• Infections
•• Canthopexy problems
•• Dipoplia
•• Canthal dystopia
•• Osteosynthesis material
removal
•• Intracranial hematomas
•• Facial asymmetries
•• Frontal surface irregularities
Hypertelorism
•• Nonsatisfactory aesthetic result
•• Infections
•• Brain injury
•• Cerebrospinal fluid leaks
•• Anosmia
•• Major relapse
•• Postoperative ptosis
Complications of Orofacial and Craniofacial Deformities

Orofacial and Craniofacial Deformities
Fernando Molina
CHAPTER
42
Craniofacial surgeons have the opportunity to offer great
possibilities for surgical reconstruction of congenital de­
formities. Even if these malformations are very complex
and include orofacial abnormalities, the surgeon has many
different surgical tools, new modalities for planning, and
increasingly objective long-term follow-up controls. Ideally
the patient’s care should be coordinated by a multidisci­
plinary team, with different professionals working together
to obtain an optimal functional and aesthetic result.
Avoiding and Managing
Unfavorable Results and
Complications in Orofacial and
Craniofacial Deformities
Hemifacial Microsomia
Distraction osteogenesis is the treatment of choice for sur­
gical correction of mandibular hypoplasia. The technique
represents a significant advancement in the field of cra­
niofacial surgery. Congenital deformities such as hemifa­
cial microsomia, Goldenhar’s and Nager’s syndromes, and
Pierre Robin’s sequence may present with mandibular hy­
poplasia of varying severity.1–4
Mandibular distraction is a less-invasive and less time-
intensive technique and has a significantly decreased
morbidity rate compared with traditional methods of man­
dibular reconstruction. Surgeons can generate new bone in
patients with unilateral or bilateral mandibular body defi­
ciency, can correct a severe hypoplastic ascending ramus, or
can reconstruct a missing condyle in an ankylotic patient.
The technique also provides the added benefit of expand­

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
740
The mandibular hypoplasia affects the gonial angle
(grade I) in less severe cases and the angle and the ascend­
ing ramus in others (grade IIa and IIb); the ramus and the
condyle are absent in more severe cases (grade III).22
Operative Considerations
The surgical procedure consists of three primary compo­
nents:
1. A vestibular incision is made and the periosteum is
elevated.
2. A corticotomy is performed on the lateral, superior, and
inferior aspects of the mandible, including the cortex to
the cancellous layer.
3. Two pins and an external distractor are inserted.
Bone vascularity should be always carefully preserved. Sev­
eral basic requirements are important when treating hemi­
facial microsomia with distraction osteogenesis:
•• Distraction vector is a critical decision. The corticotomy
and the position of the pins determine the vector,
which varies depending on the grade of mandibular
hypoplasia. Oblique, semioblique, and vertical vectors
are the most common for patients with unilateral
mandibular hypoplasia (Fig. 42.1).
•• The distraction protocol includes a 5-day latency period
followed by a rate of 1.0 mm of elongation per day
ing the overlying soft tissues. It likely represents the first
tissue-engineering surgical technique to be applied to the
craniofacial field.
Bone lengthening and distraction osteogenesis using
osteotomies and circumferential gradual distraction were
described by Ilizarov and colleagues5,6 to align fractured seg­
ments of long bones and to later elongate these bones with­
out a bone graft.
Snyder et al7 reported mandibular lengthening in a ca­
nine model using an extraoral device. A similar report using
intraoral devices followed from Italy.8 More recently, Karp
and colleagues9 reported membranous bone lengthening us­
ing external devices. Histologic examination of the zone re­
vealed a highly organized biologic process.
Since the beginning of the 1990s successful mandibular
distraction has been performed on a multitude of patients
around the world.10–16 My own experience performing man­
dibular distraction began in 1990, and I still use a corticot­
omy and external devices to achieve simultaneous skeletal
and soft tissue correction with minimal surgery.17–21
Hemifacial microsomia is the second most common fa­
cial birth disorder after cleft lip and palate, with a preva­
lence of one in 3,500 to 5,600 live births. Facial asymmetry
and microtia are the most important clinical findings in a
hemifacial microsomia patient. Deviation of the chin to the
affected side, hypoplasia of the soft tissues, and associated
disorders of other anatomic structures such as the maxilla,
the zygoma, and the muscle of mastication are present in a
wide variability.
a
b
c
d
Fig. 42.1 (a,b) Preoperative view of a 5-year-old boy who
presented with right grade IIa hemifacial microsomia. Mi­
crotia and facial asymmetry are associated with a moderate
soft tissues hypoplasia at the right side. The hypoplastic angle
and ramus in the right side of the mandible is the cause of
the deformity. The menton is deviated toward the affected
side. (c,d) At the age of 7 years, during the distraction pro­
cess. A semioblique vector was used for the reconstruction
of the mandibular ramus. Initial face changes have begun in
the right side.
Continued

741
Chapter 42 Orofacial and Craniofacial Deformities
Fig. 42.1, cont’d (e,f) The initial result 6 months after the completion of mandibular distraction. Because of the soft tissue expansion at the
right cheek, the buccal commissure has descended to the level of the contralateral side. The distance between the lateral canthus to the
mouth corner and to the menton has increased. Some volume is lacking on the right side. Mandibular overcorrection after bone regenera­
tion produced the menton medialization and an important leveling of both mandibular angles. (g,h) The result 17 years after distraction.
Facial symmetry has been maintained over time. It is still lacking volume on the right side. The patient returned asking for improvement of
this volume and definition on the right side of the face. (i) Preoperative occlusion. Orthodontics before distraction included maxillary ex­
pansion and intraoral Bionator-style dynamic appliances for 4 months. (j) Occlusion 3 years after distraction. A nice leveling of the maxillary
occlusal plane has been obtained. Posterior bite blocks were required to maintain the posterior open bite and to allow the vertical maxillary
growth. Dynamic orthodontic appliances also were used to maintain the elongation and to stabilize the occlusion.
e
f
g
h
i
j
(distraction period). Bone lengthening produces occlusal
changes that must be controlled with orthodontics;
posterior bite blocks, dynamic appliances, and other
maneuvers are commonly used.
•• Hemifacial microsomia should be overcorrected. This is
fundamental for growing patients. Occlusal deformities,
impossible to correct with orthodontics, must be
avoided when producing overcorrection. Usually, bite
blocks are required to maintain the posterior open bite,
so the blocks are gradually reduced to allow vertical
descent of the maxillae. Ideally, the maxillary occlusal
plane should be level horizontally 3 to 4 months after
distraction.
•• The consolidation period is about 8 weeks or more.
It depends on the age of the patient and the amount
of bone lengthening. More bone elongation requires a
longer consolidation period. If the patient is older than
10 years, this period can be extended (Fig. 42.2).
•• Age of treatment is also a critical factor. Initially the
procedure was always indicated at age 5 to 6 years,
including the more severe cases. Based on long-term
clinical observations, however, if an infant presents with

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
742
Suh and colleagues24 concluded after 15 years of expe­
rience that in their severe patients, the longitudinal results
achieved by distraction osteogenesis were unstable and gen­
erally led to relapse, although they were very good in the
early postoperative period. However, this paper also lacks
the methodology analysis of the postdistraction period.
I believe there is still a lack of understanding that bone
distraction does not change the hypoplastic mandible ge­
netics and growth potential. This malformation includes
muscles and other soft tissues as well. Because of this, func­
tional matrix principles do not work correctly. Well-man­
aged functional orthodontics can obtain vertical growth of
the maxilla and subsequently assist in satisfactory growth.
This fact probably is the most important prognostic factor
in the long term.
Indications for a Second Distraction
Procedure
In my experience, 18% of Pruzansky grade IIa and IIb pa­
tients require a second procedure. The most common rea­
sons are the following:
•• Erroneous distraction vector
•• Incomplete consolidation period
•• Lack of satisfactory overcorrection criteria
In all of these patients, relapse occurs 1 to 2 years later.
There is a strong tendency to retake the original ascending
ramus shape, facial asymmetry condition, and malocclu­
sion. If the patient is 12 years or older at the time of the sec­
ond bone elongation, a simultaneous maxillomandibular
distraction is indicated. In addition, a complete horizontal
maxillary osteotomy should be performed. On the affected
side, the pterygomaxillary junction is freed, but it is con­
a severe deformity (at least grade IIb), treatment should
occur at 3 or 4 years of age, simultaneously with vertical
growth of the maxilla. This prevents a constrained
mandible, which secondarily affects the growth of
the craniofacial skeleton, and avoids facial asymmetry
relapse in the short term.
Furthermore, the decision to use intraoral or extraoral
devices is based on mandibular bone stock. Generally, pa­
tients younger than 5 years who have severe or moderate
deformity and a limited bone stock are not good candidates
for internal distractors. In this group, the procedure fails to
achieve the ideal distraction vector and leads to secondary
occlusal problems, early relapse, and the need for future
surgical interventions.
The goal for all patients with hemifacial microsomia is
to obtain facial symmetry, with descent of the buccal com­
missure to the level of the contralateral side and a horizon­
tal menton located at the midline. To produce stability until
the patient reaches skeletal maturity, all of the previously
listed requirements must be fully addressed. Functional or­
thodontics should be continued until the patient reaches
skeletal maturity. In addition, ancillary procedures such as
asymmetrical menton osteotomies, dermal-fat grafts, and fat
injections should be included for select patients between 15
and 17 years of age to obtain optimal final results.
Nagy and colleagues23 studied randomized controlled
trials of early distraction osteogenesis in hemifacial micro­
somia patients, although statistical evidence to support the
use of early distraction osteogenesis for correcting hemifa­
cial microsomia as a single treatment modality is lacking.
Unfortunately, the postdistraction requirements (described
previously) were not included in this analysis. Once man­
dibular elongation is completed, additional criteria are re­
sponsible for providing good long-term results in hemifacial
macrosomia patients.
Fig. 42.2 (a) Preoperative view of a 6-year-old patient with Goldenhar’s syndrome. She presented with grade IIb mandibular hypoplasia
in the left side associated with an ear deformity, severe soft tissue hypoplasia that included the menton muscles, and upper lid coloboma.
(b) A mandibular distraction procedure was performed at the age of 8 years with a ramus elongation of 34 mm followed by a consolidation
period of 8 weeks. The result after the procedure included restoration of facial symmetry and ear correction. (c) At the age of 12 years, she
had a relapse of the deformity. A retrospective analysis of the causes for this secondary deformity revealed a short consolidation period
without a complete mineralization of the regenerate and an incorrect distraction vector (rather than a full vertical vector, a semioblique
one was used, producing an occlusal deformity that was impossible to correct with orthodontics).
a
b
c

