Chẩn Đoán Và Điều Trị Phẫu Thuật Tái Tạo Hiện Đại (CURRENT Reconstructive Surgery)

c h a p t e r 13
Cleft Palate
Peter J. Taub, MD, FACS, FAAP / Joseph E. Losee, MD, FACS, FAAP
INTRODUCTION
A palatal cleft represents one of the more commonly rec-
ognized congenital anomalies ( Figure 13-1 ). Anatomically,
the palate separates the oral and nasal cavities from the lip
to the uvula. It is divided at the incisive foramen into the
primary palate anteriorly and the secondary palate poste-
riorly. More commonly, however, the palate is described as
being composed of hard and soft portions, from the alveo-
lar ridge to the uvula. The soft portion is also referred to
as the velum.
The teeth develop within the maxilla and erupt into the
softer inferior alveolar bone. The central and lateral inci-
sors develop within the midline premaxillary portion of the
palate, separate from the canines, premolars, and molars.
The premaxilla eventually fuses with the two lateral ele-
ments, each of which houses the remaining complement of
teeth. Typical clefts occur in the region of the lateral incisor
and canine, either of which may or may not be present. 1
Among ethnic groups, the incidence of clefting is high-
est in Native Americans, followed by Caucasians, Asians,
and African Americans. Clefts of the lip, with or without a
palatal cleft, are now thought of as a separate entity from
isolated clefts of the palate. The two differ signifi cantly
in terms of their epidemiologic profi les. The incidence of
clefts of the lip with or without a cleft palate is approxi-
mately 1 in 700 live births, while that of isolated clefts of
the palate is closer to 1 in 2000 live births. The isolated
variety is also more commonly seen in females, while clefts
of the lip with or without a cleft palate are more com-
monly seen in males.
PATIENT SELECTION
At the time of consultation, a thorough history and physi-
cal examination should note any family history of relatives
with clefting or congenital anomalies and any prenatal
problems that might be related to development of the
cleft. The sequellae of an open palate include diffi culty
with feeding and maintaining adequate nutrition, collec-
tion of fl uid in the middle ear possibly leading to hearing
loss, abnormal speech production and language develop-
ment, and distortion of facial growth.
Palatal clefts are generally described in terms of their
uni- or bilaterality, their completeness, and the specifi c
structures that are affected. The lip may or may not be
involved and the alveolar ridge may or may not be part of
the cleft. A rare combination may include a cleft of the lip,
with an intervening intact alveolus, and a cleft palate. The
simplest form of a cleft palate is a bifi d uvula, which occurs
in roughly 2% of the population and may go undetected.
The bifi d uvula, however, may be a clue to the presence of
an underlying submucous cleft palate. Here, the oral and
nasal mucosal surfaces are intact, but the levator veli pala-
tini muscle fi bers are divergent. This may present in older
children without a prior diagnosis of an overt cleft palate.
Patients are referred with speech concerns despite a period
of therapy that has failed to provide adequate results. The
specifi c fi ndings that might accompany a patient’s hyper-
nasal speech include a bifi d uvula, a notch of the poste-
rior nasal spine, and a “zona pellucida” or clear area in the
middle of the soft palate.
A cleft palate may be seen in infants as part of a syn-
drome encompassing other craniofacial anomalies, such
as Apert syndrome, Stickler syndrome, Treacher Collins
syndrome, Van Der Woude syndrome, and velocardiofa-
cial syndrome. In patients with Pierre Robin sequence
( Figure 13-2 ), the cleft is usually in the secondary (or
soft) palate. Here, it arises as a result of the associated
micrognathia and inability of the tongue to remain out
of the way of the descending palatal shelves which nor-
mally fuse in the midline before birth. In such patients,
prone positioning, tongue-lip adhesion, tracheostomy, or

134 • CHAPTER 13
early distraction of the mandible may be required before
closure of the palate in order to stabilize the infant’s
airway.
Adults who present with an unrepaired cleft of the
hard and/or soft palate are likely to have greater speech
concerns than are younger patients. Adults often develop
signifi cant errors of articulation as a means of compensa-
tion for the cleft. Depending on their access to health
care, some patients may or may not have had years of
speech therapy in an attempt to overcome the physi-
ologic problem of palatal incompetence. While closure
is important to improve resonance and oral hygiene, it is
usually diffi cult to achieve near-normal speech quality in
older patients.
FIGURE 13-1. Intraoperative photograph of an infant with
a near complete cleft of the hard and soft palate.
FIGURE 13-3. Prenatal ultrasound demonstrating a cleft
of the palate.
FIGURE 13-2. Photograph of an infant with Pierre Robin
sequence demonstrating the micrognathia which contributes
to the formation of a palatal cleft.
PATIENT PREPARATION
In developed countries, the diagnosis of a cleft palate may
be made as early as 12 weeks into pregnancy by a skilled
ultrasonographer ( Figure 13-3 ). In such cases, it is helpful
for the parents to visit with a multidisciplinary team of
clinicians to discuss the nature of the problem and man-
agement strategies in advance of the birth. More often,
however, a cleft palate is diagnosed at birth as a defect “in
the roof of the mouth.”
Early problems related to a cleft palate include diffi culty
in maintaining nutrition, especially with breastfeeding, and
regurgitation. Most infants do better with head-up posi-
tioning and the use of specially designed nipples that facili-
tate intake of milk or formula by diminishing resistance to
fl ow or creating force to push fl uid from the bottle in the
presence of a weak suck.
The timing of cleft lip closure is related more to the
social implications than to the functional problems. Infants
with an open lip and an intact palate usually feed with
minimal diffi culty. Palatal clefts, on the other hand, require
closure to improve oral and nasal hygiene and allow for the
development of intelligible speech with normal resonance.
As such, cleft lip repair is generally performed at about
3 months of age, a time chosen due to the relative safety of
general anesthesia in an infant.
Prior to formal lip repair, consideration for presurgi-
cal, nasoalveolar molding of the palatal arches should be
entertained. In some children, especially those with either
a complete unilateral or bilateral cleft of the lip and pal-
ate, distortion of the palatal arch is present. This may
make lip repair problematic due to the tension required
to approximate the cleft margins. Nasoalveolar molding,
using either passive or active appliances, may dramati-
cally realign the palatal components so that the lip may
be repaired under minimal tension, the nasal contour may

CLEFT PALATE • 135
be improved, and possibly the alveolar mucosa closed at
the time of lip repair to avert bone grafting in the future
(gingivoperiosteoplasty).
Nasoalveolar molding requires obtaining an impression
of the palate as soon as possible—often within the fi rst few
days of life—from which an appliance may be fabricated
( Figure 13-4 ). Frequent follow-up visits are necessary over
the ensuing weeks to ensure correct and adequate move-
ment of the arches in anticipation of cleft lip, and eventu-
ally palate repair. Modifi cations include attachment of a
nasal bulb to facilitate recontouring of the alar rim.
Prior to the advent of nasoalveolar molding, a wide
cleft lip was closed in stages. An adhesion procedure was
performed fi rst using soft tissues from the margins of the
cleft that were not to be used for the later formal repair.
After allowing the tension from the closure to mold the
palatal arches, the lip was then repaired at a second set-
ting using standard techniques described elsewhere. The
adhesion facilitates not only lip repair but also the later
palatal closure.
The timing of palatal repair is controversial and var-
ies from center to center. It is widely accepted that repair
before 18 months of age minimizes problems related to
speech and language development while not causing
drastic effects on midfacial growth. 2 Early palate closure
creates normal anatomy for the development of proper
speech development but may interfere with midfacial
growth. Several studies, looking at cephalometric data
in patients who underwent early palatal repair, however,
failed to demonstrate a difference in midfacial growth. 3 , 4
Later repair, although ideal to minimize scar formation
during growth, leads to errors of articulation that become
more diffi cult to reverse as the child gets older.
Some surgeons have advocated repair of the palate in
stages. The soft palate, which is more intimately involved
FIGURE 13-4. Appliance fabricated from a palatal
impression for use in presurgical nasoalveolar molding.
FIGURE 13-5. Nasoendoscopic image of the velum
and posterior wall of the pharynx demonstrating incomplete
closure with phonation.
with speech production, is repaired as early as 3 to 4 months
of age, often at the time of lip repair. This is followed by
later repair of the hard palate, delayed either months or
years to minimize the detrimental affects on facial growth. 5
A palatal obturator may be used in the interim to seal off
the oral cavity. Long-term evaluation, however, has failed
to support any benefi t of this approach on normal speech
development. 6 , 7
Many patients with a submucous cleft palate do not
require surgical intervention and it is assumed that a large
percentage go undiagnosed. Studies of patients with a
known submucous cleft palate have demonstrated that
many require no treatment at all. 8 Studies for a suspected
diagnosis of a submucous cleft palate include nasophar-
yngoscopy and/or videofl uoroscopy. The former involves
passage of a small-caliber endoscope through the nose into
the nasopharynx while the patient is awake. For this reason,
younger children may not tolerate the procedure as well as
older ones and the study may need to be deferred until
the child is more cooperative. The camera is positioned
above the soft palate and is able to visualize closure of
the velum during phonation ( Figure 13-5 ). In the patient
with a suspected cleft palate, ridging of the lateral palatal
elements may be indicative of incomplete muscle union in
the midline. The soft palate may then fail to contact the
posterior pharyngeal wall at the level of Passavant ridge
opposite the second cervical vertebra during phonation.
Videofl uoroscopy provides similar information but does so
by imaging the nasopharynx with radiography. It provides
detailed information about the movement of the velum
with speech. However, a fl uoroscopic image provides only
a single, cross-sectional view of the palate, usually in the
sagittal plane. It is not possible to record multiple planes
simultaneously and therefore cannot ascertain whether
adequate velopharyngeal closure is possible.

136 • CHAPTER 13
TECHNIQUE
Palate repair is performed under general anesthesia with
the patient in a supine position. The head and neck may
be slightly extended and a shoulder roll placed across the
upper back to improve exposure. Following intubation,
a mouth prop is placed to adequately visualize the pal-
ate. The tongue blade has a groove for the endotracheal
tube and the proper size should be chosen so as to hold
the tongue out of the way in the midline yet not press
on the posterior pharynx. The attached cheek retractors
should sit just anterior to the mandibular rami. A moist-
ened throat pack is placed in the pharynx to minimize
blood loss into the stomach. A dilute epinephrine and
anesthetic solution is injected into the lateral palatal
shelves for hemostasis and the face and mouth prop are
prepped in the usual sterile fashion. Care is taken to avoid
ocular injury with lubricant and tape. Antibiotics may be
given preoperatively and continued postoperatively at the
surgeon’s discretion.
Reconstruction of the hard palate begins with eleva-
tion of the lateral mucoperiosteum using a scalpel and
periosteal elevator. The fl aps are freed from the underlying
palatal bone and advanced towards the midline for closure.
Adequate mobilization requires careful dissection poste-
riorly around the vascular pedicle. The von Langenbeck
repair leaves the anteriormost portions of the hard pal-
ate shelves attached to the alveolar ridge creating two
bipedicle fl aps that are then approximated in the midline
( Figure 13-6 ). 9 For the more common complete cleft, the
Veau-Wardill-Kilner repair creates two axial-pattern pen-
insular fl aps based on the greater palatine artery and vein.
These fl aps are similarly approximated in the midline.
They may also be fi xed back to the premaxilla or hard pal-
ate anteriorly ( Figure 13-7 ). In both instances, the resul-
tant lateral defects may be packed with a gelatin material
or left open to heal spontaneously in several weeks.
FIGURE 13-7. Completed intraoperative view of Veau-
Wardill-Kilner repair for closure of the palate.
FIGURE 13-6. Completed intraoperative view of von
Langenbeck repair for closure of the palate.
The two basic options to address closure of the cleft
of the soft palate include a straight-line repair or one that
employs double-opposing z-plasty fl aps. The straight-line
repair involves dissection of the soft palate into its compo-
nent parts: oral mucosa, palatal muscle, and nasal mucosa.
The muscular components are invariably misdirected,
inserting along the posterior edge of the hard palate. They
are detached and redirected to the midline.
Repair begins with closure of the thin nasal mucosa
anteriorly. This may not always be possible due to the size
of the defect and the limited potential for mobilization of
the lateral nasal mucosal edges. Flaps of mucosa from the
midline vomer bone may be recruited to separately close
each posterior nasal cavity. The use of vomerine mucosa
during infancy has been questioned due to its possible
adverse effects on facial growth. 10 Posterior to the vomer,
the lateral mucosa does not always reach the midline
without tension. In the past, one solution was to divide
the nasal mucosa superior to the hard palate via the nose
and allow it to advance posteriorly to close this potential
defect. Recently, acellular dermis has been used to recon-
struct this area; however, further investigation needs to
clarify whether the addition of nonautogenous tissue is
detrimental to palate repair.
The posterior soft palate normally contains the mus-
culature that acts to elevate and pull the palate posteri-
orly, thus sealing off the nasal cavity from the pharynx.
Six muscles have some attachment to the palate and all
serve slightly different functions. These include the tensor
veli palatine, the levator veli palatini, the palatoglossus, the
palatopharyngeus, the superior constrictor, and the uvu-
lus. In most cases of cleft palate, the tensor muscles, which
normally originate laterally and insert in the midline, aber-
rantly insert along the posterior edge of the hard palate. 11
The abnormal muscle position also prevents adequate reg-
ulation of air and fl uid fl ow through the distal Eustachian

CLEFT PALATE • 137
tube predisposing to fl uid buildup in the middle ear.
Re-approximation of the muscle in the midline (intravelar
veloplasty) following closure of the nasal mucosa serves to
restore the normal anatomy of the palatal musculature.
Finally, the oral mucosa is closed in the midline. Con-
trolled medial pressure on the soft tissues by a blunt
instrument laterally within the Space of Ernst may assist
in further mobilizing the soft tissues, minimizing tension
across the repair, and thus diminishing the incidence of fi s-
tula formation. The greatest area of tension is often at the
junction between the hard and soft palates. It is here that
fi stulae may develop and need to be addressed separately.
Fracture of the hamular process of the maxilla—the
small bony protruberance around which the tensor veli
palatini muscle runs—has been proposed as an adjunctive
technique. It was suggested to improve medial displace-
ment of the lateral tissues and minimize midline tension
along the repair. Fracture of the hamulus appears to have
neither obvious benefi t nor detrimental effect on clo-
sure and its continued use is questionable in cleft palate
surgery. 12
Furlow devised the double-opposing z-plasty as an alter-
native technique for closure of the soft palate. 13 The objec-
tive is to realign the muscles while lengthening the palate,
which may be inherently short and further shortened with
postoperative scarring. Opposing z-plasty fl aps are ele-
vated on either side of the defect, one anteriorly based and
the other posteriorly based. On the posteriorly based side
(usually the patient’s left for a right-handed surgeon), the
fl ap contains oral mucosa and muscle separate from nasal
mucosa. The nasal mucosa is cut as an opposing anteriorly
based fl ap. On the contralateral side of the defect, an ante-
riorly based fl ap of oral mucosa is elevated off the muscle
and nasal mucosa. The muscle and nasal mucosa are kept
together and cut as an opposing posteriorly based fl ap. The
fl aps are rotated and the nasal mucosa is re-approximated
followed by the oral mucosa ( Figure 13-8 ). The muscles
naturally realign in the midline and the length of the palate
is increased.
The most important reported benefi t of the double-
opposing z-plasty technique is the improved speech
results. 14 The dynamic lengthening of the palate is achieved
by using the principles of the z-plasty in combination with
the rearrangement of the muscle fi bers. The disadvantages
include the need for adequate mobilization of the fl aps,
since length is usually gained at the expense of width, and
the more extensive suture lines which may be sites of fi stu-
alization. The Furlow palatoplasty has also been advocated
as a conversion procedure in those patients who have
previously undergone straight-line palate repair with an
intravelar veloplasty and less than optimal speech results.
It is felt that incomplete release and transposition of the
muscles fail to produce optimal speech results. At the
time of repeat palatoplasty some patients are also noted
to have scarring of the muscles back down to the posterior
edge of the hard palate, which compromises postoperative
function. 15
FIGURE 13-8. Intraoperative view of the markings for a
double-opposing z-plasty to repair a submucous cleft palate.
The most anterior portion of the palate, the alveolar
ridge, is often closed separately. The oronasal fi stula in the
region of the cleft is usually small and, despite problems
with regurgitation, has little impact on speech production.
Reconstruction in this area requires bone graft to create a
stable palatal arch, provide bone into which the erupting
teeth may anchor, support the alar base of the nose, and
provide a base for prosthetic dental restoration.
Closure of the alveolus has been described at various
times during the management of a cleft palate. Some
authors advocate mucosal closure at the time of initial
lip repair (gingivoperiosteoplasty), noting that the bony
defect may then heal without the need for later grafting. 16
Others advocate early bone grafting, about the time of
palatal repair at around 1 year of age. 17 Others prefer to
delay grafting anywhere between 2 to 12 years of age. 18
A better gauge for timing repair may be based on dental
eruption. In this scheme, suggested times for alveolar graft-
ing include (1) prior to eruption of the permanent incisor,
(2) after eruption of the incisor but before eruption of the
cleft side canine, and (3) after eruption of the cleft side
canine. Details of the technique are beyond the scope of
this chapter and are discussed elsewhere.
COMPLICATIONS
The two signifi cant early complications of cleft palate
repair are bleeding and airway compromise. Bleeding fol-
lowing cleft palate repair is rare if care is taken to protect
the greater palatine artery and control blood loss intraop-
eratively. Infi ltration of local anesthetic with dilute lido-
caine and epinephrine is useful prior to beginning the
procedure and intraoperatively the blood pressure should
be carefully monitored and controlled. Once the palate is
closed, gentle compression over the bone may be useful
prior to emergence from anesthesia.

