Phẫu Thuật Tạo Hình (Tập 3): Sọ Mặt, Đầu Cổ Và Nhi Khoa – Neligan (Ấn bản 4)

707
Outcomes, prognosis, and complications
Fig. 30.10  Le Fort III with bipartition and internal distraction (observe transfacial pin during distraction period) and modification of angulation of the maxillary arch. (A)
Front view before. (B) Front view after procedure. (C) Computed tomography (CT) scan before Le Fort III (observe retrusion of the midface). (D) CT scan during distraction
with transfacial pin. (E) CT scan after distraction and removal of distractors. (F) Lateral view before advancement. (G) Lateral view after advancement. (H) Lateral CT scan
before advancement. (I) Lateral CT scan during distraction with internal devices. (J) Lateral CT scan after distraction.
A
B
C
D
E
F
G
H
I
J

708
SECTION II
CHAPTER 30 • Orbital hypertelorism
after a cranioplasty or after a bipartition, which tends to
deepen the temporal fossa.
Secondary procedures
Soft-tissue problems are more complex to treat than bony
irregularities.
Some craniofacial malformations involve only the skeleton,
e.g., mild symmetrical orbital hypertelorism, orbital dystopia,
frontal or malar asymmetries, and malposition. Correction
can be achieved through hidden approaches, such as bicoronal
or vestibular incisions, or through palpebral infraciliary
approaches, all of which leave almost invisible scars.
Temporal hollowness can be addressed by fat grafting
according to the Coleman technique. Repeated treatments
may be necessary.
Excess skin can be adjusted and will retract. After correc­
tion of moderate hypertelorism, the excess skin of the glabellar
region and dorsum of the nose retracts with the help of good
undermining and a bone graft to lift the dorsum of the nose.
Possible future soft-tissue correction must be considered in
the initial treatment plan such that skeletal access is achieved
without sacrificing potential cutaneous flap options. For
example, if a frontal skin flap is planned, the design of the
coronal incision may have to be altered to avoid violating the
future pedicle.
It is usually much easier to reconstruct the skeleton than to
correct soft-tissue deficiencies. All the resources of plastic
surgery can rarely achieve a perfect contour with minimal
scars. The authors briefly consider the primary soft-tissue
problems encountered in major congenital craniofacial mal­
formations at the end of this chapter.
persistent after 3 days of lumbar punctures. Rhinorrhea
represents a risk of meningitis and can be avoided by
careful dissection in the retroglabellar region.
■ Infection linked to an insufficient cranialization of the
frontal sinus (if a frontal sinus was present at the time
of surgery to require cranialization). This may be best
prevented by operating early after the age of 4 years,
when the frontal sinus has usually not yet developed.
Infection may arise anywhere from a few days to a few
months postoperatively.
■ Ophthalmological complications such as keratitis or
chemosis are best prevented by the initial tarsorrhaphy.
Blindness represents an exceptionally rare but dramatic
complication, and all patients should be warned
accordingly. The possibility of a postoperative
strabismus is more common, and care should be
taken to sufficiently medialize the medial orbital
walls posterior to the osteotomies so as to avoid a
step-off that may impinge on the medial rectus muscles.
Failure to carry the medialization far enough posteriorly
may also contribute to an insufficient medialization of the
globes despite a satisfactory medialization of the bony
orbits.
■ In the long term the most important complication is an
insufficient correction. This is most likely to occur not by
relapse, but by soft-tissue relaxation, especially at the
medial canthi. To this extent the medial canthus fixation
tends to be disappointing and frequently necessitates a
secondary correction (Fig. 30.11). The secondary
rhinoplasty greatly contributes to the aesthetic outcome,
but must be delayed until adulthood.
■ Temporal hollowing is not uncommon, especially if the
hypertelorism correction is the second major procedure
Fig. 30.11  (A–C) Secondary correction of medial canthus disinsertion.
A
B
C

709
Secondary procedures
deficiency, various nasal flaps should be considered. The
excess skin existing on the upper part of the nose can be
transferred to the lower part.
In some cases, the nose is very distorted and the shortage
of skin is obvious from the start. The frontal area is the best
zone from which to obtain the missing skin. On other occa­
sions, correction of the hypertelorism creates an excess of soft
tissue at the level of the forehead that can be used as a frontal
flap. Sometimes, a preliminary frontal skin expansion may be
necessary to provide the required soft tissue. The potential
need for nasal reconstruction should be carefully considered
early in the planning process as the usually preferred coronal
approach may require modification if soft-tissue recruitment
becomes necessary in this context.
Eyelids and orbits
Clefts of the eyelids primarily involve the lower eyelid and
are addressed during the midface reconstruction, as are ocu­
lonasal clefts. The craniofacial clefts discussed in this chapter
may also result in pathology of the orbits.
If there is anophthalmia or microphthalmia, the bony orbit
does not develop to its normal size. The preferred treatment
is a progressive enlargement of the conjunctiva,35,36 but con­
formers are very difficult to place; intraorbital expanders are
much more efficient and can produce an almost normal
growth of the orbit with a prosthesis fitted afterwards. Nev­
ertheless, this orbital expansion is difficult to execute and
requires very close follow-up. If orbital expansion fails or is
not attempted, a micro-orbit must be faced. This orbit should
first be placed in proper position, vertically and horizontally,
in relation to the other orbit. It can then be surgically enlarged
by expanding the whole circumference. Access to the orbital
roof is obtained with a localized frontal craniotomy, as for an
orbital dystopia. The secondary soft-tissue work of creating a
good cavity capable of retaining an ocular prosthesis, and
building up the short, retracted eyelids with auricular com­
posite grafts, is often more difficult and time-consuming than
the skeletal work.
Oculomotor disorders
Numerous conditions in craniofacial surgery may present
with oculomotor disorders, especially when hypertelorism is
apparent. The rule is to perform the bony work first and
secondarily to address any oculomotor disorders that are
present. Further discussion of the treatment of oculomotor
disorders is beyond the scope of this chapter.
Recurrences of hypertelorism
In our experience, hypertelorism recurrences are not frequent
if the repair has been performed after 4 years of age, at the
completion of skull growth. This recurrence rate would be dif­
ferent if the hypertelorism were corrected earlier; for instance,
at 1 year of age the brain is still undergoing significant growth
which might counteract medialization of the orbits. An
exceptional circumstance of orbital hypertelorism relapse has
been encountered with an interorbital encephalocele which
had recurred because of ventriculoperitoneal shunt dysfunc­
tion. The encephalocele acted as an interorbital expander
that progressively enlarged the tissues between the orbital
cavities.
Regardless, the final rhinoplasty will be the most important
element of the definitive aesthetic result.
Refinements in hypertelorism correction
(in clefts or craniosynostotic patients)
Irregularities are frequent in the frontal region, the nose, and
the temporal fossae. Refinements may be performed early or
delayed until the completion of craniofacial growth, after 15
years of age. Techniques to obtain a satisfactory contour have
classically included bone remodeling, bone grafting, and the
onlay of biomaterials. More recently, fat grafting as described
by Coleman has proven useful to correct minor anomalies
such as temporal hollowing.
Soft-tissue refinements are aesthetically relevant for the
final result and may be divided into several subcategories, as
follows.
Canthus correction
During the surgical correction of hypertelorism, it is preferable
to maintain the bony attachment of the medial canthus. In so
doing, one avoids the need for medial canthus reattachment,
which is always possible but is sometimes unreliable in the
long term. Even when the medial canthal ligament is pre­
served, if the medial bony resection is 2 cm, the postoperative
intercanthal distance may be reduced by only 1 cm, demon­
strating 50% efficacy at the soft-tissue level. It might be neces­
sary to reinsert the medial canthal ligaments under increased
tension (with a transnasal technique: see Fig. 30.11) and correct
an epicanthal fold (easily achieved with Y–V plasty or a Del
Campo technique (asymmetric Z-plasty).34
The lateral canthus is less well defined and it is easily
repositioned with a lateral suspension, if necessary, at the end
of the craniofacial procedure. If this correction proves insuf­
ficient in the long term, a lateral canthopexy may be performed
secondarily through a small lateral incision.
Scalp and eyebrows
Hairline distortion is frequently observed in craniofacial clefts
and reflects the continuity of the cleft at the scalp level. Hair
growing down on to the forehead can be removed. Unwanted
hair can be incorporated into a frontal skin resection that
facilitates exposure, or excision may be achieved secondarily.
Eyebrows can be clefted or displaced. Differential reposition­
ing of the forehead and scalp can correct eyebrow displace­
ment, usually by lowering the high side.
Nose
In symmetrical hypertelorism presenting with an excess of
skin at the level of the nasal dorsum, it is advisable to avoid
the easy solution of midline skin excision. Some of these
median scars do very well with time and become hardly
visible, but others stretch, become pigmented, and are very
prominent. It is therefore better to count on skin retraction, as
previously discussed.
If there is a cleft affecting the nose, it should be corrected
at the time of the craniofacial procedure to utilize the relative
excess soft tissue obtained by the undermining performed
around the nose. A cleft can be closed by reapproximation of
tissues with plasties at the alar margins, but if there is a tissue

710
SECTION II
CHAPTER 30 • Orbital hypertelorism
Craniofacial surgery is teamwork
It is obvious from the congenital craniofacial malformations
discussed in this chapter that the only possible approach to
these patients is via a craniofacial team. Once the team
members have examined the patient, and with the invalu­
able help of modern imaging, a plan of treatment is drawn
up by the plastic surgeon and the neurosurgeon to incorpo­
rate all the morphological and functional aspects of the cor­
rection. These operations are too complex to be performed
without significant previous experience with the problems
involved. Such experience can only be obtained if these
operations are performed in a limited number of specialized
centers.
Conversely, the inherent lack of growth in faciocraniosyn­
ostosis may produce relative relapse of any associated maxil­
lary retrusion, but this finding does not require further
revision.
Careful analysis with three-dimensional computed to­
mography helps tremendously in planning for precise bony
work.37 Nevertheless, minor primary undercorrection of hy­
pertelorism is more frequent than actual recurrence, despite
satisfactory bony work. This finding underscores the impor­
tance of soft-tissue refinements, especially with regard to
nasal correction, which will eventually determine the success
of the clinical outcome. In older patients, nasal deformity may
not only be the presenting chief complaint; it might also be
the final and most important step of the surgical correction.38,39
5. Tessier P. Anatomical classification of facial, craniofacial and
laterofacial clefts. J Maxillofac Surg. 1976;4:69.
7. Marchac D, Renier D, Broumand S. Timing of treatment for
craniosynostosis : a 20 year experience. Br J Plast Surg. 1994;47:211–
222. The authors report their extensive experience in this 983-patient
series. With early diagnosis, brachycephalies are corrected between 2 and 4
months of life; other craniosynostoses are addressed in the second half of
the first year of life.
9. Crouzon O. Dysostose craniofaciale héréditaire. Bull Soc Med Hôp
Paris. 1912;33:545–555.
10. Tessier P. Osteotomies totales de la face: Syndrome de Crouzon,
syndrome d’Apert, oxycephalies, scaphocephalies, turricephalies.
Ann Chir Plast. 1967;12:273.
13. Ortiz-Monasterio F, Fuente del Campo A, Carillo A. Advancement
of the orbits and the midface in one piece, combined with frontal
repositioning for the correction of Crouzon’s deformities. Plast
Reconstr Surg. 1978;61:507–516. The authors advocate composite
advancement of the orbits and midface in addition to frontal advancement
for the management of Crouzon’s syndrome. They caution that, while they
are optimistic, their data do not have sufficient follow-up to demonstrate
the longevity of their results.
16. Arnaud E, Marchac D, Renier D. Reduction of morbidity of
frontofacial advancement in children with distraction. Plast Reconstr
Surg. 2007;120:1009–1026. This is a prospective analysis of 36 patients
undergoing monobloc distraction for faciocraniosynostosis. The authors
assessed their outcomes and concluded that their use of internal
distraction reduced the risks inherent to monobloc advancement.
17. Apert E. De l’acrocephalosyndactylie. Bull Soc Med Hop Paris.
1906;23:1310.
19. Renier D, Sainte-Rose C, Marchac D, et al. Intracranial pressure
in craniostenosis. J Neurosurg. 1982;57:370–377. Pre- and
postoperative intracranial pressure (ICP) measurements were taken in
23 craniosynostosis patients. Elevated ICP normalized after surgery. A
correlation was noted between elevated ICP and lower cognitive testing.
21. Converse JM, Ransohoff J, Matthew E, et al. Ocular hypertelorism
and pseudohypertelorism. Advances in surgical treatment. Plast
Reconstr Surg. 1970;45:1. This review begins with a discussion of the
definition of hypertelorism and associated diagnoses. A detailed survey of
corrective procedures follows.
22. Tessier P. Experience in the treatment of orbital hypertelorism. Plast
Reconstr Surg. 1974;53:4.
Access the complete reference list online at