743
Chapter 42 Orofacial and Craniofacial Deformities
extra aesthetic surgical procedures. Free dermal-fat grafts
are commonly used for extra volume and mandibular angle
definition; fat injection is an excellent alternative to produce
volume in the whole hemiface and improve contour, defini­
tion, and angularity.
Mandibular distraction has evolved from the use of ex­
ternal to the use of intraoral and semiburied devices. Differ­
ent authors have evaluated the advantages and limitations
of various devices, and the perioperative problems and com­
plications have been reported25 (Table 42.1).
served at the unaffected side to serve as a pivot. Intermax­
illary fixation is performed and activation is initiated. The
result shows that together with the mandibular elongation,
the maxilla follows the bone changes, achieving simultane­
ous vertical elongation. Occlusal plane slanting varies from
12 to 18 degrees to 1 to 2 degrees after the procedure.
Especially in adults, the chin is partially medialized by
the distraction. It is not always possible to achieve a normal
central position. Therefore this procedure does not eliminate
the need for an asymmetrical sliding genioplasty or some
Table 42.1 Mandibular distraction osteogenesis incidents and complications
External devices
in native bone
(n = 129 procedures)
External devices
in grafted bone
(n = 37 procedures)
Semiburied devices
in native bone
(n = 45 procedures)
Minor incident
Difficulty in device activation
Device backup
Improper vector
Inadequate device length
Pain on activation
Hypertrophic scar
Cyst caused by pins
Psychological problem
Neurapraxia (inferior alveolar nerve)
Infection (oral antibiotics sufficient)
Trismus during treatment or device removal
Parotid gland injury
Subtotal (incident rate)
4
0
1
1
8
1
1
0
0
13
2
2
33 (26%)
3
0
0
0
4
0
0
0
0
5
2
0
14 (38%)
0
6
0
0
7
0
0
1
4
3
7
0
28 (62%)
Moderate incident
Improper vector
Device backup
Loose device
Device deformation breakage
Premature ossification
Scar requiring revision
Infection (requiring intravenous antibiotics)
Additional incision to remove device
Subtotal (incident rate)
6
1
16
1
2
2
0
0
28 (22%)
2
0
7
1
0
1
0
0
11 (30%)
2
0
1
0
3
0
1
1
8 (18%)
Major incident
Unstable device
Tooth follicle or damaged teeth
Premature consolidation
Temporomandibular joint ankylosis and
degenerative changes
Fracture
Hypertrophic scar
Subtotal (incident rate)
0
1
1
1
1
1
5 (4%)
2
0
1
2
0
0
5 (14%)
0
0
0
0
0
0
0 (0%)
Total (incident rate)
66 (51%)
30 (81%)
36 (80%)
Source: From Davidson EH, Brown D, Shetye PR, et al. The evolution of mandibular distraction: device selection. Plast Reconstr Surg
2010;126(6):2061–2070.

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
744
a cleft or as easily classified. Rare or atypical clefts come in
myriad patterns of clinical expression.
The exact incidence of rare or atypical craniofacial clefts
is unknown. Forme fruste and incomplete forms of cleft may
go unnoticed. The incidence of common clefts of the lip and
palate is better understood. Cleft lip occurs in approximately
1 in 1,000 live births among those of Western European de­
scent.26 The condition is more prevalent in the Japanese
population, with 2.1 per 1,000 births,27 and less common in
African Americans, with 0.4 per 1,000 births.
Davis28 found facial clefts have an occurrence rate of 9.6
per 1,000 for common clefts. Burian29 found an occurrence
rate of atypical facial clefts of 24.3 per 1,000. Ortiz-Monas­
terio30 reported an occurrence rate of 31 atypical clefts per
1,000 common clefts.
Historically, two theories of facial clefting have been de­
scribed: (1) the classic theory of fusion by Dursy31 and His,32
in which the clefts develop as a result of the failure of fusion
of the normally merging facial processes, and (2) the theory
of mesodermal migration, which is based on the importance
of neural crest cells in the development of the mesodermal
component of the craniofacial form.33–35
Classification
Attempts to classify craniofacial clefts have a long history
in the literature based mainly on clinical and anatomic
judgment. Some classification schemes have focused on
morphogenesis to explain the mechanism involved in the
formation of clefts.36,37 In 1962 the American Association
of Cleft Palate Rehabilitation (AACPR)38 classified rare facial
clefts into four major groups:
1. Mandibular process clefts
2. Naso-ocular clefts
3. Oro-ocular clefts
4. Oroaural clefts
In 1974, at the Interdisciplinary Workshop Conference
on Craniofacial Surgery in Chicago, Tessier presented
a comprehensive classification scheme of craniofacial
clefts. Detailed descriptions were later published in 1976
by Tessier39,40 and Kawamoto.41 This system focused on
surgical anatomy and integrating external findings with
the underlying skeletal malformation.39 This classifica­
tion does not attempt to explain the morphogenesis of
facial clefts but provides a topographic description that
has been widely accepted. It proposes a simplified nu­
meral system from 0 to 14 for all clefts depending on
their relationship to the 0 line, which is a vertical cleft of
the midline of the face. The palpebral fissures separate
facial from cranial clefts.
Operative Considerations
Given the rarity and unique nature of the clinical expres­
sion of each craniofacial cleft, treatment plans cannot be
standardized; they must be based on the individual assess­
From their paper evaluating 20 years of follow-up,
Davidson and his group25 highlighted the benefits of semi­
buried devices compared with external devices. They
pointed out that semiburied devices reduce scarring and
have the mechanical advantages of being applied directly
to the bone, being less vulnerable to dislodgment, and be­
ing more favorable for a vertical vector. However, their use
requires more bone stock, and they have the disadvantage
of requiring a second operation for removal.
Conclusion
Distraction osteogenesis has simplified the treatment for
congenital mandibular hypoplasia. Technically, it is a minor
surgical procedure that preserves the integrity of the nerve
and vascular supply. The corticotomy and pins produce
distraction vectors that allow for vertical and sagittal man­
dibular elongation that closely follows the normal direction
of mandibular growth.10–16
Orthodontics are mandatory; usually a posterior open
bite is used at the affected side, and dentoalveolar adapta­
tion and maxillary vertical growth are achieved. In the fol­
lowing years a very satisfactory craniofacial growth can be
observed.
The simultaneous expansion of the surrounding soft
tissue of the face is an additional significant benefit.19,20
Rapid descent of the buccal commissure to a normal po­
sition, chin correction, and an increase in the distance be­
tween the buccal commissure and the lateral canthus are
observed in all cases. During adolescence additional facial
volume is required, so dermal-fat grafts and fat injections
are commonly used. Chin osteotomies are used for the final
refinements to correct facial asymmetry in patients with
hemifacial macrosomia.
Craniofacial Clefts
The word face has many connotations. This can be under­
stood in the context of the relationship individuals have
with their own faces. Externally, it is their appearance as
they would like it to be, whether it is through the eyes of
others or the image they see of themselves, reflected in a
mirror. Internally it reflects a person’s worth. It is an in­
dividual’s dignity and integrity; it is also one’s facade and
mask. This inner and outer relationship individuals have
with their face is singularly entwined.
When a cleft runs through a face, the individual may
seem himself or herself reflected in a mirror as shattered. A
child born with a craniofacial cleft typically feels a sense of
disfigurement and isolation. Regardless of the surgeon’s at­
tempts to restore the physical structure of the face, it is the
child who reassembles and reconstructs his or her face from
the inside out. Perhaps no other singular deformity distorts
the face as much as a cleft.
The meaning of the word cleft is apparent and easily un­
derstood when one is faced with a clinical example, such as a
cleft lip or palate. However, not all defects in the apposition
of junctional structures are either as obviously definable as

745
Chapter 42 Orofacial and Craniofacial Deformities
to close the fissure in all cases, but our aesthetic results fell
short of expectations (Fig. 42.3).
Failure to meticulously restore the continuity across the
cleft can result in a loss of muscular animation or may leave
a persistent stigmata, such as a depressed scar, because of
the lack of appropriate soft tissue support.
When the regional tissue is believed to be insufficient
to allow correction with local flaps alone, an alternative
approach is the use of tissue expanders to restore the soft
tissue defect with adjacent expanded skin of similar color,
texture, and appearance. Patients underwent a total of 1,338
operations, or an average of 2.7 operations per patient.
Before the implementation of our current treatment phi­
losophy in 1985, 215 patients underwent 470 operations,
ment of each case. Nevertheless, there are general princi­
ples by which the sequence and timing of staged osseous
and soft tissue reconstruction can proceed.
Only in the mildest expression of a facial cleft can the
defect be corrected with a single operative procedure. Early
in our unit’s experience, success was defined by repair of a
cleft. We closed skeletal clefts with bone grafts. Soft tissue
clefts were closed by direct approximation, flap rotation, or
z-plasty. The interposition of flaps from different regions of
the face produced a patchwork effect because of the differ­
ences in color, texture, and thickness of the skin. Z-plasties
increased the patchy effect, resulting in scars crossing the
face in many directions and inconspicuous locations.42 In
reviewing our early results, we realized that we were able
Fig. 42.3 (a,b) Preoperative views of a 4-month-old boy
with a very extensive macrostoma. The cleft included the
total cheek structure, dividing the parotid gland and reach­
ing the left ear. The facial nerve was intact. (c,d) Postop­
erative result 1 year after z-plasties were used to elongate
the distance between the buccal commissure and the ear.
Although the quality of the scars is very good, it extends
across the aesthetic subunit of the cheek. Radiographic stud­
ies were completed to assess the bone deformity. (e,f) At
4 years of age, he had facial asymmetry with deviation of
the chin, inferior lip, and buccal commissure to the affected
side. The patchwork appearance of the cheek is a result of
the z-plasties. A macrotia is also obvious on the left side.
(g) At the age of 7 years, a bilateral mandibular distraction
was performed to correct the asymmetrical micrognathia.
The body has been elongated more on the left side than on
the right. The chin is located at the midline and the man­
dibular dimension is similar on both sides. (h) At 12 years
of age, the facial symmetry has been restored; however,
some stigmata is still visible in the form of scars, soft ­tissue
­deficiency, lip incompetence, and ear malposition on the
left side.
e
f
g
h
a
b
c
d

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
746
metry caused by bony and soft tissue irregularities distracts
from an aesthetically pleasing appearance. Now, we always
try to obtain the following objectives:
•• Restoration of the craniofacial skeleton
•• Reconstruction with skin and soft tissue of like color
and texture
•• Generous use of tissue expanders
•• Aesthetic unit and subunit reconstruction
•• Scar location at the limits of aesthetic subunits
•• Symmetrical repositioning of key facial landmarks
In our patients, tissue expansion is used in 64% of all primary
reconstructions and in 100% of cases involving total nasal re­
construction. Direct approximation or local flap reconstruc­
tion of soft tissues was achieved in 100% of cases, eliminating
the need for skin grafts or distant flaps (Fig. 42.4).
or 2.2 operations per patient. The 280 patients operated on
with the new treatment philosophy from January 1985 on
underwent 868 operations, or 3.1 operations per patient.
This includes placement of tissue expanders and all revisions
performed under general anesthesia; revisions performed
under local anesthesia are excluded.
In our unit, a series of 495 patients with major cranio­
facial clefts were treated43; 65% had multiple clefts and 35%
had a single cleft. In 33% of patients, the clefts were uni­
lateral, and in 67% the clefts were bilateral. The treatment
objectives were to restore the craniofacial skeleton and re­
construct with skin and soft tissue of like color and texture;
this relied on the generous use of tissue expanders.
This modern concept was developed by following our
patients over more than 4 decades; it became clear that in­
correctly placed scars do not improve with time, differences
of skin color and texture are permanent, and facial asym­
Fig. 42.4 This 5-year-old boy presented with multiple major facial clefts. He had nasal deformity, right macrostomia, eyelid and orbital de­
formity, and microtia with mandibular hypoplasia. (a) A frontal tissue expander was inserted for a nasal reconstruction. (b) Postoperatively
after nasal reconstruction, iliac bone graft for right ramus reconstruction, and macrostomia repair. (c) At 8 years of age. After radiologic
mandibular deformity assessment, unilateral distraction was performed on the residual bone stuck from the iliac graft. A simultaneous tele­
canthus correction and scar lip revision was also done. (d) Three-dimensional computed tomography was performed at the age of 14 years.
At the mandible, the final bone regenerated at the angle and ascending ramus is visible. There was important maxillary vertical growth,
almost to the level of the contralateral side. Bone grafts are present in the nasal dorsum. The right orbit has a slight lateral displacement;
however, both orbits have a very similar structure. (e) At 18 years of age, the ear reconstruction has been completed and a very reasonable
facial symmetry has been obtained after a number of surgical procedures.
a
b
c
d
e