138 • CHAPTER 13
Postoperative airway compromise in the obligate nose
breather may result from edema and blood present in an
already small nasopharynx. It may also result from swelling
due to compression beneath the Dingman mouth gag and
subsequent edema of the tongue. As a precaution, many
surgeons place a suture through the tip of the tongue at
the completion of the repair to assist in pulling the tongue
forward in the event of airway compromise. In small chil-
dren, the tongue may be diffi cult to grasp in an emergent
setting, making the suture potentially lifesaving. It is easily
removed on the fi rst postoperative day with no adverse
sequellae.
More common complications related to palatal closure
are those related to wound healing problems. Dehiscence
and fi stula formation may be minimized by closing
the medial palatal margins under as minimal tension as pos-
sible. To achieve this, wide lateral undermining is required.
The surgeon, however, must be cognizant of the location
of the pedicle to maximize dissection of the surrounding
soft tissues while avoiding vascular injury.
The mucosal repair needs to be watertight since the inci-
sion lines are continuously bathed by saliva and mucus from
the time the palate is closed. Furthermore, oral feedings
need to be restarted in the postoperative period to main-
tain adequate nutrition. To combat the diffi culty in creat-
ing a watertight seal, several authors have advocated the
use of tissue sealants in cleft palate repair. 19 Cyanoacrylate
adhesive may be instilled beneath the mucoperiosteal fl aps
to fi x the fl aps in place and minimize dead space or placed
as a topical sealant. It has yet to be shown whether such
maneuvers minimize the risk of fi stula formation.
Often, some amount of tension is placed across the
repair and a fi stula forms at the point of maximal tension
( Figure 13-9 ). Fistulae as small as 5 mm 2 may interfere
with speech production. 20 Rates of formation vary among
published series, but is approximately 5% to 10% (and
higher for patients older than 2 years). Secondary closure
FIGURE 13-9. Chronic fi stula of the hard palate.
FIGURE 13-10. Photograph of the nasopharynx
demonstrating purulent fl uid arising from the unseen
Eustachian tube.
of a palatal fi stula is diffi cult due to the scarring that is
present at the margins of the defect. Alloplastic material
(acellular dermis), local fl aps (adjacent palate), or distant
fl aps (tongue fl ap), may be used to cover the defect and
better results are obtained with a two-layer closure.
Wound dehiscence may result from infection at the
time of repair. Generally, the palate tolerates the normal
colonization of bacterial fl ora within the oral cavity and
does not become infected. In the oral cavity, common fl ora
include staphylococcal and streptococcal species, as well
as Enterobacter , Neisseria , Bordetella , and Corynebacterium .
However, children with clefts often have fl uid in the mid-
dle ear which may harbor more virulent organisms and act
as a source of contamination ( Figure 13-10 ). Since repair is
considered elective, deferring surgery until after any infec-
tion has subsided is often prudent.
OUTCOMES ASSESSMENTS
Following successful cleft palate closure, the major param-
eter to observe is the patient’s speech development and
language production. Children born with a cleft palate
are at risk for speech and language problems related to
velopharyngeal insuffi ciency. Reports of insuffi ciency range
from 7% to 30%. Frequently, adjunctive speech therapy is
needed to correct errors of articulation that develop as a
compensatory mechanism. Despite advances in the surgi-
cal management of cleft palate, preschoolers continue to
require speech therapy as an integral component of their
global care.
Speech impairments specifi c to velopharyngeal incom-
petence affect those sounds that require pressure in the
oral cavity, either anteriorly or posteriorly. These include
the sibilant consonants soft g, /s/, /z/, /ch/, /sh/, and /zh,
and the intraoral pressure consonants /b/, /d/, hard g,

CLEFT PALATE • 139
/k/, /p/, and /t/. Such problems have been shown to be
more prevalent in patients with a complete cleft of the
hard and soft palate or those with associated congenital
anomalies. 21
Technique and surgeon skill may each play a role in the
ultimate success of cleft palate repair. Longitudinal closure
with an intravelar veloplasty was compared with Furlow
palatoplasty in nonsyndromic cleft palate patients operated
upon by a single surgeon. 22 Patients who had undergone
straight-line closure had a 34% higher incidence of hoarse-
ness, nasal air escape, and hypernasality at 3 years of age
than those who underwent Furlow palatoplasty. No differ-
ence was noted in the rate of fi stula formation. In a sepa-
rate study, nonsyndromic patients with an incomplete cleft
palate demonstrated a higher rate of adequate velopharyn-
geal function following Furlow palatoplasty as opposed to
von Langenbeck palatoplasty. 23 The Furlow technique was
also noted to have fewer complications and the results of
velopharyngeal function translated to improved results in
speech and language production.
Midfacial growth is also a concern following repair of
the palate. It is thought that the production of scar tis-
sue near the growth centers of the face interferes with
normal skeletal development. This fi nding has been noted
since the early 1900s when Gilles and Fry observed poste-
rior displacement and narrowing of the maxillary arch in
patients who underwent early hard palate repair. Similarly,
Ortiz-Monasterio et al., 24 among others, 25 noted excellent
jaw relationships in adult patients who did not undergo
timely palate closure. As a result, surgical protocols were
developed that closed only the soft palate at an early stage
while the hard palate was obturated with a prosthesis until
facial growth was complete. 26 The belief was that closure
of the more pliable soft palate had less of an effect on
facial growth than did closure of the hard palate. It should
be noted however that other studies have failed to show
an adverse affect on facial growth by repair of the palate. 27
To date, prospective, well-controlled studies are still lack-
ing to establish the long-term effect of hard palate repair
on facial growth. Creation of such a study would likely
be unethical in light of the speech concerns that would
develop in the control group of patients with portions of
their cleft left unrepaired.
Currently, modern interventions for patients with a
cleft palate result in a good outcome in terms of speech
development and facial appearance with a low risk of com-
plication. Management should be directed by a team of
clinicians with experience in treating children with clefts
of the lip and palate. Continuing long-term evaluations are
equally important to analyze the choices that are made
with respect to the timing and techniques of cleft pal-
ate repair.
REFERENCES
1. Vichi M, Franchi L. Eruption anomalies of the maxillary
permanent cuspids in children with cleft lip and/or palate.
J Clin Pediatr Dent. 1996;20:149.
2. Witzel MA, Salyer KE, Ross RB. Delayed hard palate clo-
sure: The philosophy revisited. Cleft Palate J. 1994;21:263.
3. Randall P, LaRossa DD, Fakhraee SM, et al. Cleft palate clo-
sure at 3 to 7 months of age: A preliminary report. Plast
Reconstr Surg. 1983;712:624.
4. Semb G. A study of facial growth in patients with unilateral
cleft lip and palate treated by the Oslo Cleft Lip and Palate
Team. Cleft Palate Craniofac J. 1991;28:1.
5. Schweckendiek H. Zur Frage der Fruh-und Spat-operationen
der angeborenen Lippen-Kiefer-Gaumen-Spalten (mit Dem-
onstrationen). Z Laryng Rhin Oto. 1951;30:51.
6. Schweckendiek W. Primar veloplasty: Long-term results
without maxillary deformity. A twenty-fi ve year report. Cleft
Palate J. 1978;15:268.
7. Bardach J, Morris HL, Olin WH. Late results of prima-
ry veloplasty: The Marburg Project. Plast Reconstr Surg.
1984;73:207.
8. McWilliams BJ. Submucous clefts of the palate: How like-
ly are they to be symptomatic? Cleft Palate Craniofac J.
1991;28:247.
9. Trier WC. Primary palatoplasty. Clin Plast Surg. 1985;12:659.
10. Friede H, Johanson B. Adolescent facial morphology of ear-
ly bone grafted cleft lip and palate patients. Scand J Plast
Reconstr Surg Hand Surg. 1982;14:41.
11. Braithwaite G, Maurice DG. Importance of levator veli
palatine muscle in cleft palate closure. Br J Plast Surg.
1968;21:60.
12. Kane AA, Lo LJ, Yen BD, et al. The effect of hamulus frac-
ture on the outcome of palatoplasty: a preliminary report
of a prospective, alternating study. Cleft Palate Craniofac J.
2000;37:506.
13. Furlow LT. Cleft palate repair by double opposing Z-plasty.
Plast Reconstr Surg. 1986;78:724.
14. Gunther E, Wisser JR, Cohen MA, Brown AS. Palatoplasty:
Furlow’s double-reversing Z-plasty versus intravelar velo-
plasty. Cleft Palate Craniofac J. 1998;35;546.
15. Noorchashm N, Dudas JR, Ford M, et al. Conversion Furolw
palatoplasty. Ann Plast Surg. 2006;56:505.
16. Santiago PE, Grayson BH, Cutting CB, et al. Reduced need
for alveolar bone grafting by presurgical orthopedics and
primary gingivoperiosteoplasty. Cleft Palate Craniofac J.
1998;35:175.
17. Dado DV, Rosenstein SW, Alder ME, Kernahan DA. Long-
term assessment of early alveolar bone grafts using three-
dimensional computer-assisted tomography: a pilot study.
Plast Reconstr Surg. 1997;99:1840.
18. Cohen M, Polley JW, Figueroa AA. Secondary (intermedi-
ate) alveolar bone grafting. Clin Plast Surg . 1993;20:691.
19. Turkaslan T, Ozcan H, Dayicioglu D, Ozsoy Z. Use of adhe-
sives in cleft palate surgery: A new fl ap fi xation technique.
J Craniofac Surg. 2005;16:719.

140 • CHAPTER 13
20. Witt PD, D’Antonio LL. Velopharyngeal insuffi ciency and
secondary palatal management: A new look at an old prob-
lem. Clin Plast Surg. 1993;20:707.
21. Persson C, Elander A, Lohmander-Agerskov A, Söderpalm
E. Speech outcomes in isolated cleft palate: impact of cleft
extent and additional malformations. Cleft Palate Craniofac
J. 2002;39:397.
22. Gunther E, Wisser JR, Cohen MA, Brown AS. Palatoplasty:
Furlow’s double reversing Z-plasty versus intravelar velo-
plasty. Cleft Palate Craniofac J. 1998;35:546.
23. Yu CC, Chen PK, Chen YR. Comparison of speech results
after Furlow palatoplasty and von Langenbeck palatoplasty
in incomplete cleft of the secondary palate. Chang Gung
Med J. 2001;24:628.
24. Ortiz-Monasterio F, Serrano Reveil A, Valderrama M, Cruz
R. Cephalometric measurements on adult patients with
nonoperated cleft palates. Plast Reconstr Surg. 1959;24:53.
25. Mestre JC, De Jesus J, Subtelny JD. Unoperated oral clefts at
maturation. Angle Orthodon. 1960;30:78.
26. Schweckendiek W. Primary veloplasty: long-term results
without maxillary deformity. A twenty-fi ve year report. Cleft
Palate J. 1984;15:268.
27. David DJ, Anderson PJ, Schnitt DE, et al. From birth to ma-
turity: a group of patients who have completed their proto-
col management. Part II. Isolated cleft palate. Plast Reconstr
Surg. 2006;117:515.

14
c h a p t e r
Secondary Deformities
of the Cleft Lip, Palate, and Nose
Jamal M. Bullocks, MD, FACS / Samuel Stal, MD
INTRODUCTION
Secondary deformities of the cleft patient are inevitable.
Despite timely and appropriate care, the intrinsic congen-
ital insult confers overwhelming forces on the anatomi-
cal structures associated with the cleft. Throughout the
lifetime of the patient, theses forces are not overcome,
conversely growing with the patient causing continual
deformation and challenges to repair efforts.
Once of controversy was the chronological age at which
clefts were repaired. Arguments for delayed repair touted
the benefi ts of improved structural outcomes leading to
fewer secondary deformities. However, it is without a
doubt that early primary repair of the cleft lip and palate
produce signifi cant improvements in speech and subse-
quent cognitive and language development. Additionally,
the social and psychological advantages have proven to be
innumerable. Therefore, early primary repair of the cleft
lip and palate is now the standard despite its potential neg-
ative effects on the growth of the maxilla and subsequent
secondary deformities. 1
At the root of secondary deformities of the cleft lip,
palate, and nose is the deformed maxilla. The maxilla is
hypoplastic, and retruded with outward rotation of the
cleft elements. With regard to the bilateral cleft, there is
inherent premaxillary instability. These structural anoma-
lies results in a midface confi guration that defi es the ability
to harmoniously reconstruct the soft tissues of this region.
The soft tissues are displaced by the cleft and are aber-
rantly inserted in the bony cleft margin which effectively
tethers these structures. Lastly, the associated soft tissue
structures are themselves hypoplastic and deformed dur-
ing development, making primary repair diffi cult.
The secondary deformities of the cleft patient are not
only the result of this overwhelming congenital disposition,
but are also infl uenced by the other factors. Of greatest
importance is the overall health and associated comorbidi-
ties of the patient. Functional primary repairs of the lip
and palate are plagued when there is a strong history of
morbidity in the upper airway. Chronic coughing, gagging,
and infections delay wound healing after primary repair
and predispose the patient to failure and to the develop-
ment of secondary deformities. The type and technique of
the primary repair will also infl uence the development of
secondary deformities. This aspect is a direct correlation
of the cleft surgeons judgment and ability, which is the
most controllable variable in this equation.
The evaluation and treatment of secondary deformities
of the cleft lip, nose and palate requires an astute apprecia-
tion of the evolution of the cleft. Secondary deformities
are predictable; the presenting embryologic insult dictates
the development of deformities despite repair. Finally,
because of the three-dimensional confi guration of the lip,
nose, and palate along the cleft, it is appreciated that these
deformities do not occur in isolation. Properly timed con-
comitant repair of all defects will increase the ability to
restore anatomic congruity and ultimately minimize the
need for additional secondary procedures.
SECONDARY DEFORMITIES
OF THE CLEFT LIP
Secondary deformities of the lip after primary repair are
a spectrum of anomalies that range from superfi cial scar
abnormalities to full-thickness derangement of the skin,

142 • CHAPTER 14
muscle, and mucosa. Evaluation of the lip involves care-
ful assessment of the mucosa, vermilion, orientation of
the orbicularis, symmetry of the philtral complex, and
examination of the lip scar with special attention to the
alignment of the white roll. Normally, in the adult, the
philtrum is 17 mm in length with the philtral columns
diverging from the collumela and ending at the peaks of
the Cupid’s bow. 2 At the superior aspect of the philtrum,
the columns are 6 to 9 mm apart, while at the base are 8 to
12 mm apart. 2 In the young patient, these measurements
are appreciably smaller but similar in proportion. Ideally
there should also be an uninterrupted white roll with a
smooth transition of the contiguous lip skin and vermilion
mucosa. The orbicularis muscle is oriented circumferential
to the vermilion without bulges or tethering to either the
lip skin or mucosa. Additionally important to the lip anat-
omy, is the anterior projection of the upper lip compared
with the lower. When the patient is relaxed the upper lip
projects 2 to 3 mm anterior to the lower lip. 1
The most frequent secondary anomalies after primary
repair include vermilion defi ciency contributing to the
“whistle” deformity, lip skin excess (long lip), lip skin defi -
ciency (short lip), orbicularis disorientation, and vermil-
ion misalignment. 3 Although these secondary deformities
are seen after both unilateral and bilateral repair, patients
with bilateral cleft lip are predisposed to another subset
of secondary deformities. In patients with bilateral cleft
lip, secondary deformities are either a problem of rela-
tive excess or defi ciency. At the time of primary repair, the
integrity of the prolabium as well as the surgical plan con-
trols the ability to create a symmetric philtrum with the
proportions described above. In a situation in which there
is adequate prolabial substance, errors in planning contrib-
ute to a lip that will ultimately be wide. The philtrum is in
excess of tissue, appearing wide without the discrepancy in
the collumelar and base distances of the philtral columns.
When there is a relative defi ciency of prolabial tissue a short
retracted philtrum will result despite adequate technique
during primary repair. 4 The so-called “tight lip” results from
horizontal defi ciency of the philtrum. 1 These aberrations of
the philtral skin also occur in the mucosa, conferring either
a defi ciency or excess of vermilion. Lastly, both unilateral
and bilateral cleft patients are susceptible to abnormalities
associated with the scars from the primary repair. The scar
may be hypertrophic, widened, or abnormally contracted,
thereby distorting the symmetry of the repair.
Modalities for preventing secondary cleft lip deformi-
ties stem from appropriate presurgical treatment, choice
of operative technique, and meticulous attention to detail
during operative repair. Over the past two decades, the
advent of presurgical orthopedics has improved our ability
to perform primary repair on patients with very wide
clefts. 5 , 6 An intraoral appliance is fi xed to the maxilla along
the cleft margins. The maxillary segments are then actively
or passively aligned into a more normal anatomic relation-
ship. Presurgical orthopedics is initiated early in the post-
natal period in order to shorten the distance between the
cleft margins so that primary repair can be performed at
FIGURE 14-1. The spectrum of secondary cleft lip
deformities: malalignment of the white roll, vermilion excess
with notching, long lip deformity, and hypertrophic scarring.
3 months of age. 5 – 7 The surgical technique for lip repair
should address the full height of the lip extending into
the nasal sill, as well as the orbicularis muscle. The tension
should be placed on the upper third of the repair as in
the Millard rotation advancement. At the time of primary
repair every effort should be made to free all the soft tis-
sues completely from the cleft in order to create a tension-
free repair. It is also at this point where there is the best
chance to align the white roll and vermilion to prevent
subsequent deformities that are more diffi cult to correct
secondarily.
Secondary deformities of the cleft lip, once identi-
fi ed, should be addressed when there has been adequate
development of the soft tissue to allow easy manipulation.
Once the wounds of the primary repair have healed, an
integral growth period is allowed for maturation of the lip
elements. This will establish an appropriate consistency of
soft tissue and accurately defi ne the deformity to increase
the likelihood of success with secondary repair. Repair of
secondary cleft lip deformities should then be approached
in one operation with as many procedures necessary. 1
Cleft Lip Scar Abnormalities
The scar of the secondary cleft lip deformity represents
abnormalities of superfi cial and deep components of the
lip, including the skin, vermilion, and underlying orbicu-
laris muscle. Isolated superfi cial scar abnormalities along
the skin portion of the repair are in the form of hyper-
trophy, contracture, notching, and tethering of the vermil-
ion ( Figure 14-1 ). Deformities in the postoperative period
may not be present until the onset of scar maturation, at
which time the intrinsic characteristics of wound healing
predispose the incisions to the development of such defor-
mities. In the early postoperative period, these abnormal
scars should be treated conservatively. The parents are
instructed to constantly massage the scars with the appli-
cation of coca butter, vitamin E, or Mederma. 1