710.e1
References
References
1. De Myer W, Zeman W, Palmer CA. The face predicts the brain:
diagnostic significance of median facial anomalies for
holoprosencephaly (arrhinencephaly). Pediatrics. 1964;34:256–263.
2. Patten BM. Human Embryology. 3rd ed. New York: McGraw Hill;
1968.
3. Cohen MM Jr, Jirasek JE, Guzman RT, et al. Holoprosencephaly, and
facial dysmorphia: nosology, etiology and pathogenesis. Birth
Defects. 1971;7:125.
4. Sedano HO, Cohen MM Jr, Jirasek J, et al. Frontonasal dysplasia.
J Pediatr. 1970;76:906.
5. Tessier P. Anatomical classification of facial, craniofacial and
laterofacial clefts. J Maxillofac Surg. 1976;4:69.
6. Cohen MM Jr. Craniosynostosis: Diagnosis, Evaluation and
Management. New York: Raven Press; 1986.
7. Marchac D, Renier D, Broumand S. Timing of treatment for
craniosynostosis : a 20 year experience. Br J Plast Surg. 1994;47:211–
222. The authors report their extensive experience in this 983-patient
series. With early diagnosis, brachycephalies are corrected between 2 and 4
months of life; other craniosynostoses are addressed in the second half of
the first year of life.
8. Marchac D, Arnaud E. Midface surgery from Tessier to distraction.
Child’s Nerv Syst. 1999;15:681–694.
9. Crouzon O. Dysostose craniofaciale héréditaire. Bull Soc Med Hôp
Paris. 1912;33:545–555.
10. Tessier P. Osteotomies totales de la face: Syndrome de Crouzon,
syndrome d’Apert, oxycephalies, scaphocephalies, turricephalies.
Ann Chir Plast. 1967;12:273.
11. Marchac D, Renier D. Le front flottant, traitement précoce des
faciocraniosténoses. Ann Chir Plast. 1979;24:21.
12. Marchac D, Renier D. Craniofacial Surgery for Craniosynostosis.
Boston: Little, Brown; 1982:125.
13. Ortiz-Monasterio F, Fuente del Campo A, Carillo A. Advancement
of the orbits and the midface in one piece, combined with frontal
repositioning for the correction of Crouzon’s deformities. Plast
Reconstr Surg. 1978;61:507–516. The authors advocate composite
advancement of the orbits and midface in addition to frontal advancement
for the management of Crouzon’s syndrome. They caution that, while they
are optimistic, their data do not have sufficient follow-up to demonstrate
the longevity of their results.
14. Mühlbauer W, Anderl H, Marchac D. Complete Frontofacial
Advancement in Infants with Craniofacial Dysostosis: Transactions of the
Eighth International Congress of Plastic Surgery, Montreal. Montreal:
McGill University; 1983:318–320.
15. Marchac D, Renier D. Early monobloc frontofacial advancement. In:
Marchac D, ed. Craniofacial Surgery: Proceedings of the First
International Congress of Cranio-maxillo-facial Surgery. Berlin:
Springer-Verlag; 1987:130–136.
16. Arnaud E, Marchac D, Renier D. Reduction of morbidity of
frontofacial advancement in children with distraction. Plast Reconstr
Surg. 2007;120:1009–1026. This is a prospective analysis of 36 patients
undergoing monobloc distraction for faciocraniosynostosis. The authors
assessed their outcomes and concluded that their use of internal
distraction reduced the risks inherent to monobloc advancement.
17. Apert E. De l’acrocephalosyndactylie. Bull Soc Med Hop Paris.
1906;23:1310.
18. Cinalli G, Renier D, Sebag G, et al. Chronic tonsillar herniation in
Crouzon’s and Apert’s syndromes. The role of the premature
synostosis of the lambdoid suture. J Neurosurg. 1995;83:575–582.
19. Renier D, Sainte-Rose C, Marchac D, et al. Intracranial pressure in
craniostenosis. J Neurosurg. 1982;57:370–377. Pre- and postoperative
intracranial pressure (ICP) measurements were taken in 23
craniosynostosis patients. Elevated ICP normalized after surgery. A
correlation was noted between elevated ICP and lower cognitive testing.
20. Tessier P, Guiot J, Rougerie JP, et al. Ostéotomies cranio-naso-orbito-
faciales. Hypertelorisme Ann Chir Plast. 1967;12:103.
21. Converse JM, Ransohoff J, Matthew E, et al. Ocular hypertelorism
and pseudohypertelorism. Advances in surgical treatment. Plast
Reconstr Surg. 1970;45:1. This review begins with a discussion of the
definition of hypertelorism and associated diagnoses. A detailed survey of
corrective procedures follows.
22. Tessier P. Experience in the treatment of orbital hypertelorism. Plast
Reconstr Surg. 1974;53:4.
23. Van der Meulen J. Medial faciotomy. Br J Plast Surg. 1979;32:339.
24. Van der Meulen J, Vaandrager JM. Surgery related to the correction
of hypertelorism. Plast Reconstr Surg. 1983;71:6.
25. Tessier PL. Facial bipartition: A concept more than a procedure. In:
Marchac D, ed. Proceedings of the First International Congress of the
International Society of Cranio-maxillo-facial Surgery. Berlin: Springer-
Verlag; 1987:217–245.
26. David DJ, Moore MH, Cootes RD. Tessier clefts revisited with a
third dimension. Cleft Palate J. 1989;26:163.
27. Kawamoto H. Rare craniofacial clefts. In: McCarthy J, ed. Plastic
Surgery. Philadelphia: W. B. Saunders; 1990:2922–2973.
28. Tessier P. Chirurgie Orbito-palpebrale. Paris: Masson; 1977.
29. Jackson I, Munro I, et al. Atlas of Craniofacial Surgery. St-Louis:
Mosby; 1982.
30. Marchac D, Renier D, Arnaud E. Infrafrontal correction of
teleorbitism. In: Whitaker L, ed. Proceedings from the Seventh Meeting
of the ISCFS. USA: Santa Fe; 1997:173–175.
31. Wolfe SA. Facial Surgery of the Facial Skeleton. Boston: Little Brown;
1989.
32. Raveh J, Vuillemin T. Advantages of an additional subcranial
approach in the correction of craniofacial deformities.
Craniomaxillofac Surg. 1988;16:350.
33. Gillies HD, Harrison SM. Operative correction by osteotomy of
recessed malar maxillary compound in a case of oxycephaly. Br J
Plast Surg. 1950;2:123.
34. Fuente Del Campo A. Surgical treatment of the epicanthal fold.
Plast Reconstr Surg. 1984;73:566–570.
35. Marchac D, Cophignon J, Achard E, et al. Orbital expansion for
anophthalmia and micro-orbitism. Plast Reconstr Surg. 1977;59:486.
36. Marchac D. Surgical enlargement of the anophthalmic pocket. In:
Toth B, Keating R, Stewart W, eds. An Atlas of Orbitocranial Surgery.
London: Martin Dunitz; 1999:203–208.
37. Moreira Gonzalez A, Elahi M, Barakat K, et al. Hypertelorism: the
importance of three dimensional imaging and trends in the surgical
correction by facial bipartition. Plast Reconstr Surg.
2005;115:1537–1546.
38. Ortiz-Monasterio F, Fuente Del Campo A. Nasal correction in
hypertelorbitism. Scand J Plast Reconstr Surg. 1981;15:27.
39. Marchac D, Sati S, Renier D, et al. Hypertelorism correction: what
happens with growth? Evaluation of a series of 95 operated cases.
Plast Reconstr Surg. 2012;129:713–727.

SECTION II • Craniofacial
31 
Craniofacial clefts
James P. Bradley and Henry K. Kawamoto Jr.
SYNOPSIS
■ Congenital craniofacial clefts are abnormal disfigurements of the face
and cranium occurring in a variety of patterns and varying degrees of
severity.
■ Craniofacial clefts are thought to occur spontaneously, except for
syndromes with clefting combinations numbers 6, 7, and 8, like
Treacher Collins syndrome or hemifacial microsomia.
■ If normal embryologic neuroectoderm migration and penetration do not
occur, the epithelium breaks down to form a facial cleft. The severity
of the cleft is proportional to the failure of penetration by the
neuroectoderm.
■ Tessier’s numeric classification from number 0 to number 14 (facial
clefts = numbers 0–8 and cranial clefts = numbers 9–14) offers a
descriptive, easy-to-understand system for rare craniofacial clefts and
is treatment-oriented.
■ Median craniofacial dysplasias consist of hypoplastic (tissue
deficiency), dysraphia (normal tissue but clefted), and hyperplastic
(tissue excess) malformations.
■ During infancy (3–12 months), functional problems, soft-tissue clefts,
and midline cranial defects (e.g., encephaloceles) may be corrected.
■ In older children (6–9 years), midface and orbital reconstruction with
bone grafting or a facial bipartition may be performed.
■ At skeletal maturity orthognathic and soft-tissue remodeling are offered
as final corrective procedures.
Introduction
Congenital craniofacial clefts are abnormal disfigurements of
the face and cranium with deficiencies, excesses, or even a
normal (but separated) amount of tissue occurring along
linear regions.1–4 Of all congenital facial anomalies, craniofa­
cial clefts are among the most disfiguring. They may be seen
in a variety of patterns and varying degrees of severity.
Although at first glance they appear to defy definable pat­
terns, most craniofacial clefts occur along predictable embryo­
logic lines.5 Craniofacial clefts may be either unilateral or
bilateral, and one cleft type can manifest on one side of the
face, while a different type is present on the other side.
Classifications of craniofacial clefts
These craniofacial malformations are rare and have multiple
variations and a spectrum of severity. For diagnostic and
treatment purposes, the use of similar terminology describing
embryologic maldevelopment, genetic etiology, or anatomic
landmarks is beneficial. Organization of the seemingly hetero­
geneous clefting malformations is necessary for both morpho­
genetic understanding and knowledge of surgical anatomy
for treatment. Therefore, orderly systems of classification
have been described.1–4
The American Association of Cleft Palate Rehabilitation
(AACPR) separated craniofacial clefts into four categories
based on pathologic location: (1) mandibular process clefts;
(2) naso-ocular clefts; (3) oro-ocular clefts; and (4) oroaural
clefts.6 First, mandibular process clefts group malformations
of the mandible and lower lip. Second, naso-ocular clefts
include malformations located between the nasal ala and the
medial canthus. Third, oro-ocular clefts consist of clefting
anomalies connecting the oral cavity to the orbit between the
medial and lateral canthus. Fourth, oroaural clefts describe
anomalies involving the region from the oral commissure to
the auricular tragus. Subsequent to the AACPR classification,
Boo-Chai made modifications based on surface anatomic
landmarks, including skeletal components.7 The oro-ocular
clefts were subdivided into two types, the oromedial canthus
and orolateral canthus, with the infraorbital foramen as a
reference point.
The Karfik classification has an embryologic and morpho­
logic basis and is divided into five groups: (1) group A:
rhinencephalic malformations; (2) group B: anomalies of the
first and second branchial arch; (3) group C: orbitopalpebral
malformations; (4) group D: craniocephalic malformations
(e.g., Apert and Crouzon syndromes); and (5) group E: atypi­
cal deformities from congenital tumors, atrophy, hypertrophy,

712
SECTION II
CHAPTER 31 • Craniofacial clefts
tissue agenesis and holoprosencephaly at one end (the hypo­
plasias), and frontonasal hyperplasia and excessive tissue (the
hyperplasias) at the other end, median anomalies with normal
tissue volume occupy the middle portion of the spectrum.10
Noordhoff et al. limited the term “holoprosencephaly” to
cases of alobar brain and preferred the term “dysplasia”
rather than “dysgenesis”.11 Thus, for clarification, median
craniofacial dysplasias have been organized and divided into:
I. median craniofacial hypoplasia (tissue deficiency or
agenesis)
II. median craniofacial dysraphia (normal tissue volume
but clefted)
III. median craniofacial hyperplasia (tissue excess or
duplication).
Under each division further subclassification is used to
describe the specific anomalies of the number 0–14 clefts
(Table 31.1).
I:  Median craniofacial hypoplasia (tissue
deficiency and/or agenesis)
A.  Holoprosencephalic spectrum (alobar brain)
1. Cyclopia: described as a single eye in a single orbit.
Arrhinia exists with proboscis often located above the
single orbit. Microcephaly is also a component.
2. Ethmocephaly: severe hypotelorism exists but the orbits
are separate anatomically. Again, arrhinia is present;
however, the proboscis is located in between the
orbits.
3. Cebocephaly: moderate to severe hypotelorism is noted.
There is a proboscis-like rudimentary nose in a more
typical nasal position.
4. Primary palate agenesis: the primary palate, including
the premaxillary segment and associated midline
structures, is absent or severely deficient. Hypotelorism
is seen.
B.  Median cerebrofacial hypoplasia (lobar brain)
In this condition, midline facial hypoplasia and midline
cerebral malformations exist. Unilateral or bilateral cleft lip
and palate may be present.
C.  Median facial hypoplasia
Midline facial hypoplasia exists without gross cerebral
involvement. Unilateral or bilateral cleft lip and palate may
again be present.
D.  Microforms of median facial hypoplasia
Microform variants of median facial hypoplasia may occur
when there are mild deficiencies from maldevelopment of the
median facial structures. Unilateral or bilateral cleft lip and
palate can also be found. This group also includes:
1. Binder syndrome or anomaly (maxillonasal dysplasia)
patients have characteristic flat nasomaxillary facial
region with deficient or absent nasal spine and a
negative overjet from class III anterior incisor
relationship.
and true oblique clefts which cannot be related to any
embryologic fusion line.8 Group A, the rhinencephalic malfor­
mations, was further divided into two subtypes: group A1,
axial malformations derived from frontonasal prominence,
and group A2, paraxial malformations adjacent to the nasal
region. Group B, anomalies of the first and second branchial
arch, was also further divided into two subtypes: group B1,
composed of the lateral otocephalic malformations (craniofa­
cial microsomia, Treacher Collins syndrome, Pierre Robin
sequence, and auricular malformations) and group B2, includ­
ing the mandibular midline malformations.
The Van der Meulen classification used the term “dyspla­
sia” since some of the malformations did not represent true
clefts.9 The defects were labeled by the name of the develop­
mental area (facial processes and bones) that is involved. The
malformations are believed to occur before or during the
fusion of the facial processes, but before the start of ossifica­
tion (Fig. 31.1).
Median craniofacial clefts require special consideration
because of variation in malformations and deformations. With
Fig. 31.1  Embryology of the face. Illustration depicting facial development at
(A) 27 days; (B) 33 days; (C) 39 days; and (D) 46 days with migration of median
nasal prominence, lateral nasal prominence, and maxillomandibular region.
C
D
A
B
Median nasal prominence
Lateral nasal prominence
Maxillomandibular region

713
Classifications of craniofacial clefts
2. Abnormalities of the maxillary central incisors: three
variants of this include:
(a) absent central maxillary incisors
(b) single maxillary central incisor
(c) hypoplastic central maxillary incisors.
3. Absence of upper lip frenulum.
II:  Median craniofacial dysraphia
Median craniofacial dysraphia describes a midline anomaly
that has normal tissue volume, but clefting or an abnormal
separation of midline structures is present. Within the spec­
trum of median craniofacial dysplasia there is a group of
malformations which have normal tissue volume but have
abnormally split (true median cleft lip) or displaced (encepha­
locele). This group is better placed as median tissue defects
midway between hypoplasia and hyperplasia.
A.  True median cleft
A true median cleft may manifest as an isolated cleft of the
upper lip, “0 cleft”, not associated with either hypoplasia
or hyperplasia. Alternatively, a true median cleft may have
tissue deficiency or agenesis, e.g., absent nasal septum. A true
median cleft may also occur with tissue excess, e.g., dupli­
cated nasal septum. With median craniofacial dysraphia a
true median cleft has separated, but there is a normal tissue
amount. The upper lip deformity is a true median cleft lip
with a split between the median globular processes. This
is opposed to a “false” median cleft with agenesis of the
globular processes. With the true median cleft the separation
passes between the central incisors. The cleft may continue
posteriorly as a cleft of the primary and/or secondary palate.
When the cleft encroaches into the interorbital region, orbital
hypertelorism may be seen.
B.  Anterior encephaloceles
An encephalocele is a cystic congenital malformation in which
central nervous system structures have herniated through a
defect in the cranium in communication with cerebrospinal
fluid pathways. They occur between normally developed
zones, where a weakness permits brain to escape. The mass
further pushes surrounding fields apart.
Anterior encephaloceles are divided into frontoethmoidal
and basal groups. In frontoethmoidal encephaloceles, the
defect occurs at the junction of the frontal and ethmoidal
bones (the foramen cecum).12 Nasoethmoidal encephalo­
cele is considered a Tessier number 14 cleft or frontonasal
dysraphia in Mazzola’s morphological classification. Basal
encephaloceles are associated with a defect at or behind the
crista galli and, in some cases, they may protrude through a
defect in the sphenoid bone and are called trans-sphenoidal
encephalocele.13
III:  Median craniofacial hyperplasia (tissue
excess or duplication)
This spectrum of anomalies includes all forms of excess tissue
starting from just thickened or duplicated nasal septum
towards the more severe forms of frontonasal dysplasia.
Subclassification
Description
I. Median craniofacial hypoplasia
Tissue deficiency.
A. Holoprosencephalic spectrum (alobar brain)
Single holistic brain with midline facial hypoplasia or agenesis. Four subclassifications:
cyclopia, ethmocephaly, cebocephaly, primary palate agenesis.
1. Cyclopia
1. Single eye in a single orbit, arrhinia with proboscis often located above the single
orbit and microcephaly.
2. Ethmocephaly
2. Severe hypotelorism but separate orbits. Arrhinia with proboscis located in between
the orbits.
3. Cebocephaly
3. Severe hypotelorism. Proboscis-like rudimentary nose.
4. Primary palate agenesis
4. Premaxillary segment missing of hypoplastic.
B. Median cerebrofacial hypoplasia (lobar brain)
Separate lobes to brain but with midline cerebral malformation; midline facial
hypoplasia.
C. Median facial hypoplasia
Midline facial hypoplasia without gross cerebral involvement.
D. Microforms of median facial hypoplasia
1. Binder anomaly (maxillonasal dysplasia).
2. Central maxillary incisor anomaly.
3. Absent upper lip frenulum.
II. Median craniofacial dysraphia
Normal tissue volume but clefted.
A. True median cleft
Isolated cleft of the upper lip or abnormal split between the median globular process. It
can be an incomplete or complete form.
B. Anterior encephalocele
Cystic malformation in which central nervous structures are abnormally displaced or
herniated through a defect in the cranium.
III. Median craniofacial hyperplasia
Tissue excess or duplication. All forms of excess tissue starting from just thickened or
duplicated nasal septum to the more severe forms of frontonasal dysplasia.
Table 31.1  Classification of median craniofacial dysplasia