747
Chapter 42 Orofacial and Craniofacial Deformities
terization. There were no perioperative deaths and no cases
of perioperative blindness.
The overwhelming majority of patients underwent elec­
tive operations. However, there were three severe cases of
Treacher Collins’ syndrome in which urgent mandibular
distraction osteogenesis was used to improve upper airway
mechanics and avoid the need for tracheostomy. Twelve pa­
tients with upper eyelid colobomas also underwent urgent
closure of the colobomas to protect the globe and preserve
vision. Of the 30 patients with upper eyelid colobomas in
this series, 11 were blind in the affected eye because of a
delay in treatment. Twelve patients presenting with large
enough colobomas to cause blindness were treated urgently
with preservation of vision. Seven had colobomas that were
either too peripheral or too small to cause blindness.
Short-term solutions for upper eyelid colobomas
included an ocular moistening chamber or temporary tar­
sorrhaphy. Definitive treatment consisted of direct closure,
Complications
The results of this study and the associated complications
are shown in Table 42.2.
Our overall complication rate was 6.2%. Hypertrophy or
widening of scars occurred in 18 cases (3.6 %) and was usu­
ally located in the region of the lip. Infections occurred in 17
cases (1.3%) and consisted of eight wound infections, seven
deep surgical site infections, and two cases of bacterial men­
ingitis. Transnasal canthopexy had to be revised in 11 cases.
New-onset diplopia occurred in 6 cases (0.4%), and late-re­
current canthal dystopia occurred in 10 cases (0.8%). Nine
patients (0.7%) developed tissue expander problems—five
infections and four extrusions—that required its removal.
Four patients (0.3%) required removal of osteosynthesis ma­
terial. Two patients had acute intracranial hematomas that
required evacuation, and one patient had a nasopharyngeal
bleed that required a return to the operating room for cau­
Table 42.2 Results
Percentage of patients
Number of cases
(n = 495)
Multiple clefts
65
321.75
Single clefts
35
173.25
Complication rate
6.2
30.69
Hypertrophy (widening of scar)
1.3
18
Infection
  Wound infection
  Deep surgical site infection
  Bacterial meningitis
1.3
17
8
7
2
Transnasal canthopexy
–
11
New-onset diplopia
.4
6
Late-recurrent canthal dystopia
.8
10
Tissue expander issues
  Infection
  Extrusion
.7
9
5
4
Osteosynthesis material
removal
.3
4
Acute intracranial hematoma
–
2
Nasopharyngeal bleed
–
1
Deaths
–
0
Perioperative blindness
–
0
Total complications
–
78

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
748
cases (22%): four cases of hemifacial rotation, four cases of
orbital translocation, and three cases of medialization of the
medial orbital walls.
Although the cranial vault grows rapidly in the first year
of life, it levels off early. The upper midface grows at a slower
rate in infancy but it continues to grow later in childhood
and early adolescence. In contrast, the growth of the lower
midface and mandible, determined by eruption of the den­
tition, continues well into adolescence. This overall growth
allows the cranio-orbitozygomatic region to reach approxi­
mately 85% of its adult size by age 5.46
Consequently, skeletal reconstructive surgery involving
the upper midface (cranio-orbitozygomatic region) should
be relatively stable after age 5 or 6 years. In contrast, re­
construction of a patient’s maxillary–mandibular complex
should be postponed until adolescence, although there are
exceptions. Unless significant functional concerns arise, the
timing of reconstruction should be based on an understand­
ing of the normative residual growth expected within the
operated region of the facial skeleton. Basic principles apply
to all facial clefts, but specific problems may arise as indi­
vidual clefts are addressed.
Conclusion
The laws of normal growth are best formulated and under­
stood when the causes of their exceptions can be estab­
lished. The experimental method itself rests on the notion
that induced and controlled departures from the ordinary
can unveil the laws of order. Consequently, the congeni­
tally malformed are nature’s experiment, uncontrolled by
intentional human art to be sure, but a source of insight
nevertheless. The early teratologists sought to understand
malformations by classifying them. The early French anato­
mists developed three categories: missing parts (monstres
par defaut), extra parts (monstres par exces), and normal
parts in the wrong places. But a classification is no more
than a set of pigeon holes until the causes of the ordering
can be specified.
Hypertelorism
Hypertelorism is a descriptive term commonly used to
signify any abnormal increase in intraorbital distance.47
Strictly defined, hypertelorism is an abnormally great dis­
tance between any two paired body parts; for this reason,
Tessier et al48,49 preferred the term orbital hypertelorism,
or hypertelorbitism. Telecanthus refers to an increased in­
tercanthal distance, and although telecanthus always ac­
companies hypertelorism, the reverse is not always true;
in some instances, there may be an increased intercanthal
distance without true hypertelorism, commonly after facial
trauma or blepharophimosis.
During embryologic development, the optic placodes
arise laterally and then medially translocate. Hypertelorism
results when any condition interrupts this medial move­
local flap closure, or a lid-switch operation. Of the 66 pa­
tients with lower eyelid colobomas, only four were blind
and all had corresponding upper lid colobomas. No patient
with an isolated lower eyelid coloboma went blind, even in
cases of delay in treatment.
All 183 patients who underwent primary reconstruc­
tion with local flaps and z-plasties had a secondary re­
construction using expanders and the aesthetic subunit
principle, meaning that all scars were located at the limits of
aesthetic subunits and facial symmetry was improved signif­
icantly (see Fig. 42.4). Only 50 (52%) of the secondary cases
achieved reconstruction by aesthetic subunits and were left
with scars crossing aesthetic subunits. Tissue expansion was
used in 64% of all primary reconstructions and in 100% of
cases involving total nasal reconstruction.
Direct approximation or local flap reconstruction of soft
tissues was achieved in 100% of cases, eliminating the need
for skin grafts or distant flaps. Formal craniofacial osteotomy
was necessary in 53% of patients, and 24% required bone
grafting to restore the craniofacial skeleton. Bone graft was
used on all bony fissures that did not require major craniofa­
cial osteotomies. Most patients requiring bone grafting also
required soft tissue work, and these reconstructive proce­
dures were combined when possible. Thus, as with soft tis­
sue work, we completed most bony reconstruction before
the child entered school.
Hypertelorism was the most common indication for ma­
jor craniofacial osteotomy. In 51 cases of median and para­
median clefts, the combination of hypertelorism, anterior
open bite, and shortened midface was treated with hemifa­
cial rotation with satisfactory resolution. Hemifacial rotation
does not retard midfacial growth44 and thus was performed
after age 3 years, at which time the frontozygomatic suture
is sufficiently strong, and before the child enters school.
In contrast, 64 adolescent patients underwent orbital repo­
sitioning by means of orbital translocation.
Optimal timing for intervention remains unclear. Early
manipulation of the underlying bony skeleton in the region
may potentially disturb normal midface development. Bone
grafting of the bony defect early may lock the maxillary seg­
ments in place and interfere with the normal direction of
growth and remodeling. Although, arguably, the inherent
growth potential in these cases remains unknown and may
possibly be limited because of the severity of the anomaly,
early restoration of the skeletal contour can significantly re­
duce the deformity and allow the child to be mainstreamed
earlier into society. However, what may be an acceptable
result at age 5 or 6 years may be less than ideal in the long
term.44,45
Because it is especially true that orbital translocation
retards midfacial growth, it was delayed in this study until
skeletal maturity. Seventy-five patients with mild hyper­
telorism, Tessier grade II, underwent medialization of the
medial orbital walls before entering school. Transnasal can­
thopexy with 28-gauge stainless steel wire was performed
in every case, and the canthopexy had to be revised in 11

749
Chapter 42 Orofacial and Craniofacial Deformities
The timing for surgical intervention is based on a num­
ber of factors. Babies with encephaloceles may require early
treatment for resection of the encephalocele sac. Extreme
care must be taken in basal encephaloceles, because they
may contain the pituitary gland or the hypothalamus and
a gross resection may lead to panhypopituitarism. Children
with severe hypertelorism may not be able to develop ste­
reoscopic vision if treatment is delayed.
Surgical treatment at early ages can potentially affect
midface growth. However, some reports have suggested
that this is only partially true.53–57 It seems intuitive that
the larger the surgical procedure and the younger the pa­
tient, the more likely that growth centers will be affected,
and that subsequent growth will be diminished. Most expe­
rienced surgeons will delay surgical correction until a signif­
icant amount of growth has occurred, but before any serious
psychological issues have had time to develop (usually at 6
to 8 years of age depending on development).
Surgical procedures at a later age will be more stable,
resulting in better outcomes. The greater the degree of hy­
pertelorism, the less likely that a single surgical procedure
will achieve an optimal final result.
A number of surgical techniques can be used to treat hy­
pertelorism. The cause and the degree for hypertelorism will
influence the decision of which technique should be used.
Ortiz-Monasterio and Molina55 suggested that surgical cor­
rection for telorbitism is essentially an extended rhinoplasty
through an intracranial approach. In fact, surgical treatment
of hypertelorism is one of the more complex operations by
craniofacial surgeons.
The mildest degree of hypertelorism may be treated
with subcranial two-wall osteotomies. The two medial walls
are then medially translocated after removing a central strut
of the nasal dorsum, perpendicular plate, and posterior eth­
moid sinuses sufficient to permit medial globe transposition.
This medial movement is primarily responsible for distance
reduction between the orbits, and in these patients, no sig­
nificant effect on globe position is observed.
The U-shaped technique (three-wall osteotomy) in­
cludes the orbital floor with the medial and lateral walls as
a single unit. The osteotomy excludes the orbital roof, obvi­
ating an intracranial approach. The nasal airway should be
always preserved. The disadvantages of this procedure are
the difficulty of the infraorbital osteotomy, the risk of injury
to the infraorbital nerve and the lacrimal fossa, and the at­
tempted tooth buds.
“On block” osteotomies are indicated for the most severe
cases. In this technique, the orbit is circumferentially cut in
the coronal plane posterior to the eye globe equator and a
portion of nasal bone, the perpendicular plate, and the pos­
terior ethmoid sinuses is removed. This approach provides
complete medialization of the orbits and allows for more
significant translocations that may result in a better final
stability.
Facial bipartition is usually reserved for children who
have any condition with a midline separation of the face
ment or when some structure, usually the brain, expands
and pushes the orbits apart. The following are the most com­
mon causes for hypertelorism:
•• Craniofrontonasal dysplasia
•• Facial clefting
•• Encephaloceles
•• Facial trauma
•• Craniosynostosis
•• Tumors
Frontonasal dysplasia sequence, more common in girls
than boys, is the most common cause of hypertelorism.
A widow’s peak and broad nasal tip are common mani­
festations.50,51 The nose is quite broad and truly bifid.
Craniofrontonasal dysplasia is a combination of coronal
synostosis with frontonasal dysplasia.52,53
Midline facial clefting may be associated with other mid­
line intracranial anomalies. Facial clefting associated with
encephaloceles, which may protrude from the cranial cavity
through a single or multiple locations, is a very complex de­
formity. Isolate encephaloceles may be the one exception to
the belief that, in all cases of hypertelorism, the distance be­
tween the optic canals is normal, or only slightly increased.48
A frontal encephalocele usually exits through the foramen
cecum, and a basal encephalocele through the skull’s floor;
the midline migration of the orbits is affected, resulting in
hypertelorism.
Significant facial trauma most often results in telecan­
thus, but it may also cause hypertelorism; usually a compo­
nent of vertical globe dystopia and significant asymmetry
accompany the increased interorbital distance.
Syndromic craniosynostosis, particularly Apert’s and
Pfeiffer’s syndromes, may present with an increased in­
terorbital distance. Tumors such as fibrous dysplasia and
syndromes and Greig’s and Opitz’s syndromes share hyper­
telorism as a common phenotypic component.
For young adults, the normal values for interorbital dis­
tance have been described based on radiologic measure­
ments, between the lacrimal crests.54,55 Giinther56 described
the following grading system:
1. First degree (30 to 34 mm)
2. Second degree (35 to 39 mm)
3. Third degree (40 mm or more)
The surgical approach varies based on the severity.
Operative Considerations
Hypertelorism does not always require surgical treatment.
In fact, mild hypertelorism is considered by many to be an
attractive trait. In addition, the epicanthic folds, exotropia,
widely spaced eyebrows, narrow palpebral fissures, and
dystopia canthorum may contribute to an illusion of hy­
pertelorism.52