SECONDARY DEFORMITIES OF THE CLEFT LIP, PALATE, AND NOSE • 143
Scars that are refractory to conservative therapy are
readdressed with more aggressive, invasive treatment at
an interval of 12 months after primary repair. After at
least 1 year, the scar has been given ample opportunity to
mature, at which point revision should be considered. The
elevated scar may be treated simply with corticosteroid
injection or dermabrasion. However, these therapies are
less effective on widened scars as compared with raised
scars. Surgical revision of abnormal scars entails elliptical
or diamond-shaped excision of the scar with linear re-
approximation ( Figure 14-2 ). If notching of the vermil-
ion is a problem this reorientation will serve to lengthen
the scar and relieve minor problems. Often, full-thickness
excision is required with re-approximation of the orbicu-
laris, when muscular derangement is encountered on eval-
uation. As painful as it is to the family, aggressive revisions
are sometimes warranted.
Malalignment of the Vermilion
Continuity of the white roll is often the most judged com-
ponent of the lip repair ( Figure 14-3A,B ). Inaccurate align-
ment of the vermilion at the time of primary repair is the
culprit, causing lack of color continuity along the skin and
vermilion border. Discrepancies of as small as 2 mm are
noticeable at conversational distances. 1 , 3 , 8 Surgical correc-
tion is the only remedy ( Figure 14-4 ). The vermilion should
be used to reconstruct the vermilion step-off instead of
mucosa with tissue rearrangement opposing like-with-like
tissue. Z-plasty rearrangement is effi cacious in reorienting
the skin vermilion step-off. This is accomplished with the
z-plasty designed along the vertical component of the step
off with horizontal extensions at 45 degrees. Transposition
of the two skin fl aps creates a smooth continuous white
roll without distortion of other components of the lip.
A
B
C
FIGURE 14-2. Diamond-shaped excision of philtral scars are suitable for minor
superfi cial scar revisions, and are particularly useful in increasing the scar length and
reducing vermilion height.
A
B
FIGURE 14-3. Alignment of the white roll is a major component of determining
success in primary cleft lip repair. (A) Poor alignment of less than 3 mm is obvious at
conversational distances. (B) Proper alignment includes reconstruction of the Cupid’s bow.
A
B

144 • CHAPTER 14
When elevation of the vermilion at the site of repair
occurs ( Figure 14-5 ), this may be due to varying severity of
deformity. This includes superfi cial scar contracture caus-
ing shortening of the involved philtral column or muscu-
lar malalignment tethering the skin superiorly toward the
cleft. In minor superfi cial cases, conservative therapy with
scar massage is instituted. If there is persistent deformity
12 months after primary repair, the scar margins are excised
along the length of the philtrum across the white roll into
the vermilion in a diamond shaped ( Figure 14-2 ). Closure
is then preceded in a pants-over-vest fashion to maintain
fullness along the margin ( Figure 14-6 ). 8 Full-thickness
deformities representing malalignment of the orbicularis
result from inadequate rotation of the lateral lip elements
at the time of primary repair. 8 – 10 This deformity can only
be corrected by disassembling the reconstruction and re-
rotating the lip elements with careful re-approximation of
the orbicularis.
FIGURE 14-5. Elevation of the vermilion produces
notching of the scar margin.
Orbicularis Oris Deformities
The importance of orbicularis continuity cannot be over-
stated. Proper restoration of orbicularis orientation not
only improves the appearance and function of the lip but
also provides a stable base for correction of cleft palate
and nasal deformities. Prevention of secondary muscle
deformity is most effectively accomplished with proper
primary repair. The deformed muscle inserts vertically
along the cleft margin and the alar base. The initial recon-
struction should involve complete release of the muscular
attachments to the cleft followed by direct approximation
of both the superfi cial and deep layers of the orbicularis.
Restoring continuity during primary repair additionally
provides a driving force for shaping the premaxilla and
palatal shelves. Therefore, orbicularis deformities have a
profound effect on the lip, palate, and nose as well as the
subsequent prognosis of the repair of these structures.
Orbicularis diastasis is evident by the presence of bulg-
ing along the incision of the cleft repair. This is particu-
larly evident with attempted animation. Once secondary
orbicularis deformities are identifi ed, repair should be
performed by completely releasing the scar and resort-
ing continuity of the muscle. After the scar is opened the
orbicularis is separated from the skin and mucosa in sub-
dermal and submucosal planes, respectively. The muscle is
then rotated form its vertical position to a more anatomi-
cal horizontal position. In the unilateral cleft, the muscle
is re-approximated to the contralateral orbicularis without
distortion of the philtrum. In bilateral cleft deformity, the
muscle is reoriented horizontally and attached to the lat-
eral aspect of the prolabial skin.
Vermilion Defi ciency
Vermilion defi ciencies occur in unilateral and bilateral
cleft patients in different forms. In unilateral deformity,
defi ciency of the vermilion on the medial lip element is a
common problem. This causes an apparent fullness on the
A
B
C
FIGURE 14-4. Malalignment of the white roll corrected with superfi cial z-plasty
rearrangement.

SECONDARY DEFORMITIES OF THE CLEFT LIP, PALATE, AND NOSE • 145
vermilion of the lateral lip element, the “whistle” defor-
mity or vermilion notch. In addition to the defi ciency of
the vermilion in the bilateral cleft lip patient, there is also
appreciable shortening of the philtrum. The philtrum is
usually normal in height in the unilateral cleft patient,
making techniques for repair quite different in these two
patient populations.
Mild to moderate defi ciencies of the vermilion may be
simply camoufl aged with fi llers. Traditionally, dermal fat
grafts and injectable autologous fa, has served this pur-
pose. 11 More recently, a variety of nonautologous fi llers
have been described, including bovine collagen, acellular
dermal matrix, and hyaluronic acid. Fillers represent the
least invasive technique for correcting minor deformities
and are particularly useful when there is not overwhelm-
ing scar contracture along the lip repair. In the severely
scarred lip, there will be considerable diffi culty gener-
ating enough force to overcome the tissue forces and
appropriately disperse the implanted material. Variable
absorption of theses fi llers may mandate repeat or multiple
applications to sustain their structural effect. 11
More signifi cant defi ciencies of the vermilion require
local tissue rearrangement to properly address the deformity.
The most common confi guration in the unilateral cleft lip
patient is vermilion defi ciency medial adjacent to vermilion
excess laterally along the scar. Repair involves fi rst resecting
the entire scar, then the excess lateral vermilion is redistrib-
uted to the medial deformity. This can be easiest accom-
plished with a simple z-plasty along the upper border of the
vermilion without disturbing the white roll ( Figure 14-7 ).
If distortion of the remaining lip element, particularly along
the white roll, is predicted with redistribution of the lateral
lip vermilion, tissue may be recruited by V-Y advancement
of the mucosa to replace the defi cient vermilion. 12
Vermilion defi ciencies in bilateral cleft secondary defor-
mities respond less favorably to fi llers and local tissue
FIGURE 14-6. Pants-over-vest closure prevents scar depression and adds bulk to
maintain fullness along the scar.

146 • CHAPTER 14
rearrangement, unless these defects are minor. Due to the
concomitant philtral defi cit, favorable results are better
obtained with lower to upper lip switch procedures, such
as an Abbe fl ap ( Figure 14-8 ). The Abbe fl ap helps correct
projection discrepancies between the upper and lower lips,
in which the lower lip excess is transferred to the upper
lip defi ciency. Although lip switch procedures are reliable
procedures for correcting vermilion and philtral defi cien-
cies, in patients with reasonable dimensions, these fl aps
should be considered second line to scar revision and local
tissue rearrangement.
CUPID’S BOW: SECONDARY
DEFORMITIES
The caudal portion of the philtrum represents a complex
and aesthetically intricate structure, the Cupid’s bow.
Secondary scar and mucosal deformities distort the phil-
tral tubercle and the slight peak of the vermilion at the
base of the philtral column. These deformities are a variety
of abnormalities of vermilion discrepancy, ranging from
notching ( Figure 14-5 ) to complete absence of Cupid’s
bow. For patients with vertical defi ciency lateral to the
cleft and subsequent loss of alignment of the white roll at
the peak of the bow, simple triangular skin excision and
A
C
B
FIGURE 14-7. Recruitment of the local vermilion into an
area of vermilion defi ciency may be accomplished by varying
techniques: (A) local V-Y advancement; (B) z-plasty; (C) V-Y
advancement of the labial vermilion.
superior advancement of the vermilion will treat this prob-
lem effectively ( Figure 14-2 ). In cases of smaller step-offs,
z-plasty reorientation of the white roll as described ear-
lier may suffi ce. When performing these techniques that
advance the vermilion, care is taken not to rotate the inside
“wet” vermilion to the dry external surface. Although,
these techniques of skin excision and vermilion advance-
ment are effective, the associated scarring of the wounds
may induce an artifi cial appearance to the lip. Therefore,
in these instances, particularly in patients with abnormal
scarring after primary repair, the repair should be taken
down to repeat rotation advancement of the lateral lip.
When the Cupid’s bow portion of the vermilion is
totally absent, as is frequently the case in bilateral cleft lip
deformities, then reconstruction with a Abbe fl ap should
be considered to provide adequate vermilion and vermil-
ion ridge to reshape the Cupid’s bow on the cleft side. In
certain instances, the vermilion portion of the Cupid’s bow
may be correctly aligned but have poor superior projection.
In these cases, elevation of the white roll is performed with
tissue fi llers (hyaluronic acid or autologous fat) to accentu-
ate the peak of the Cupid’s bow. When there is vermilion
defi ciency, the vermilion portion of the Cupid’s bow can
be similarly augmented with dermal fat grafts or injectable
fi llers with a modest degree of overcorrection at the time
of implantation.
The Short Lip
In unilateral cleft lip deformity, the short lip refers to
positioning of the lateral lip elements at a defi cient ver-
tical height with respect to the medial lip elements and
is usually accompanied by asymmetry of Cupid’s bow. 8
The short lip is often noted to be 3 to 4 mm shorter in
length as compared with the normal lip. 1 , 3 This secondary
deformity is usually the result of scar contracture, but also
results from inadequate rotation and advancement at the
time of primary repair. 1 , 3 , 8 The most effective way of cor-
recting this deformity is by releasing the scar completely
and performing rotation and advancement of the lateral
elements similar to the technique described by Millard for
primary repair. 13 During secondary rotation and advance-
ment, attention to additional downward positioning is
paramount. If a rotation and advancement repair was per-
formed as the initial repair, then a full-thickness z-plasty,
including the muscle and mucosa, can also be effective.
Minor length discrepancies can be treated with superfi cial
diamond-shaped skin excision and closure to lengthen the
scar.
The short lip is also occasionally secondary to vermilion
and labial mucosa defi ciencies. 1 , 3 , 8 In these instances, cor-
rection with z-plasty and V-Y mucosal advancement is not
adequate, as it is the case with vermilion length discrepan-
cies alone. There is an additional tissue requirement that
can be addressed with a dermal fat graft or lateral buccal
fat. This type of deformity is more commonly seen in bilat-
eral secondary cleft lip deformities and is also amenable
to Abbe fl ap reconstruction when the shortness is both in

SECONDARY DEFORMITIES OF THE CLEFT LIP, PALATE, AND NOSE • 147
the lateral and vertical dimension. The Abbe fl ap provides
composite tissue to replace the vertical skin and mucosa
defi ciency. 14
The Long Lip
In long lip deformity, the lateral lip elements are inferiorly
positioned compared with the medial lip elements. This
occurs more commonly after unilateral compared with
bilateral cleft lip repair. The Tennison triangular fl ap repair
of the cleft lip often resulted in the long lip. 15 This is due
to the inadequately addressed upper third of the lip during
primary repair. With the increasing utilization of the rota-
tion advancement technique being performed for primary
repair of the cleft lip, this secondary deformity is seen in
less frequently.
Correction of long lip deformity requires opening the
entire lip from the base of the nose to the vermilion. The
skin, muscle, and musocal elements are re-approximated
from the bottom up. The associated dog ear is incorpo-
rated into the nasal sill and alar base. The excess tissue
is then excised along the lateral alar base. Although it is
tempting to perform a lesser simple excision of the tis-
sue below the alar base and pexy, the elongated lip to
the piriform aperture, this technique is doomed to failure.
The overwhelming forces of gravity and underlying mus-
culature will pull down on the base of the ala on the cleft
side, making the lower border on the cleft side appear
long.
The Tight Lip
The tight lip represents tissue defi ciency in the horizontal
plane after primary lip repair. 8 This deformity is more com-
monly seen after bilateral cleft lip repair. 8 The width of the
cleft and the degree of prolabial protrusion predisposes to
the formation of this secondary deformity after primary
repair. After primary repair, the prolabium remains short
A
C
B
D
FIGURE 14-8. (A–D) Lip switch procedure (Abbe fl ap), based on the inferior labial
artery. Transfer of a central lower lip segment to augment the central upper lip and
reconstruction of the vermilion.

148 • CHAPTER 14
compared with the lateral lip elements ( Figure 14-9 ). This
is an example in which the initial congenital insult is diffi -
cult to overcome secondary to the relative paucity of tissue
available for primary repair. With the advent of presurgical
orthopedics which attempts to decrease the cleft distance
before primary repair, hopefully this deformity will be
seen less frequently.
In order to correct the problem of upper lip defi ciency,
tissue must be recruited into this region. Historically, this
has been done most successfully with a lip switch pro-
cedure (ie, Abbe fl ap). 16 The Abbe fl ap will reduce the
tightness of the upper lip and reduce the projection dis-
crepancy of the lower lip. 14 , 16 In addition to providing skin
and vermilion to the upper lip, an Abbe fl ap will also pro-
vide addition muscle, which has been shown to improve
orbicularis function. 14 Technically, the fl ap should always
be placed centrally to replace the philtral portion of the
lip regardless of where the upper lip defi ciency exists. If
there is relative lateral defi ciency contributing to a tight lip
deformity, the fl ap is again placed centrally in order to pro-
vide symmetry of the upper lip, lateral elements are rotated
and advanced to restore orbicularis continuity. Depending
on the vermilion defi cit that is present in the upper lip,
the fl ap can be designed with either V- or M-shaped donor
excision to recreate the upper lip tubercle or Cupid’s bow
region. 16
The Wide Lip
Contrary to the tight lip, the wide lip represents horizontal
excess of the upper lip. This deformity again more com-
monly occurs after bilateral cleft lip repair. This second-
ary deformity is the result of both technical design errors
during initial repair and mechanical forces stretching the
dimensions of the repair. 1 , 3 At the time of initial lip repair
in the 3-month-old infant, the philtral width should be
designed to be approximately 6 mm. 1 , 3 Additionally, dur-
ing primary repair, the orbicularis muscle must be fi rmly
attached to itself across the midline. If the muscle fails to
unite with the prolabium, the orbicularis will pull either
side of the lateral lip elements away for the prolabium
widening this region over time. If this is the instance, there
will be concomitant hypertrophy and excess fullness of the
lateral lip.
To correct the wide lip deformity, there must be reduc-
tion of the wide prolabium in conjunction with correction
of the orbicularis. A new philtrum is designed after excision
of the excess philtral tissue. The orbicularis is redirected
into a horizontal position and fi rmly affi xed to the prola-
bium. If possible, depending on the adequacy of tissue in
the philtrum, an attempt should be made to reconstitute
the orbicularis across the cleft. Orbicularis reconstitution
is easily performed in patients with wide unilateral cleft
FIGURE 14-9. Secondary bilateral cleft lip deformity demonstrating “festooning,”
philthral defi ciency, and long lateral lip elements.

SECONDARY DEFORMITIES OF THE CLEFT LIP, PALATE, AND NOSE • 149
deformities. Finally, it is equally important to design the
new philtrum smaller than the fi nal desired size to antici-
pate the inevitable stretching that occurs during healing.
CLEFT PALATE: SECONDARY
DEFORMITIES
Secondary deformities of the cleft palate represent a com-
plex conglomeration of soft tissue and bony structural
abnormalities that results in both cosmetic and func-
tional abnormalities. The misaligned palatal shelves and
the hypoplastic maxilla are the culprits of these second-
ary deformities. Despite timely primary repair of the pal-
ate, the predisposing structural aberrations decrease the
chance for an acceptable primary repair. The cleft palate
can be either V or U shaped with aberrant insertion of
the palatal soft tissues into the cleft margins, particularly
the tenor veli palatine muscle which serve to elevate the
palate and close the communication between the nose and
mouth during speech and deglutination ( Figure 14-10 ).
The relative distance of the palatal shelves and the length
of the palatal diastasis will predict the occurrence of sec-
ondary deformities. For instance, a wide, complete cleft of
the palate that requires multiple procedures to close will
inevitably develop secondary deformities after primary
repair.
Confounding the development of secondary palatal
deformities is the negative effect the primary repair has
on growth of the maxilla. Inhibition of maxillary growth
is believed to result from the transient devasculariza-
tion of the palate that occurs in most techniques used
for palatoplasty. Delaying the primary repair to allow the
maxillary elements to become more robust may minimize
the development of secondary deformities. However, delay
in primary palate repair is not an option because of the
overwhelming negative effect it has on speech, language,
and cognitive development. 1 Therefore, early repair of the
cleft palate is the rule and secondary deformities are inevi-
table. Understanding the management of these deformities
is essential knowledge for a cleft surgeon.
The palate is embryologically separated into primary
and secondary components by the incisive foramen. The
premaxillary segment forms from the fusion of the medial
nasal process, a derivative of the frontal nasal process and
the lateral maxillary elements. The premaxillary segment
will form the midportion of the lip and house the alveolar
segment of bone containing the incisors. Failure of fusion
of this structure produces a cleft of the primary palate. It
is important to recognize that the primary cleft is a three-
dimensional structure that extends from the nose to the
anterior alveolar segment ( Figure 14-10 ). The resultant
secondary deformities of the primary cleft palate are in
the form of oronasal fi stulas, anterior alveolar fi stulas, and
a varying array of dentolalveolar aberrations.
Posterior to the incisive foramen is the secondary pal-
ate. The secondary palate forms from the fusion of the
palatine shelves and mesenchymal infi ltration across the
cleft margins. There can be complete or incomplete for-
mation along the length of the cleft. Repair of the second-
ary palate is designed not only for coverage of the defect
but more importantly to reorient the deranged levator veli
palatini muscle. Secondary deformities of the secondary
palate present with varying degrees of palatal fi stulae and
velopharyngeal incompetence.
Auditory tube
Aponeurosis
Vomer
Maxilla
Alveolus
Tensor
Soft palette
Superior constrictor
Premaxilla
Alveolus
Palatine process of
maxilla
Tensor
Superior constrictor
Auditory tube
Aponeurosis
A
B
FIGURE 14-10. (A) Normal compared to (B) a cleft morphology of the palate and the
pharyngeal musculature.