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SECTION II
CHAPTER 31 • Craniofacial clefts
migration and resulted in zygomatic abnormalities. Constric­
tion limb deformities (amniotic band syndrome) have also
been associated with rare facial clefting. Coady et al. found a
statistically significant association between craniofacial clefts
and limb ring constrictions.20
Based on animal and human clinical studies, many envi­
ronmental factors have also been shown to cause facial clefts.
These investigations produced four major categories: (1) radia­
tion21,22; (2) infection23,24; (3) maternal metabolic imbalances25;
and (4) drugs and chemicals.26 A considerable number of
drugs and chemicals have teratogenic potential, but few have
been shown to cause craniofacial malformations in humans.
Some medications, including those containing retinoic acid,
are being looked at as a cause of facial malformations.27
Although the teratogenic potential of drugs and their effect
on facial development are known, the critical phase of
embryologic differentiation and development occurs when a
mother may be unknowingly pregnant. Teratologists are
confronted with the problem of multiple factors acting on
numerous pathways and have no simple answer that univer­
sally explains the formation of a particular cleft.
Embryologic craniofacial development
The understanding of normal morphogenesis occurring in the
embryo and fetus allows the clinician to describe and classify
craniofacial clefts of infants and adults. Likewise, the study of
rare craniofacial clefts lends clues to facial and neuroembryol­
ogy. A traditional summary of normal facial development and
newer understanding of genetically determined development
zones of the face based on neuroembryology is outlined
below.
The three primary germ layers, ectoderm, mesoderm, and
endoderm, are the basis for tissue and organ formation.28 In
the third week of gestation, the primitive tissues of the tri­
laminar embryo give rise to notochordal and prechordal
mesoderm. Simultaneously, the rostral ectoderm differenti­
ates to form highly specialized neural crest cells, which are
responsible for the ultimate development of the brain and
midline facial structures.29 The ectoderm forms a neural plate
with bilateral folds which conjoin into a neural tube. During
closure of the neural tube, neural crest cells (mesenchyme)
migrate into underlying tissue, forming pluripotential stem
cells. The embryonic prominences of the face are formed by
the migration of these neural crest cells. A segmental pattern
of ventral migration of neural crest, termed rhombomeres,
provides the precursors of cartilage, bone, muscle, and con­
nective tissue of the face and head.
Any defect in the quantity and quality of this migrating
ectomesenchyme is manifest as a craniofacial malformation
from severe holoprosencephaly to minor clinical stigmata of
craniofacial clefts like dimples or skin tags.30 Another cause
for arrested development causing dysplasias and dystopias
(the abnormal formation and location of structures) is abnor­
mal development or involution of embryologic arteries.31
Starting at the fourth week of gestation, the face assumes
a recognizable form.32 Between 4 and 8 weeks of gestation the
crown–rump length increases from approximately 3.5 mm to
28 mm. The double-layer stomodeal membrane creates the
opening for the primitive mouth. An overhanging frontonasal
prominence represents the superior border of the stomodeum.2
Five prominences (the frontonasal and paired maxillary and
“Frontonasal dysplasia” was the most widely known of
these types of hyperplasias.14 Objections are raised about the
terminology of this condition. Typically, the term “dysplasia”
refers to the whole spectrum of abnormal tissue development
starting from tissue agenesis and hypoplasia all the way to
the other extreme of hyperplasia and excess tissue.
The basic defect of median craniofacial hyperplasia is not
known. Embryologically, if the nasal capsule fails to develop
properly, the primitive brain vesicle fills the space normally
occupied by the capsule, thus producing anterior cranium
bifidum occultum and leading to morphokinetic arrest in the
positioning of the eyes and nostrils, which tend to maintain
their relative fetal positions.15,16 Experiments have shown that
a reduction in the number of migrating neural crest cells
results in these multiple defects.17,18
Other nasal findings may range from a notched broad nasal
tip to completely divided nostrils with hypoplasia and even
absence of the prolabium and maxilla with a median cleft lip.
In addition, variable notching of the ala is described. Occa­
sionally, associated abnormalities include accessory nasal
tags, low-set ears, conductive hearing loss, mild to severe
retardation, basal encephalocele, and agenesis of the corpus
callosum. Importantly, a high incidence of ocular abnormali­
ties is described. More distant anomalies include tetralogy of
Fallot, absence of the tibia, and others. When hypertelorism
is severe or when extracephalic anomalies occur, mental
deficiency appears to be more likely and more severe.14,15,17
The Tessier classification of craniofacial clefts was described
in 1976.1 This classification has proven to be the most complete
and has withstood the test of time. This insightful classifica­
tion was based on the extensive personal experience by Tessier
in the anatomy laboratory, in the operating room, and from
his embryologic knowledge. The terminology is uniform, and
the characterizations of specific features are detailed. It is also
reproducible by clinicians evaluating craniofacial clefts. In
addition, the classification links the clinical observations with
underlying skeletal deformity documented by preoperative
three-dimensional (3D) computed tomography (CT) scan
imaging and confirmed during surgery. Correlation of the
clinical appearance with the surgical anatomic findings
improves the clinical utility of this system for the craniofacial
surgeon. Distinctive features such as Tessier craniofacial clefts
are described below.
Epidemiology and etiologic factors
The true incidence of craniofacial clefts is unknown because
of their rarity and because of the difficulty in recognizing
sometimes subtle physical findings in mild malformations.
However, the incidence of craniofacial clefts has been esti­
mated at 1.4–4.9 per 100 000 live births.1–3 The incidence of rare
craniofacial clefts compared to common cleft lip and palate
malformations may range from 9.5 to 34 per 1000.3
Most rare craniofacial clefts occur sporadically. However,
the role of heredity in the causation of rare craniofacial clefts
seems to occur in Treacher Collins syndrome and in some
familial cases of Goldenhar syndrome. A dominant gene
defect (TCOF-1) causes Treacher Collins syndrome.19 Although
penetrance is somewhat variable, the malformation is very
consistent. In a TCOF-1 gene knock-out animal model,
regional massive cell death affected crest cell mesenchymal

715
Epidemiology and etiologic factors
form the oral commissures. Inadequate neural crest cells
result in macrostomia while excessive tissue produces micro­
stomia or macrostomia. The mandibular prominence lies
between the stomodeum and the first branchial groove, which
delineates the caudal limits of the face. The paired free ends
of the mandibular arch enlarge and converge ventrally during
the sixth week. The lower lip and mandible are developed
from this arch. Paired lateral pharyngeal elevations of the arch
unite to form the anterior portion of the tongue.
The external and middle ear are also formed during the
sixth week of gestation. The tragus and the crus of the helix
are derived from three hillocks at the caudal border of the first
branchial arch. The malleus and incus of the middle ear are
also formed by the first branchial arch. The remainder of the
external ear is formed from three hillocks on the cephalic
border of the second branchial arch. The stapes of the middle
ear is also formed by the second branchial arch.
During a short 4-week period, there is a coordination of cell
migration, cellular interaction, and apoptosis. Failure of this
intricate program will result in clefts that will usually fall
along predictable embryonic lines.
Failure of fusion
Two theories exist that describe how embryologic failure or
errors result in craniofacial cleft malformations. First, the
“fusion failure” theory suggests that clefts are created when
fusion of facial processes fail.36 Second, the “failure of meso­
dermal penetration” theory implicates the lack of mesoderm
and neuroectoderm migration and penetration into the
bilaminar ectodermal sheets as the cause of craniofacial
clefts.37 Although most of the current knowledge is based on
animal cleft lip and palate studies, rare craniofacial clefts may
be produced by similar mechanisms.
The “failure of fusion” theory, proposed by Dursy in 1869
and His in 1892, purported that the free edges of the facial
processes unite in the central region of the face.35 As various
processes fuse, the face gradually forms. When epithelial
contact is established between opposing facial processes,
mesodermal penetration completes the fusion. Dursy sug­
gested that the upper lip is created when finger-like advancing
ends of the maxillary process and the paired global process
unite. He asserted that disruption of this sequence results in
craniofacial cleft anomalies.
Proponents of the mesodermal penetration theory believed
that the finger-like ends of the facial processes do not exist.
Warbrick38 and Stark and Ehrmann39 suggest that the central
facial processes are composed of bilamellar sheets of ecto­
derm. This bilamellar membrane is bordered by epithelial
seams, which delineate the principal processes. During devel­
opment, the mesenchymal tissue migrates and penetrates this
double-layered ectoderm, called the “epithelial wall”. Caudal
to the stomodeum, the lower face is formed by the branchial
arches. The arches consist of a thin sheet of mesoderm, which
lies between the ectodermal and endodermal layers. The
neural crest cells of neuroectodermal origin, which arise from
the dorsolateral surface of the neural tube, migrate under the
ectoderm and supplement the mesoderm of the frontonasal
process and branchial arches.40 Most of the craniofacial skel­
eton is believed to be formed by these neural crest cells. If
neuroectoderm migration and penetration do not occur, the
epithelium breaks down to form a facial cleft. The severity of
mandibular) formed by neural crest migration surround the
stomodeum (see Fig. 31.1). The frontonasal prominence is
formed by neural crest cells migrating ventrally from the
mesencephalic region and contributes to the frontal and nasal
bones. The maxillary and mandibular prominences are formed
by more caudally located migrating neural crest cells that
encounter pharyngeal endoderm in their ventral migration
around the aortic arches.
Optic vesicles appear from lateral invaginations of the
diencephalons and induce lens placodes in ectoderm and
neural crest migration to form the sclera. Defective optic
vesicle formation results in microphthalmos, or anophthal­
mos. The movement of this optic tissue from lateral to medial
results from the narrowing frontonasal prominence and
expansion of the lateral face. Inadequate transition of the eyes
produces hypertelorism, and overmigration produces hypo­
telorism or even median cyclopia.33
During the sixth week, the medial nasal processes enlarge
and coalesce in the midline. The nasal placodes arise from
ectodermal tissue inferolateral to the frontonasal prominence
and cephalad to the stomodeum. Nasal placodes invaginate
into the face to form nasal pits and elevations at the margins
produce a horseshoe-shaped median and lateral nasal promi­
nences. The caudal extensions of the medial nasal processes,
the globular processes, are united with the developing maxil­
lary processes to form the upper lip. The medial nasal process
gives rise to the nasal tip, the columella, the philtrum, and the
premaxilla. The nasal alae are derived from the lateral nasal
processes. The frontonasal process contributes the bridge and
the root of the nose.
The posterior aspect of each nasal pit is separated from the
oral cavity by an oronasal membrane. Failure of this mem­
brane to disintegrate normally leads to choanal atresia.34 The
paired median nasal processes merge with the frontonasal
prominence to form the majority of the frontal process. These
structures gradually enlarge and superiorly displace the
frontonasal prominence. During the sixth week, the two
median nasal processes coalesce in the midline, and their most
caudal limbs, the premaxillary prominence, expand above the
stomodeum. The nasal tip, philtrum, columella, cartilaginous
septum, and primary palate are derived from these paired
median elements. Cephalad to the medial nasal process, the
frontonasal process persists to form the nasal dorsum and
root. Elevation of the lateral nasal prominence creates the
nasal alae. Defects during this development may be midline
and produce arrhinia or a bifid nose.
The maxillary processes are paired mesodermal masses
that lie cephalad to the mandibular arch and ventral to
the optic neuroectoderm. These triangular masses enlarge,
separate from the mandibular arch, and then migrate ven­
trally. The maxillary process ultimately coalesces with the
mesoderm of the globular processes to form the upper lip.
The cheek, maxilla, zygoma, and secondary palate are also
derived from the maxillary processes. Between the maxillary
prominence and lateral nasal prominence a depression exists
with a solid rod of epithelial cells.35 The ends of this rod form
a connection from the nasal pit to the conjunctival sac (naso­
lacrimal duct). With inadequate neural crest cell migration a
fissure within the line of this duct may persist as an oblique
facial cleft.
The stomodeal aperture is reduced by migrating mesen­
chyme fusing the maxillary and mandibular prominences to

716
SECTION II
CHAPTER 31 • Craniofacial clefts
reinforces the importance of Tessier’s descriptions. With
progression of the neuromeric theory, the value of Tessier’s
organization of rare craniofacial clefts should become appar­
ent to embryologists and geneticists.
Patient selection
Distinctive features of Tessier
craniofacial clefts
Tessier developed a classification for rare craniofacial clefts
based on his anatomical and operative observations and
experiences (Fig. 31.2A,B). The clefts are numbered from 0 to
14, follow well-defined zones of the face and orbit, and cor­
relate with embryologic developmental maps (Fig. 31.2C).
The numbered clefts relate soft-tissue clinical features to
underlying bony involvement verified by operative findings
and more recently preoperative 3D CT assessments.
The eyelids and orbits define the horizontal axis dividing
the face into upper and lower hemispheres. Tessier used these
landmarks, because the orbit belongs to both the cranium and
the face. Thus, the orbit separates the numbered cranial clefts
from the facial clefts. In addition, the following combinations
of numeric clefts are often clinically observed: 0 and 14, 1 and
13, 2 and 12, 3 and 11, 4 and 10, 5 and 9, and 6 and 8. Clefts
5–9 are considered lateral clefts, because they pass lateral to
the infraorbital foramen. Tessier cleft number 7 is the most
lateral craniofacial cleft.
The clinical expression of the craniofacial cleft is highly
variable. Tessier reported that the soft-tissue and skeletal
components were seldom affected to the same extent. Skeletal
landmarks are more constant and reliable than the soft-tissue
landmarks. Typically, facial clefts located medial to the infra­
orbital foramen had greater soft-tissue involvement than
clefts found lateral to the foramen. By contrast, facial clefts
located lateral to the infraorbital foramen had greater bony
disruption than clefts found medial to the foramen. Finally,
bilateral forms of the clefts are found in varying combinations,
often creating an asymmetric malformation.
The craniofacial clefts, as described below, use the Tessier
classification to relate soft-tissue features to underlying skel­
etal involvement. The severity of involvement of regional
structures influences the therapeutic strategy. The order of
description below consists of facial clefts from medial to
lateral followed by cranial clefts from lateral to medial.
Number 0 cleft
The number 0 cleft has been called median craniofacial
dysraphia, centrofacial microsomia, frontonasal dysplasia,
median cleft face syndrome, or holoprosencephaly; but, for
accuracy, it is the facial manifestation or lower half of “median
craniofacial dysplasia”, as described above.43,44 Patients with
this midline facial cleft may have a cranial extension or a
number 14 cleft. As mentioned above, the number 0 Tessier
craniofacial clefts are unique in that there may be a deficiency,
normal, or excess tissue. With tissue agenesis and holopros­
encephaly at one end (the hypoplasias), and frontonasal
hyperplasia and excessive tissue (the hyperplasias) at the
other end, median anomalies with normal tissue volume
occupy the middle portion of the spectrum.
the cleft is proportional to the failure of penetration by the
neuroectoderm. Unfortunately, the precise nature of the pro­
posed mechanisms in the formation of rare craniofacial clefts
is not known. Nevertheless, the concepts of fusion and meso­
dermal penetration provide an understanding of the problems
of the rare craniofacial cleft.
Neuromeric theory
Newer understanding of neuroembryology suggests that a
direct relationship exists between the development of the
nervous system and those structures to which its contents are
dedicated. The neural tube is conceived as a series of devel­
opmental zones within the central nervous system.41,42 Six
prosomeres provide a Cartesian system to organize the tracts
and nuclei of the prosencephalon (forebrain). The mesen­
cephalon (midbrain) and rhombencephalon (hindbrain) are
subdivided into two mesomeres and 12 rhombomeres, respec­
tively. Each of these neuromeres is defined by a unique overlap
of several genetic coding zones along the axis of the embryo.
In the hindbrain and caudally to the coccyx these neuromeric
units are defined by the Homeobox series of genes (Hox
genes). In the forebrain, a more complex series of genes is
used, such as Sonic hedgehog (Shh), Wingless (Wnt), and
Engrailed (En).
The unique “barcode” for each neuromeric zone is shared
with all cells exiting from a particular level to form the meso­
derm and endoderm of the embryo. For example, the Hox
gene which codes for the rhombomeres 2 and 3 (which make
up the first pharyngeal arch – PA1) is shared with the meso­
derm that makes up those arches. This same Hox gene is also
shared with the neural crest cells which subsequently move
into the mesodermal “zones” of PA1. The migrating neural
crest cells then provide the instructions for differentiation into
the appropriate facial tissues. Thus, all the bones and soft
tissues of the face can be thought of as genetically determined
“fields” with defined cellular content and a fixed position in
space. With the folding of the embryo, these fields are placed
into their correct topologic positions and a three-dimensional
form results.
This system permits the “mapping” of the face into devel­
opmental zones with distinct spatial origins in their precursor
tissue units. The midline mesoderm of the nasal and ocular
fields is of different origin, innervation, and blood supply
from all surrounding mesodermal elements. When all the
developmental zones are accounted for, the occurrence of
craniofacial clefts is nothing more than an orderly progression
of deficiency states in the precursor fields, resulting in varying
degrees of absence of soft-tissue functional matrix or underly­
ing bone. The anatomic and clinical observations of Tessier
and his classification system are exactly compatible with this
map. Variations include (1) clefts number 2 and 3 fall within
the same zone (cleft number 3 being more posterior) and (2)
clefts number 4 and 5 represent varying degrees of involve­
ment in the same zone of the maxilla. Tessier’s classification
system has been widely adopted by surgeons and other clini­
cians and has stood the test of time. However, geneticists and
embryologists have been slow to embrace his numeric orga­
nization of craniofacial clefts because it could not previously
be understood by existing theories of embryologic develop­
mental. The consistency of these newer neuroembryologic
theories with Tessier’s classification of craniofacial clefts