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
750
Complications
The most common complication after hypertelorism cor­
rection is a nonsatisfactory aesthetic result in which the
child’s appearance is compromised (Fig. 42.5).
An average reduction between the orbits of 5.3 mm
postoperatively (only 2.7 mm between the centers of the
globes)60 and an interorbital reduction of 6 mm61 have been
reported. Despite these very modest improvements after
major surgical corrections, patients and families have re­
ported satisfaction with the results62 (Fig. 42.6).
Infection is one of the more common complications. The
infection rate is higher because the required osteotomies
must cross sinus cavities. Most craniofacial infections do not
present clinically until about 10 days postoperatively, and
early antibiotic treatment should be initiated.63
In hypertelorism associated with basal encephaloceles,
brain injury is the most common complication. In addi­
tion to infections, cerebrospinal fluid leaks, anosmia, ma­
(clefts and encephaloceles). An intracranial route is required.
In addition to bringing the orbits closer together, the rota­
tional movement of the two lateral facial halves increases
the midface vertical dimension and corrects the anterior
open bite.56,58,59
Osteotomies are performed in the orbital roofs, medial and
lateral walls, and the floors. Instead, a pterygomaxillary dys­
junction is performed to allow the maxilla to move in continuity
with the orbits. In some patients, the dysjunction may interfere
with future midface growth. The larger bone-segment medi­
alization can enhance final stability and preserve unerupted
tooth buds. Medial canthopexies are always performed, but the
decision regarding bone grafting the nasal dorsum depends on
the dorsal height of the paired nasal bones. For children with a
significantly increased intraoptic nerve distance, a staged cor­
rection should be considered to avoid potential loss of vision.
Surgeons without significant pediatric congenital craniofacial
experience should only undertake these procedures with the
assistance of an experienced surgeon.
Fig. 42.6 (a) This 5-year-old girl presented with craniofrontonasal dysplasia and hypertelorism. A failed attempt at surgical correction left
frontal and paranasal scars. She had lateral canthal dystopia and a vertical shortness of the face. (b) Result 1 year after hemifacial rotation.
Skin resection was performed at the frontonasal area. The interorbital distance was reduced 26 mm, the anterior open bite was corrected,
and the lateral ligaments were moved to a proper position. (c) Long-term result after 7 years. The interorbital distance has reverted to its
original position. A late hypertelorism relapse, probably secondary to ethmoid sinus pneumatization, occurred. The frontal deformity is
very similar to the initial condition.
a
b
c
Fig. 42.5 (a) This 9-year-old girl had severe hypertelorism and
craniofrontonasal dysplasia. The deformity was associated with
a cleft lip and palate that was corrected early. (b) Result 2 years
postoperatively. Although an important orbital medialization
has been obtained, facial asymmetries and soft tissue undercor­
rection remain. Epicanthal folds, a marked difference between
the palpebral apertures, telecanthus, and nose asymmetry are
still evident.
a
b

751
Chapter 42 Orofacial and Craniofacial Deformities
However, in many cases the intracranial route is used to
medially mobilize both orbits or the two halves of the face.
Conclusion
Every patient who presents with orofacial and craniofacial
deformities is a candidate for correction. Patients should
receive a multidisciplinary treatment in which the experi­
ence, skills, and knowledge of a group of health profession­
als provide different treatment protocols to obtain optimal
aesthetic and functional results.
jor relapse, and postoperative ptosis have been reported;
many more serious complications may have gone unre­
ported.57,64–66
Conclusion
Hypertelorism is a condition of increased intercanthal dis­
tance. The surgical correction results in excellent aesthetic
and functional results for most patients. Minor deformi­
ties can be easily corrected with subcranial approaches.
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54. Mulliken JB, Kaban LB, Evans CA, Strand RD, Murray JE.
Facial skeletal changes following hypertelorbitism correc­
tion. Plast Reconstr Surg 1986;77(1):7–16
55. Ortiz-Monasterio F, Molina F. Orbital hypertelorism. Clin
Plast Surg 1994;21(4):599–612
56. Tessier P. Facial bipartition: a concept more than a pro­
cedure. In: Marchac D, ed. Craniofacial Surgery. Berlin:
Springer-Verlag; 1987:217
57. McCarthy JG, La Trenta GS, Breitbart AS, Zide BM, Cutting
CB. Hypertelorism correction in the young child. Plast Re­
constr Surg 1990;86(2):214–225, discussion 226–228
58. van der Meulen JC. Medial faciotomy. Br J Plast Surg 1979;
32(4):339–342
59. Ortiz Monasterio F, Molina F, Sigler A, Dahan P, Alvarez L.
Maxillary growth in children after early facial bipartition.
J Craniofac Surg 1996;7(6):440–448
60. Panchal J, Kim YO, Stelnicki E, Pilgram T, Marsh JL. Quanti­
tative assessment of osseous, ocular, and periocular chang­
es after hypertelorism surgery. Plast Reconstr Surg 1999;
104(1):16–28
61. Songür E, Mutluer S, Keçeci Y, Alper M, Güner U, Totan S.
Late results of hypertelorism correction. J Craniofac Surg
1997;8(1):29–31
62. Poole MD, Walters E, Beardsworth E, Griffiths P. Orbital
dystopia: attempts to evaluate the results of surgery. Br J
Plast Surg 1991;44(2):81–86
63. Fearon JA. Rigid fixation of the calvaria in craniosynostosis
without using “rigid” fixation. Plast Reconstr Surg 2003;
111(1):27–38, discussion 39
64. Munro IR, Das SK. Improving results in orbital hyper­
telorism correction. Ann Plast Surg 1979;2(6):499–507
65. Ortiz Monasterio F, Medina O, Musolas A. Geometrical
planning for the correction of orbital hypertelorism. Plast
Reconstr Surg 1990;86(4):650–657
66. van der Meulen JCH, Vaandrager JM. Surgery related to
the correction of hypertelorism. Plast Reconstr Surg 1983;
71(1):6–19
30. Ortiz Monasterio F, Fuente del Campo A, Dimopulos A.
Nasal clefts. Ann Plast Surg 1987;18(5):377–397
31. Dursy E. Zur Entwicklungsgeschichte des Kopfes des
Menschen und der hoheren Wirbeltheire. Tubingen: Ver­
lag der H. Lauppschen-Buchhandlung; 1869
32. His W. Die entwicklung der menschlichen und thierischer
physiognomen. Arch Anat Entwicklungsgesch; 1892:S384
33. Stark RB. The pathogenesis of harelip and cleft palate.
Plast Reconstr Surg (1946) 1954;13(1):20–39
34. Johnston MC. The neural crest in abnor-malities of the
face and brain. Birth Defects Orig Artic Ser 1975;11(7):
1-18
35. Vermeij-Keers C, Mazzola RF, Van der Meulen JC, Strickler
M. Cerebro-craniofacial and craniofacial malformations:
an embryological analysis. Cleft Palate J 1983;20(2):128–
145
36. Niemeyer MF, van der Meulen J. The genetics of cranio­
facial malformations. In: Stricker M, vander Meulen J,
Rápale B, Mazzola R, eds. Craniofacial Malformations.
London: Churchill Livingstone; 1990
37. van der Meulen JC, Mazzola R, Vermey-Keers C, Stricker
M, Raphael B. A morphogenetic classification of craniofa­
cial malformations. Plast Reconstr Surg 1983;71(4):560–
572
38. Harkins CS, Berlin A, Harding RL, Longacre JJ, Snodgrasse
RM. A classification of cleft lip and cleft palate. Plast Re­
constr Surg Transplant Bull 1962;29:31–39
39. Tessier P. Anatomical classification facial, cranio-facial
and latero-facial clefts. J Maxillofac Surg 1976;4(2):69–92
40. Tessier P. Anatomical classification of facial, craniofacial,
and lateral facial clefts. In: Tessier P, ed. Symposium on
Plastic Surgery in the Orbital Region. St Louis: CV Mosby;
1976
41. Kawamoto HK Jr. The kaleidoscopic world of rare cranio­
facial clefts: order out of chaos (Tessier classification).
Clin Plast Surg 1976;3(4):529–572
42. Tessier P. [Complete and forme fruste vertical and oblique
orbito-facial clefts (colobomas)]. Ann Chir Plast 1969;
14(4):301–311
43. Monasterio FO, Taylor JA. Major craniofacial clefts: case se­
ries and treatment philosophy. Plast Reconstr Surg 2008;
122(2):534–543
44. Kawamoto HK. Rare craniofacial clefts. In: McCarthy J, ed.
Plastic Surgery. Vol 4. Philadelphia: WB Saunders; 1990
45. Resnick JI, Kawamoto HK Jr. Rare craniofacial clefts: Tes­
sier no. 4 clefts. Plast Reconstr Surg 1990;85(6):843–849,
discussion 850–852
46. Kazanjian VH, Holmes EM. Treatment of median cleft lip
associated with bifid nose and hypertelorism. Plast Re­
constr Surg Transplant Bull 1959;24:582–587
Hyperteleorism
47. Greig DM. Hypertelorism: a hitherto undifferentiated con­
genital craniofacial deformity. Edinburgh Med J 1924;31:
560