150 • CHAPTER 14
Generally, fi stulae are the most common defects of
the hard palate after repair. Anterior to the alveolar ridge,
the defect is termed an oronasal fi stula and posterior it is
termed a palatal fi stula. 17 The majority of fi stulae are small
and present with regurgitation of food and liquid through
the nose. Speech is rarely affected by small fi stulae; how-
ever, larger defects can cause variable degrees of hyper-
nasality. Fistulae also result from orthodontic treatment,
specifi cally after rapid expansion of the dental arches. Most
commonly, fi stulae occur at the junction of the primary
and secondary palate followed by the hard palate alone.
There is an increased incidence of fi stula formation with
increasing severity in the cleft. Of all fi stulae, 61% occur
after repair of bilateral deformities of the lip and palate,
26% after unilateral deformities of the lip and palate, and
15% after isolated palatal defects. 18 , 19
Repair of Oronasal Fistulae
Oronasal fi stulae most frequently represent a residual cleft
in the maxillary alveolus that was not closed at the time of
primary palatoplasty and is not a true fi stula. If the fi stula
is small and without functional signifi cance, closure may
occur spontaneously with growth; therefore, intervention
should be delayed. Small symptomatic fi stulae anterior
to the alveolar arch can be closed by primary gingivope-
riosteoplasty at the time of lip repair. 8 , 20 This technique
may eliminate or minimize the need for bone grafting.
Placement of bone into the oronasal fi stula serves to pro-
vide a matrix into which the teeth can erupt, as well as to
stabilize the maxillary arch to allow proper alignment of
the maxillary teeth. Closure of the oronasal fi stula with
bone grafting is performed between 6 and 12 years of age
during the period of mixed dentition. 21 – 23
As a general rule, fi stulae should be closed by re-approx-
imating the oral and nasal layers separate from one another.
Most fi stulae are closed with local tissues within which the
bone graft is contained ( Figure 14-11 ). Frequently, a turn-
over fl ap can be employed to close the nasal layer after
which cancellous bone is packed into the recesses of the
cleft and covered with a shield of cortical bone, if available.
The iliac bone, harvested from the posterior surface of the
crest, is easily procured and is a plentiful source for graft-
ing. Palatal, buccal, or labial fl aps are then used to close the
oral layer ( Figure 14-12 ).
Palatal Fistulae
Palatal fi stulae vary in size and location and have a high
recurrence rate. Repair of palatal fi stulae involve rearrange-
ment of local tissues to close the defect in a double-layered
fashion. Large or recalcitrant fi stula may require the use
of regional oral fl aps or free fl aps for closure. Small palatal
fi stulae can be closed in two layers, using one turnover fl ap
from the palatal side to close the nasal mucosa and a second
large palatal fl ap to close the oral mucosa ( Figure 14-13 ).
The nasal mucosa can also be closed in a purse-string fash-
ion if the fi stula is small and the adjacent tissue is supple
and mobile. The oral mucosa fl aps should be designed large
enough to compensate for their scarred and inelastic qual-
ity. The design can be a unipedicle or a bipedicle fl ap and be
performed unilaterally or bilaterally ( Figure 14-14 ). These
fl aps often mimic those used in primary palatoplasty.
If there is a relative paucity of reliable adjacent tissue or
the fi stula is large, consideration should be given to recruit-
ing tissue from regional sources. Large anterior defects
can be closed using buccal mucosa fl aps. A portion of the
buccal sulcus is tunneled through the nasal fossa onto the
defect in the hard palate, which provides single-layer clo-
sure of the nasal mucosa. The oral mucosa is then closed
using large palatal fl aps. Fistulae in the posterior palate,
particularly the soft palate, can be closed either primar-
ily or with pharyngeal fl aps, depending on the amount of
tension. Pharyngeal fl aps provide fresh vascular tissue to
close the defect and obturate the nasophayngeal aperture,
which is of particular benefi t in patients with velopharyn-
geal incompetence.
For large recurrent defects, other regional fl aps from the
tongue and the buccal mucosa are employed when previous
attempts at repair have failed. The tongue fl ap described by
Guerrerosantos and Altamirano 24 ( Figure 14-15 ) mobilizes a
superfi cial portion of the tongue based on a posterior pedicle.
At a second procedure 3 weeks later, the fl ap is then divided
and inset. A larger portion of the lateral buccal mucosa may
be transferred to cover the entire oral palate based on the
facial artery ( Figure 14-16 ). 25 , 26 The facial artery myomu-
cosal fl ap transfers a portion of buccinator and lateral buccal
mucosa based either antegrade off the facial artery or retro-
grade off the buccal branch of the maxillary artery. 25 – 27
Crippling fi stulae that have failed repeated closure
attempts may require free tissue transfer. Reserved as
FIGURE 14-11. Gingivoperiosteoplasty utilizing soft
tissue fl aps from the cleft margin to close the fl oor (oral
mucosa) and the roof (nasal fl oor) of an oronasal fi stula. (From
Kelly P, Taylor T, Hollier L, Stal S. Alveolar bone grafting. In: McCarthy
JG, Galiano RD, Boutros SG, eds. Current Therapy in Plastic Surgery.
Philadelphia: Saunders Elsevier; 2006.)

SECONDARY DEFORMITIES OF THE CLEFT LIP, PALATE, AND NOSE • 151
A
B
C
FIGURE 14-13. Two-layer closure of a small palatal fi stula (A). First, elevation of the
fi stula margin and turnover closure of the nasal mucosa (B). Second, rotation of a large
oral mucosa fl ap (unipedicle) over the nasal closure (C).
C
B
A
FIGURE 14-14. Two-layer closure of a palatal fi stula (A). The nasal mucosa is closed
(B) and bipedicle oral mucosa fl aps are advanced over the defect (C).
B
C
A
D
Labial-alveolar
sulcus
FIGURE 14-12. (A) Oronasal cleft after cancellous bone grafting. (B, C) Cortical
bone fragments are utilized to strut the nasal and alveolar margin reducing tension on
the fl ap closure. (D) The fi nal closure of the oronasal defect. (From Kelly P, Taylor T, Hollier L,
Stal S. Alveolar bone grafting. In: McCarthy JG, Galiano RD, Boutros SG, eds. Current Therapy in
Plastic Surgery. Philadelphia: Saunders Elsevier; 2006.)

152 • CHAPTER 14
FIGURE 14-15. Regional fl aps for closure of recalcitrant
palatal fi stulae. The tongue fl ap described by Guerrerosantos
and Altamirano.24
A
B
C
FIGURE 14-16. (A–C) Closure of large palatal defect with the facial artery
myomucosal (FAMM) fl ap.
the last option, the radial forearm free fl ap has been used
to close resistant large defects. This thin fasciocutane-
ous fl ap provides composite tissue to reconstruct both
the oral and nasal linings. The fl ap is superior to other
thin facsiocutaneous fl ap due to its long pedicle that can
reach a variety of recipient vessels in the neck. The use
of acelluar dermal matrix has recently been advocated
for closure of palatal fi stulae. 28 , 29 The alloderm essentially
serves as a scaffold for the migration of granulation tissue
from the surrounding vascularized structures that even-
tually differentiates into dermis. Alloderm can provide a
layer of tissue in cases where reconstruction of only one
layer is possible ( Figure 14-17 ). Lastly, a prosthesis can
be fabricated to obturate the fi stula and should be con-
sidered when multiple attempts at closure have proven
to be futile. 8 The prosthesis can serve multiple functions,
including closure of the fi stula, camoufl age of the missing
teeth, and improved velopharygeal competence when a
posterior extension is included.
MAXILLARY GROWTH DISTURBANCE
As mentioned previously, the mandatory early repair of
the cleft palate has a negative effect on maxillary growth.
This exacerbates the abnormal development of the already
deformed maxilla. 30 The result is the development of a
hypoplastic maxilla that causes noticeable cosmetic defi -
ciencies and abnormal occlussal relationships. 30 The extent

SECONDARY DEFORMITIES OF THE CLEFT LIP, PALATE, AND NOSE • 153
of the maxillary hypoplasia is secondary to the timing of
the primary repair, the extent of the defect (with bilat-
eral defects being more injurious than unilateral defects),
and the amount of secondary scarring. The maxilla is also
narrow, causing crowding, crossbite, and apertognathia.
Occlussal abnormalities frequently require orthodontic
intervention. Maxillary expansion may also be required
to achieve adequate width to accommodate permanent
dentition.
In the majority of cases, the maxillary hypoplasia is local-
ized to the base of the nose on the cleft side. Orthodontic
correction of the occlussal abnormalities along with bone
grafting of the cleft will provide adequate contour to
the lip and base of the nose. 31 In wide cleft deformities,
the lateral maxillary arch may collapse and not be amena-
ble to orthodontic treatment. In these instances, a trans-
verse unilateral osteotomy through the maxillary sinus
is created on the cleft side. 31 This allows mobilization of
the collapsed arch segment inferiorly and buccally into
occlusion. Subsequent maxillary defects are bone grafted
and the reconstruction is immobilized for 6 weeks using
intermaxillary fi xation. An additional secondary effect of
maxillary hypoplasia is an apparent maxillary retrusion
and class II malocclusion. This is corrected by performing
a transverse maxillary osteotomy (LeFort I osteotomy).
The mobilized maxillary segment is then moved anterior
and inferior to correct the retrusion and vertical height
defi ciency. Again, bone grafting may be required to fi ll
any gap and to stabilize the malar-maxillary complex.
This technique is often needed to restore facial contour
and proper occlusion with the mandibular dentition.
Generally, maxillary osteotimies are performed in the late
teens after the completion of maxillary and mandibular
growth. 8
A
D
E
B
C
FIGURE 14-17. Three-layer closure of palatal fi stulae using acellular dermal matrix.
(A) Fistula. (B) Incisions for oral mucosa fl ap. (C) Elevation of bilateral peninsular fl aps
around the fi stula based on the greater palatine arteries. (D) Placement of the acelluar
matrix. (E) Final three-layer closure.

154 • CHAPTER 14
CLEFT NASAL DEFORMITY
The cleft nasal deformity is infl uenced greatly by the extent
of the cleft deformity and the result and technique used
for primary lip repair and palate repair. The lip and nose
share a common defi ciency in soft tissue and the abnormal
insertion of the orbicularis muscle pulls and widens the
nasal deformity. At the base of the cleft nasal deformity,
there is a hypoplastic nasal spine that opposes adequate
projection. There is also a negative effect caused by the
displacement of the weaker, lower lateral cartilage. The
lateral crus is displaced outward, further fl attening the tip-
defi ning point. The cleft nose is deviated with a resultant
tilting of the nasal bones and defl ection of the septum. The
vomer is crooked and often displaced off the vomerine
crest. If inadequately addressed at the time of lip repair,
the collumella is shortened secondary to a defi ciency of
skin and nasal lining. In the complete bilateral cleft, sup-
port for the nasal tip is defi cient on both sides, causing
retro-positioning of the entire tip complex and producing
a broad fl at bulbous tip with no projection.
Overall, the most apparent distortion of the cleft nose
is the obvious abnormalities of the lower lateral cartilages
( Figure 14-18 ). The fl attened, laterally displaced lower lat-
eral cartilages do not form the acute angle at the genu nec-
essary for proper tip defi nition, leading to inadequate tip
projection. Poor tip projection is secondary to the weak,
malformed lower lateral cartilage and the lowered angle of
septum ( Figure 14-19 ). Maintenance of projection of the
tip can only be accomplished with proper support of the
lower lateral cartilage.
Beginning in the neonatal period, nasoalveolar molding is
used to remodel the lower lateral cartilage. 1 , 32–34 During this
period the cartilage has a remarkable ability to be reshaped
due to the high levels of circulating estrogens. 35 , 36 Using a
nasal extension to the passive palatal presurgical devices and
appliance is placed to shape the nose in the fi rst months
of life. The appliance elevates the lower lateral cartilage
and stretches the nasal lining ( Figure 14-20 ). Nasoaveolar
molding is the fi rst step in a series of procedures in early
childhood that helps increase the prognosis of defi nitive rhi-
noplasty when the patient is of appropriate age. In the fi rst
year of life, the cleft lip-nose patient will have a number of
procedures to correct the lip deformity. It is advantageous
to perform additional molding techniques on the lower
lateral cartilage. At the time of lip repair, the lower lateral
cartilage is freed from the overlying skin; the lower lateral
cartilage is then suspended to the dermis in the desired posi-
tion ( Figure 14-21 ). These dermal suspension sutures serve
to defi ne the apex of the alae and reposition the skin and
mucosal envelope opening the nasal aperture.
While minor procedures may be performed in early
childhood, formal septorhinoplasty to correct the residual
sequellae of the cleft nasal deformity is best deferred until
growth of the nose is complete. This age is approximately
11 to 12 years in girls and 13 to 14 years in boys. 32 In early
adolescence, patients are likely to be mature enough to
consider septorhinoplasty. The goals of the procedure are
focused on correcting the nasal bones and septal deviation
and resorting tip support. However, before reshaping of the
tip is to occur, the alar base is addressed. The poorly pro-
jected tip is based on a hypoplastic maxilla. Consideration
for augmentation of the piriform should be made using
a bone graft, diced cartilage, or hydroxyapatite. The aug-
mentation is performed using an intraoral incision and the
material is secured in a subperiosteal location below the
cleft side of the alae. In patients, with maxillary retrusion
contributing not only to poor alar base support but also
Septum
Vomerian crest
Laterally displaced
lower lateral cartilage
FIGURE 14-18. Characterization of the cleft nasal deformity. Lateral and inferiorly
displacement of the lower lateral cartilage, septal deviation, and rotation of the vomerian
crest secondary to deforming forces of the cleft (down arrow).

SECONDARY DEFORMITIES OF THE CLEFT LIP, PALATE, AND NOSE • 155
FIGURE 14-19. Cleft nose deformity, illustrating poor tip projection and defi nition
secondary to the weak, fl attened, laterally displaced lower lateral cartilages and the
lowered angle of the septum.
A
B
FIGURE 14-20. (A, B) Nasoalveolar device applied in the neonatal period elevates
the lower lateral cartilage and stretches the nasal lining as the fi rst step in a series of
procedures in early childhood that helps increase the prognosis of defi nitive rhinoplasty
when the patient is of appropriate age.

156 • CHAPTER 14
to functional malocclusion, a LeFort I osteotomy with
advancement should ideally be performed before defi ni-
tive rhinoplasty. 8
Defi nitive rhinoplasty is best performed through an
open approach to carefully analyze and directly address
the lower lateral cartilages. The soft tissues are completely
released and the support structures are visualized. 37 The
septum is dissected and associated anomalies corrected.
At this point, septal cartilage can be harvested for later
use as either a columellar strut, an alar batten graft, or a
spreader graft. Generous attention is given to reshaping
the lower lateral cartilages. Symmetry to the lower lat-
eral cartilages is created using intradomal and interdomal
suture techniques ( Figure 14-22 ). 38 The tip may also
need additional support in the form of a collumelar strut,
which also serves as scaffold for equalizing the domes.
Tip projection may require additional augmentation with
a separate graft. To correct any deviation of the nasal
bones, osteotomy and repositioning can be performed. 37 , 38
Defi nitive septoplasty is performed to correct deviations
and alterations of the septum on the vomerian crest.
In the bilateral cleft lip nasal deformity, the collumella
is defi cient, the nostrils are obliquely oriented and the alar
base is widened. Correction requires advancement from
either the bilateral nasal sill or the lateral aspect of the
philtrum for lengthening ( Figure 14-23 ). The columella is
also wide with loss of the central waist. The collumella can
be reshaped by direct excision or narrowing with plica-
tion sutures placed between the medial crura. The bilat-
eral cleft lip deformity includes bilateral fl aring of the
lower lateral cartilages, causing abnormally elongated and
enlarged nostrils. The elongated appearance is secondary
to overall poor projection, and is repaired by recreating
the middle crura and supporting the tip with a columellar
strut. A widened alar base is corrected with caudal lateral
crus spanning sutures. 38
FIGURE 14-22. Suture techniques for the correction of tip and alar base deformities
in cleft rhinoplasty.
A
B
C
FIGURE 14-21. Lower lateral cartilage dermal suspension. (A) Vestibular infra
cartilaginous nasal incision. (B) Dissection and exposure of the lower lateral cartilage.
(C) Suture placement through the cartilage then skin and brought through the same site
and tied in the nasal vestibule.

SECONDARY DEFORMITIES OF THE CLEFT LIP, PALATE, AND NOSE • 157
CONCLUSION
Secondary deformities of the cleft lip, palate, and nose are
multiple. They have multiple etiologies, with inherent con-
genital deformation and subsequent primary repair being the
two most infl uential. Presurgical orthopedics can have a pro-
found benefi cial effect in preventing these deformities and
improving outcome of primary repair. Once a diagnosis is
established, multiple procedures may be required to correct
the deformity. In planning reconstruction, careful attention
should be placed on the hypolastic maxilla and the insertion
of the soft tissues into the margins of the cleft. The timing of
repair is dictated by requirements for function and growth.
REFERENCES
1. Stal S, Hollier LH. Secondary deformities of the cleft lip,
nose, and palate. In: Mathes SJ, ed. Plastic Surgery. 2nd ed.
Philadelphia: WB Saunders; 2005:339–363.
2. McCarthy JG, Cutting CB. Secondary deformities of cleft
lip and palate. In: Georgiade GS, Riefkohl R, Levin LS, eds.
Georgiade: Plastic, Maxillofacial, and Reconstructive Sur-
gery . 3rd ed. Baltimore, MD: Williams & Wilkins; 1997:
247–257.
3. Stal S, Hollier LH. Correction of secondary cleft lip deformi-
ties. Plast Reconstr Surg . 2002;109(5):1672–1681.
4. Mulliken JB, Wu JK, Padwa BL. Repair of bilateral cleft
lip: review, revisions, and refl ections. J Craniofac Surg.
2003;14(5):609–620.
5. Georgiade NG, Latham RA. Maxillary arch alignment in the
bilateral cleft lip and palate infant, using the pinned coaxial
screw appliance. Plast Reconstr Surg. 1975;56(1):52–60.
A
B
FIGURE 14-23. Collumelar lengthening techniques. (A) Inferiorly based fl ap using
the superior vestibular tissue and (B) superiorly based fl ap rotating the soft tissue of the
nasal sill.