717
Patient selection
to a severe form of cyclopia, ethmocephaly, or cebocephaly.
The subcategories (see Table 31.1) demonstrate that the sever­
ity of facial anomalies correlate well with the severity of brain
abnormality and mental retardation. A CT scan of the brain
can differentiate between alobar and lobar brain anomalies
and clarify the spectrum of holoprosencephalic patients.
Clinically it may be important to distinguish among patients
Median craniofacial hypoplasia (deficiency of
midline structures)
A deficiency may manifest as hypoplasia or agenesis in which
portions of midline facial structures are missing (Fig. 31.3).
This developmental arrest may range from the mildest form
of hypoplasia of the nasomaxillary region and hypotelorism
Fig. 31.2  Tessier classification of craniofacial clefts. Soft-tissue landmarks are
outlined in (A); skeletal locations of numeric clefts are depicted in (B). Facial clefts
are numbers 0 through 7, and cranial clefts are numbers 8 through 14. Mandibular
midline facial cleft is number 30. (C) Embryology correlation to Tessier facial clefts:
Tessier-numbered craniofacial clefts are shown correlating with growth center
junctions in this 45-day-old fetus.
A
B
14 13 12
11
10
9
8
7
6
5
4
1
1
0
2 3
1 2
3
0
30
1
23
4
5
6
7
7
8
9
10
11
12
13
14
0
C

718
SECTION II
CHAPTER 31 • Craniofacial clefts
Skeletal deficiencies
Skeletal deficiencies range from separation between the upper
central canines to absence of the premaxilla and a cleft of the
secondary palate (see Fig. 31.3C). Nasal deficiency may
include partial or total absence of the septal cartilage and even
nasal bones. The bone defect may extend cephalad into the
area of the ethmoid sinuses and result in hypotelorism or
cyclopia. Nasomaxillary deficiencies like these may be seen in
Binder syndrome.
Median craniofacial dysraphia (normal tissue
volume but clefted)
These Tessier 0 clefts placed between the hypoplasias and
hyperplasias have normal tissue volume but are abnormally
split (true median cleft lip) or displaced (encephalocele).
with poor brain differentiation who may die in infancy from
those with a better prognosis.
Soft-tissue deficiencies
Soft-tissue deficiencies with Tessier 0 clefts include the
upper lip and nose. Agenesis or hypoplasia may result in
a false median cleft lip and absence of philtral columns.
When a wide central cleft exists, it typically extends the
length of the upper lip and up into the nasal floor (see Fig.
31.3A,B). With nasal anomalies, the columella may be nar­
rowed or totally absent. The nasal tip may be depressed
from lack of septal support. The septum may often be
vestigial with no caudal attachment to the palate. Dental
abnormalities may include absent central maxillary incisors,
single maxillary central incisor, and/or hypoplastic central
maxillary incisors.
Fig. 31.3  Number 0 cleft. (A,B) Patient with a true median cleft lip, cleft palate,
hypertelorbitism, and sphenoethmoidal encephalocele (not seen). (A) Preoperative view.
(B) Postoperative view after median cleft lip repair. (C,D) Patient with excessive midline
tissue manifested by bifid nose and an accessory band of skin on the nasal dorsum.
(C) Preoperative view. (D) Postoperative view after initial nasal surgery. (E) Illustration of
skeletal involvement demonstrates separation between the central incisors, widening of the
nasal region, and orbital hypertelorism.
A
B
C
D
E

719
Patient selection
Number 1 cleft
This paramedian facial cleft was first delineated by Tessier.1
Van der Meulen et al. nominated this cleft as a type 3 nasos­
chisis nasal dysplasia.9 The number 1 facial cleft continues
cranially as a number 13 cleft.
Soft-tissue involvement
The number 1 cleft, similar to the common cleft lip, passes
through the cupid’s bow and then the alar cartilage dome.
Notching in the area of the soft triangle of the nose is a distinct
feature (Fig. 31.4A). The columella may be short and broad.
The nasal tip and nasal septum deviate away from the cleft.
Soft-tissue furrows or wrinkles may be present on the nasal
dorsum if the cleft extends in a cephalic direction. The cleft is
evident medially to a malpositioned medial canthus, and
telecanthus may result. With a cranial extension as a number
13 cleft, vertical dystopia is present.
Skeletal involvement
A keel-shaped maxilla exists with the anterior incisors facing
toward the cleft creating an anterior open bite. An alveolar
cleft is rare but would pass between the central and lateral
incisors. This paramedian cleft separates the nasal floor at the
pyriform aperture just lateral to the nasal spine (Fig. 31.4C).
The cleft may extend posteriorly as a complete cleft of the
hard and soft palate. Extension of the cleft in a cephalad
direction is through the junction of the nasal bone and the
frontal process of the maxilla. The nasal bones are displaced
and flattened. Ethmoidal expansion leads to hypertelorism.
Also, there is asymmetry of the greater and lesser sphenoid
wings, the pterygoid plates, and anterior cranial fossa.
Number 2 cleft
Soft-tissue involvement
This other paramedian facial cleft also may begin in the region
of the common cleft lip. However, the nasal deformity is in
the middle third of the alar rim and distinguishes the number
2 cleft (Fig. 31.5). In the number 2 cleft, the ala is hypoplastic,
whereas in the number 1 cleft, the ala is merely notched at the
dome, and in the number 3 cleft, the alar base is displaced.
The lateral aspect of the nose is flattened, and the dorsum is
broad. The eyelid is not involved; the cleft passes medially to
the palpebral fissure. Although the medial canthus is dis­
placed, the lacrimal duct is usually not involved. If the cleft
continues in a cephalad direction as a cranial number 12 cleft,
then distortion of the medial brow is noted.
Skeletal involvement
The number 2 cleft begins between the lateral incisor and the
canine. It extends into the pyriform aperture, lateral to the
septum and medial to the maxillary sinus. A hard- and soft-
palate cleft may occur. The nasal septum may be deviated
away from the cleft. The cleft distorts the nasal bones as it
passes between the nasal bones and the frontal process of the
maxilla. Ethmoidal sinus involvement may result in orbital
hypertelorism. Asymmetry of the greater and lesser sphenoid
wings and anterior cranial base is present.
Soft-tissue involvement
When an isolated cleft of the upper lip is not associated with
tissue deficiency (e.g., absent nasal septum) or tissue excess
(e.g., duplicated septum) it is considered a “true” median cleft
lip. With a true median cleft lip there is a split between the
median globular processes, whereas with a false median cleft
lip an agenesis of the globular processes may occur. An
encephalocele is a cystic congenital malformation in which
central nervous system structures herniate through a defect
in the cranium in communication with cerebrospinal fluid
pathways.45 They occur between normally developed zones,
where a weakness permits the brain to escape the cranial
space. The mass may further push the developmental fields
apart.42
Skeletal involvement
When the true median cleft passes between the central inci­
sors, the cleft can continue posteriorly as a midline cleft palate.
When the cleft encroaches into the interorbital region, hyper­
telorbitism may occur. Anterior encephaloceles are divided
into basal and frontoethmoidal groups. In frontoethmoidal
encephaloceles, the defect occurs at the junction of the frontal
and ethmoidal bones (the foramen cecum).12 Basal encephalo­
celes are associated with a defect at or behind the crista galli
and, in some cases, they may protrude through a defect in the
sphenoid bone.
Median craniofacial hyperplasia (excess of
midline tissue)
This spectrum of midline anomalies includes all forms of
excess tissue starting from just thickened or duplicated nasal
septum (see Fig. 31.3C) towards the more severe forms of
frontonasal dysplasia.
Soft-tissue midline excess
Soft-tissue midline excess tissue may be manifested in the lip
with broad philtral columns or a duplication of the labial
frenulum. The nose may be bifid with a broad columella and
mid-dorsal furrow. The alar and upper lateral cartilages may
be displaced laterally.
Skeletal excess
Skeletal excess in a widened number 0 facial cleft can be
seen as a diastema between the upper central incisors.
A duplicate nasal spine may exist. A characteristic keel-
shaped maxillary alveolus is seen. Anterior teeth are angled
toward the midline creating an anterior open bite. Central
midface height is shortened. The cartilaginous and bone
nasal septum is thickened or duplicated. The nasal bones
and nasal process of the maxilla are broad, flattened, and
displaced laterally from the midline. Ethmoidal and sphe­
noidal sinuses may be enlarged, contributing to symmetrical
widening of the anterior cranial fossa and hypertelorism.
The cribriform plate is low, and the breadth of the crista
galli is exaggerated. The body of the sphenoid is broadened
with displacement of the pterygoid plates away from the
midline.

720
SECTION II
CHAPTER 31 • Craniofacial clefts
occurrence of one-third on the right, one-third on the left, and
one-third bilateral. When bilateral clefting occurs, a number
4 or 5 facial cleft may be seen contralateral to the number
3 cleft.
Soft-tissue involvement
The number 3 cleft begins similar to a number 1 and 2 cleft,
passing through the philtral column and floor of the nose.
Number 3 cleft
The number 3 cleft is the most common of the Tessier
craniofacial clefts. Morian reported the first case and later
classified this as a Morian type I cleft.46 It is also referred to
as a Tessier oro-naso-ocular cleft. The cephalad continuation
of the cleft is a number 11 cleft. In contrast to a common
cleft lip and palate, a number 3 cleft has the following: (1)
an equal distribution between males and females and (2) an
Fig. 31.4  Number 1 cleft. (A) Patient with notched left alar dome and orbital dystopia.
(B) Same patient at maturity after left soft triangle reconstruction with composite graft.
(C) Skeletal involvement is through the piriform aperture just lateral to the nasal spine and
septum. The orbit is displaced laterally.
A
B
C

721
Patient selection
may occur directly on to the cheek instead of into the nasal
cavity.
The medial canthus is inferiorly displaced and may be
hypoplastic. Colobomas of the lower eyelid are medial to the
inferior punctum. In mild forms, colobomas may be the only
obvious evidence of this cleft. In mild cases it is important to
check a CT scan for bony involvement and maintain an index
Deficiency of tissue between the alar base and lower eyelid
results in a shortened nose on the affected side. The cleft
passes between the medial canthus and the inferior lacrimal
punctum (Fig. 31.6A). The lacrimal system, particularly the
lower canaliculus, is disrupted. Blockage of the nasolacrimal
duct and recurrent infections of the lacrimal sac are common.
The inferior punctum is displaced downward, and drainage
A
Fig. 31.5  Number 2 cleft. (A) Patient with hypoplasia of the middle third of the
right nostril rim causing the appearance of alar base retraction. The lateral nose is
flattened. The medial border of the eyebrow is also distorted as evidence of a
number 12 cranial cleft. There is also orbital dystopia and displacement of the right
medial canthus. (B) Skeletal involvement shows deformity of the piriform aperture
and nasal bone.
B
A
Fig. 31.6  Number 3 cleft. (A) Patient with complete form has a right cleft lip and
palate and severe shortening of tissues between the right alar base and medial
canthus. The right nasal ala is displaced superiorly, the medial canthus is displaced
inferiorly, and the nasolacrimal system is disrupted. (B) Skeletal involvement is
between the lateral incisor and the canine extending up through the lacrimal groove.
The cleft creates a direct communication among the orbital, maxillary sinus, nasal,
and oral cavities.
B