753
Chapter 42 Orofacial and Craniofacial Deformities
Discussion by Philip Kuo-Ting Chen and Susie Lin
Avoiding and Managing Unfavorable
Results and Complications in
Hemifacial Microsomia
The problems associated with hemifacial microso­
mia (HFM) involve both the skeleton and soft tissues.
Patients with HFM may have a lateral facial cleft with
macrostomia; hypoplasia of the craniofacial skeleton,
which mainly affects the mandible; soft tissue defi­
ciency, which may involve different tissue planes of the
face and palate; facial nerve palsy; and/or ear defor­
mity. The treatment protocol for HFM should be com­
prehensive; treatment of different problems should
be performed at different stages of development. The
treatment protocol followed at Chang Gung Craniofa­
cial Center is outlined in Table 42D.1.
Macrostomia is repaired around 3 to 6 months of
age. A small vermilion flap is transferred from the lower
lip to the upper lip across the commissure to prevent
possible commissure distortion from scar contracture.
The cheek muscle is repaired in an overlapping fashion
with the lower musculature on top of the upper mus­
culature to bring the mouth angle upward and prevent
possible downward displacement of the mouth angle
by the scar. A z-plasty is designed on the skin closure
with the medial limb going downward, the central limb
vertically along the nasolabial fold, and the lateral limb
horizontally to form the least conspicuous scar.1
Hemipalatal hypoplasia may affect velopharyngeal
closure, resulting in velopharyngeal insufficiency. This
condition is different from the velopharyngeal insuffi­
ciency in patients with a cleft palate, which results from
the displacement of the levator veli palatini muscles. The
velopharyngeal insufficiency in HFM is caused by asym­
metry of the soft palate, with different degrees of hy­
poplasia on the affected side. A pharyngeal flap rather
than levator muscle repositioning is usually required
to correct the velopharyngeal insufficiency in patients
with HFM.2
Significant asymmetry in the orbital region can re­
sult in significant psychological stress for the patient.
A corrective osteotomy is usually planned after 5 years
of age when the cranio-orbitozygomatic region reaches
approximately 85% of its adult size.
A midface degloving dissection is approached
through bicoronal, infraciliary, and intraoral incisions.
Discrepancy in the horizontal dimension is easier to cor­
rect than that in the vertical or sagittal dimensions. A C-
shaped zygomatic osteotomy is performed through the
frontozygomatic suture, zygomaticomaxillary buttress,
and zygomatic arch. The zygoma is moved laterally to
expand the orbital width.
For surgical correction of the vertical and sagittal
orbital dystopia, our preference is to perform distrac­
tion osteogenesis using an intraoral vertical distractor
for vertical lengthening of the zygomaticomaxillary
Table 42D.1 The treatment protocol for hemifacial microsomia at
Chang Gung Craniofacial Center
Area treated
3 to 6 months
of age
3 to 4 years
of age
5 years of age
and older
Adolescents
Adults
Macrostomia
✔
Hemipalatal
hypoplasia
✔
Cranio-orbital
✔
Maxilla–mandible
✔
✔
Ear
✔
✔
Soft tissue
*
✔
✔
Facial nerve
✔
✔
*Soft tissue augmentation might be considered in preschool children if they already have psychological problems.

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
754
complex, moving the infraorbital rim upward and us­
ing a rigid external distraction (RED) device to move
the whole complex forward. The supraorbital rim is also
moved upward during the initial osteotomy through the
bicoronal incision. These procedures seem complicated;
however, they are less technically demanding than the
box-type orbital osteotomy, which involves moving the
orbits upward then filling in the dead space beneath
the infraorbital rim by performing other additional
­procedures.
Since the mid-1990s, we have been using distrac­
tion osteogenesis to correct mandibular discrepancy in
HFM for early correction of skeletal problems around
preschool age. The long-term results of our earlier cases
are not as ideal as the results shown in Chapter 42. We
performed a complete osteotomy at the mandibular an­
gle and applied a single-vector or multivector external
distractor for mandibular distraction. Despite overcor­
rection in mandibular distraction and maintenance of
the mandibular symmetry for 1 year after distraction,3
the patients showed gradual relapse at longer follow-up.
The cause of the relapse was attributed to the residual
canting in the maxilla. As a result of these observations,
we have changed our protocol to a two-jaw distraction
in preschool children. Le Fort I osteotomy is performed
at the same time as mandibular osteotomy. The maxilla
is wired to the mandible with intermaxillary fixation
and an external multivector distractor is then applied.
Although the maxillary canting has been successfully
corrected, we still see surgical relapses during long-term
follow-up.
The age of the patient at the time of distraction
osteogenesis plays a determining role in surgical re­
lapses.4–6 Our current protocol is to perform two-jaw
distraction in adolescents. These patients usually have
Pruzansky grade IIa or IIb deformities. A vertical inter­
nal distractor is used for the maxilla, and a multivector
external distractor is used for the mandible. These pa­
tients also have orthodontic treatment, and the occlu­
sion is maintained until skeletal maturity. We have seen
much more favorable outcomes in our patients treated
with this protocol.7
The coronoid process in Pruzansky grade IIb pa­
tients may be longer than the remnant of the condyle.
Extreme caution should be exercised in treating this
group of patients, because the coronoid process may
fuse with the skull base during distraction, resulting in
ankylosis.
Distraction osteogenesis is not the only way to cor­
rect skeletal problems in patients with HFM. Orthogna­
thic surgery can achieve good symmetry for grade I and
some grade IIa patients. The treatment course is much
shorter and simpler compared with distraction osteo­
genesis. The skeletal deformity in HFM is a combination
of facial asymmetry and class II malocclusion. The hemi­
maxilla and mandible on the affected side need both
downward and forward movement to achieve better fa­
cial symmetry. Overcorrection of the occlusal cant is of­
ten required to level the mouth angle. If overcorrection
is not built into the surgical treatment plan, it is often
unreliable. Soft tissue symmetry of the face should be
achieved intraoperatively to obtain a better long-term
surgical outcome. Bone grafting for gaps at the Le Fort I
osteotomy site is also highly recommended to prevent
postoperative relapse.
Neither distraction nor orthognathic surgery can
correct the bony deficiency in the transverse dimen­
sion. Le Fort I osteotomy cannot correct malar asym­
metry. The contour deficiency at the mandibular angle
and malar prominence will need ancillary procedures
at the second stage of treatment. All of these second­
ary contouring procedures have their own limitations.
Alloplastic implants such as Medpor (Stryker) are easy
to perform but introduce the risk of implant infection.
Autologous bone graft may resorb over time and is as­
sociated with donor site morbidity. It is also difficult to
create a definitive jaw line or mandibular angle with
autologous fat injections.
An irregular chin contour presents another chal­
lenge after skeletal correction of the jaw discrepancy.
It is difficult to restore chin symmetry and achieve a
smooth chin contour with osseous genioplasty. Our pre­
ferred technique is to contour the inferior border of the
chin to create a more symmetrical and smoother outline.
The use of a costochondral graft is our preferred
technique in treating Pruzansky grade III patients. Tim­
ing of surgery is still controversial. Early surgery may
provide a functional joint on the affected side; however,
the subsequent growth of the costochondral graft is of­
ten unpredictable. Our preference is to perform costo­
chondral grafting in adolescent or adult patients.
Ear reconstruction is performed with Nagata’s
technique8 using costochondral graft after the age of 12
years with a chest circumference greater than 60 cm.
The three-dimensional framework needs rigid fixation
with small-gauge wires to prevent warping or distortion
of the graft in the long run. Delicate soft tissue handling
and careful flap design are the key factors in preventing
flap necrosis and subsequent salvage procedures.
Even after skeletal correction with distraction os­
teogenesis, these patients almost always need soft tis­
sue augmentation on the affected side. The soft tissue
procedure may be performed as a camouflage procedure
before skeletal correction in preschool children to de­
crease the psychological impact of the facial deformity.
Although free flap transfer can provide a greater volume
augmentation, the color mismatch of the skin paddle
with the surrounding facial skin and the ­subsequent flap

755
Chapter 42 Orofacial and Craniofacial Deformities
sagging are often problematic. Our preferred method for
soft tissue augmentation is a serial fat injection. Fat in­
jection can provide a more even soft tissue augmenta­
tion compared with free flap transfer. Small fat parcels,
each around 1/60 mL in size, should be injected in dif­
ferent planes of the facial soft tissue to achieve the de­
sired outcome.
A facial reanimation procedure is needed if the HFM
patient has a significant facial nerve disorder. This pro­
cedure is somewhat complex and thus is not discussed
here.
Conclusion
The pathology of HFM involves both the facial soft
tissue and skeleton. The severity and presentation
of this deformity differ among patients. To achieve a
successful outcome, a multidisciplinary approach to
treat different pathologies in different time frames
with different protocols is usually needed. Choosing
the correct treatment protocol, careful preoperative
planning, and delicate soft tissue handling are keys to
avoiding unfavorable results and complications.
Avoiding and Managing Unfavorable
Results and Complications in Major
Craniofacial Clefts
Rare craniofacial clefts are a challenge to all craniofa­
cial surgeons, not just because of their rarity, but also
because of their complex pathology.
The nomenclature for craniofacial clefts can be con­
fusing. Some forms of craniofacial clefts may have the
same pathology but be classified under different names.
Tessier No. 0 to 14 clefts are often associated with or­
bital hypertelorism. Tessier No. 7 cleft, for example, de­
scribes HFM with macrostomia. Tessier No. 1 and No. 2
clefts, although similar to common cleft lips in Tessier’s
drawings, are different in their nasal deformities and
are quite rare. The paramedian clefts (Tessier No. 3 and
No. 4) are the more common craniofacial clefts com­
pared with the rest of the cleft deformities described
by Tessier.9
A facial cleft involves both the soft tissue and under­
lying skeleton with different degrees of severity. The soft
tissue cleft results in distortion of the anatomic land­
marks and exposure of some underlying structure. The
bony deficiency in the maxilla results in a bony cleft
in different regions. Because the Tessier classification is
based on the eye, the orbit is often involved, with orbital
dystopia and eyeball exposure from eyelid defects except
in true median facial clefts (No. 0–14, or No. 1–2) or lat­
eral facial clefts (No. 7). The cheek cleft in No. 3 also has
exstrophy of the nasal cavity.9
The treatment priorities in rare facial clefts are as
follows10:
•• Protection of the globes
•• Restoring the normal facial soft tissue anatomy
•• Repairing the bony cleft at a suitable age
Although the eyeball is exposed at birth, an emergency
operation is seldom needed to repair the eyelid soon
after birth. The soft tissue repair is usually performed
around 3 to 6 months of age. Conservative manage­
ment is recommended for globe protection before sur­
gical repair of the soft tissue.
A facial cleft often results in shortening of the facial
dimension along the cleft; soft tissue lengthening is the
key to restore the facial anatomy. Z-plasty is a funda­
mental plastic surgery technique for tissue lengthening.
The lengthening effect of a z-plasty varies in different
facial regions because of varying soft tissue quality.
For a facial cleft within the same facial unit, the re­
sult of a z-plasty is usually good. A z-plasty above the
white skin roll is required in repairing the median cleft
lip (Tessier No. 0) in addition to approximation of the
cleft. The z-plasty can accentuate the shape of the Cu­
pid’s bow and prevent postoperative scar contracture.
For Tessier No. 1 and No. 2 clefts, a marginal flap is de­
signed to bridge over the alar rim. The cleft margins are
approximated with multiple z-plasties for leveling of
the alar rim. This is also true in repairing Tessier No. 5
facial clefts. The shortened oculo-oral distance is best
lengthened by multiple z-plasties. The scars are usu­
ally inconspicuous because the tissue along the cleft is
within one facial unit.9
For facial clefts involving more than one facial unit,
such as in Tessier No. 3 and No. 4 clefts, multiple z-plas­
ties along the junction of different facial units will result
in unsightly scars with different skin color, quality, and
thickness. We had been using the tissue expansion tech­
nique for secondary revision of the zigzag scars since
before the early 1990s. However, the z-plasty scar can
still be too wide, which cannot be totally corrected with
tissue expansion.
After seeing these drawbacks, we changed our
approach using a technique called midface rotation
advancement.11 The lip markings are the same as in tra­
ditional cleft lip repair. The redundant lip tissue, es­
pecially in No. 4 facial clefts, is discarded to avoid an
unnatural look after lip repair. Local turnover flaps are
developed for the lining of the nasal cavity and eye­
lid. On the midface, the laterally based cheek flap is el­
evated subperiosteally with a relaxing incision on the
periosteum lateral to the infraorbital neurovascular
bundle. The dissection of the cheek flap is then car­
ried superiorly to include the orbicularis oculi muscle.