158 • CHAPTER 14
6. Latham R, Qusy R, Georgiade N. An extraorally activated
expansion appliance for cleft palate infants. Cleft Palate J.
1976;13:252–261.
7. Grayson BH, Santiago PE, Brecht LE, et al. Long term
effects of nasoalveolar molding in infants with cleft lip and
palate. Cleft Palate Craniofac J . 1999;36:486–498.
8. Stal S, Spira M. Secondary reconstructive procedures
for patients with clefts. In: Sursin D, Georiade NG, eds.
Pediatric Plastic Surgery . St. Louis, MO: CV Mosby; 1984:
352–378.
9. Jackson IT, Fasching MC. Secondary deformities of cleft
lip, nose, and cleft palate. In: McCarthy JG, ed. Plastic and
Reconstructive Surgery . Vol. 4. Philadelphia: WB Saunders;
1990.
10. Cohen M. Secondary correction of the nasal deformity asso-
ciated with cleft lip. In: Cohen M, ed. Mastery of Plastic and
Reconstructive Surgery. New York: Little, Brown, and Com-
pany; 1994:702–719.
11. Coleman SR. Facial recontouring with lipostructure. Clin
Plast Surg. 1997;24:347–367.
12. Kapetansky DI. Double pendulum fl aps for whistling
deformities in bilateral cleft lips. Plast Reconstr Surg. 1971;
47:321–323.
13. Millard DR Jr. The Unilateral Deformity. Boston, MA: Little,
Brown & Co; 1976. Cleft Craft: the Evolution of its Surgery;
Vol 1.
14. Jackson IT, Soutar DS. The sandwich Abbe fl ap in secondary
cleft lip deformities. Plast Reconstr Surg. 1980;66:38–45.
15. Stal S, Klebuc M, Taylor T, et al. Algorithms for the treatment
of cleft lip and plate. Clin Plast Surg. 1998;25(4):493–507.
16. Abbe R. A new plastic operation for the relief of deformity
due to double harelip. Med Rec. 1898;53:477–478.
17. Oneal RM. Oronasal fi stulas. In: Grabb WC, Rosenstein SW,
Brock KR, eds. Cleft Lip and Palate . New York: Little, Brown,
and Company; 1971.
18. Schultz RC. Management and timing of cleft palate fi stula
repair. Plast Recontr Surg. 1986;78:739.
19. Schultz RC. Cleft palate fi stula repair. Improved results by
the addition of bone. J Craniomaxillofac Surg. 1989;17:34.
20. Randall P. Discussion of “Management and timing of cleft
palate fi stula repair” by RC Schultz. Plast Reconstr Surg.
1986;78:746.
21. Abyholm FE, Bergland O, Semb G. Secondary bone grafting
or alveolar clefts. Scand J Plast Reconstr Surg. 1981;15:127.
22. Jackson IT, Vandervord JG, McLennan JG, et al. Bone graft-
ing of the secondary cleft lip and palate deformity. Br J Plast
Surg. 1982;35:345.
23. El Deeb M, Messer LB, Lehnert MW, et al. Canine eruption
into grafted bone in maxillary alveolar cleft defects. Cleft
Palate J. 1982;19:9.
24. Guerrerosantos J, Altamirano JT. The use of lingual fl aps
in repair of fi stulas of the hard palate. Plast Reconstr Surg.
1966;38:123–128.
25. Ashtiani AK, Emami SA, Rasti M. Closure of complicated
palatal fi stula with facial artery musculomucosal fl ap. Plast
Reconstr Surg . 2005;116(2):381–386.
26. Nakakita N, Maeda K, Ando S, et al. Use of a buccal mus-
culomucosal fl ap to close palatal fi stulae after cleft palate
repair. Br J Plast Surg . 1990;43(4):452–456.
27. Joshi A, Rajendraprasad JS, Shetty K. Reconstruction of in-
traoral defects using facial artery musculomucosal fl ap. Br J
Plast Surg . 2005;58(8):1061–1066.
28. Steele MH, Seagle MB. Palatal fi stula repair using acellular
dermal matrix: the University of Florida experience. Ann
Plast Surg . 2006;56(1):50–53.
29. Clark JM, Saffold SH, Israel JM. Decellularized dermal graft-
ing in cleft palate repair. Arch Facial Plast Surg . 2003;5(1):
40–44.
30. Smahel Z, Mullerova Z. Effects of primary periosteoplasty
and facial growth in unilateral cleft lip and palate: 10 year
follow-up. Cleft Palate J. 1988;20:356.
31. Vargervik K. Growth characteristics of the premaxilla and
orthodontic treatment principles in bilateral cleft lip and
palate. Cleft Palate J. 1983;20(4):289–302.
32. Stal S, Hollier L. Correction of secondary deformities of the
cleft lip nose. Plast Reconstr Surg . 2002;109(4):1386–1392.
33. Tan S, Abramson D, MacDonald D, et al. Molding therapy
for infants with deformational auricular anomalies. Ann
Plast Surg. 1997;3:263–268.
34. Cutting C, Grayson B, Brecht L. Presurgical columella elon-
gation with one-stage repair of the bilateral cleft lip and
nose. Proceedings of the American Cleft Palate-Craniofacial
Association. 1995;52:58.
35. Brown F, Colen L, Addante R, et al. Correction of congenital
auricular deformities by splinting in the neoneonatal period.
Pediatrics. 1986;78:406–411.
36. Matsuo K, Hayashi R, Kiyono M, et al. Nonsurgical cor-
rection of congenital auricular deformities. Clin Plast Surg.
1993;17:333–395.
37. Stal S, Hollier LH. Correction of secondary deformi-
ties of the cleft lip nose. Plast Reconstr Surg. 2002;109(4):
1386–1392.
38. Van Beek AL, Hatfi eld AS, Schnepf E. Cleft rhinoplasty.
Plast Reconstr Surg. 2004;114:57e.

c h a p t e r 15
INTRODUCTION
Normal speech depends on the functional and structural
integrity of the velopharynx. This complex and dynamic
structure allows for uncoupling of the oral and nasal cavi-
ties during speech. Dysfunction of the velopharyngeal
valve (velopharyngeal dysfunction, or VPD) may result
in hypernasality, nasal air emission, and compensatory
articulation errors, all of which may impair speech intel-
ligibility and lead to social stigmatization. The goal of sur-
gical intervention is to restore velopharyngeal competence
while avoiding the complications of upper airway obstruc-
tion. Successful surgical management of VPD requires
careful preoperative assessment and individualization of
treatment. Optimization of surgical outcome is critically
dependent on a thorough analysis of each patient’s history,
structural anatomy, and velopharyngeal dynamics. This
analysis is best performed with close interdisciplinary col-
laboration of the surgeon, the speech pathologist, and the
other members of the cleft-craniofacial team.
The velopharyngeal port is composed of several striated
muscles that play a role in velopharyngeal valving. These
include the superior pharyngeal constrictor, the musculus
uvulae, and, most importantly, the levator veli palatini. If
these muscles are structurally abnormal, as in cleft palate,
or if their function is impaired, the oral and nasal cavities
may not be adequately uncoupled during sound produc-
tion. The cranial base likewise contributes to the overall
confi guration of the velopharyngeal port. If the cranial
base angle is abnormally obtuse, the resulting increase in
pharyngeal depth may lead to velopharyngeal dispropor-
tion and, consequently, to valvular dysfunction. The proce-
dures most often employed for the physical management
of VPD are the Furlow palatoplasty, the sphincter palato-
plasty, and the posterior pharyngeal fl ap (PPF). Each of
these techniques will be discussed below.
PATIENT EVALUATION AND SELECTION
Patient Evaluation
Patients with known or suspected VPD are best evalu-
ated by an interdisciplinary team of specialists. A com-
prehensive history, physical examination, and speech and
language evaluation, including appropriate instrumental
measures and imaging studies, are essential to the evalua-
tion of patients with VPD.
The elicited history should focus on patient/family con-
cerns with speech; pregnancy and birth history; primary
medical/genetic diagnoses (eg, cleft palate, syndromes, car-
diac defects, neuromuscular disease, etc); family history of
cleft lip/palate or VPD; feeding or swallowing diffi culties;
hearing loss or ear disease; snoring or symptoms of obstruc-
tive sleep apnea (OSA); surgical history, including prior cleft
palate repair, pharyngoplasty, tonsillectomy, or adenoidec-
tomy; hearing loss, developmental delays, or learning dis-
abilities; and history of speech therapy. A history of snoring
or other signs of upper airway obstruction should alert the
physician to obtain a preoperative polysomnogram. Each
patient should undergo direct craniofacial and intraoral
examination, focusing on craniofacial symmetry, oral/facial
movement and symmetry, dentition and occlusion, structural
integrity of the palate (eg, fi stulae or clefts), signs of submu-
cous cleft palate, including bifi d uvula, zona pellucida, and
notching of the posterior hard palate, velar length and eleva-
tion during phonation, and tonsil size and symmetry.
As noted above, the size of the tonsils and adenoids
should also be evaluated and documented. Rarely, enlarged
tonsils or an enlarged, irregular adenoid pad can cause dys-
function of the velopharyngeal valve. In such instances,
patients may benefi t from tonsillectomy or adenoidectomy
alone. Conversely, adenoidectomy may result in VPD in
predisposed patients, such as those with submucosal cleft
Velopharyngeal Insuffi ciency
Rachel A. Ruotolo, MD / Richard E. Kirschner, MD, FACS, FAAP

160 • CHAPTER 15
palate or velopharyngeal disproportion (as in patients with
22q11.2 deletion syndrome). In such patients, adenoidec-
tomy should be avoided. Patients with 22q11.2 deletion syn-
drome often have associated anomalies of the great vessels,
including medially deviated carotid arteries ( Figure 15-1 ),
and such patients should undergo preoperative MRI evalua-
tion for anatomic documentation and to guide surgical man-
agement. 1 – 4 If PPF surgery is performed in a patient with
medial carotid deviation, the base of the fl ap can be skewed
in order to avoid vascular injury. 5 , 6
It is important to obtain information about any prior
surgical procedures (eg, Furlow palatoplasty, adenoidec-
tomy, PPF) and elucidate the timing of any changes in the
patient’s speech. Each patient should be evaluated by a
speech pathologist familiar with cleft speech disorders.
Perceptual speech assessment remains the gold standard
in the diagnosis of VPD. The components of a standard
speech evaluation for the assessment of velopharyn-
geal closure for speech should include an assessment of
resonance, nasal emission, voice, and articulation. When
clinical speech evaluation suggests the presence of VPD,
instrumental assessment of speech and velopharyngeal
closure may be useful as an adjunct to perceptual judg-
ments. Instrumental measures can provide confi rmation
of perceptual judgments and further evidence of the need
for intervention, as well as allow for objective pre- and
post-treatment measurements. The most popular clinical
tools for indirect instrumental evaluation include acoustic
assessment of nasality and aerodynamic testing.
Imaging of the velopharynx is critical for making the
most appropriate treatment decision, as optimization
of surgical outcome is critically dependent on tailoring
FIGURE 15-1. Preoperative magnetic resonance
angiogram (MRA) showing medially deviated left carotid artery
in coronal section (A) and axial section (B).
A
B
the surgical procedure to each patient’s velopharyngeal
anatomy and function. In addition, imaging provides for
assessment of the adjacent structures of the upper air-
way, which could impact treatment planning, such as
the tonsils and adenoid pad. Objective assessment of the
velopharyngeal valve is conducted using multiview vid-
eofl uoroscopy ( Figure 15-2 ) and/or nasopharyngoscopy
( Figure 15-3 ). With either technique, the velopharyn-
geal closure pattern, contribution of lateral pharyngeal
wall motion, gap size, and site of attempted velar closure
on the posterior pharyngeal wall should be noted and
documented.
Procedure Selection
Following thorough assessment, the proper surgical pro-
cedure for management of VPD may be determined to be
one of three surgical procedures: Furlow double-oppos-
ing z-palatoplasty, PPF, or sphincter pharyngoplasty.
Leonard Furlow fi rst described the double-opposing
z-plasty technique for the treatment of cleft palate. 7 This
technique allows for anatomic reorientation of sagit-
tally oriented levator veli palatini fi bers, and at the same
time reduces the potential for velar shortening that can
occur with a longitudinal scar. A patient with a submu-
cous cleft and small gap VPD will benefi t from levator
repositioning and velar lengthening and thus represents
the ideal candidate for Furlow palatoplasty. In contrast,
patients with horizontally oriented levator muscles and
those who have had prior reconstruction of the levator
sling should not be considered candidates for the Furlow
technique.

VELOPHARYNGEAL INSUFFICIENCY • 161
The PPF serves as an obturator to nasopharyngeal air
escape. Patients with good lateral wall motion, or sagit-
tal velopharyngeal closure patterns, are ideally suited for
management using this technique. In contrast, patients
with coronal closure patterns may derive greater ben-
efi t from a sphincter pharyngoplasty. Previous surgical
intervention must be taken into account, however. For
A
B
FIGURE 15-2. Videofl uoroscopy during rest (A) and attempted closure, with arrows
showing velar bulge (B).
example, if the patient has had a previous tonsillectomy,
the posterior tonsillar pillars may be scarred, making a
sphincter pharyngoplasty an unfavorable option. Both
PPF surgery and sphincter pharyngoplasty carry some
risk of postoperative sleep apnea, thus any history of
snoring or airway obstruction warrants a preopera-
tive polysomnogram, and the decision to proceed with
A
B
FIGURE 15-3. Nasopharyngoscopy at rest (A) and with complete closure during
speech without evidence of velopharyngeal insuffi ciency (VPI) (B).

162 • CHAPTER 15
surgical management of VPD must be tempered by the
study results.
As noted above, surgical outcomes are critically depen-
dent on individualizing surgical technique. Proper patient
evaluation and selection includes both subjective and
objective measures, and every aspect of the preopera-
tive evaluation must be weighed in determining the best
modality of treatment for each patient.
PATIENT PREPARATION
Informed Consent
A detailed informed consent must be obtained from the
parents preoperatively. Risks of VPD surgery include
bleeding, dehiscence, OSA, hyponasal speech, and per-
sistent VPD/hypernasal speech. Patients with preopera-
tive symptoms of airway obstruction are at an increased
risk for OSA postoperatively and should be monitored
accordingly. Patients with 22q11.2 deletion syndrome and
a medially deviated internal carotid artery are at a theo-
retical increased risk for arterial injury when performing
either PPF surgery or sphincter pharyngoplasty, and par-
ents/patients should be made aware of this increased risk
preoperatively.
Anesthesia
General anesthesia is standard in surgical correction of VPD.
Pediatric anesthesiologists familiar with treating patients
with complicated airways should be available, especially
when patients have a history of preoperative airway obstruc-
tion, Pierre Robin sequence, or syndromic diagnoses.
Equipment and Positioning
The patient is positioned supine on the operating table
with the neck slightly extended. Exception to this should
be made in patients who have cervical spine abnormali-
ties that predispose to cervical instability. In such cases,
hyperextension of the neck should be avoided. A Dingman
mouth gag is placed to allow for a reliable, unobstructed
view of the palate and velopharynx. Once adequate visual-
ization of the operating fi eld is achieved, the operation can
proceed with effi ciency.
TECHNIQUE
Furlow Double-Opposing
Z-Palatoplasty (Figure 15-4)
This technique involves the creation of mirror image
z-plastys on the nasal and oral sides of the velum; the cen-
tral limb is placed along the palatal midline. On the oral
side, the posteriorly based triangular fl ap is composed of
muscle and mucosa. The deep plane of dissection is the
nasal submucosa. The tip of the fl ap is placed at the junc-
tion of the hard and soft palate, and its most posterior
extent is placed the hamulus. Once the oral myomucosal
fl ap is elevated, the anteriorly based nasal mucosa fl ap can
be created. This fl ap extends from the base of the uvula
medially toward the hamulus laterally. On the contralat-
eral side, the anteriorly based fl ap is an oral mucosal fl ap,
while the posteriorly based fl ap is a nasal myomucosal fl ap.
In essence, the oral and nasal z-plastys are mirror images of
one another. It is important to note that proper design of
the z-plastys is based on the palatal anatomy and not on a
predetermined geometric pattern. The palatal aponeurosis
must be freed completely from the posterior hard palate in
order for the posteriorly based fl aps to be properly rotated
and the levator fi bers to be properly retrodisplaced. The
palate is then closed in two layers: the nasal fl aps are trans-
posed and sutured, followed by the oral fl aps.
Sphincter Pharyngoplasty (Figure 15-5)
The most widely used technique today is a variation of the
technique fi rst described by Hynes. 8 – 10 The posterior ton-
sillar pillars, made up of the palatopharyngeus muscles and
overlying mucosa, are dissected and elevated as superiorly
based fl aps. These fl aps are rotated 90 degrees and inset
into the posterior pharynx. The level of fl ap inset should
be at the level of attempted velopharyngeal closure, as
determined by preoperative imaging. Of note, the supe-
rior-most extent of fl ap inset may be limited by the inferior
portion of the adenoid pad. If the level of attempted velar
closure is determined to be above this level, preoperative
adenoidectomy is advised, thereby allowing for inset of the
pharyngoplasty at the proper level of the posterior pharyn-
geal wall. Once both fl aps are inset, their respective donor
sites are closed.
Posterior Pharyngeal Flap (Figure 15-6)
PPF width (and, conversely, lateral port size) is determined
preoperatively, based on velopharyngeal imaging studies.
Flap width should be tailored to gap size and to the extent
of lateral pharyngeal wall motion. The base of the fl ap
should be designed at the level of attempted velar con-
tact with the posterior pharyngeal wall. This fl ap may be
superiorly or inferiorly based, although the former is the
technique most often utilized. Flap length is determined
as that necessary for proper inset, depending on the spe-
cifi c technique used. The posterior pharyngeal mucosa and
underlying superior pharyngeal constrictor are divided to
the prevertebral fascia. The fl ap is then elevated to its base
in this avascular plane. The distal end of the fl ap is sutured
into the soft palate, which may be split in a sagittal or in a
horizontal (“fi sh mouth”) orientation. In general, a wider,
shorter fl ap has less of a tendency to tube on itself, resulting
in less narrowing of the fl ap postoperatively. The undersur-
face of narrower fl aps, in contrast, should be lined by nasal
mucosal fl aps based on the posterior velum in order to
prevent such tubing. The donor site in the posterior phar-
ynx may be closed primarily or left open to remucosalize
secondarily.