722
SECTION II
CHAPTER 31 • Craniofacial clefts
orbital cavity but not the nasal cavity. The cleft then passes
medial to the infraorbital foramen. This landmark defines
the boundary between the medial number 4 facial cleft and
lateral number 5 facial cleft. The number 4 cleft terminates
at the medial aspect of the inferior orbital rim. With an
absent medial orbital floor and rim, the globe may prolapse
inferiorly. In bilateral cases the medial midface and premax­
illa are protrusive. The sphenoid body is asymmetric, and
pterygoid plates are displaced but the anterior cranial base
is unaffected.
Number 5 cleft
This facial cleft is the rarest of the oblique facial clefts. It has
been called the oculofacial cleft II, Morian III cleft, a lateral
maxillary dysplasia, or an oro-ocular type II cleft (AACPR
classification).1,48 The cephalad progression of the number 5
cleft is the number 9 cleft. One-quarter of cases are unilateral,
one-quarter of cases are bilateral, and one-half of cases are
combined with another facial cleft.
Soft-tissue involvement
The number 5 facial cleft begins just medial to the oral com­
missure and courses along the cheek lateral to the nasal ala
(Fig. 31.8A,B). The cleft terminates in the lateral half of the
lower eyelid. Although the globe is typically normal, microph­
thalmia may occur.
Skeletal involvement
The alveolar cleft begins lateral to the canine in the region of
the premolars. In contrast to the number 4 cleft, the number
5 cleft then courses lateral to the infraorbital foramen and
terminates in the lateral aspect of the orbital rim and floor (see
Fig. 31.8A,C). The cleft is separated from the inferior orbital
fissure. The maxillary sinus may be hypoplastic. Prolapse of
orbital contents through the lateral orbital floor defect into the
maxillary sinus causes vertical orbital dystopia. The lateral
orbital wall may be thickened and the greater sphenoid wing
abnormal. The cranial base is normal.
Number 6 cleft
This zygomaticomaxillary cleft represents an incomplete form
of Treacher Collins syndrome. It was nominated a maxillozy­
gomatic dysplasia by Van der Meulen et al.44 Similar and often
more severe cleft facial features are seen in Nager syndrome.
Nager syndrome patients also may have radial club deformi­
ties of the upper extremities.
Soft-tissue involvement
The cleft is often identified as a vertical furrow, due to hypo­
plastic soft tissue, from the oral commissure to the lateral
lower eyelid (Fig. 31.9A). This line of hypoplasia runs through
the zygomatic eminence along an imaginary line from the
angle of the mandible to the lateral palpebral fissure. The
lateral palpebral fissure is pulled downward. The lateral
canthus is displaced inferiorly. This may create an appearance
of a severe lower lid ectropion and an antimongoloid slant.
Colobomas appear in the lateral lower eyelid and mark the
cephalic end of the cleft.
of suspicion for disruption of the lacrimal system. Involve­
ment of the globe is rare, but microphthalmia may occur.
Typically, the eye is malpositioned inferiorly and laterally.
Injury to the eye, including corneal erosions, ocular perfora­
tion, and loss of vision, may result from desiccation unless the
globe is protected.
Skeletal involvement
Osseous characteristics of this facial cleft include involvement
of the orbit and direct communication of the oral, nasal, and
orbital cavities (Fig. 31.6B). The cleft begins between the
lateral incisor and the canine. In contrast to the number 1 and
2 facial clefts, the anterior maxillary arch is flat in the number
3 cleft. The number 3 cleft disrupts the frontal process of the
maxilla and then terminates in the lacrimal groove. In the
severest form, the cleft is bilateral and skeletal disruption is
significant. With bilateral cases the contralateral facial cleft
may be a number 4 or 5 cleft. There may be narrowing of the
ethmoid and sphenoid sinuses. Both the orbital floor and
anterior cranial base are displaced inferiorly.
Number 4 cleft
The number 4 cleft occurs lateral to the nose and other
median facial structures. The cleft has been called melos­
chisis (separation of cheek). Dick reported the first case in
the English literature and von Kulmus may have recorded
the initial description in Latin in 1732.47 The cleft has also
been classified or referred to as oro-ocular (AACPR), oro­
facial cleft,1 and medial maxillary dysplasia.1,44 The cranial
continuation of the cleft is the number 10 cleft. For unilateral
number 4 facial clefts it is estimated that a distribution exists
for right to left side of 2 to 1.3 and a distribution exists for
male to female of 2.5 to 1. In contrast, bilateral cases occur
in equal numbers of males and females. Bilateral cases are
associated with contralateral craniofacial clefts numbers 3, 5,
and 7.
Soft-tissue involvement
As opposed to numbers 1, 2, and 3 facial clefts, the number 4
cleft begins lateral to Cupid’s bow and the philtral column
and medial to the oral commissure (Fig. 31.7A). The orbicu­
laris oris muscle is located in the lateral lip element with no
muscle centrally. The cleft passes lateral to the nasal ala.
Although the ala is not involved and the nose is intact, it is
displaced superiorly. Bilateral involvement pulls the nose
upward (Fig. 31.7B). The cleft extends through the cheek and
into the lower eyelid lateral to the inferior punctum. The
lower eyelid and lashes may extend directly into the lateral
aspect of the cleft. The medial canthus and nasolacrimal
system are normal. The globe is typically normal, but
microphthalmia and anophthalmos may be seen.
Skeletal involvement
Skeletal involvement is usually less extensive than the
number 3 cleft. The alveolar cleft begins between the lateral
incisor and the canine (Fig. 31.7C). The cleft extends lateral
to the pyriform aperture to involve the maxillary sinus.
The medial wall of the maxillary sinus is intact. A conflu­
ence exists between the oral cavity, maxillary sinus, and

723
Patient selection
Fig. 31.7  Number 4 cleft. (A) Patient with left-side cleft that begins lateral to Cupid’s bow
and terminates in the lower eyelid medial to the punctum. (B) Bilateral clefting of the upper lip
lateral to Cupid’s bow and malar extension lateral to the nose up to the lower eyelids with
asymmetric involvement. (C) Skeletal involvement begins between the lateral incisor and
canine and extends through the maxilla between the infraorbital foramen and the piriform
aperture. The orbit, maxillary sinus, and oral cavities communicate.
A
B
C
Skeletal involvement
The number 6 facial cleft is along the zygomaticomaxillary
suture separating the maxilla and zygoma (Fig. 31.9B). There
is no alveolar cleft, but a short posterior maxilla may result in
an occlusal tilt. Choanal atresia is common. The cleft enters
the orbit at the lateral third of the orbital rim and floor. It
connects to the inferior orbital fissure. The zygoma is hypo­
plastic with an intact zygomatic arch. There is narrowing of
the anterior cranial fossa. The sphenoid is normal.
Number 7 cleft
This temporozygomatic facial cleft is the most common
craniofacial cleft. Other descriptive terms of this cleft
include craniofacial microsomia, hemifacial microsomia,
otomandibular dysostosis, first and second branchial arch
syndrome, auriculobranchiogenic dysplasia, hemignathia
and microtia syndrome, oroaural cleft (AACPR), a group B1
lateral otocephalic branchigenic deformity, and zygotemporal
dysplasia.48–51 Goldenhar syndrome (oculo-auriculo-vertebral
spectrum) is an autosomal-dominant more severe form addi­
tionally with epibulbar dermoids and vertebral anomalies.52
The number 7 cleft is also seen in Treacher Collins syndrome.
The incidence is approximately 1 in 5600 births. There is a
slight (3 : 2) male predominance and bilateral involvement.
Soft-tissue involvement
The cleft begins at the oral commissure and runs to the preau­
ricular hairline. The intensity of expression varies from a mild
broadening of the oral commissure with a preauricular skin tag
to a complete fissure extending toward the microtic ear (Fig.
31.10). Typically the cleft does not extend beyond the anterior

724
SECTION II
CHAPTER 31 • Craniofacial clefts
junction. Tessier believed that the cleft is centered in the region
of the zygomaticotemporal suture. The posterior maxilla and
mandibular ramus are hypoplastic in the vertical dimen­
sion, creating an occlusal plane that is canted cephalad on
the affected side. The coronoid process and condyle are also
often hypoplastic and asymmetric, contributing to a posterior
open bite on the affected side. The zygomatic body is severely
malformed, hypoplastic, and displaced. In the most severe
form, the zygomatic arch is disrupted and is represented by
a small stump. The malpositioned lateral canthus is caused
by a hypoplastic zygoma that results in the inferiorly dis­
placed superolateral angle of the orbit. Occasionally, severely
deforming number 7 clefts can cause true orbital dystopia.
The abnormal anterior zygomatic arch continues posteriorly
as a normal zygomatic process of the temporal bone. The
cranial base is asymmetric and tilts causing an abnormally
positioned glenoid fossa. The anatomy of the sphenoid is
border of the masseter. However, the ipsilateral tongue, soft
palate, and muscles of mastication (cranial nerve V) may be
underdeveloped. The parotid gland and parotid duct may
be absent. Facial nerve weakness (cranial nerve VII) may be
present. External ear deformities range from preauricular
skin tags to complete absence. External ear and middle-ear
abnormalities have been documented by Longacre et al.,51
Grabb,53 and Converse et al.54 Preauricular hair is usually
absent in patients with craniofacial microsomia. Patients with
Treacher Collins syndrome often have preauricular hair from
the temporal region pointing to the oral commissure. The
ipsilateral soft palate and tongue are often hypoplastic.
Skeletal involvement
Osseous anomalies in a number 7 cleft include a wide range.
The skeletal cleft passes through the pterygomaxillary
Fig. 31.8  Number 5 cleft (left) and number 4 cleft (right). (A) This patient demonstrates
bilateral facial clefts: The number 5 cleft begins just medial to the oral commissure and extends
up the lateral cheek to the middle of the eyelid, while the more medial number 4 cleft begins
lateral to Cupid’s bow and extends up to the medial third of the lower eyelid. (B) Postoperative
view of same patient after repair of bilateral clefts. (C) Skeletal involvement in the number 5
cleft (left) begins at the premolars and extends lateral to the infraorbital foramen, while the
number 4 cleft begins between the lateral incisor and canine and passes medial to the
infraorbital foramen.
A
B
C

725
Patient selection
divides the facial clefts from the cranial clefts. The number 8
cleft rarely occurs alone but is usually associated with other
craniofacial clefts. It appears to be the cranial extension of the
number 6 cleft. The bilateral occurrence of the combination of
numbers 6, 7, and 8 craniofacial clefts is unique. Tessier
believed that this pattern of clefts best describes Treacher
Collins syndrome (Fig. 31.12). Infants with Goldenhar syn­
drome will typically have more soft-tissue involvement, while
those with Treacher Collins syndrome tend to have more
severe bony abnormalities.
Soft-tissue involvement
The number 8 cleft extends from the lateral canthus to the
temporal region. A dermatocele may occupy the coloboma of
the lateral commissure. Occasionally hair markers can be seen
abnormal, and there can be a rudimentary medial and lateral
pterygoid plate.
Number 8 cleft
This frontozygomatic cleft located at the lateral canthus is the
equator of the Tessier craniofacial time zones (Fig. 31.11). It is
the temporal continuation of the orolateral canthus cleft
(AACPR), the commissural clefts of the ophthalmo-orbital
disorders, and zygofrontal dysplasia.44 The number 8 cleft
A
Fig. 31.9  Number 6 cleft. (A) Patient with an incomplete form of Treacher Collins
syndrome shows bilateral linear malar hypoplasia. (B) Skeletal involvement occurs
in the region of the zygomaticomaxillary suture. The zygoma is hypoplastic.
B
Fig. 31.10  Number 7 cleft. Patient with a complete fissure of the right oral
commissure which extends toward the external ear, resulting in macrosomia.
(Courtesy of D Hurwitz)
Fig. 31.11  Number 8 cleft. In this patient’s left eye the lateral commissure of the
palpebral fissure is obliterated by a dermatocele. This cleft separates the facial and
cranial clefts.

726
SECTION II
CHAPTER 31 • Craniofacial clefts
this defect there is soft-tissue continuity of the orbit and
temporal fossa.
Number 9 cleft
This upper lateral orbit cleft is the rarest of the craniofacial
clefts. The number 9 cleft begins the medial movement
through the cranial clefts. This defect was nominated fronto­
sphenoid dysplasia by Van der Meulen.44 It is the cranial
extension of the number 5 facial cleft.
Soft-tissue involvement
The number 9 cleft is manifested by abnormalities of the
lateral third of the upper eyelid and eyebrow. The lateral
canthus is also distorted. In the severe form, microphthalmia
is present (Fig. 31.13). The superolateral bony deficiency of the
along a line between the temporal area and the lateral canthus.
The soft-tissue malformation presents as a true lateral com­
missure coloboma (dermatocele) with absence of the lateral
canthus. Abnormalities of the globe, in the form of epibulbar
dermoids, are also often present, especially in Goldenhar
syndrome.
Skeletal involvement
The bony component of the cleft occurs at the frontozygomatic
suture. Tessier noted a notch in this region in patients with
Goldenhar syndrome (combination number 6, 7, and 8 clefts).
In the complete form of Treacher Collins syndrome (combina­
tion number 6, 7, and 8 clefts) the zygoma may be hypoplastic
or absent and the lateral orbital wall missing (see Fig. 31.12C).
Thus, the lateral palpebral fissure’s only support is the greater
wing of the sphenoid and downward slanting occurs. With
Fig. 31.12  Combination number 6, 7, and 8 cleft. (A) Patient with Treacher Collins
syndrome demonstrates malar hypoplasia and antimongoloid slant to palpebral fissure.
(B) Postoperative image after malar reconstruction with cranial bone grafts and eyelid
reconstruction with lid switch flaps. (C) Skeletal involvement in the complete form includes
absence of the zygoma, lateral orbital wall (greater wing of sphenoid provides remaining
portion of the lateral wall), and lateral orbital floor.
A
B
C

727
Patient selection
Number 10 cleft
This upper central orbital cleft has also been classified as a
frontal dysplasia group.44 The number 10 cleft is the cranial
extension of a number 4 cleft.
Soft-tissue involvement
The number 10 cleft begins at the middle third of the upper
eyelid and eyebrow. The lateral eyebrow may angulate tem­
porally (Fig. 31.14A). The palpebral fissure may be elongated
with an amblyopic eye displaced inferolaterally (Fig. 31.14B).
The entire upper eyelid may be absent in severe forms (able­
pharia). Colobomas and other ocular anomalies may be
present. Frontal hair projection may connect the temporopa­
rietal region to the lateral brow.
Skeletal involvement
The bony component of the number 10 cranial cleft occurs in
the middle of the supraorbital rim just lateral to the superior
orbital foramen (Fig. 31.14C). Often an encephalocele occupies
the defect through the frontal bone and a prominent bulge is
observed in the forehead. The orbit may be deformed with
a lateroinferior rotation. Orbital hypertelorism may result
in severe cases. The anterior cranial base may also be
distorted.
Number 11 cleft
This upper medial orbital cleft is the cranial extension of the
number 3 cleft. Van der Meulen included this malformation
in the frontal dysplasia group.44
Soft-tissue involvement
The medial third of the upper eyelid may show involvement
with a coloboma. The upper eyebrow may have a disruption
evident which extends up to the frontal hairline (Fig. 31.15).
A tongue-like projection at the medial third of the frontal
hairline may also be identified.
Skeletal involvement
The number 11 cleft may be seen as a cleft in the medial third
of the supraorbital rim if it passes lateral to the ethmoid bone.
If the cleft passes through the ethmoid air cells to produce
extensive pneumatization then orbital hypertelorism is seen
clinically. The cranial base and sphenoid architecture, includ­
ing the pterygoid processes, are symmetric and normal.
Number 12 cleft
The number 12 cleft is the cranial extension of the number 2
cleft.
Soft-tissue involvement
The soft-tissue cleft lies medial to the medial canthus, and
colobomas extend to the root of the eyebrow. There is a lateral
displacement of the medial canthus with an aplasia of the
medial end of the eyebrow. There are no eyelid clefts. The
forehead skin is normal with a short downward projection of
the paramedian frontal hairline (Fig. 31.16A).
orbits allows for a lateral displacement of the globes. The cleft
then extends cephalad into the temporoparietal hair-bearing
scalp. The temporal hairline is anteriorly displaced, and a
temporal hair projection is often seen in the number 9 cleft.
Furthermore, a cranial nerve VII palsy in the forehead and
upper eyelid is common.
Skeletal involvement
The bony defect of the number 9 cranial cleft extends through
the superolateral aspect of the orbit. Distortion of the upper
part of the greater wing of the sphenoid, the squamosal
portion of the temporal bone, and surrounding parietal bones
may be present. This hypoplasia of the greater wing of the
sphenoid results in a posterolateral rotation of the lateral
orbital wall. The pterygoid plates may be hypoplastic. There
may be a reduction in the anteroposterior dimension of the
anterior cranial fossa.
A
Fig. 31.13  Number 9 cleft. (A) Patient with left-side rare number 9 cleft through
the superolateral orbital roof with anophthalmia. (B) Same patient at maturity after
nasal and orthognathic reconstruction.
B

728
SECTION II
CHAPTER 31 • Craniofacial clefts
Soft-tissue involvement
There is typically a paramedian frontal encephalocele, which
is located between the nasal bone and the frontal process of
the maxilla. The soft-tissue cleft is medial to intact eyelids and
eyebrows. The medial end of the eyebrow, however, can be
displaced inferiorly. A V-shaped frontal hair projection can
also be seen.
Skeletal involvement
Changes in the cribriform plate are the hallmark of a number
13 cleft. The paramedian bony cleft traverses the frontal bone,
then courses along the olfactory groove. There is widening of
the olfactory groove, the cribriform plate, and the ethmoid
sinus, which results in hypertelorism. A paramedian frontal
encephalocele can cause the cribriform plate to be displaced
inferiorly, leading to orbital dystopia. Unilateral and bilateral
Skeletal involvement
The number 12 cleft passes through the flattened, frontal
process of the maxilla (Fig. 31.16B). It then travels superiorly,
increasing the transverse dimension of the ethmoid air cells,
producing orbital hypertelorism and telecanthus. The frontal
and sphenoid sinuses are also pneumatized and enlarged. The
remainder of the sphenoid and frontal bones are normal.
The frontonasal angle is obtuse. The cleft is located lateral to
the olfactory groove, thus the cribriform plate is normal in
width. Encephaloceles have not been observed with this cleft.
The anterior and middle cranial fossae are widened on the
cleft side, but otherwise normal.55
Number 13 cleft
The number 13 cleft is the cranial extension of the paramedian,
facial number 1 cleft (Fig. 31.17).
Fig. 31.14  Number 10 cleft. (A) Patient with bilateral cleft in the center of the left superior
orbital rim and microphthalmia. (B) Patient with fronto-orbital encephalocele in the mid right
forehead that fills the void from the cleft defect. The right globe is displaced downward.
(C) Skeletal defect and asymmetric hypertelorism are demonstrated on the right.
A
B
C