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
756
The ­periosteum on the inferior orbital rim is opened to
facilitate the medial advancement of the cheek flap. A
lateral canthotomy can be performed for further tissue
release. This allows the cheek flap to be advanced me­
dially to reach the proposed medial canthus on the up­
per wound margin, the lateral edge of the nasal flap on
the nose, and the philtral column on the lip. The rem­
nant of the medial canthal tendon can be easily identi­
fied during the dissection. The medially based nasal flap
and alar base are raised off the underlying cartilage and
frontal process of the maxilla to allow a downward rota­
tion of the shortened oculoalar tissue. The lengthening
effect is achieved by downward rotation of the medially
based nasal flap and medial advancement of the later­
ally based cheek flap, similar to a rotation-advancement
cheiloplasty in cleft lip repair. A medial canthopexy is
performed by transnasal wiring for a stronger support
of the lower eyelid. The canthopexy requires overcorrec­
tion, because relapse is quite common. The nasal flap is
rotated downward to match the alar base on the noncleft
side, also with overcorrection. The nasalis, orbicularis
oris, and facial expression muscles are repaired sepa­
rately. The skin edge is approximated to the suture lines
placed along the junction of the facial units.
We compared the results of z-plasties versus mid­
face rotation advancement using a 5-point visual ana­
log scale. Group II (midface rotation advancement, nine
patients) had a significantly better score compared with
group I (multiple z-plasties, five patients).
If the lengthening effect is inadequate with the mid­
face rotation-advancement technique, additional tissue
lengthening can be achieved during a revision surgery
with the same approach. The concept is similar to a lip
revision in cleft lip patients.
The timing of the bone graft surgery should be
­determined by the location of the cleft and the affected
tooth. The usual timing is similar to the alveolar bone
grafting in typical cleft patients, which is around 7 to
11 years of age.
Orbital dystopia caused by a facial cleft is ­managed
by orbital osteotomy as described in the previous
­section (HFM).
Conclusion
The technique used for repairing a rare facial cleft differs
based on the pathology. For facial clefts within one facial
unit, multiple z-plasties is the technique of choice. For
Tessier No. 3 and No. 4 facial clefts, a midface rotation-
advancement technique can provide a better outcome
compared with the multiple z-plasties technique.
Avoiding and Managing Unfavorable
Results and Complications in
Hypertelorism
Hypertelorism can occur in rare craniofacial clefts
without craniosynostosis; craniofacial dysostosis such
as Crouzon’s, Apert’s, or Pfeiffer’s syndrome; or pa­
tients with frontonasal encephalocele.
Not all hypertelorism patients require surgical treat­
ment. In East Asia, parents seldom request surgery if
the degree of hypertelorism is mild, with an intercan­
thal distance around 30 to 34 mm. We seldom perform
simpler operations such as medial wall mobilization or
two-wall osteotomies, even though these procedures are
effective in correcting mild hypertelorism.
For more severe hypertelorism, we perform two
types of operations: (1) box-shift osteotomies and (2)
facial bipartition. Both procedures require an intracra­
nial approach. The choice of the procedure is mainly
­determined by the maxillary arch and orbital axis. If
the patient has a V-shaped face with a narrow max­
illa, ­facial bipartition is the preferred technique, be­
cause it can correct both the orbits and the maxilla.
This technique is particularly useful in the presence
of a facial cleft.12
Timing of the surgery is an important factor.
­Although there is a consensus among cleft surgeons that
6 to 8 years of age is suitable for hypertelorism correc­
tion, a box-shift osteotomy at this age can still have an
adverse effect on facial growth. Facial bipartition per­
formed at this age also carries the risk of injuring the
permanent dentition.
Infection is the most common complication associ­
ated with hypertelorism correction. When the skull base
is opened between the intracranial space and the nasal
cavity, a dead space is created. Bony approximation dur­
ing orbital correction will still leave this space open. The
infection may result in the loss of frontal bone flap or
even meningitis. We routinely use a galea-frontalis flap
to obliterate the dead space or a pericranial flap plus
­fibrin glue in secondary repair.
Optic nerve injury is another potential risk, because
the two orbits are approximated in the front but left
unchanged at the orbital apices. Compression of the
orbital nerves can occur with this surgical movement.
With the help of computer-assisted surgical planning,
simulation, or navigation, this risk can be greatly re­
duced. The case illustrated in Fig. 42D.1 is an example
of computer-assisted surgical planning and navigation
in a patient with a rare facial cleft with hypertelorism.

757
Chapter 42 Orofacial and Craniofacial Deformities
a
b
h
i
c
d
e
f
Fig. 42D.1 (a,b) A 5-year-old girl with a rare craniofa­
cial cleft and hypertelorism. (c) Preoperative comput­
ed tomography (CT). (d,e) Computer-assisted surgical
planning. (f) Postoperative CT. (g) Computer-assisted
surgical navigation to prevent possible optic nerve
injury during surgery. (h,i) Soft tissue repair was per­
formed 1 month after the hypertelorism correction.
Her postoperative appearance 2 weeks after the soft
tissue surgery is shown.
g

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
758
Conclusion
Choice of the technique for correction of orbital hyper­
telorism is determined by the facial shape, maxillary
arch, and orbital axis. Complete sealing of the bony
dead space at the skull base is critical to prevent post­
operative infection. Computer-assisted surgical plan­
ning is helpful in achieving a better result and reducing
the complication rate.
References
1. Yu CC, Goh RCW, Lo LJ, Chen PK, Chen YR. Surgical
repair for macrostomia: significance of Z-plasty limb
directions. Ann Plast Surg 2010;64(6):751–754
2. Tan YC, Chen PK. Hemipalatal hypoplasia. J Craniofac
Surg 2009;20(4):1150–1153
3. Ko EW, Hung KF, Huang CS, Chen PK. Correction of
facial asymmetry with multiplanar mandible distrac­
tion: a one-year follow-up study. Cleft Palate Cranio­
fac J 2004;41(1):5–12
4. Nagy K, Kuijpers-Jagtman AM, Mommaerts MY. No
evidence for long-term effectiveness of early osteo­
distraction in hemifacial microsomia. Plast Reconstr
Surg 2009;124(6):2061–2071
5. Meazzini MC, Mazzoleni F, Bozzetti A, Brusati R.
Comparison of mandibular vertical growth in hemi­
facial microsomia patients treated with early dis­
traction or not treated: follow up till the comple­
tion of growth. J Craniomaxillofac Surg 2012;40(2):
105–111
6. Suh J, Choi TH, Baek SH, Kim JC, Kim S. Mandibu­
lar distraction in unilateral craniofacial microsomia:
­longitudinal results until the completion of growth.
Plast Reconstr Surg 2013;132(5):1244–1252
7. Lu TC, Yao C, Chen PK. Simultaneous maxilla-mandib­
ular distraction in preadolescent hemifacial microso­
mia patients. Presented at the 16th Congress of Inter­
nal Society of Craniofacial Surgery, Tokyo, Sep, 2015
8. accessed 2/2017
9. Tessier P. Anatomical classification facial, cranio-facial
and latero-facial clefts. J Maxillofac Surg 1976;4(2):
69–92
10. Bradley JP, Kawamoto HK. Craniofacial clefts. In:
­Neligan, ed. Plastic Surgery. Vol 3. 3rd ed. Elsevier-
Saunders; 2013
11. Chen PK, Chang FC, Chan FC, Chen YR, Noordhoff MS.
Repair of Tessier no. 3 and no. 4 craniofacial clefts
with facial unit and muscle repositioning by mid­
face rotation advancement without Z-plasties. Plast
­Reconstr Surg 2012;129(6):1337–1344
12. Arnaud E, Marchac D, Rocco FD, Renier D. Orbital
­hypertelorism. In: Neligan, ed. Plastic Surgery. Vol 3.
3rd ed. Elsevier-Saunders; 2013

759
Orthognathic Surgery and
Maxillofacial Distraction Surgery
Pravin K. Patel
CHAPTER
43
Orthognathic surgery involves the surgical manipulation
of the elements of the facial skeleton to restore the nor­
mal anatomic and functional relationship in patients with
anomalies of the dentofacial skeleton. Since its evolution
from the mid-20th century with its classic procedures to
the incorporation of distraction, osteogenesis has become
a powerful tool in the surgeon’s armamentarium that can
create dramatic changes in facial form and function that
few other procedures can achieve.
Achieving this desired goal involves a detailed archi­
tectural blueprint and construction details. Furthermore,
it requires a close working relationship between the or­
thodontist and the surgeon, each manipulating their own
component of the dentofacial unit in a complementary,
time-specified, integrated approach. Speech and language
pathologists and psychologists play an important role
in guiding the treatment planning. As an elective proce­
dure, orthognathic surgery places significant demands on
­patients and their families—with an overall length of treat­
ment that spans years—in the form of financial resources
and a multitude of appointments that require time off
from employment and educational activities beyond the
brief period of surgery and its immediate recovery. It is a
significant commitment from the patient, caregivers, and
professionals. Thus, practitioners must clearly define the
surgical and dental goals and then concisely translate those
goals to the patient and the family, including a discussion
regarding why those goals may not be reached. The probable
is what usually happens, but at this point the focus should
be on the improbable, the unintended, and the unexpected.
Thus understanding the unfavorable outcomes and surgi­
cal and orthodontic complications that can and do occur
with maxillofacial skeletal procedures is the subject of this
chapter.
There is a relevant distinction between the “unfavor­
able” and the “complication.” An unfavorable outcome can
occur without a specific complication, and not all compli­
cations lead to an unfavorable outcome. A perfectly well-
executed orthognathic procedure from blueprint to con­
struction without any complications can still lead to an
unfavorable or unsatisfactory outcome. A face with a perfect
occlusal and functional result may not be pleasing to either
its owner or to the architects who designed it. Moreover,
unlike many surgical procedures, the outcome depends not
only on the surgical procedure itself, but also on a multitude
of factors that exist long before the actual surgery, as well as
on the ability to control variables long after the surgical pro­
cedure has taken place. Whether the desired long-term out­
come is achieved in terms of occlusal function and aesthetics
of the dental and facial form depends on whether the goals
of each of the phases of treatment were achieved. Moreover,
the success of each phase depends on the preceding phase
of treatment. For example, inadequate incisor decompensa­
tion limits the amount of sagittal repositioning possible and
compromises the final facial aesthetics. If mobilization of the
maxilla at the time of surgery is inadequate and a less-than-
ideal occlusal relation obtained, the postsurgical orthodontic
phase is prolonged, and relapse toward the initial condition
is likely to occur. Even when facial skeletal and dental goals
are achieved, the functional goals may not be. Moreover, un­
intended consequences may occur in terms of velopharyn­
geal insufficiency, airway compromise, or oromotor function
while optimizing facial appearance.
Each orthognathic case has a stepwise sequence of di­
agnosis, dentosurgical treatment planning, presurgical
orthodontic preparation, surgery, postsurgical orthodontic
detailing, and retention. Thus, unlike many of the surgical
procedures, the scale of time is not measured in weeks to
months; rather, it is measured in years. The surgical compo­
nent must be taken within the context of the overall treat­
ment from diagnosis to retention. Within this lengthy time
frame of a complex choreography between orthodontist and