VELOPHARYNGEAL INSUFFICIENCY • 163
COMPLICATIONS
In general, complications are infrequent following any
of the above surgical techniques and include bleeding,
dehiscence, oronasal fi stula, nasal airway obstruction,
and sleep apnea. The incidence of each of these com-
plications varies signifi cantly depending upon the tech-
nique used.
Furlow Palatoplasty
Postoperative complications specifi c to the double-
opposing z-plasty technique include bleeding, oronasal
fi stula, and nasal airway obstruction. Bleeding can often
be avoided simply by meticulous attention to hemostasis.
Although some have reported a relatively high incidence
of oronasal fi stula formation following use of the Furlow
technique for repair of cleft palate, the incidence of fi stula
formation following its use for management of VPD has
not been reported but can be presumed to be quite low.
Risk factors for fi stula formation include previous cleft
surgery, comorbid conditions, and, most importantly, ten-
sion on the repair. Finally, although airway obstruction can
occur following Furlow palatoplasty, it is less frequent and
usually transient, when compared to PPF surgery. 11 , 12 It is
important to monitor the respiratory status of all patients
postoperatively, particularly those with a prior history of
upper airway obstruction.
Sphincter Pharyngoplasty
Bleeding, fl ap dehiscence, nasal airway obstruction, and
OSA have all been reported following sphincter pharyngo-
plasty. In cases where VPD was noted to persist postopera-
tively, partial or total fl ap dehiscence was most often cited
as the etiologic factor. 13 Sphincter pharyngoplasty has often
been as “dynamic” or “physiologic.” Whereas dynamism of
Oral mucosal flap
Nasal mucosal flap
Oral
musculo-mucosal flap
Nasal
musculo-mucosal flap
Nasal flaps rotated
Oral flaps rotated
FIGURE 15-4. Furlow double-opposing z-palatoplasty. (From Kirschner RE. Palatal
anomalies in 22q11.2 deletion syndrome. In: Murphy K, Scambler P, eds. Velo-Cardio-Facial Syndrome:
Understanding Microdeletion Disorders . Cambridge; New York: Cambridge University Press; 2005.)

164 • CHAPTER 15
sphincter pharyngoplasty is not a constant fi nding, reduc-
tion in velopharyngeal port size is assured. 14 , 15 Nasal air-
way obstruction, which may present as hyponasal speech,
nasal airway congestion, or OSA are potential unwanted
outcomes, although some have reported that nasal airway
obstruction may be less frequent after sphincter pharyn-
goplasty than after PPF surgery. Patients most at risk are
those with comorbid conditions such as tonsillar hyperpla-
sia or a history of Pierre Robin sequence or OSA. When
airway obstruction does occur, however, it is most often
transient. Persistent upper airway obstruction may often
be successfully treated using continuous positive airway
pressure (CPAP) at night. 16 , 17
Posterior Pharyngeal Flap
The most commonly reported complications of PPF
surgery include bleeding, fl ap dehiscence, and airway
obstruction. Rare cases of death have been reported. 18 , 19
Bleeding often occurs during the fi rst 24 to 48 hours
at the open donor site in the posterior pharynx. This
risk can be reduced with careful hemostasis, use of
electrocautery in fl ap elevation, and primary closure of
the donor site. Many patients show signs upper airway
obstruction in the immediate postoperative period, but
this often resolves in several days to weeks as edema
resolves. Patients at greatest risk for airway complications
include those with a history of Pierre Robin sequence,
hypotonia (22q11.2 deletion syndrome), and enlarged
tonsils. Preoperative or concomitant tonsillectomy is
recommended in patients with signifi cant tonsillar
hyperplasia. All patients should be monitored with con-
tinuous pulse oximetry overnight. Patients with wide
fl aps should be considered for placement of a temporary
nasopharyngeal airway and observation in an intensive
care unit.
Palatopharyngeus flaps
Flaps interdigitated
Posterior pharynx
Posterior adenoid pad
Palatoglossal arch
Soft palate
Palatopharyngeal arch
FIGURE 15-5. Sphincter pharyngoplasty. (From Kirschner RE. Palatal anomalies
in 22q11.2 deletion syndrome. In: Murphy K, Scambler P, eds. Velo-Cardio-Facial Syndrome:
Understanding Microdeletion Disorders . Cambridge; New York: Cambridge University Press; 2005.)

VELOPHARYNGEAL INSUFFICIENCY • 165
OUTCOMES ASSESSMENT
Successful outcome of surgical management of VPD is
measured by the effective elimination of hypernasality
and nasal air emission while avoiding overcorrection that
may lead to hyponasality or sleep apnea. Postoperatively,
each patient should undergo formal speech evaluation at
6 months, at 12 months, and then at yearly intervals. In
addition, careful monitoring of the upper airway is essen-
tial. Patients who demonstrate signs or symptoms of OSA
that persist beyond the immediate postoperative period
should undergo polysomnographic evaluation. Patients
with signifi cant OSA that persists postoperatively may
require CPAP therapy or, in some cases, surgical revi-
sion. Obstructing PPFs may be narrowed or converted to
sphincter pharyngoplasty, based on the results of studies
that document a lower incidence of OSA following the
latter procedure. 20
Although hypernasality is successfully reduced or elim-
inated in the majority of patients who undergo surgery,
approximately 10% to 20% of patients will demonstrate
persistent hypernasality requiring revisional surgery. 13 All
such patient should undergo the same comprehensive
evaluation that was performed before the primary proce-
dure. Persistent VPD may be due to fi stula, fl ap dehiscence,
scarring and tethering of the fl ap, poor selection of primary
technique, or improperly low placement of the fl ap or
sphincter on the posterior pharyngeal wall. Patients should
undergo objective assessment via diagnostic imaging using
either nasendoscopy or videofl uoroscopy. Revisional sur-
gery for the management of persistent VPD should only
be performed in patients with a stable airway and no signs
of OSA.
Patients with persistent VPD after Furlow palatoplasty
may be managed by PPF or sphincter pharyngoplasty,
the selection of which should be based on the fi ndings of
postoperative imaging studies. In some patients, persistent
VPD after PPF may be found to be secondary to narrow-
ing and/or inferior migration of the fl ap. If the fl ap is noted
to be too narrow, the fl ap may be augmented or divided
and a new, wider fl ap created. If the fl ap is noted to be
Suture in uvula
Transverse incision
in soft palate
Posterior
pharyngeal
flap
FIGURE 15-6. Posterior pharyngeal fl ap. (From Kirschner RE. Palatal anomalies
in 22q11.2 deletion syndrome. In: Murphy K, Scambler P, eds. Velo-Cardio-Facial Syndrome:
Understanding Microdeletion Disorders . Cambridge; New York: Cambridge University Press; 2005.)

166 • CHAPTER 15
tethered inferiorly, it can be advanced using a V-Y tech-
nique, divided and replaced by a new fl ap, or taken down
and replaced by a sphincter pharyngoplasty. Persistent VPD
following sphincter pharyngoplasty is most often managed
by re-elevating the fl aps and tightening the central port.
Close monitoring of speech and airway stability is critically
important following secondary surgery. The risk of OSA
has not been found to be greater after secondary speech
surgery. 13 , 21
It is essential to base the operative plan on the preop-
erative assessment of velopharyngeal motion and closure
patterns, both for optimizing speech outcomes and limit-
ing the risk of complications. This is equally important in
the setting of primary and revisional surgery. Because the
nature of VPD varies from patient to patient, reliably suc-
cessful outcomes depend on careful execution of the surgi-
cal technique as well as diagnostic precision and selection
of technique.
REFERENCES
1. D’Antonio LL, Marsh JL. Abnormal carotid arteries in the
velocardiofacial syndrome. Plast Reconstr Surg . 1987;80:471.
2. MacKenzie-Stepner K, Witzel MA, Stringer DA, et al.
Abnormal carotid arteries in the velocardiofacial syndrome:
a report of three cases. Plast Reconstr Surg . 1987;80:347.
3. Mitnick RJ, Bello JA, Golding-Kushner KJ, et al. The use of
magnetic resonance angiography prior to pharyngeal fl ap
surgery in patients with velocardiofacial syndrome. Plast Re-
constr Surg . 1996;97:908.
4. Ross DA, Witzel MA, Armstrong DC, et al. Is pharyngo-
plasty a risk in velocardiofacial syndrome? An assessment
of medially displaced carotid arteries. Plast Reconstr Surg .
1996;98:1182.
5. Tatum SA 3rd, Chang J, Havkin N, et al. Pharyngeal fl ap and
the internal carotid in velocardiofacial syndrome. Arch Facial
Plast Surg . 2002;4:73.
6. Witt PD, Miller DC, Marsh JL, et al. Limited value of preop-
erative cervical vascular imaging in patients with velocardio-
facial syndrome. Plast Reconstr Surg . 1998;101:1184.
7. Furlow LT Jr. Cleft palate repair: preliminary report on
lengthening and muscle transposition by z-plasty. South-
eastern Society of Plastic and Reconstructive Surgery. Boca
Raton, FL; 1978.
8. Hynes W. Pharyngoplasty by muscle transplantation. Br J
Plast Surg . 1950;3:128.
9. Hynes W. The results of pharyngoplasty by muscle trans-
plantation in failed “cleft palate” cases, with special reference
to the infl uence of the pharynx on voice production. Ann R
Coll Surg Engl . 1953;13:17.
10. Hynes W. Observations on pharyngoplasty. Br J Plast Surg .
1967;20:244.
11. Liao YF, Noordhoff MS, Huang CS, et al. Comparison of
obstructive sleep apnea syndrome in children with cleft
palate following Furlow palatoplasty or pharyngeal fl ap
for velopharyngeal insuffi ciency. Cleft Palate Craniofac J .
2004;41:152.
12. Liao YF, Yun C, Huang CS, et al. Longitudinal follow-up of
obstructive sleep apnea following Furlow palatoplasty in
children with cleft palate: a preliminary report. Cleft Palate
Craniofac J. 2003;40:269.
13. Witt PD, Myckatyn, T, Marsh JL. Salvaging the failed phar-
yngoplasty: intervention outcome. Cleft Palate Craniofac J .
1998;35:447.
14. Witt PD, Marsh JL, Arlis H, et al. Quantifi cation of dynamic
velopharyngeal port excursion following sphincter pharyn-
goplasty. Plast Reconstr Surg . 1998;101:1205.
15. Kawamoto HK Jr. Pharyngoplasty revisited and revised. In:
Jurkiewicz MJ, Culbertson JH Jr, eds. Operative Techniques
in Plastic and Reconstructive Surgery . Philadelphia: W.B.
Saunders; 1995:239.
16. Witt PD, Marsh JL, Muntz HR, et al. Acute obstructive sleep
apnea as a complication of sphincter pharyngoplasty. Cleft
Palate Craniofac J . 1996;33:183.
17. Saint Raymond C, Bettega G, Deschaux C, et al. Sphincter
pharyngoplasty as a treatment of velopharyngeal incompe-
tence in young people: a prospective evaluation of effects
on sleep structure and sleep respiratory disturbances. Chest .
2004;125:864.
18. Hofer SO, Dhar BK, Robinson PH, et al. A 10-year review of
perioperative complications in pharyngeal fl ap surgery. Plast
Reconstr Surg . 2002;110:1393.
19. Valnicek SM, Zuker RM, Halpern LM, et al. Perioperative
complications of superior pharyngeal fl ap surgery in chil-
dren. Plast Reconstr Sur g. 1994;93:954.
20. Pensler JM, Reich DS. A comparison of speech results after
the pharyngeal fl ap and the dynamic sphincteroplasty proce-
dures. Ann Plast Surg . 1991;26:441.
21. Barone CM, Shprintzen RJ, Strauch B, et al. Pharyngeal fl ap
revisions: fl ap elevation from a scarred posterior pharynx.
Plast Reconstr Surg . 1994;93:279.

16
c h a p t e r
Craniosynostosis
Mitchel Seruya, MD / Stephen B. Baker, MD, DDS, FACS / Jeffrey Weinzweig, MD, FACS
PATIENT EVALUATION AND SELECTION
Craniosynostosis is defi ned as the premature closure of
a cranial suture which causes abnormal calvarial growth.
Virchow’s law states that skull growth is arrested in the
direction perpendicular to the fused suture and expanded
at the sites of unaffected sutures, leading to characteris-
tic calvarial deformations. 1 This condition can be classi-
fi ed into simple (single suture) versus complex (multiple
sutures) and nonsyndromic versus syndromic subtypes
( Tables 16-1 and 16-2 ).
Preoperative assessment for craniosynostosis includes a
detailed medical history, physical examination, and radio-
graphic imaging. Medical history should elicit skull irregu-
larities, associated syndromes, family history of calvarial
deformities, and symptoms of intracranial hypertension
(headache/vomiting, developmental changes, irritability,
and oculomotor paresis). Physical examination should
evaluate for characteristic calvarial shapes and asymme-
tries, premature closure of the anterior fontanelle (nor-
mally open until 8–12 months of age), perisutural ridging,
and signs of intracranial hypertension (papilledema,
supraorbital retrusion, severe towering [turricephaly], and
severe frontal/occipital bossing). Head circumferences,
cranial indices, and anthropometric measurements should
be documented.
Radiological investigation may be necessary to cor-
roborate the diagnosis and/or rule out any associated
intracranial abnormalities. Computed tomography (CT)
remains the most sensitive barometer of bony fusion and
may provide evidence for elevated intracranial pressure,
as noted by erosion of the inner calvarial table (“copper-
beaten” appearance also referred to as “thumb printing”)
( Figure 16-1 ). CT imaging and MRI are also helpful in
evaluating the underlying brain for structural or functional
abnormalities, including hydrocephalus, holoprosenceph-
aly, cortical dysplasias, and Chiari malformations.
Surgical intervention is indicated in craniosynostosis
both for the correction of calvarial contour deformities
and the prevention of psychosocial dysfunction, intrac-
ranial hypertension, and/or mental retardation. 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
approximately 47% in multisuture synostosis. 2 – 3 Sutural
release in simple craniosynostosis has been advised due to
the concerns regarding increased intracranial pressure as
well as the mild but signifi cant developmental delay in the
aging child. Patients with complex synostoses present with
increased severity of physical and neurological symptoms;
therefore, surgical intervention is even more imperative.
PATIENT PREPARATION
Preoperative considerations include the optimal type and
timing of surgical correction for craniosynostosis. A broad
range of surgical options exist in the armamentarium of
contemporary craniofacial surgical reconstruction, all with
the primary objective of releasing the affected suture(s)
to permit normalization of skull growth in the setting
of accelerated cerebral growth. An open craniofacial
approach remains the mainstay of therapy, relying on wide
scalp dissection, extensive calvarial osteotomies, and skull
reconfi guration that is individually tailored to each cranial
vault deformity. 4 To address concerns regarding incision
length, operative blood loss, and length of stay for open
craniofacial procedures, minimally invasive techniques
have been proposed. These techniques include endoscopic
sutural release, 5 spring-assisted cranioplasty, 6 and distrac-
tion osteogenesis. 7
The optimal surgical age has been a source of contention,
given its differential effects on intraoperative hemodynam-
ics, postoperative cranial growth, and subsequent mental

168 • CHAPTER 16
development. While the literature is inconclusive regard-
ing the appropriate timing for correction of craniosynosto-
sis, the majority of craniofacial surgeons operate between
3 and 12 months of age. Surgical age is also dependent on
the type of surgical approach employed. Minimally inva-
sive techniques, which rely on dynamic cranial vault altera-
tion during rapid calvarial growth, are generally performed
at an earlier age than open surgical correction.
Given the senior authors’ extensive experience with
open craniofacial reconstruction for craniosynostosis, surgi-
cal considerations, operative steps, and outcomes shall be
described for this type of surgical approach in the accom-
panying sections. In preparation for open craniosynostosis
surgery, measures for ensuring adequate blood resuscita-
tion should be undertaken. These include obtaining a blood
type and cross, autologous blood donation, and/or allo-
genic blood direct donation from a family member. Parents
should be instructed that open craniosynostosis surgery is
performed under general anesthesia. For most types of open
craniosynostosis surgeries, patients are placed in the supine
position to facilitate calvarial exposure. However, if the pos-
terior vault is being addressed, as in a lambdoidal synostosis
or some sagittal synostoses, the patient is ideally placed in
the prone position. In the prone position, care must be taken
to ensure adequate protection of the globes, facilitated by
bilateral tarsorrhaphies and periorbital cushioning.
A plating system is used by most surgeons to main-
tain the proper position of the osteotomized bone seg-
ments after they have been placed in the desired position.
Historically, titanium was used but fell out of favor when
it was noted that the plates translocated with continued
TABLE 16-1. Classifi cation of
Craniosynostosis
Affected Suture
Phenotypic Presentation
Sagittal
Coronal (unilateral)
Coronal (bilateral)
Metopic
Lambdoid
Multiple sutures
Dolichocephaly, scaphocephaly
Anterior plagiocephaly
Brachycephaly
Trigonocephaly
Posterior plagiocephaly
Cloverleaf (Kleeblatschädel),
acrocephaly, oxycephaly
TABLE 16-2. Craniofacial Dysostosis Syndromes
Syndrome
Involved Suture
Morphological Presentation
Crouzon
Coronal, sagittal
Midface hypoplasia, shallow orbits, proptosis, hypertelorism
Apert
Coronal, sagittal, lambdoid
Midface hypoplasia, shallow orbits, proptosis, hypertelorism, symmetrical
syndactyly of hands and feet , choanal atresia, ventriculomegaly,
genitourinary/cardiovascular anomalies
Pfeiffer
Coronal, sagittal
Midface hypoplasia, proptosis, hypertelorism, broad great toe/thumb
calvarial growth. Resorbable plates eliminate the prob-
lems associated with translocation. They maintain strength
across the osteotomy long enough for the bone to heal but
are resorbed by the body after several years. Most surgeons
today employ some type of resorbable fi xation for cranial
vault remodeling.
TECHNIQUE
Metopic Synostosis (Trigonocephaly)
Metopic synostosis is marked by a variable degree of phe-
notypic severity, ranging from mild ridging to the formation
of a triangular shaped 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 premature
suture fusion. Weinzweig termed this radiographic fi nding
FIGURE 16-1. CT fi ndings of elevated intracranial
pressure. This sagittal CT image demonstrates the classic
“copper-beaten” appearance, also referred to as “thumb
printing,” resulting from increased intracranial pressure.