729
Patient selection
fetal position. Flattening of the glabella and extreme lateral
displacement of the inner canthi are also seen. The periorbita,
including the eyelids and eyebrows, are otherwise normal. A
long midline projection of the frontal hairline marks the
superior extent of the soft-tissue features of this midline
cranial cleft.
Skeletal involvement
The frontal encephalocele herniates through a medial frontal
defect. The caudal aspect of the frontal bone is flattened,
giving the glabellar region a flattened and indistinct position.
forms of the number 13 cleft exist, similar to most of the other
craniofacial clefts. When the cleft is bilateral, some of the most
extreme cases of hypertelorism can be seen.2
Number 14 cleft
The number 14 cleft occurs in the midline forehead and
cranium as an extension of number 0 clefts. As in the number
0 clefts or median craniofacial dysraphia, described above,
there may be a tissue deficiency, tissue excess, or normal
amount of tissue but clefted in some way.
Soft-tissue involvement
Similar to its facial counterpart, the number 14 cleft can be
produced as an agenesis or an overabundance of tissue. When
agenesis occurs, orbital hypotelorism is generally seen.
Included in this group of craniofacial malformations are the
holoprosencephalic disorders, which include cyclopia, ethmo­
cephaly, and cebocephaly (Fig. 31.18A). The cranium is typi­
cally microcephalic, and there is hypotelorism. A complete
absence of midline cranial base structures can occur, causing
the orbits to coalesce. Malformations of the forebrain are
usually proportional to the degree of facial abnormality. An
extensively involved number 14 cleft can severely handicap
the newborn, and life expectancy is usually limited from
hours to months.
At the other end of the spectrum, hypertelorism is associ­
ated with the number 14 cleft (Fig. 31.18B). The terms fronto­
nasal and frontonasoethmoid dysplasia were used by Van der
Meulen to categorize this group.44 Lateral displacement of the
orbits can be produced by midline masses such as a frontona­
sal encephalocele or a midline frontal encephalocele (Fig.
31.18C,D). Cohen et al. thought that the basic fault in embryo­
logic development lies in the malformation of the nasal
capsule, and the developing forebrain remains in a low posi­
tion.56 A morphokinetic arrest of the normal medial movement
of the eyes occurs and the orbits remain in their widespread
Fig. 31.15  Number 11 cleft. Patient with coloboma in medial third of left upper
eyelid extending through the medial third of the eyebrow.
A
Fig. 31.16  Number 12 cleft. (A) Patient with right cleft has orbital hypertelorism
and a disturbance of the left medial eyebrow. (B) Skeletal involvement of right-side
clefting through the frontal process of the maxilla displacing the orbit laterally and
hypertelorism results.
B

730
SECTION II
CHAPTER 31 • Craniofacial clefts
Number 30 cleft
The median cleft of the lip and mandible, first described by
Couronne, are extremely rare with less than 100 cases
reported.58 These median clefts of the lower lip and mandible
are caudal extensions of the number 14 cranial cleft and
number 0 facial cleft (Fig. 31.19). The number 30 clefts include
mandibular process clefts, midline branchiogenic syndrome,
and intermandibular dysplasia.
Soft-tissue involvement
Soft-tissue involvement of this midline cleft may be as mild
as a vermillion notch in the lower lip. However, often the
entire lower lip and chin may be involved. The anterior
tongue may be bifid and attached to the split mandible by
a dense fibrous band. Ankyloglossia and total absence of
the tongue have been reported with midline mandibular
clefts. The anterior neck strap muscles are often atrophic
and replaced by dense fibrous bands which may restrict
chin flexion.
Skeletal involvement
Skeletal involvement is typically a cleft between the central
incisors extending into the mandibular symphysis. This
anomaly is thought to be caused by failure of fusion of the
first branchial arch. However, associated neck anomalies are
felt to be caused by failure of fusion of other lower branchial
arches. The hyoid bone at times is absent and the thyroid
cartilages may fail to form completely.
Number 31 cleft
The paramedian cleft of the lip and mandible are even more
rare than the median lower jaw clefts. Despite Dr. Tessier’s
vast experience, he did not evaluate a patient with a lower
jaw paramedian cleft and, thus, did not complete his number­
ing system. For these newly described lower jaw paramedian
clefts the numbering system counts up from number 30.
Number 31 clefts involve the lower lip and mandible just
adjacent to the midline (Fig. 31.20).59,60
Soft-tissue involvement
Soft-tissue involvement may be a vermillion notch or depres­
sion in the lower lip just off the midline. A linear scar and skin
tag may also be noticed to the left or right of the median lip
and chin.
Skeletal involvement
Skeletal involvement may involve alveolar notching between
the central incisor and lateral incisor. The cleft may also
extend further into mandible in this parasymphyseal
region.
Number 32 cleft
This other paramedian lower lip and mandibular cleft is
located just lateral to the number 31 cleft. The number 32 cleft
involves a lower lip defect approximately halfway between
the midline and oral commissure (Fig. 31.21).
No pneumatization of the frontal sinus is evident; however,
the sphenoid sinus is extensively pneumatized. The crista
galli and the perpendicular plate of the ethmoid are bifid, and
there is an increased distance between the olfactory grooves
(Fig. 31.18E). When the crista galli is severely enlarged, pres­
ervation of the olfactory nerve is often not possible during the
surgical correction of hypertelorism. The crista galli and eth­
moids are widened and caudally displaced. Consequently, the
cribriform plate, which is normally located 5–10 mm below
the level of the orbital roof, can be caudally displaced up to
20 mm.57 The greater and lesser wings of the sphenoid are
rotated and result in a relative shortening of the middle cranial
fossa. The anterior cranial fossa is upslanting, causing a har­
lequin eye deformity on plain radiographs.
A
Fig. 31.17  Number 13 cleft. (A) Patient with a right cleft which begins cleft
through the right alar dome (number 1 cleft) and extends to the frontal bone to
cause right-sided telorbitism. (B) Postoperative image after corrective facial
bipartition and nasal reconstruction with forehead flap.
B

731
Patient selection
may be seen because of the mandibular separation near the
molar region (Fig. 31.22).61
Soft-tissue involvement
Soft-tissue involvement may be a vermilion notch or depres­
sion in the lower lip just medial to the oral commissure.
Skeletal involvement
Skeletal involvement may involve alveolar notching or com­
plete separation in the area of the premolar just posterior to
the mental foramen and anterior to the first molar.
Summary
The clinical expression of craniofacial clefts is highly variable
and ranges from a mild, barely noticeable forme fruste
Soft-tissue involvement
Soft-tissue involvement may be a vermilion depression and
regional attenuation or hypoplasia of the orbicularis oris
in the lower lip halfway between the midline and oral
commissure.
Skeletal involvement
Skeletal involvement may involve alveolar notching lateral to
the lateral incisor or extend into the mandible parasymphyseal
region just medial to the mental foramen.
Number 33 cleft
The number 33 cleft is lateral to the number 32 cleft and may
be associated with other severe facial deformities like Golden­
har (oculoauriculovertebral) syndrome. Severe malocclusion
Fig. 31.18  Number 14 cleft. (A) Patient with number 14 cleft, holoprosencephaly, a form of
median craniofacial hypoplasia (tissue deficiency). (B) Patient with number 14 cleft,
frontonasoethmoidal encephalocele, a form of median craniofacial dysraphia (normal tissue
but clefted). (C) Patient with number 14 cleft, craniofrontonasal dysplasia, a form of median
craniofacial hyperplasia (excess tissue). (D) Postoperative image after corrective
encephalocele repair, medial orbit repositioning, and nasal bone grafting. (E) Skeletal
involvement shows displacement of the frontal process of the maxilla, the nasal bones, and
medial orbital walls laterally. This large defect is often occupied by an encephalocele.
A
B
C
D
E

732
SECTION II
CHAPTER 31 • Craniofacial clefts
proper timing and stages for corrective surgery.2–4 If there are
functional problems, like ocular exposure, or airway prob­
lems, or if the malformation is severe, then surgery should be
performed early. If the malformation is mild, surgery should
be delayed. During infancy (3–12 months), soft-tissue clefts
and midline cranial defects (e.g., encephaloceles) may be cor­
rected. Midface and orbital reconstruction with bone grafting
may be performed in older children (6–9 years). Orthognathic
procedures are delayed until skeletal maturity (14 years or
greater).
Surgical techniques used for correction of craniofacial clefts
depend upon the anatomic regions that are involved. For
timing and general corrective protocols, the deformities may
be grouped into (1) midline and paramedian clefts (numbers
0–14, numbers 1–13, 2–12, or other combinations); (2) oro-
naso-ocular clefts (numbers 3–11, 4–10, 5–9); and (3) lateral
(microform) to a disfiguring, complete defect of skeletal and
soft tissue. Tessier’s description of craniofacial clefts based on
bony and soft-tissue landmarks provides a classification
which can be validated by neuroembryology. Craniofacial
clefts, far from being considered oddities, will allow plastic
surgeons in the future to refine and understand the develop­
mental architecture of the face.
Treatment
Treatment of craniofacial clefts
Standardized treatment plans are not always possible because
of the variety of craniofacial clefts and levels of severity.
However, guiding principles are helpful in determining the
Fig. 31.19  Number 30 cleft. (A) Close-up intraoral view of patient with number 30 cleft showed dental
separation of central incisors and bony cleft. (B) Preoperative view of patient with number 30 cleft with a
deep tongue groove and fusion to the clefted mandible. (C) Postoperative view of same patient with number
30 midline mandibular cleft after skeletal and soft-tissue repair. (Courtesy of Cassio Raposo do Amaral, MD.)
A
B
C

733
Treatment
muscular continuity as in a common cleft lip repair. Microform
variations may be corrected with intraoral muscle repair,
Z-plasty of the white roll, and no cutaneous scar (Fig. 31.23).
For lateral clefts, lip reconstruction is performed with a
rotation-advancement repair.
For the nose, a median cleft correction may be done in early
childhood with a primary rhinoplasty used to unify bifid
nasal cartilages after excision of intervening fibrofatty tissue.
For paramedian defects, the nose is reconstructed with carti­
lage grafts or composite skin-cartilage grafts, particularly for
asymmetries and soft triangle notching. Alar retraction may
be corrected with local rotation flaps or Z-plasty flaps. Sec­
ondary nasal reconstruction may be performed with a septo­
plasty or even cantilevered cranial bone grafts. Correction of
clefts including constellation of numbers 6, 7, and 8 clefts like
Treacher Collins syndrome of craniofacial microsomia.
First, for midline clefts, proper diagnosis into median cra­
niofacial hypoplasia (tissue deficiency), median craniofacial
dysplasia (normal tissue volume but separated), or median
craniofacial hyperplasia (tissue excess) is helpful for proper
treatment planning. Midline clefts may have symmetrical
deformities like bifidity of the nasal cartilages or symmetrical
hypertelorbitism, while paramedian clefts manifest asym­
metry with unilateral nasal notching or horizontal and vertical
orbital dystopia. For the upper lip, correction of cleft defects
involves aligning the white roll and vermillion, and restoring
Fig. 31.20  Number 31 cleft. Left side paramedian lower lip cleft just off the
midline with a full-thickness break in the vermilion, a linear skin scar and a chin
skin tag. (Courtesy of Steve Wall, MD.)
Fig. 31.21  Number 32 cleft. Left side paramedian cleft of the lower lip and skin
midway between the midline and left oral commissure.
A
Fig. 31.22  Number 33 cleft. (A) Intraoral view of paramedian mandibular cleft
between premolar and first molar with redundant gingiva. (B) 3D cone beam CT
scan shows osseous defect in left side mandibular body but with inferior border
intact.
B

734
SECTION II
CHAPTER 31 • Craniofacial clefts
down-fracture). Anterior encephalocele reduction and cranial
base bone graft may be necessary before midline fixation (Fig.
31.24).62 For these large anterior encephaloceles, subsequent
cleft palate repair may require a pharyngeal flap for nasal
lining. With paramedian (numbers 1–13, 2–12) clefts, vertical
adjustment of a facial bipartition segment may be necessary
to correct vertical, as well as horizontal, orbital dystopia (Fig.
31.25). For these procedures, the misaligned occlusal plane is
usually temporary and correctable. Subsequent medial can­
thoplasty may also be necessary.
Second, for oro-naso-ocular clefts (numbers 3–11, 4–10,
5–9), the original descriptions for correction of the soft-tissue
component of these clefts involved the use of Z-plasty flaps.
These flaps were designed to lengthen the foreshortened
distance between the medial canthus and nasal alar base on
the affected side. Presently, flaps are designed to respect aes­
thetic units by leaving scars along aesthetic lines (Fig. 31.26).55
For repair, complete dissection of the cleft is necessary. Small
orbital floor bone graft or bone graft matrix may be used to
fill the cleft defect and separate the orbit from the maxillary
sinus. A banner flap from along the median nasal sidewall
may be rotated into a subciliary incision beneath the affected
lower eyelid, and a transnasal medial canthopexy may be
performed. Both vertical and horizontal orbital dystopia may
be corrected with a facial bipartition (Fig. 31.27). Abnormal
canthal anatomy should be addressed with a medial and/or
lateral canthopexy. The goal during mid-childhood is orbital
correction even if malocclusion is not corrected or temporarily
made worse. Oro-naso-ocular clefts may also present with
large encephaloceles from cranial defects (Fig. 31.28). In these
cases a staged approach in infancy may be appropriate for
optimal correction. Of note, for the lateral lip cleft repair
(numbers 4 and 5 clefts), the intervening tissue between the
philtral column and lateral cleft should be excised.
The third treatment group, the lateral craniofacial clefts
(numbers 6, 7, and 8) include the constellation of clefts like
Treacher Collins syndrome and craniofacial microsomia. For
the oral commissure (as in cleft number 7), macrostomia may
affect feeding, saliva control, and speech acquisition and
should be addressed at an early age. The oral commissure
should be corrected for symmetry and fall approximately
below the medial canthal vertical line. Orbicularis oris muscle
fibers should be reoriented and interlaced at the neo-oral
commissure, and the cleft should be closed in a straight line.
The medial aspect may be closed with a small Z-plasty so that
the vertical limb is along the nasolabial fold. For the lower lip
(as in cleft number 30), a vertical excision with bilateral exten­
sions (not crossing the labiomental fold) with a layered closure
is performed.63
For mandibular reconstruction (as in cleft combinations 6,
7, and 8 in both craniofacial microsomia and Treacher Collins
syndrome), costochondral grafts may be used for severe
deformities, and distraction osteogenesis for moderate defor­
mities in mid-childhood (6–8 years).64 For mild maxillary
deformities resulting in an occlusal cant, correction can wait
until adulthood with a Le Fort I osteotomy with or without a
concomitant mandibular procedure.
Surgical correction of the periorbital region is necessary in
many craniofacial clefts. For the eye, urgent intervention is
necessary if the eye is exposed and at risk of corneal ulcer­
ation. However, early reconstructive procedures for globe
protection must be balanced to allow enough eye opening to
duplication of the nasal septum should be reserved until the
end of nasal growth.
For orbital dystopia, correction with an orbital box osteot­
omy has been described for these deformities; however, a
facial bipartition allows for more versatility, especially in
patients with mixed dentition when occlusal correction is not
paramount. A facial bipartition requires bilateral monobloc
osteotomies: in the anterior zygomatic arch, lateral orbital
wall, orbital roof, medial orbital wall, orbital floor, pterygo­
maxillary buttresses, and septum (during the midface
A
Fig. 31.23  Number 0 cleft correction. (A) Preoperative frontal view of patient with
midline incomplete cleft lip. (B) Postoperative frontal view of patient after intraoral
repair of orbicularis oris muscle without cutaneous scar.
B