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
760
(Fig. 43.2). Each must understand the other’s objective and
abilities to achieve those goals within any limitations or
compromises that may occur when reaching common sur­
gical and occlusal goals. Moreover, anatomic dentosurgical
goals may negatively affect functional goals. An example
is a patient with a severe class III cleft in whom a maxil­
lary advancement procedure may put the patient at risk for
velopharyngeal incompetence (VPI) while improving the
anterior dental relationship, the aesthetic appearance, and
speech articulation. A mandibular setback procedure used
to minimize VPI may put the patient at risk for obstructive
sleep apnea and a less-than-ideal facial appearance. Thus
the goals of the speech and language pathologist and sleep
medicine specialist may come into play. In many cleft pa­
tients, the lateral incisor is not present, and incorporating
the need for either prosthodontic consideration or segmen­
tal closure with a canine-to-lateral substitution needs to be
addressed from the beginning. It is more than simply filling
a dental gap, because each approach will have a different
outcome in perioral aesthetics of the arch form and smile.
In a patient with a class II cleft in whom significant man­
dibular advancement would improve both aesthetic ap­
pearance and airway, the surgeon’s goal may be limited by
insufficient dental decompensation that could have been
achieved with mandibular first molar extractions and re­
traction of the anterior dentition. A bimaxillary advance­
ment procedure could surgically compensate for improving
the airway, but at the expense of the facial aesthetics.
surgeon, errors occur and accumulate. The list of errors that
can lead to unfavorable outcomes and complications is ex­
tensive and is well reviewed in the existing literature.1–5 A
meaningful way to discuss these is to consider what can go
wrong with each phase of treatment (see the Summary Box).
Avoiding Unfavorable
Results and Complications
in Orthognathic Surgery
and Maxillofacial Distraction
Surgery
Preoperative Planning
Errors in Communication
Unfavorable outcomes occur primarily because of poor
communication between the surgeon and the orthodontist
and the patient6,7 (Fig. 43.1). For the vast majority, patients
are referred to the surgeon by the orthodontist, whose goal
is primarily to achieve occlusion that cannot be achieved
without the aid of the surgeon. The surgeon assesses the pa­
tient from the perspective of optimizing the aesthetic goals
of the soft tissue draping the skeletal framework within the
context of achieving the orthodontist’s expected occlusion
Summary Box
Causes of Complications and Unfavorable ­Outcomes
Errors in Diagnosis and Treatment Planning
•• Unrealistic expectations
•• Lack of understanding of the complexity of the
procedure
•• Lack of teamwork between the orthodontist and the
surgeon
•• Failure of including speech, psychology, and sleep
medicine
•• Wrong diagnosis
•• Errors in diagnostic setup
Errors in Preoperative Orthodontic
Management
•• Inappropriate timing of intervention
•• Inadequate orthodontic setup
•• Insufficient decompensation
•• Inadequate transverse width coordination
•• Inadequate root divergence for segmentation
•• Inappropriate extraction versus nonextraction
Complications in Operative Phase
of Management
•• Errors in surgical planning
•• Intraoperative complications (see Box 43-1)
Errors in Postoperative Orthodontic
Management
•• Insufficient coordination of the dental arches
•• Inadequate postoperative posterior buccal-lingual
torque
•• Failure of retention
Unsatisfactory Outcome
•• Velopharyngeal insufficiency
•• Issues related to hardware
•• Chronic maxillary sinusitis
•• Osteotomy nonunion
•• Late malocclusion and relapse
•• Unsatisfactory facial appearance
•• Unacceptable nasal appearance and breathing
•• Unsatisfactory smile and perioral aesthetics
•• Issues related to temporomandibular joint

761
Chapter 43 Orthognathic Surgery and Maxillofacial Distraction Surgery
were unhappy with what I considered an improved ap­
pearance. In two decades of experience, I have found the
involvement of a psychologist from the initial discussion for
orthognathic surgery to be critical. This is especially true in
adolescence, when many patients are defining their identity
and facing stress at home and social and academic stress at
school. Our psychologists have uncovered suicidal ideations,
drug and alcohol abuse, sexual orientation, and pregnancy
unknown to the orthodontist, the surgeon, and the par­
ents. Such sensitive issues within the context of dentofacial
From the patient’s perspective, the options available are
bewildering, and the potential to negatively affect the facial
appearance and function is daunting. Even when the profes­
sionals feel that the ideal occlusion and facial appearance are
achieved, the patient may be unhappy with the appearance
despite the often dramatic improvement in appearance. In­
corporating the change within the patient’s psyche can be
difficult. I have had adolescents who were not sufficiently
prepared for the physical changes to return to school and
older patients in the third decade and beyond who equally
Fig. 43.1 This patient was referred “ready for jaw surgery.”
However, when the patient learned what was involved with
a mandibular body advancement (bilateral sagittal split
osteotomy), she elected not to proceed with surgery be­
cause of concerns regarding the possibility for the need for
a blood transfusion, even if remote. As a compromise, an
osseous genioplasty was performed with a compromised
outcome. The decompensation was reversed. This case il­
lustrates communication failure; the treatment goals were
not understood by the patient and family.
Fig. 43.2 This patient was referred “ready for jaw surgery.” The patient expressed concerns to the
surgeon at the initial consultation regarding the perioral aesthetics of protuberant lips. The maxil­
lary incisors were excessively flared by the routine sequence of orthodontic arch wires. Maxillary
advancement surgery would worsen the appearance, whereas mandibular setback would not alter
the upper lip. However, there was no communication by the surgeon at the initial planning stage.
The option of first bicuspid extraction and anterior segmental repositioning would have achieved
the occlusal goal and the patient’s aesthetic goals. The outcome was compromised by a lack of
communication and understanding what each can achieve. A mandibular setback was offered to
achieve occlusion but at the risk of an unfavorable ramal osteotomy and poor fixation, because
the third molars would have been ideally extracted 6 months before the bilateral sagittal split
osteotomy procedure.

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762
on the chin projection without considering occlusion and
retroglossal airway space.
The origin of the occlusal discrepancy can unfavorably
influence the outcome of surgery if the underlying cause is
not addressed or taken into account in the treatment plan­
ning. Concerns of relapse, vascularity, and secondary impact
on velopharyngeal dysfunction are greater for patients with
facial clefts requiring maxillary advancement than for pa­
tients with developmental dentofacial skeletal deformities.
Patients with developmental dentofacial deformities who
present for correction of an anterior open bite deformity
are at an increased risk of relapse if the underlying cause of
tongue thrust is not managed. Patients with condylar hy­
perplasia who present with prognathic mandibular asym­
metry and occlusal class III cross-bite may exhibit contin­
ued asymmetric growth with a resulting recurrence of the
condition if the underlying pathology remains active. Pa­
tients who undergo mandibular advancement surgery may
progressively relapse with time as a result of underlying
temporomandibular joint (TMJ) pathology, such as juvenile
rheumatoid arthritis or idiopathic progressive condylar re­
sorption unrecognized before surgery. Optimizing the out­
come demands an understanding of the underlying causes
of the dentofacial skeletal deformity whether of congenital,
developmental, or traumatic origin, each of which influence
the outcome differently despite the similarity in surgical
procedure and desired goals.
Records, Diagnosis, and Simulation
Accurate records are needed for diagnosis and to detail the
orthodontic and surgical treatment planning at two critical
time points: (1) at the beginning before there is any inter­
vention, a diagnostic setup, and (2) at the time of surgical
planning weeks before the procedure, a surgical setup. Too
often the surgeon is brought into the picture in the middle
of orthodontic treatment, and the only involvement is at
the time of the surgical setup (the model surgery), result­
ing in compromises that could have been addressed at the
time of the diagnostic setup. At both junctures, the setup
must be accurate to reflect the patient’s initial dentoskel­
etal anatomy and allow the team to predictably plan the
optimal end point.
Historically, the complexity of the three-dimensional fa­
cial architecture was reduced to a series of two-dimensional
records captioning the soft tissue envelope using photogra­
phy and the underlying skeletal framework with plain ra­
diographs in multiple orthogonal planes. The dental models
were the only component that was recorded in three di­
mensions, and they required using a face-bow for position­
ing in the three-dimensional space. However, developing an
accurate three-dimensional composite model of a patient’s
condition by registering each of the two-dimensional orthog­
onal radiographic planes (lateral cephalometric and fron­
tal cephalometric) and the two-dimensional ­photographs
(frontal and profile views) with the mounted three-dimen­
sional dental models had its own difficulties. Registration
surgery, if missed, can lead to disastrous outcomes. Under­
standing the patient’s motivation for surgery is an important
factor in a successful outcome.8,9
Taking the time to communicate the complexity of the
orthodontic and surgical treatment and their impact is criti­
cal to optimizing the outcome with the end result of a sat­
isfied patient comfortable with his or her new appearance
and functional bite. The patient must have an understanding
of limitations and compromises that may occur. Orthogna­
thic surgery, whether needed for cleft or craniofacial condi­
tions or for the more common developmental deformities,
should be approached as a multidisciplinary team perspec­
tive and not just involve the orthodontist and surgeon inde­
pendently driving the entire treatment plan. In addition, we
have found it enormously beneficial to establish a dedicated
“jaw” clinic, in which the vast majority of the patients are at
various stages of jaw treatment. We have found that patients
and their families become more comfortable with surgery
after talking to each other in such settings. The supportive
group that naturally develops is a powerful tool in commu­
nication that leads to a successful outcome.
Errors in Diagnosis
The wrong diagnosis will lead to the wrong surgery and
to an unsatisfactory or unfavorable outcome. A classic ex­
ample is patients who feel that their mandible is “too prog­
nathic.” They come to the surgeon wanting to have their
mandible and chin brought back. In a true profile view this
may at first seem acceptable. But many of these patients
will have subtle signs of midfacial deficiency that would be
missed with this treatment plan and would be better served
by a maxillary advancement procedure as the primary pro­
cedure to optimize their aesthetic appearance. The surgeon
and the orthodontist should note the lack of dental display,
the perialar deficiency, the acute nasolabial angle, the de­
ficient columellar projection, and the convexity of the face
that may extend to the zygomatic region. These can only
be assessed when seeing the patient clinically in three di­
mensions and are often missed in the standard orthodontic
two-dimensional photographs that often accompany the
patient at the time of referral. Of all the images, the three-
quarter oblique view often best reveals the midfacial defi­
ciency that is commonly missed in the true lateral profile
view. Another relevant example is when patients come
to the surgeon initially for a recessed chin wanting a chin
augmentation. Without assessing the dental occlusion, the
aesthetic outcome from symphyseal surgery, whether os­
seous or alloplastic, may be unsatisfactory. If the patient
has a significant class II dental relation indicating a man­
dibular body deficiency, then a “camouflage” genioplasty
may result in an unnaturally deep labial mental sulcus if
advanced to the desired projection. Such patients would
benefit from an orthodontic assessment and consideration
for a mandibular body advancement and an osseous genio­
plasty. There also may be symptoms of obstructive sleep
apnea (OSA) that can be missed if the surgeon focuses only