CRANIOSYNOSTOSIS • 169
the metopic notch , a morphologic abnormality that is seen in
93% of synostotic patients ( Figure 16-2 ). 8 A metopic notch
is not seen in any nonsynostotic patients and, therefore, can
be used to diagnose metopic synostosis even after the period
physiologic suture closure. This notch represents the ana-
tomic site of attachment of the falx, a dural refl ection off
the crista galli with basicranial origins, and suggests a role
for the cranial base in metopic suture fusion.
Whereas a metopic notch describes the endocranial
fi nding on axial CT images in patients with metopic synos-
tosis, a corresponding three-dimensional groove is found
on the endocranial surface of the actual skull that extends
from the nasion to the anterior fontanelle in these patients.
Weinzweig termed this clinical fi nding the metopic groove ,
an anatomic abnormality that can reliably be found in
patients with metopic synostosis ( Figure 16-3 ).
FIGURE 16-2. The metopic notch. Axial CT images demonstrate the endocranial
bony spur associated with normal metopic suture fusion (A), and the V (omega)-shaped
metopic notch (B). Moderate ectocranial ridging is also appreciated in this patient.
A
B
FIGURE 16-3. The metopic groove. Intraoperative evaluation of the inner table
of the anterior cranial vault of a previously untreated 9-year-old child with metopic
and bicoronal synostosis and Crouzon syndrome demonstrates a metopic groove.
While Crouzon syndrome is commonly associated with bicoronal synostosis, this child
demonstrated both a metopic notch and metopic groove, pathognomonic fi ndings
consistent with metopic synostosis. The metopic groove typically extends from the
nasion to the anterior fontanelle. Note the severe depressions of the inner table of the
skull indicating increased intracranial pressure in this older child.

170 • CHAPTER 16
In general, the goals of surgery are the normalization
of the forehead with reconstitution of a normal supraor-
bital rim when necessary. Individuals presenting solely
with a prominent midline keel may be best served by sim-
ple contouring of the frontal bone or by removal of the
frontal bone fl ap followed by reconfi guration. Conversely,
patients with signifi cant trigonocephaly and hypotelorism
will require a fronto-orbital reconstruction, recontouring
the frontal bone, and laterally expanding the orbits, often
with cranial bone graft placed in the midline of the frontal
bandeau at the level of the nasion.
The essentials of fronto-orbital reconstruction involve
a standard “stealth” (zig-zag) coronal incision, providing
for adequate exposure of the fronto-orbital region while
minimizing any postoperative scar. Perioperative antibiot-
ics and steroids are given prior to the start of surgery. The
incision is infi ltrated with 0.25% lidocaine and 1:400,000
parts epinephrine to minimize intraoperative bleeding.
The frontal and temporal regions are dissected in the sub-
galeal plane and care is taken to preserve the periosteum
on the surface of the bone, which helps minimize blood
loss and may be used to stabilize osteotomized bony seg-
ments. The dissection is taken down to the level of the
periorbital tissues, with caution taken to avoid any injury
to the underlying globes. Following exposure of the
frontal and orbital regions, the frontal bone is removed,
providing access to the intracranial compartment. The
supraorbital rim is then removed in one piece to facili-
tate reconstruction of the previously triangular-shaped
supraorbital bar. Care is taken to remove suffi cient bone
in the region of the sphenoid bone to allow for growth
at the midface and orbits. If the orbits require correc-
tion of hypotelorism, it will be necessary to displace the
lateral walls of the orbit as well as to split the midline
and interpose a calvarial bone graft. Reconfi guration of
the supraorbital bar often requires a midline osteotomy
to facilitate a fl attened forehead with additional partial-
thickness bone cuts at the lateral (pterional) angle to
promote normalization of the lateral supraorbital angle.
The supraorbital reconfi guration is maintained by the uti-
lization of intervening bone grafts as well as resorbable
hardware. Following placement of the supraorbital bar as
a foundation, the frontal bone is reconstructed using the
remaining portions of bone. It is often possible to reverse
the original frontal bone fl ap (posterior portion is now
in an anterior position) to obtain an adequate width and
contour with the new frontal bone fl ap. It is important to
provide adequate enhancement at the pterional region to
avoid long-term supratemporal hollowing or recession.
Sagittal Synostosis (Scaphocephaly,
Dolichocephaly)
Children with sagittal synostosis will present with a narrow,
elongated skull ( dolichocephaly , long headedness; scaphoceph-
aly , boat shaped). Depending on the region of greatest pre-
mature fusion of the sagittal suture, the child may manifest
frontal or occipital bossing, or a combination of both.
The treatment for sagittal synostosis remains contro-
versial, with surgical approaches ranging from minimal
removal of the involved suture and bone to extensive total
calvarectomy and reconfi guration. Simple synostectomy or
simple strip craniectomy is safe and well tolerated, provid-
ing adequate cosmetic results in select patients with mild
deformities. This procedure, however, has several disadvan-
tages stemming from the fact that it strictly addresses the
fused suture and not the compensatory changes in skull
shape. It also leaves a large unprotected area over the ver-
tex of the skull, an area with a high rate of restenosis and
renewed growth restriction.
An extended synostectomy provides immediate resto-
ration of normal skull contour by shortening the antero-
posterior dimension, expanding the biparietal dimension,
and addressing the frontal and occipital prominences.
Amongst extended synostectomy techniques, the pi pro-
cedure has become a widely utilized approach for older
infants (3–12 months of age) with scaphocephaly. The
essential steps involve removal of bone along both sides of
the sagittal suture as well as over the coronal suture, in the
design of the Greek letter pi (P). Cranial bone overlying
the sagittal sinus is left intact to minimize bleeding. Drill
holes are placed in the adjacent osteotomized bone fl aps,
allowing for placement of an absorbable suture that helps
narrow the anteroposterior dimension while expanding
the biparietal length.
Staged or partial vault remodeling procedures also work
well in indicated patients. In some cases, the majority of the
deformity occurs anteriorly or posteriorly. In these cases,
vault remodeling may be limited to the most involved area
(posterior vs anterior) of the cranium. In properly selected
cases, this approach is less morbid and produces excellent
results.
To address older infants beyond the period of maximal
cerebral growth (.12–18 months of age), as well as those
children with signifi cant frontal or occipital prominence,
procedures with more aggressive craniectomies and recon-
struction must be undertaken. 9 Total calvarectomy and
reconstruction in the setting of severe or late presentation
scaphocephaly has been proposed to offer superior cos-
metic results with a minimal increase in morbidity. This
may be accomplished by removal of the frontal, occipital,
and both parietal bones. Subsequently, reconfi guration is
carried out and aimed at providing a shortened antero-
posterior dimension in addition to a widened biparietal
diameter. The use of rigid fi xation is indispensable in these
cases, providing greater three-dimensional conformational
stability and decreased intraoperative time, bleeding, and
postoperative infection.
Coronal Synostosis (Anterior
Plagiocephaly/Brachycephaly)
Patients with unicoronal synostosis present with anterior,
or frontal, plagiocephaly whereas those with bilateral cor-
onal involvement demonstrate brachycephaly. Phenotypic
features of anterior plagiocephaly include ipsilateral

CRANIOSYNOSTOSIS • 171
perisutural ridging, forehead fl attening, and orbital reces-
sion, coupled with contralateral compensatory frontal
bossing. Facial deformities are also common, including
nasal root displacement toward the ipsilateral side, ante-
rior displacement of the ipsilateral ear, increased orbital
aperture, and chin deviation toward the contralateral side.
Pathognomonic radiographic fi ndings diagnostic of uni-
coronal synostosis include elevation of the ipsilateral orbit
which is seen secondary to superior displacement of the
greater wing of the sphenoid, also known as the “harle-
quin” deformity ( Figure 16-4 A–C). Phenotypic features of
brachycephaly include forehead retrusion and fl attening,
frontal towering, and biparietal widening. The nasal dor-
sum can be low and hypertelorism may be present.
Surgical intervention in unicoronal and bicoronal synos-
toses aims to correct both the frontal and orbital asymme-
tries. With the current understanding that unilateral coronal
synostosis presents with bilateral dysmorphic changes,
bilateral correction is now believed to be the optimum
approach. For anterior plagiocephaly, a bilateral fronto-
orbital advancement procedure is employed for expansion
of the affected forehead and orbit with concomitant reces-
sion of the contralateral orbit ( Figure 16-4 D–I). Excellent
symmetry can be achieved in this manner ( Figure 16-4 J,K).
Bilateral fronto-orbital advancement also serves in the cor-
rection of bicoronal synostosis.
The bilateral fronto-orbital advancement reconstruc-
tion (FOAR) involves the release of both coronal sutures
while providing bilateral frontal and orbital correction
( Figure 16-5 ). A standard coronal skin incision is made. A
bifrontal bone fl ap is removed typically in one piece, leav-
ing a 1–2 cm wide supraorbital bandeau. Extending the
osteotomy posterior to the coronal suture will often pro-
vide adequate width and a satisfactory new frontal recon-
struction when this bone fl ap segment is inverted. The
sphenoid wing is osteotomized and the coronal sutures are
FIGURE 16-4. Bilateral fronto-orbital reconstruction
for unicoronal synostosis. Three-dimensional CT scans
demonstrate right coronal synostosis and a harlequin
deformity (A, B), and a normal, patent contralateral coronal
suture (C).
A
B
C

172 • CHAPTER 16
FIGURE 16-4. (continued) Intraoperative exposure
demonstrates right coronal synostosis, ipsilateral frontal
fl attening, and contralateral frontal bossing (D–F).
D
E
F

CRANIOSYNOSTOSIS • 173
opened to the level of the skull base, serving to prevent
continued growth restriction and resultant postoperative
hollowing of the pterional regions. Caution must be exer-
cised when performing the osteotomy across the sphenoid
wing on the affected side, as its superior displacement may
cause technical diffi culty upon frontal bone fl ap removal
and lead to dural laceration if caution is not exercised.
After removal of the frontal bone fl ap, the orbital bandeau
is freed with osteotomies performed across the lateral
orbit at the frontozygomatic suture, the orbital roof, and
the nasion just above the nasofrontal suture. Care is taken
to protect the underlying brain as well as orbital contents
with judicious placement of retractors. The bandeau is
then reconfi gured using partial osteotomies at the midline
while using absorbable hardware on the interior surface
to maintain the newly contoured shape. The newly con-
fi gured supraorbital bar is replaced, its lateral aspects and
midline fi xed to the calvarial foundation with resorbable
fi xation for improved healing and postoperative mainte-
nance of the surgical construct. It is frequently necessary
to overcorrect the expansion of the affected side by 5%
to 10% while also providing a convex shape at the lateral
border for a satisfactory reconstruction. Often, the unaf-
fected orbit is mildly set back to correct for preoperative
compensatory overgrowth. The frontal bone fl ap is then
attached to the supraorbital bar, taking care to match it
to the previously overcorrected (5%–10%) orbital bandeau
on the affected side. Remaining portions of bone are fi xed
FIGURE 16-4. (continued) Bilateral fronto-orbital
advancement is performed for expansion of the affected
frontal bone and orbit with concomitant recession of the
contralateral orbit. Fixation is performed using resorbable
plates and screws (G–I).
G
H
I

174 • CHAPTER 16
with absorbable plates or suture, and closure is performed
in a routine fashion with placement of subgaleal drains.
Lambdoid Synostosis and Posterior
Deformational Plagiocephaly
Posterior plagiocephaly due to lambdoid suture synosto-
sis is rare, with the majority of observed posterior plagio-
cephaly secondary to positional molding. Understanding
the phenotypic differences between lambdoid synosto-
sis and posterior deformational plagiocephaly is critical
toward making the appropriate diagnosis and designing
the proper course of treatment. Children with lambdoid
synostosis characteristically have a trapezoid-shaped head
in association with posterior displacement of the ipsilat-
eral ear, contralateral occipital bossing, and frequent ridg-
ing of the affected lambdoid suture. In contrast, posterior
deformational plagiocephaly is marked by a parallelogram-
shaped head, anterior displacement of the ipsilateral ear,
and ipsilateral frontal bossing in the absence of palpable
ridging along the lambdoid sutures.
Infants with true lambdoid synostosis may benefi t from
a variety of surgical approaches, aiming to release the
affected suture(s) and normalize the posterior calvarial
vault contour. Options include simple synostectomy, uni-
lateral reconfi guration of the affected occipital region, and
bilateral occipital reconstruction with or without the use
of an occipital bandeau. The majority of lambdoid surgical
candidates have signifi cant parietal and frontal compen-
satory changes in addition to their occipital deformation;
therefore, they are best served by a more extended calva-
rectomy and reconstruction.
For an extended calvarial reconfi guration, the globes are
protected with bilateral tarsorrhaphies and patients are
placed in a prone position. Perioperative antibiotics and
steroids are administered. Intraoperative bleeding is mini-
mized with the use of local anesthetic mixed with epi-
nephrine as well as controlled hypotensive anesthesia. A
coronal incision is then undertaken followed by subgaleal
dissection to expose the occipitoparietal regions. Both
parietal bone fl aps are subsequently removed with osteot-
omies taken posterior to the coronal and anterior to the
lambdoid sutures. A midline strip of bone is then left to
protect the underlying sagittal sinus. After removal of the
parietal bone fl aps, dissection is carried out at the level
of the lambdoid suture under direct visualization, taking
great caution at the level of the transverse, sagittal and
sigmoid sinuses. Osteotomies are brought to within 1 cm
on either side of the midline, with the fi nal cut made after
the underlying dura and sinus have been clearly dissected
free under direct visualization. Inadvertent entry into the
sinus, particularly at the region of the asterion, may lead
to signifi cant blood loss over a short period and consti-
tutes the greatest risk encountered with this approach.
FIGURE 16-4. (continued) Preoperative (J) and postoperative (K) photographs
demonstrate correction of the orbital and frontal bone asymmetries. Note the increased
aperture of the right eye in the preoperative photo and subsequent correction.
J
K

CRANIOSYNOSTOSIS • 175
Nevertheless, with appropriate care and meticulous dis-
section, this complication may be avoided in the majority
of individuals. The removed calvarial plates are reconfi g-
ured to provide adequate reshaping of the occipital con-
tour and stabilized with resorbable hardware, deliberately
leaving open the region of the prior lambdoid suture.
Subgaleal drains are then placed and closure is carried
out in routine fashion.
Treatment of posterior deformational plagiocephaly is a
function of both the age and severity at presentation. When
presenting before the age of 6 months, therapy consists
of positional modifi cations combined with physiotherapy
in the case of constrained neck movements or asymmetric
neurological development. In cases of no improvement or
progression of the deformity despite repositioning after 2 to
3 months, cranial orthotic (helmet) therapy is indicated.
COMPLICATIONS
Perioperative morbidity may include wound infection,
dural laceration, superfi cial brain injury, cerebrospinal
fl uid leak, encephalocele formation, subgaleal hematoma,
and ocular injury. Intraoperative blood loss and transfusion
requirements, leading to hemodynamic instability, con-
stitute great dangers to the patient and should never be
underestimated. It is imperative to accurately gauge the
extent of blood loss and match accordingly with packed
red blood cells. Other serious perioperative complications
consist of ischemic brain injury, venous air embolism, epi-
dural and subdural hemorrhage, and severe transfusion
reactions.
Long-term postoperative concerns include recurrent
calvarial deformities, cranial bone defects that fail to fi ll in
over time, and hardware-related problems. Depending on
the extent of morphologic asymmetry, intracranial hyper-
tension, and developmental delay, recurrent calvarial defor-
mities may require a minor or major reoperation. Defects
larger than 2 cm in patients older than 18 to 24 months will
often persist and may need eventual correction with split
calvarial bone graft or bone substitute. Persistent hardware
may also be problematic, particularly in patients in whom
resorbable hardware was used. It is not uncommon for the
polylactic/polyglycolic constructs to remain in place for 12
to 18 months before eventual resorption. In rare individuals,
FIGURE 16-5. Bilateral FOAR for bicoronal synostosis. The FOAR procedure
involves release of both coronal sutures while providing bilateral frontal and orbital
correction. A bifrontal bone fl ap is removed, typically as a single segment, leaving a
1–2 cm wide supraorbital bandeau. A tongue-and-groove osteotomy of the bandeau
facilitates advancement and fi xation (A, B). Fixation of the frontal bone to the supraorbital
bar is then performed (C, D).
A
C
B
D

176 • CHAPTER 16
sterile abscesses may develop at sites of hardware resorp-
tion and subsequently require exploration for debridement.
Postoperative mortality rates are low and continue to decline
with technological advancements and experience.
OUTCOMES ASSESSMENT
Based on outcomes data on open craniosynostosis surgery,
estimated blood loss spans 25% to 500% of estimated
blood volume (EBV), transfusion requirement range from
25% to 500% of EBV, complication rates are between
6.8% and 23.3%, and mortality rates center around 0% to
1.1%. 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 indeed
higher for complex synostoses.
Outcome studies have also demonstrated that con-
servative therapy is benefi cial in the reduction of calva-
rial deformities secondary to positional molding. Postural
changes and helmet therapy are most effective between
4 and 12 months of age, during the period of rapid brain
growth. Approximately 95% of infants can be expected to
have satisfactory cosmetic improvement with conservative
management. Counterpositioning with or without phys-
iotherapy or helmet therapy may reduce skull deformity,
with better results noted with increasing compliance.
REFERENCES
1. Virchow R. Uber den Cretinismus, namentlich in Franken
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. Whitaker LA, Munro IR, Salyer KE, Jackson IT, Ortiz-
Monasterio F, Marchac D. Combined report of problems and
complications in 793 craniofacial operations. Plast Reconstr
Surg. 1979;64(2):198–203.
5. Jimenez DF, Barone CM, Cartwright CC, Baker L. Early
management of craniosynostosis using endoscopic-assisted
strip craniectomies and cranial orthotic molding therapy.
Pediatrics. 2002;110(1 Pt 1):97–104.
6. Lauritzen CG, Davis C, Ivarsson A, Sanger C, Hewitt TD.
The evolving role of springs in craniofacial surgery: the
fi rst 100 clinical cases. Plast Reconstr Surg. 2008;121(2):
545–554.
7. Akai T, Iizuka H, Kawakami S. Treatment of craniosynos-
tosis by distraction osteogenesis. Pediatr Neurosurg. 2006;
42(5):288–292.
8. Weinzweig J, Kirschner R, Farley A, et al. Metopic synosto-
sis: defi ning the temporal sequence of normal suture fusion
and differentiating it from synostosis on the basis of com-
puted tomography images. Plast Reconstr Surg . 2003;112:
1211–1218.
9. Weinzweig J, Baker SB, Whitaker LA, Sutton LN, Bartlett SP.
Delayed cranial vault reconstruction for sagittal synostosis in
older children: an algorithm for tailoring the reconstructive
approach to the craniofacial deformity. Plast Reconstr Surg.
2002;110(2):397–408.