735
Treatment
Fig. 31.24  Number 0–14 cleft correction.
(A,B) Frontal views of patient with Tessier
numbers 0–14 craniofacial cleft.
(A) Preoperative image demonstrating
large midline frontonasal encephalocele.
(B) Postoperative image after gradual orbital
contraction procedure and median cleft lip
and nose repair. (C,D) Lateral views of
patient with Tessier numbers 0–14
craniofacial cleft. (C) Preoperative image
demonstrating the anterior displacement of
the encephalocele with functional problems
of independent ocular movement and
drooling. (D) Postoperative image after
corrective procedures. Functional
improvements in ocular, oral competence,
and speech were noted. (E) Preoperative
three-dimensional computed tomography
scan with large central osseous defect
and 81-mm interdacryon distance.
(F) Postoperative image after orbital
contraction (midline device in place) with
interdacryon distance narrowed to 17 mm.
A
B
C
D
E
F
A
Fig. 31.25  Number 2–12 cleft correction.
(A) Preoperative view shows left nasal alar
cephalad malposition, orbital dystopia, and
hairline malformation. (B) Postoperative
view after facial bipartition and medial
canthoplasty. Future nasal correction will be
needed.
B

736
SECTION II
CHAPTER 31 • Craniofacial clefts
prevent deprivation amblyopia. For the eyelid, “lid switch”
transposition flaps are used for skin/muscle deficiencies.
Palatal grafts may be used for lower lid conjunctival lining.
The orbit may be reconstructed with cranial bone grafting to
restore orbital continuity and correct dystopia. Accurate
repositioning of the medial canthus may be performed with
transnasal wiring. Lateral canthopexies are sometimes needed
to achieve symmetry. Correction of number 8 (lateral eye)
clefts are performed with Z-plasty flaps. For the lacrimal
apparatus, disruption of the canalicular system may be cor­
rected with Silastic stents or formal dacryocystorhinostomy.
Fig. 31.26  Number 3 cleft correction. (A) Preoperative view shows wide left cleft lip and plate and shortening between the notched alar base and distorted medial canthus.
Left-side cleft number 7 and number 8 are also present. (B) 3D CT scan reveals left-side skeletal defect with unification of orbital, maxillary sinus, nasal, and oral cavities.
(C) Postoperative view after left-side downward rotation of nose, medial transnasal wire canthopexy, medial upper to lower lid switch flap, and cleft lip repair. Future cleft
palate, macrostomia repair, and ear reconstruction will be needed.
A
B
C
Outcomes and complications
Proper timing and technique of staged corrective procedures
will minimize perioperative complications and long-term
sequelae. If early functional problems like globe exposure are
not addressed, then corneal ulceration and blindness may
result. Likewise, speech problems may develop, as in more
common cleft lip and palate deformities. Complications may
occur for specific soft- or hard-tissue correction because of the
difficult nature of these procedures.
A
Fig. 31.27  Number 10 cleft correction. (A) Preoperative
view shows right vertical and horizontal orbital dystopia
with notching of midlateral brow. (B) Postoperative view
after facial bipartition and downward rotation of midlateral
brow.
B

737
Summary
Fig. 31.28  Number 3–10 cleft correction.
(A) Preoperative left lateral image
demonstrated a large right
frontoencephalocele. A right-side numbers
3 and 10 and a left-side number 3
craniofacial cleft were present.
(B) Computed tomography scan revealed
significant bony defect of the right
fronto-orbital region, encephalocele cyst,
and bony deformity. (C) After the first
procedure of encephalocele repair and right
fronto-orbital reconstruction, the patient
showed improvement but still had
orbital and facial cleft deformities.
(D) Postoperative frontal images after
bilateral cleft lip repair and right
nasoforehead rotation and medial canthal
repositioning. Future reconstructive
procedures will be necessary.
A
B
C
D
Summary
Craniofacial clefts are highly variable and range from mild
(barely noticeable) forme fruste (microform), to disfiguring,
complete defects of the skeletal and soft tissue. Tessier’s
description of craniofacial clefts based on bony and soft-tissue
landmarks provides a classification which has been validated
by neurometric theory of neuroembryology. For corrective
surgery, guiding principles are helpful in determining the
proper timing and stages, like addressing functional problems
(ocular exposure, airway problems) early. General corrective
protocols may be grouped into (1) midline and paramedian
clefts (numbers 0–14, 1–13, 2–12); (2) oro-naso-ocular clefts
(numbers 3–11, 4–10, 5–9); and (3) lateral clefts (numbers 6, 7,
8, like Treacher Collins syndrome of craniofacial microsomia).

738
SECTION II
CHAPTER 31 • Craniofacial clefts
1. Tessier P. Anatomical classification of facial, cranio-facial and
latero-facial clefts. J Maxillofac Surg. 1976;4:69–92. Tessier introduces
his now ubiquitous classification scheme for craniofacial clefts in this
account. Cleft position is described in reference to the orbit.
2. Kawamoto HK Jr. The kaleidoscopic world of rare craniofacial
clefts: order out of chaos (Tessier classification). Clin Plast Surg.
1976;3:529–572.
3. Kawamoto HK Jr. Rare craniofacial clefts. In: McCarthy JG, ed.
Plastic Surgery. Philadelphia: Saunders; 1990:2922–2973.
4. Bradley JP, Kawamoto HK. Rare craniofacial clefts. In: Grabb WC,
Smith JW, eds. Plastic Surgery. Philadelphia: Saunders;
1990:2922–2973.
5. Carstens MH. Functional matrix repair: a common strategy for
unilateral and bilateral clefts. J Craniofac Surg. 2000;11:437–469.
9. Van der Meulen JC, Mazzola R, Vermey-Keers C, et al. A
morphogenetic classification of craniofacial malformations. Plast
Reconstr Surg. 1983;71:560–572. The authors describe a new classification
scheme for craniofacial clefts. Pathogenesis and cerebral involvement are
emphasized.
10. Allam K, Wan D, Kawamoto HK, et al. The spectrum of median
craniofacial dysplasia. Plast Reconstr Surg. 2011;127:812–821. Midline
craniofacial malformations are further defined from an embryological
perspective. The authors separate these entities into hypoplasias (tissue
deficiency), dysraphias (normal amount of tissue, but clefted), and
hyperplasias (tissue excess).
11. Noordhoff SM, Huang CS, Lo LJ. Median facial dysplasia in
unilateral and bilateral cleft lip and palate: a subgroup of median
cerebrofacial malformations. Plast Reconstr Surg. 1993;91:996–1005. A
group of patients characterized by midface anomalies without cerebral
involvement is identified. Topics ranging from anatomical considerations
to growth potential are addressed.
55. Longaker MT, Lipshutz GS, Kawamoto HK Jr. Reconstruction of
Tessier number 4 clefts revisited. Plast Reconstr Surg. 1997;99:
1501–1507.
57. Tessier P. Orbital hypertelorism. 1. Successive surgical attempts,
material and methods, causes and mechanisms. Scand J Plast
Reconstr Surg. 1972;6:135–155. Orbital hypertelorism is described. An
extensive case series informs observations on diagnosis and management.
Access the complete reference list online at

738.e1
References
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2. Kawamoto HK Jr. The kaleidoscopic world of rare craniofacial
clefts: order out of chaos (Tessier classification). Clin Plast Surg.
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738.e2
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Tessier number 4 clefts revisited. Plast Reconstr Surg.
1997;99:1501–1507.
56. Cohen MM Jr, Jirasek JE, Guzman RT, et al. Holoprosencephaly and
facial dysmorphia: nosology, etiology and pathogenesis. Birth
Defects Orig Artic Ser. 1971;7:125–135.
57. Tessier P. Orbital hypertelorism. 1. Successive surgical attempts,
material and methods, causes and mechanisms. Scand J Plast
Reconstr Surg. 1972;6:135–155. Orbital hypertelorism is described. An
extensive case series informs observations on diagnosis and management.
58. Senan M. Case Report: Tessier Number 30. Indian J Plastic Surg.
2007;40:57–60.
59. Morritt DG. A paramedian cleft of the lower lip. J Craniofac Surg.
2007;18:704–706.
60. Oka M. A bilateral total cleft of lip and hard and soft palate and
transverse cleft face with an atypical paramedian defect of the
lower lip and lower jaw on the right side. J Maxillofac Surg.
1983;11:232–235.
61. Tanna N, Wan DC, Perry AD, et al. Paramedian mandibular cleft:
revisiting the Tessier classification. J Craniofac Surg. 2012;23:38–40.
62. Kumar A, Heling E, Guenther D, et al. Correction of
frontonasoethmoidal encephalocele: the HULA procedure. Plast
Reconstr Surg. 2009;123:661–669.
63. Zide BM, McCarthy J. The mentalis muscle an essential component
of chin and lower lip position. Plast Reconstr Surg. 1989;83:413–420.
64. Keagle J, Bradley JP. Craniofacial microsomia. In: Thaller SR,
Bradley JP, Garri JI, eds. Craniofacial Surgery. New York, NY:
Informa Healthcare; 2009.

SECTION II • Craniofacial
32 
Nonsyndromic craniosynostosis
Patrick A. Gerety, Jesse A. Taylor, and Scott P. Bartlett
SYNOPSIS
■ Craniosynostosis is the pathologic fusion of one or more cranial vault
sutures, usually resulting in an abnormal head shape.
■ Nonsyndromic craniosynostosis occurs in a sporadic, non-familial
fashion, in the absence of an associated genetic syndrome.
■ The fused cranial suture results create a cranial deformity, with areas
of restricted growth and compensatory bossing, as well as the
potential for functional and organic issues (increased intracranial
pressure (ICP) being the most significant).
■ Diagnosis entails a clinical examination and computed tomography
(CT) corroboration. The optimal timing of treatment is during infancy,
between 6 and 9 months.
■ Conventional open techniques and new modalities (including springs
and distraction osteogenesis) can be entertained.
■ The choice of operation depends on the specific suture fused and the
degree of dysmorphology. In general, the technical surgical goals entail
releasing the area of sutural fusion, repositioning the bone in an
anatomic but overcorrected location, eliminating secondary
compensatory changes, filling in osteotomy gaps with bone dust
slurry, and closing the soft tissue relatively tension-free.
■ The physiologic goal is to mitigate functional problems (e.g.,
intracranial hypertension and developmental delay, optic disc atrophy,
and strabismus).
■ Complications can be divided into early or late events.
■ Secondary revisions may be necessary involving either the soft tissue,
bone, or both.
■ On rare occasions, the complete intracranial procedure must be
repeated. However, major morbidity and mortality are exceedingly
uncommon in the modern approach and management of
craniosynostosis.
Introduction
Craniosynostosis is a disease involving the early and patho­
logic fusion of any of the cranial sutures, most often one of
the six major sutures. This early fusion results in recognizable
patterns of abnormal head shape. In the absence of a known
heritable or genetic syndrome as well as other medical prob­
lems, it is termed nonsyndromic. This is in contrast to a
number of other genetic syndromes, which cause suture
fusion as well as other craniofacial abnormalities (see Chapter
33: Syndromic craniosynostosis). Nonsyndromic patients
most often present with the fusion of only one suture, but
there are instances in which multiple sutures have fused. The
incidence of nonsyndromic craniosynostosis is approximately
1 in 2500 live births.1 These patients represent approximately
80% of cases of craniosynostosis, and this incidence is
approximately 10 to 50 times more common than syndromic
causes (e.g. Apert and Crouzon syndromes). Sagittal cranio­
synostosis occurs most frequently while lambdoid synostosis
occurs least frequently. A review of the experience at the
Children’s Hospital of Philadelphia in 2008 suggests that the
relative incidence of metopic synostosis has been increasing
particularly relative to diagnoses of unicoronal synostosis.2
This finding has been reported by other groups as well,
demonstrating that sagittal synostosis is approximately twice
as common as metopic and four times as common as unicoro­
nal.3,4 The etiology of nonsyndromic craniosynostosis is
unknown but it is believed to be multifactorial.
Basic science
Nonsyndromic craniosynostosis has an uncertain etiology,
but a host of factors have been identified that may cause or
contribute to the premature fusion of cranial sutures. It has
been theorized that suture fusion may either be a primary
event innate to the suture’s biology or a secondary event due
to external factors.
There are known cases of inherited and familial nonsyn­
dromic craniosynostosis. Autosomal dominant inheritance
is present in approximately 10% of cases. Coronal and
metopic suture fusion has a greater likelihood of being
familial than sagittal (10% vs 2%). Some data have suggested
that rising paternal age may contribute to the increasing
Access video lecture content for this chapter online at expertconsult.com