763
Chapter 43 Orthognathic Surgery and Maxillofacial Distraction Surgery
expectations on what they see in the media of models and
actors. The surgeon must balance the patient’s expecta­
tions with what can be realistically achieved by defining
the new skeletal framework and the occlusion simultane­
ously. It is important to understand that no patient wishes
to look “average” and that the use of normative cephalo­
metric data should only be used as a guide.20,21 As an il­
lustration, although a sella–nasion–A point (SNA) angle of
82 degrees is considered an average, it may be unattractive
compared with an SNA angle of 84 degrees or more if this
would result in an attractive facial appearance with fullness
and better support of the midfacial soft tissue envelope. An
SNA angle greater than 82 degrees may also have a signifi­
cant favorable effect on the patient’s smile. Buccal corri­
dors that are empty before the advancement can be better
filled with the dentition from canine to canine for a more
attractive, fuller smile. A lesser advancement would result
in a less full smile. Because the dentition can be adjusted
orthodontically and the dental arch as a unit surgically, the
final desired maxillary and mandibular incisor inclinations
and thus the orthodontist and the surgeon determine the
smile aesthetics to be achieved by the treatment. Compar­
ing for the patient the difference between the patient and
his or her siblings and parents serves as a guide toward
what goals can realistically be achieved.
Once the final skeletal position is determined relative
to the skull base, the surgeon and the orthodontist need to
work backward. The orthodontist needs to determine what
to do with the teeth to allow the surgeon to achieve the fi­
nal skeletal position in the construction of the desired face.
A classic example is in cleft patients who present with a
class III dentofacial skeletal relationship, in which the skel­
etal deficiency is more than the dental discrepancy. The sur­
geon would like to significantly advance the midface, but the
skeletal advancement is limited by the minimal discrepancy
at the occlusal level (insufficient negative overjet). The out­
come would be less than ideal without orthodontically in­
creasing the negative overjet. If the orthodontic preparation
was not ideal, alloplastic implants may be needed as a com­
promise. Similarly, for class II patients who would benefit
from significant mandibular body advancement, the occlu­
sal relationship may be insufficient to allow for the desired
advancement, and the projection of the facial plane must be
made up by an osseous genioplasty. There must be adequate
dental decompensation to make the occlusal relationship
worse to achieve the desired skeletal movement to optimize
a favorable outcome.
Although the treatment planning options are well es­
tablished to optimize outcome in the developmental dento­
facial skeletal cases, for congenital conditions ranging from
minor facial clefts to syndromic conditions as Goldenhar and
Treacher Collins, the path to the final goals is not as clear.
Classic approaches are challenged by severe tissue deficiency
at multiple tissue planes and severely abnormal architec­
ture, so simple rearrangement of the existing skeletal ele­
ments cannot be achieved in a stable manner without bone
grafts. Unlike developmental cases, for which surgical inter­
errors would build with each step, and any simulation of
the treatment planning (sectioning and repositioning the
plaster-mounted models) would lead to uncertainty about
the accuracy of transferring the surgical plan to the operat­
ing room.10,11 Beyond the laboratory technical errors is the
fundamental difficulty in the consistency of the head po­
sition and in the difficulty in accurate face-bow mounting
in the cases of asymmetry that would involve the ear, the
skull base, the orbits, and dual-plane maxillary–mandibular
asymmetry. Traditional model surgery worked well in the
routine developmental dentofacial skeletal cases, in which
the vast majority of patients’ problems could be captured
within the two-dimensional sagittal plane. However, when
the problem extends to the third dimension, where yaw,
roll, and pitch of the maxilla and the mandible play a role, it
becomes less reliable, and successful orthognathic surgery
becomes more of an art than a series of well-defined steps
to a desired outcome. In cases of asymmetry, the roll and
the pitch are easier to correct, but the yaw is missed be­
cause of the inability to accurately visualize the problem in
the transverse plane.
Today, surgical-orthodontic management has become
increasingly reliable with the ability to visualize structures
using three-dimensional radiologic cone beam computed
tomography (CBCT) and software that allows the surgeon
to simulate surgery with three-dimensional movement of
the skeletal components.12,13 The next generation of max­
illofacial surgeons will be fully immersed in three-dimen­
sional technology, allowing a virtual orthodontic and surgi­
cal simulation that is specific to the patient’s anatomy.14–16
Although the technical execution of the planning and model
surgery would be expected to improve with elimination of
registration errors, it is important to remember Obwege­
ser’s Principle Number 35,17 “The computer can show you
what you can achieve with your planning but cannot plan for
you.” Knowing where to position the maxillary–mandibular
skeletal bases and the dental arches relative to the desired
soft tissue envelope is dependent on the surgeon’s and or­
thodontist’s eye, experience, and intuition, which cannot be
replaced by a computer.17
Treatment Planning
Accurate clinical and radiographic diagnostic assessment
of the facial appearance and the underlying dentofacial
skeletal structure will point to the structural regions that
are malpositioned and malsized relative to the skull base
and to each other. The problem is not in determining what
is wrong, but determining where the skeletal elements
and consequently the dental component should be posi­
tioned.18,19 The surgeon and orthodontist will each have
their own notion of the normative facial structure, and
the patient will likely have a differing internally biased ex­
pectation of the desired facial appearance. Orthodontists
often rely on normative cephalometric numbers, which is
understandable because cephalometric analysis is histori­
cally rooted in the profession. Patients typically base their

SECTION VI PEDIATRIC AND CRANIOFACIAL SURGERY
764
across the cleft site, and improve gingival-mucosal vascu­
larity.
In many cleft patients, the lateral incisor is congeni­
tally missing, has been extracted because of inadequate
bone support, or is lost because of dental caries. A decision
must be made with the family, the orthodontist, and the
restorative prosthodontist whether to maintain the dental
space for an implant-retained dental prosthesis or a bridge
versus segmental maxillary osteotomy and closure of the
dental space by moving the canine into the lateral incisor
position. If a canine substitution is planned by advancing
the lesser alveolar segment, then the cleft site may not need
to be grafted before segmentation unless bone is needed to
support the central incisor and canine root.
In many cleft patients, there is a transverse width dis­
crepancy even after phase I orthodontics that is significant
in adolescents at the time of consideration for phase II final
orthodontic-surgical management. Although a segmental
Le Fort I osteotomy can correct a residual transverse width
discrepancy, my experience is that immediate surgical ex­
pansion at the time of surgery can be accomplished but dif­
ficult to maintain despite orthodontic retention because of
the relapsing forces of an inelastic scarred palatal mucosa.
Our preference is to correct the transverse width with slow
expansion using a transpalatal expander and when needed
unilateral or bilateral maxillary osteotomy (surgically as­
sisted palatal expansion) before formally correcting the sag­
ittal and vertical skeletal discrepancies.
Thus my goals for cleft patients include the following:
1. Convert a multisegmented cleft alveolus to a noncleft
single dental maxillary alveolar arch.
2. Address the missing lateral incisor early in the planning
in terms of substitution versus replacement.
3. Correct the transverse width discrepancy before sagit­
tal and vertical advancement (with surgically assisted
palatal expansion if needed).
4. Optimize the facial skeletal aesthetics by maximal den­
tal decompensation with a treatment plan established
in early adolescence well before skeletal maturity.
Preoperative Orthodontic Stage
There are pitfalls in the preparation of the dentition for or­
thognathic surgery that must be well understood by the or­
thodontist and the surgeon.6,22,23 Less-than-ideal orthodon­
tic preparation will lead to a less-than-ideal surgical and
occlusal outcome (see Fig. 43.2). In patients with skeletal
discrepancies requiring orthognathic surgery, dental com­
pensations occur as a natural biologic response to optimize
occlusion in the face of maxillary–mandibular discrepancy.
These dental compensations occur in all three planes: an­
terior-posterior, transverse, and vertical. A surgeon whose
goal is to optimize the aesthetic skeletal outcome and an
orthodontist who seeks to achieve maximal coordination
(intercuspal relationship) of the dental arches must agree
on the extent to which the teeth must be decompensated
before skeletal surgery. Failure to communicate at the outset
vention primarily takes place at skeletal maturity, the need
to intervene in the actively developing immature skeleton
is motivated by functional concerns and the need to limit
compensatory or progressive distortion of the anatomy. Dis­
traction osteogenesis allows the surgeon to use the biologic
machinery to lengthen bone and expand the hypoplastic
surrounding soft tissue matrix: muscle, nerve, and integu­
ment. It is in such cases that the surgeon enlists the aid of
the orthodontist to support the functional and reconstruc­
tive goals with the understanding that the occlusal outcome
may initially be less than ideal with distraction alone. Solv­
ing the three-dimensional nature of the problem is hindered
by the limited ability to control the vector of the distraction
process because of the simplicity of the mechanical devices.
It is generally unidirectional and at best bidirectional. Thus,
in the end, classic orthognathic procedures are likely needed
to fully control the three-dimensional nature to optimize
the outcome. The surgeon must therefore communicate to
the patient and the family that a distraction may need to be
multistaged to address the different vectors through differ­
ent periods of growth and development, and that classic or­
thognathic surgery is commonly needed at skeletal maturity
to allow the surgeon and the orthodontist full three-dimen­
sional control over the reconstruction. Expectations must be
managed. Skeletal surgery is “carpentry,” and that carpentry
does not restore normal growth and development in these
children. It puts the bones where they should be, but in a
growing child the arrangement is not likely to remain ideal,
either because of relapse or lack of growth. Relapse can be
limited by distraction, but may not restore growth because
of the underlying pathology.
Optimizing the outcome of orthognathic surgery for cleft
patients begins long before skeletal maturity (Fig. 43.3). In
my practice, it begins in early adolescence at age 12 to 14
years, when it is important that both the surgeon and the
orthodontist assess the cleft patient and begin the discus­
sion with the family of the possible need for skeletal cor­
rection and occlusion depending on mandibular growth
and on the maxillary deficiency that is common at this age.
Early discussion with the family, monitoring growth, and a
close working relationship with the orthodontist will allevi­
ate some of the difficulties that may be encountered in late
adolescence if treatment planning is delayed. The surgeon
and the orthodontist must address several considerations
in early adolescence:
1. Status of the alveolar bone graft
2. Need for cleft orthognathic surgery
3. Management of the typically missing lateral incisor
Ideally patients have undergone phase I orthodontics that
would involve transpalatal expansion, initial alignment of
the dentition, and iliac bone grafting of the primary al­
veolar cleft in early mixed dentition. If this has not been
addressed, it should be done before initiating phase II or­
thodontics when the patient is in permanent dentition.
This is needed to support the dentition adjacent to the cleft
margin, allow canine eruption and orthodontic movement