17
c h a p t e r
INTRODUCTION
The term hemifacial microsomia (HFM) syndrome refers
to a spectrum of congenital anomalies development and
growth of craniofacial structures derived from the fi rst and
second branchial arches. The malformations include micro-
tia and facial asymmetry secondary to hypoplasia of struc-
tures such as the mandible, temporal bone, lateral parts
of the face, and the cranium. The syndrome can vary in
severity from almost unnoticeable to severely disfi guring,
and in some cases it may be associated with malformations
in other regions of the anatomy. Bilaterality occurs in 15%
to 30% of cases and may also present with asymmetrical
involvement.
The most commonly used term, hemifacial microsomia,
was popularized by Gorlin and Pindborg. 1 Other terms
that have been used include otomandibular dysostosis,
fi rst and second branchial arch syndrome, and auriculo-
branchiogenic dysplasia. 2 If the deformity includes orbital
dystopia and skull asymmetries, then it is referred to as
craniofacial microsomia. In any case, associated craniofacial
anomalies may include macrostomia, micrognathia, cleft
lip (with or without cleft palate), preauricular skin tags
or pits, and ocular dermoid cysts ( Figure 17-1 A,B). The
occurrence of epibulbar dermoid cysts with this pattern of
anomaly, especially when accompanied by vertebral anom-
alies, has been designated Goldenhar syndrome. 3
EPIDEMIOLOGY
The incidence of HFM has not been established, although
it is known to be the second most common group of birth
defects after cleft lip and palate. 4 It occurs more commonly
on the right side. Because half of all cases of microtia occur
in association with syndromes, in particular HFM, some
authors concluded that microtia should be considered a
microform of HFM. 5 Other investigators support this view.
A family history of HFM, auricular anomalies, or hear-
ing loss was associated with a greater risk of HFM. A posi-
tive familial association of approximately 50% has been
observed in large series, and patterns of both autosomal
dominant and autosomal recessive inheritance have been
described.
PATHOGENESIS
The clinical signs spectrum seen in HFM arises when the
normal developmental processes in the fi rst and second
branchial arches are disrupted. Under normal circum-
stances, the fi rst branchial arch forms Meckel cartilage,
which further develops into the mandible, the muscles of
mastication, a major portion of the malleus, and the incus.
The second branchial arch forms Reichert cartilage, which
further develops into the hyoid bone, the styloid process,
and the muscles of facial expression. A major portion of
the stapes is also derived from this cartilage.
In 1973, Poswillo 6 was able to induce phenocopies of
HFM by administering triazines to mice and thalidomide
to monkeys. Drugs induced hematomas in the region
of the stapedial artery. Secondary tissue necrosis caused
by the ischemia had produced similar facial anomalies in
the animals. As major was the hemorrhage, more severe
is the syndrome and for these reasons we can explain the
broad spectrum of abnormalities that can be seen. Retinoic
acids produce anomalies in the axial skeleton and cranio-
facial region, as well as in the cardiovascular and central
nervous systems, similar to those seen in HFM. 7 , 8
PATIENT EVALUATION AND SELECTION
Classifi cation
The most effective classifi cation should be univer-
sally acceptable to allow clinicians and investigators to
Hemifacial Microsomia
Fernando Molina, MD

178 • CHAPTER 17
communicate data unambiguously, and should also help
in treatment planning and prognosis. The earliest classifi -
cation by Pruzansky 9 and Murray et al. 10 focused on the
mandibular deformity into four grades:
Grade I—hypoplasia affects only the gonial angle
Grade II-A—the angle and the ascending ramus are
affected
Grade II-B—hypoplasia is a more severe affecting
angle, ascending ramus with a fl at and rudimentary
condyle
Grade III—Complete absence of the ramus and
condyle
The OMENS classifi cation is an acronym that means
o rbit, m andible, e ar, n erve, and s oft tissue. 11 Later revised
to OMENS-Plus, 12 it further differentiated between
the mandible and orbit skeletal components, and distin-
guished between facial nerve involvement and soft tissue
involvement. The expansion of OMENS to indicate the
presence of extracranial anomalies by adding “plus” to the
classifi cation creates a more complete system.
Clinical Presentation
Mandibular hypoplasia and microtia remains the hallmarks
of this deformity. The more affected mandibular structure
is the ascending ramus. In the severe cases, the lack of bone
at the mandible secondarily affects the growth of the ipsi-
lateral maxilla and orbit. Approximately 15% of patients
have orbital involvement. In the affected side, the orbit is
lower when compared with the orbit on the opposite side.
Microtia is the most common auricular malformation and
frequently is situated in a very low position. Middle ear
anomalies are also observed. Seventh nerve involvement is
present in less than half of the patient; the more affected
is the marginal branch with unilateral lip palsy. The second
FIGURE 17-1. (A) A 7-year-old girl with right hemifacial microsomia. Facial
asymmetry, microtia, soft tissue defi ciency, and chin deviation to the affected side are the
typical signs. (B) A 6-year-old girl with Goldenhar syndrome. Ocular dermoid cysts and
vertebral anomalies are characteristic clinical features together with the facial asymmetry.
B
A

HEMIFACIAL MICROSOMIA • 179
is the frontal branch and the patients are unable to elevate
the eyebrow.
Whereas a soft tissue defi ciency is present in most
patients, the masticatory muscles are affected in differ-
ent degrees. Usually there is no relationship between the
bone hypoplasia and the muscles involvement. However,
in grade III HFM, the masticatory group of muscles are
severely affected and in many cases absent.
Other commonly associated anomalies include mac-
rostomia, preauricular ear tags, palatal deviation, epibul-
bar dermoids, vertebral and long bone anomalies, and rib
anomalies. Palatal deviation is usually the result of muscu-
lar palsy and hypoplasia and is not related to any cranial
nerve weakness.
PATIENT PREPARATION
The objective in the treatment of the patients with HFM
is to obtain facial symmetry and to correct the functional
alterations. Children must be seen regularly by a craniofa-
cial team consisting of a craniofacial surgeon, an orthodon-
tist team, a geneticist, a speech pathologist, an audiologist,
an otolaryngologist, an ophthalmologist, and a social
worker. This will ensure a more thorough assessment of
the underlying problem and better treatment planning.
Frequent ear examinations and hearing tests are recom-
mended to identify and treat ear infections and/or hearing
loss. Microtia is usually associated with a 50 to 55 dB hear-
ing loss on the affected side.
Velopharyngeal insuffi ciency is present in up to one
third of children with HFM, and speech evaluation should
be recommended.
Patients should fi rst be clinically examined with the
intention of providing a quantitative and qualitative assess-
ment protocol for determining soft and hard tissue compo-
nents of the facial deformity.
In patients with facial asymmetry, with the use of a
compensatory head posture corrected, the clinician subjec-
tively assesses the level of the asymmetry. One should note
forehead, orbital, zygoma, and external ear position and
relationships by viewing the patient frontally. The qual-
ity and thickness of the cheek soft tissue should also be
observed. Additional anthropometric measurements must
be made, the distance between the lateral canthus to the
buccal commissure and from a central point of the inferior
orbital rim to the buccal commissure are recorded before
and after distraction. The position and contour of the chin
(pogonion), as well as the inferior border and angle of the
mandible (gonion), are likewise recorded.
The intraoral examination documents the status of the
occlusion: the occlusal plane/cant, molar relationship, and
presence of crossbites. Evaluation of the temporoman-
dibular joint is routinely performed: mandibular excur-
sions, including maximum interincisal opening, and lateral
movements are documented.
Preoperatively, lateral and posteroanterior cephalomet-
ric studies are done. Panorex views are used to compare
both sides of the mandible and to locate the tooth buds
and dental nerve, in order to avoid their injury during sur-
gery. In the fi rst cases, the superimposing method of Björk
and Skieller 13 was used to obtain a mandibular growth
prediction to assess the ideal dimension of the mandible
and to reproduce the rotational movements (anterior and
posterior) that it performed during growth. 3D-CT offers
refi nement in anatomic and morphologic mapping to aid
in the diagnosis, treatment planning, and postsurgical con-
trol in the long term of patients.
TECHNIQUE
Macrostomia and preauricular skin tags are the most obvi-
ous deformities that may need to be addressed early in
life. Untreated macrostomia may cause feeding diffi culties
because of the incompetence of the mouth. Not infre-
quently, preauricular tags have cartilage remnants extend-
ing into the deeper subcutaneous tissue, and to avoid
depression deformity of the scar, it is important that the
cartilage remnants be completely removed using blunt dis-
section with the deeper layers approximated. For repair of
macrostomia, the distance from midline to the oral com-
missure on the noncleft side is measured and used as a
reference for the future oral commissure on the cleft side.
Frequently there is a distinct demarcation at the site of
macrostomia, and this usually coincides with the other side.
Revisions may be necessary, although one may be able to
minimize the risk associated with revision by reconstruct-
ing an oral commissure 1 to 2 mm close to the midline
to compensate for laxity. Incision at the mucocutaneous
junction exposes the underlying muscle in the cleft, which
is then properly repaired. A z-plasty at the mucosal com-
missure is included to recreate the crease of the mouth. In
the skin, the surgeon should avoid the presence of z-plasty
since the fi nal result is always anesthetics.
It may be possible to excise epibulbar dermoid cysts
with minimal scarring of the cornea. Children with these
cysts should be referred to an experienced ophthalmolo-
gist for management to avoid secondary symblepharon.
Auricular reconstruction is usually performed after the age
of 9 years. Using costochondral graft to fabricate a cartilage
framework, the ear reconstruction is achieved. Six months
later the new ear is separated from the cranium using a
skin graft. There after, surgical refi nements are done to
obtain a satisfactory fi nal ear. Mandibular distraction may
be considered before reconstruction of a low-set microtia
in an attempt to position the reconstructed ear in a sym-
metrical location.
Velopharyngeal insuffi ciency is very seldom observed.
If a patient requires a treatment related to palatal muscle
hypoplasia, it should be performed on the basis of nasoen-
doscopic fi ndings. Upper airway compromise resulting
from severe mandibular micrognathia in bilateral HFM
may require consideration of bilateral mandibular distrac-
tion or the need for tracheostomy early in life.
The mandibular hypoplasia is treated with distraction
osteogenesis to achieve simultaneous skeletal and soft tis-
sue correction with minimal surgery. The technique has

180 • CHAPTER 17
only recently become popular in the craniofacial skeleton
and present a treatment modality with unique properties
that offer a superior clinical and functional results not only
by augmenting the craniofacial skeleton but also the over-
lying soft tissue. 14 – 17 The bones of the craniofacial skeleton
are membranous in embryologic origin. As such, they are
of a relatively smaller dimension, have a much richer blood
supply, and are more accessible to surgical manipulation
due to wide subperiosteal dissection.
Under general anesthesia and a dilute solution of xylo-
caine and epinephrine is injected over the oblique line and
buccal surface of the ramus. A 3-cm incision is made on
the lateral vestibule oral mucosa. Subperiosteal dissection
is performed exposing the gonial angle and the neighbor-
ing area of the ascending ramus. This maneuver is simple
and provides an excellent exposure of the bone hypoplasia
area. The site of the planned external corticotomy and pin
placement is marked with gentian violet, avoiding the men-
tal nerve and tooth buds. This step is critical since the selec-
tion of the pin hole sites dictate the vector of distraction.
The corticotomy is not just external, but rather is an
external and extended corticotomy that attempts to pre-
serve the nerve, the intramedullary vascularity, and the
tooth buds. It should be done with a side-cutting burr. The
osteotomy begins at the retromolar angle, cutting along
the medial and lateral buttresses and extending through
the lateral aspect of the mandibular angle, including the
entire cortex down to the cancellous layer. Along the infe-
rior aspect, the corticotomy is extended widely around the
angle where the bone is quite thick ( Figure 17-2 ). Only
FIGURE 17-2. Diagram showing the external and
extended corticotomy. The cortical bone is widely cut from
the retromolar triangle to the mandibular angle exposing the
cancellous bone. Two points has been selected to introduce
the pins through the full thickness of the mandible.
FIGURE 17-3. The diagram shows the remaining 6 to
8 mm of the internal cortical layer protecting the neurovascular
bundle. Different zones of bone resistance are encountered,
minor at the angle but major resistance at the alveolar ridge,
that will be elongated differently under a perpendicular
distraction vector. The remaining lingual cortex eventually will
be disrupted by the distraction forces. This fact usually occurs
during the second week of the distraction period.
6 to 8 mm of the internal cortical layer is preserved to
protect the nerve and artery ( Figure 17-3 ). In fact, with
this external extended corticotomy, different zones of
bone resistance are encountered, less at the angle and more
at the alveolar ridge, which will be elongated differently
with a perpendicular distraction vector to achieve multi-
dimensional mandibular lengthening ( Figure 17-4 A,B). In
fact, this technique will produce bone lengthening with
simultaneous bone remodeling. Increases in the vertical
height of the ramus and the new bone formation at the
mandibular angle allow clockwise mandibular rotational
growth movements. During the consolidation period, the
patient is allowed use of the mandible to eat, talk, etc. and
the masticatory muscles work on the regenerate bone to
modify the mandibular morphology. The bigonial distance
is increased in the vertical dimension of the coronoid pro-
cess and also in the musculature. All of the changes explain
the role of the functional matrix on mandibular distraction
osteogenesis and it is clearly observed when the neurovas-
cular bundle and intramedullary vascularity are preserved.

HEMIFACIAL MICROSOMIA • 181
Vectors of Distraction
The position of the pins placement will determine the vec-
tor of distraction. This is different in each patient accord-
ing to the grade of mandibular hypoplasia ( Figure 17-5 ).
In patients with grade I HFM, the corticotomy extends
obliquely from the posterior edge of the hypoplastic gonial
angle to the retromolar angle in the alveolar ridge. The pins
must be placed perpendicular to the corticotomy to obtain
an oblique vector of distraction that produces greater bone
elongation in the angle and less in the alveolar ridge.
In patients with grade II-A HFM, the distraction process
must remodel and elongate the angle and the initial por-
tion of the ascending ramus. For this reason, the cortico-
tomy is placed obliquely at the junction of the angle and
ramus and the pins must be inserted in an intermediate
position between a vertical and oblique distraction vector.
In patients with grade II-B HFM, the corticotomy is
placed horizontally at the base of the ascending ramus and
the pins must follow a vertical distraction vector in order
to obtain more elongation in the hypoplastic ascending
ramus.
The Semirigid External Devices
Two pins, from 2.0 to 3.5 mm in diameter (age depend-
ing), are introduced percutaneously through the whole
thickness of the mandible (bicortical), 3 to 5 mm in front
and behind the corticotomy. The diamond tip design of the
pins makes it unnecessary to drill a hole before its intro-
duction in the bone. Care must be taken to position the
pins parallel to each other and facilitate their fi xation to
the distraction device and one must avoid injuries in the
unerupted dental follicles.
The mucosa is closed with a fi ne absorbable suture and
the distraction device is applied. The device is made of
two hollow plates with a central perforation to allow the
entrance of the mandibular pins. The two plates are joined
by another stainless steel screw running free in one of
them and transversing the whole length of the plate at the
other, so when it is turned the distance between the plates
is increased or decreased ( Figure 17-6 A,B). The steel resis-
tance of the distractor device is softer than the pins; this
produces a fl exible device that can bend under the masti-
catory muscle action and externally refl ects the shape of
the elongated bone area.
THE POSTOPERATIVE PERIOD
Elongation is commenced on the fi fth postoperative day
(latency period) at a rate of approximately 1 mm per day
(rhythm). It is done by the parents with minimal discom-
fort to the patient. In the event that the patient is resistant
to turning the distractors, an age-appropriate dose of acet-
aminophen may be given 20 minutes prior to turning. The
elongation is usually completed in 3 to 4 weeks (distraction
period), and the device is left in place for an additional 6 to
FIGURE 17-4. (A) Bone elongation and remodeling should be achieved at the
gonion and the ascending ramus in the hypoplastic hemifacial microsomia mandible.
If the bone elongation area does not recreate the normal mandible anatomy, major
occlusion problems will occur and facial asymmetry will remain. (B) The diagram shows
the multidimensional new bone formation area at the mandible after the proper use
of distraction vectors. Neurovascular components and tooth buds always should be
preserved.
A
B