740
SECTION II
CHAPTER 32 • Nonsyndromic craniosynostosis
in life between 4 and 7 months of age.13 These two diagnoses
are often confused by healthcare providers.
A complete history should be obtained including prenatal
information (birth position, multiple gestation, duration of
gestation), affected parents or siblings, the presence of any
other identified anomalies, and achievement of milestones. In
older children and adults, elevated intracranial pressure (ICP)
may lead to symptoms such as headaches, visual disturbances,
lethargy, and vomiting. In neonates and infants these symp­
toms are difficult to elicit. An ophthalmologic exam can
identify papilledema, a sign of elevated ICP, and should be
obtained in all patients with suspected craniosynostosis.
Physical examination will nearly always make the diagno­
sis.14 The exam should focus on head shape, ear position,
orbital shape and symmetry, fontanelles, cranial sutures, and
measures of cranial morphology (head circumference, cephalic
index; Table 32.1). Patterns of specific suture fusion are char­
acteristic and have classically been described using
Virchow’s law.15 This concept describes cranial growth as
perpendicular to sutures. When sutures fuse abnormally,
compensatory growth occurs parallel to the fused suture. This
produces characteristic craniofacial patterns. The patterns of
individual suture fusion diagnoses can be seen in Fig. 32.1 and
are described in detail below.
Deformation (positional) plagiocephaly is almost entirely a
non-surgical diagnosis, and a full discussion is beyond the
scope of this chapter.16 However, it is critical to include in this
discussion because it also results in a characteristic head
shape and is often confused with craniosynostosis. Craniofa­
cial surgeons are often consulted on, provide counseling for,
and coordinate care of patients with deformational plagio­
cephaly (positioning, positional aids, helmet orthotics).17 The
successful campaign to reduce the rates of sudden infant
death syndrome (SIDS) by promoting back sleeping in infants
has lead to an observed rise in deformational plagiocephaly.18–20
The characteristic deformity involves occipital flattening that
is often asymmetric with the ipsilateral ear displaced anteri­
orly. This diagnosis can be confused with lambdoid cranio­
synostosis; a comparison of these two diagnoses can be seen
in Table 32.2. Another significant contributor to deformational
plagiocephaly is torticollis which may either precede (and
thus cause) or result from deformational plagiocephaly.21
The most sensitive and specific diagnostic test for cranio­
synostosis is computed tomography (CT) with three-
dimensional reconstructions. This allows the visualization of
all sutures as well as an examination of ventricular morphol­
ogy, brain stem position (Chiari), and midline brain abnor­
malities (corpus callosum). Radiologic signs of increased ICP
may also be identified. The characteristic “thumb-printing” or
incidence of metopic synostosis. Several syndromes (Muenke,
Saethre–Chotzen) have variable expressivity and may have
a mild phenotype. Thus, they may be assumed to be non­
syndromic upon presentation. In fact, these patients are
affected by FGFR3 or TWIST mutations. Any patients who
present with possible familial pattern of craniosynostosis
should receive a genetic consultation for counseling and
possible testing.
The cranial sutures play a critical role particularly early in
life by allowing significant cranial deformation during birth,
allowing expansion for rapid early brain growth, and for
controlling complex signaling to lead overall calvarial growth
and maturation. The precise etiology of nonsyndromic cranio­
synostosis and the underlying cranial suture biology remain
poorly understood. Animal model experimentation has lead
to a greater understanding of the complex nature of sutures.
The sutures represent a complex interaction of tissues (dura,
bony front, mesenchymal tissue, periosteum), and within this
organ are complex molecular signaling and growth processes.5
Several important molecules have been identified, including
transforming growth factor beta (TGFB), bone morphogenetic
proteins (BMP), TWIST, MSX2, and fibroblast growth factor
receptors (FGFR).6 While all of these molecules can be shown
experimentally to affect suture fusion, their precise role in the
nonsyndromic patient remains unknown.7 Gene regulation
and the products of these genes are clearly different in abnor­
mally fused versus open sutures.8 Sophisticated genetic
analysis continues to emerge on possible genetic contribution
to nonsyndromic craniosynostosis.9
Environmental factors have been shown to affect suture
fusion. This is best illustrated by diagnoses of craniosynostosis
in which cranial growth has been externally restricted or in
which cerebral/cranial growth does not occur normally.
Uterine shape (bicornuate), intrauterine position (breech),
large fetal size, and multiple gestations have all been associ­
ated with premature suture fusion and its sequelae. These
external forces apparently cause or increase the risk of prema­
ture suture fusion. This concept has been validated in experi­
mental and animal data. Lack of outward forces from brain
growth or restricted growth of other areas of the cranium are
also thought to contribute to craniosynostosis. Cranial base
growth restriction has long been theorized as a cause for
adjacent suture fusion and craniosynostosis. Similarly,
improper brain growth may also impart abnormally low
forces on the cranial sutures causing premature fusion.
In addition to mechanical forces, other environmental risk
factors have been identified to increase the risk of nonsyn­
dromic craniosynostosis. Maternal smoking, white maternal
race, advanced maternal age, gestation at high altitude, use of
nitrosatable drugs (e.g., nitrofurantoin), paternal occupation
(e.g., agriculture, forestry), fertility treatments, endocrine
abnormalities (e.g., hyperthyroidism), and warfarin ingestion
during gestation have all been linked to craniosynostosis.10–12
Patient presentation and diagnosis
Craniosynostosis may be diagnosed at or immediately follow­
ing the birth of a baby. The parents or pediatrician will often
notice a distinctly abnormal head shape and will be referred
to a craniofacial or neurosurgeon. In contrast, patients with
deformational (positional) plagiocephaly often present later
Table 32.1  Cephalic index (CI).
The ratio of biparietal diameter (BPD)and fronto-occipital diameter
(FOD). The measurements are taken where the distances are
maximum. CI = BPD/FOD × 100
Cephalic index
Cranial morphology
<76
Scaphocephaly or dolichocephaly
76–81
Normocephaly
>81
Brachycephaly

741
Patient presentation and diagnosis
“copper-beaten” patterns, when evident on 3D scan or the
scout film, suggest raised cranial pressure22 (Fig. 32.2). Simi­
larly, axial cuts can show loss of gyral folding and blunted
cisternae along the endocranial surface, consistent with the
brain expansion against an immobile, restrictive cranial vault.
The CT scan also may aid in surgical planning and serves as
a baseline study to compare against postoperative changes.
This may also be used as data for virtual surgical planning.23
Concern has arisen in the last decade of the deleterious effects
of ionizing radiation particularly in young children.24 This has
lead institutions such as our own to use dose-reduction pro­
tocols and to avoid CT scans when possible including for
routine postoperative results.
Patterns of altered head shape develop when one or more
of the six major sutures of the cranium fuses prematurely
(sagittal, metopic, coronal, lambdoid). These patterns are now
described in detail and can be visualized in Fig. 32.1. There
are other minor cranial sutures, which may also fuse prema­
turely but are beyond the scope of this chapter.
Sagittal synostosis develops following fusion of the midline,
sagittal suture (Fig. 32.3). This restricts bitemporal growth and
promotes anteroposterior expansion resulting in a character­
istic “boat-shaped” head. This deformity is alternately referred
to as dolichocephaly or scaphocephaly. Cephalic index (CI),
the ratio of bitemporal distance (BPD) to fronto-occipital
distance (FOD), is the most common measurement used in
assessing scaphocephaly. CI is normally 76–82 and in sagittal
synostosis typically measures <76. On exam, the sagittal
suture may be palpated as a bony ridge, and one or both
fontanelles may have closed. The head shape can be affected
by variable fusion of the sagittal suture (i.e. entire, anterior,
posterior). Fusion of the anterior sagittal suture contributes to
significant frontal bossing. Similarly, an occipital bulge results
from involvement of the posterior sagittal suture. Varying
degrees of frontal or occipital prominence can occur
Fig. 32.1  Characteristic skull shape depending on sutural involvement in
craniosynostosis.
Lambdoid synostosis
(posterior plagiocephaly)
Sagittal synostosis
(scaphocephaly or
dolichocephaly)
Unicoronal synostosis
(anterior plagiocephaly)
Bicoronal synostosis
(brachycephaly)
Metopic synostosis
(trigonocephaly)
Normal cranial
(normocephaly)
Table 32.2  Differentiating deformational plagiocephaly from lambdoid and unicoronal craniosynostosis.
Flat forehead
Flat occiput
Unicoronal synostosis (UCS)
Unilateral lambdoid synostosis
Deformational plagiocephaly
Primary feature
Ipsilateral forehead retrusion
Ipsilateral occipital flatness
Ipsilateral occipital flatness
Compensatory
changes
Contralateral forehead may be
bossed
Ipsilateral mastoid bulge (canted
skull base)
Ipsilateral forehead may be
bossed
No change in occiput
Ipsilateral forehead, no change or
recessed
Contralateral forehead may be
retruded
Ipsilateral ear
position
Anterior and superior
Varied
Varied
Posterior and inferior
Anterior
Periorbital region
Ipsilateral
Wide palpebral fissure
Higher retruded supraorbital rim,
brow
Harlequin on X-ray
Usually unaffected in anatomic
head position
Usually unaffected
May have contralateral retrusion
of fronto-orbital zygomatic
region
Nasal radix
Deviated towards affected side
Midline in anatomic head position
Midline
Head shape
from above
Ipsilateral forehead retrusion
Trapezoid
Parallelogram
Incidence
Rare
Very rare
Common

742
SECTION II
CHAPTER 32 • Nonsyndromic craniosynostosis
two lines drawn from nasion to pterion on axial cuts of a
CT scan. Normal is >104 degrees and severe is <89 degrees.
Frontal stenosis measures the severity of biparietal narrowing
and is the ratio of interparietal distance to the intercoronal
distance.27 Persing et al. have described two different severi­
ties of metopic synostosis; this delineation may also help
guide treatment.28
Unicoronal synostosis (UCS) has in the past been called
“anterior plagiocephaly”. Because this term can be confus­
ing, most surgeons now advocate specifying the suture when
discussing a diagnosis of craniosynostosis. Plagiocephaly is
a generic term denoting an asymmetric flattening of part of
the head. This occurs in positional plagiocephaly, unicoro­
nal synostosis, and lambdoid synostosis (see Table 32.2).
This confusing terminology is still often encountered in the
literature. UCS results in an asymmetric head shape with
contrasting ipsilateral and contralateral effects (Fig. 32.5).
Ipsilaterally, anterior growth of the forehead is restricted,
resulting in a flattened forehead and supraorbital rim with
temporal deficiency. The ipsilateral orbital dysmorphology
can be quite significant, with a taller and narrower orbit that
A
B
Fig. 32.2  Radiologic signs of elevated intracranial pressure. Thumbprinting and moth-eaten appearance. (Reproduced from Weinzweig J, Baker SB, Whitaker LA, et al.
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;110:397–408.)
C
D
depending on the extent and location of synostosis along the
sagittal suture.
Metopic synostosis results in a characteristically “keel”-
shaped forehead and triangular head shape termed trigono­
cephaly (Fig. 32.4). In contrast to the other cranial sutures,
the metopic suture normally fuses by 8 months,25 so mild
head shape abnormalities detected in late infancy do not
necessarily warrant aggressive diagnostics and surgery. The
other features of metopic synostosis include bitemporal nar­
rowing, parietal expansion, supraorbital and lateral orbital
retrusion, and hypotelorism. The orbital morphology of
metopic synostosis is a teardrop shape with the apex pointed
toward the metopic suture. Metopic synostosis is also associ­
ated with midline brain aberrations, and one report suggests
a higher than usual concomitant presentation of Chiari I
malformation.26 Severity of metopic synostosis is fairly vari­
able. While some think of metopic ridge as the most mild
form of the disease, it does not share the orbital and other
features of true synostosis. The severity may be measured
by the degree of supraorbital retrusion, trigonocephaly, and
biparietal narrowing. The frontal angle is the angle between

743
Patient presentation and diagnosis
normal posterior shape should signal that the process is not
positional plagiocephaly, however these two diagnoses may
be coincident.
Bicoronal synostosis results in a symmetric head shape (Fig.
32.6). This diagnosis is much more likely to occur in the setting
of a syndrome or familial inheritance. Lack of AP growth
causes remarkable bossing and expansion of the parietal skull
resulting in a large biparietal diameter (BPD). The head shape
is termed brachycephaly.30 This leads to a blunted forehead
and supraorbital ridge. In certain cases, there can be a com­
pensatory increase in parietal height causing turribrachy­
cephaly (tall flat head).
produces the appearance of a raised eyebrow and wide-open
eye in contrast to the contralateral side. This results in the
harlequin deformity, defined by the orbital shape with a
peak in the superolateral aspect. Contralaterally, compensa­
tory overgrowth occurs, producing forehead bossing. The
ipsilateral ear is typically positioned anterior and superior
secondary to normal contralateral anteroposterior (AP)
growth. The effects of UCS can be seen in twisting of the
middle and lower thirds of the face with the nasal root
moving ipsilaterally. When present, the chin deviates to the
unaffected side secondary to changed position of the glenoid
fossa.29 The posterior head shape is typically unaffected. This
Fig. 32.3  Sagittal craniosynostosis. This results in an elongated, narrow head termed scaphocephaly or dolichocephaly. These patients may have frontal and/or occipital
prominences.
A
B
C
D

744
SECTION II
CHAPTER 32 • Nonsyndromic craniosynostosis
variable and in some instances can be anteroinferiorly dis­
placed making ear position an unreliable feature.33 The con­
sistent features of lambdoid synostosis are in reality ipsilateral
occipital flattening and notable contralateral parieto-occipital
bossing. This posterior abnormality can translate into a degree
of facial asymmetry but this is variable.
Unilateral lambdoidal synostosis is a rare diagnosis (1 in
40 000 live births31), which results in a posteriorly based asym­
metric head shape. The pattern of deformity is often compared
and contrasted with that of positional plagiocephaly (see
Table 32.2). It has traditionally been characterized by ipsilat­
eral occipital flatness, posteriorly displaced ear, and mastoid
bulging. In the past, there was also belief that compensatory
frontal bossing arose in the contralateral forehead, providing
an overall trapezoid head shape when viewed from above32
(Fig. 32.7). This trapezoid shape was in contrast to the paral­
lelogram shape commonly found in deformational plagio­
cephaly. Clinically, the altered ear position can be quite
A
Fig. 32.4  Metopic craniosynostosis. This results in a triangular or keel-shaped
head termed trigonocephaly. Important features are bitemporal narrowing,
hypotelorism, and a teardrop-shaped orbit. The point of the teardrop is
superomedial and points towards the fused metopic suture.
B
Fig. 32.5  Unicoronal craniosynostosis. This results in an asymmetric head shape
termed anterior plagiocephaly. Important features include ipsilateral forehead and
supraorbital retrusion, a tall ipsilateral orbit with wide-open eye, and nasal root
(radix) which deviates toward the affected suture. Contralaterally there is
compensatory forehead bossing. The chin deviates away from the affected suture.
A
B

745
Patient selection
strabismus. Neurosurgeons are key partners during the entire
process. The neurosurgeon is an intraoperative participant in
the care of these patients and preoperatively evaluates the
status of the brain parenchyma and associated abnormalities
(e.g., hydrocephalus, Chiari malformation).
The fundamental goals of surgical treatment are two-fold:
(1) to relieve abnormally high intracranial pressure (ICP) and
allow room for normal brain growth and (2) to produce an
aesthetic head shape. Patients with mild head shape abnor­
malities, partial fusion of one suture, and no indication of
elevated ICP may be candidates for observation. In contrast,
patients with clear evidence of elevated ICP, who are most
often those with severe head shape abnormalities, should
receive operative correction.
Intracranial hypertension remains a challenging topic. At
extreme levels, elevated ICP can lead to blindness. There is
literature to suggest that, when unabated, it contributes to
developmental delay, even at lower levels. ICP poses a
Patient selection
The treatment of craniosynostosis, like other craniofacial
disorders, requires a multidisciplinary team approach. In par­
ticular, the craniofacial surgeon will work with neurosurgery,
genetics, ophthalmology, audiology, child psychology, the
child’s pediatrician, and other specialists during the course of
diagnosis and treatment. Because of specific gene mutations
(FGFR, TWIST) and the presence of syndromic patients, these
patients should be offered genetic counseling. The presence of
other findings, familial pattern, bicoronal synostosis, multiple
sutures, and other unusual presentations warrant a genetics
evaluation. A fundoscopic exam by a neuro-ophthalmologist
is required to assess for papilledema. Their exam can also
reveal baseline visual information and identify preoperative
A
Fig. 32.6  Bicoronal craniosynostosis. This results in a head shape with a
shortened fronto-occipital dimension termed brachycephaly. This features bilateral
supraorbital retrusion. The entire forehead may be retrusive with excessive
backwards slope. The vertex and cranium may be tall termed turricephaly or
turribrachycephaly.
B
A
Fig. 32.7  Lambdoid craniosynostosis. This results in an asymmetric head shape
termed posterior plagiocephaly. This results in ipsilateral occipital flattening and a
pathognomonic ipsilateral mastoid bulge. Contralaterally the occipital and frontal
regions may have a compensatory bulge.
B

746
SECTION II
CHAPTER 32 • Nonsyndromic craniosynostosis
tranexamic acid; and data in other invasive pediatric surgeries
supports that estimated blood loss, need for transfusion, and
volume transfused are lower when these agents are used.40,41
In order to avoid transfusion, some surgeons have advocated
preoperative treatment with erythropoietin which appears to
also have an excellent safety profile.42,43 Numerous techniques
have been used to attempt to avoid or decrease the amount
of blood transfusion during these case. The literature appears
to demonstrate, however, that modern use of blood transfu­
sion is very safe.
Positioning is supine for anterior procedures and prone in
a cerebellar headrest for posterior procedures. A modified
prone position (“sphinx”) may be used for simultaneous
anterior and posterior cranial vault remodeling.44 The authors
prefer a staged approach in such cases if possible, to minimize
blood loss, and to give more complete access to a single
anatomic site (and therefore a more complete correction),
rather than a somewhat limited access to two anatomic areas.
A coronal incision is required for most cranial vault remodel­
ing procedures. This typically begins just above the ear aligned
with the helical root or tragus. The design is non-linear (e.g.
zigzag, sinusoidal) to avoid a conspicuous scar and distorting
scar contraction.45
The choice of procedure is dependent upon the specific
suture involved, the degree of deformity, and the age at
presentation. Procedures for each deformity are discussed in
depth below. The procedures can be divided into several broad
categories: conventional vault remodeling, suturectomy, dis­
traction osteogenesis, and spring-mediated cranioplasty. An
endoscope can be employed to decrease incisional size and to
directly visualize dural dissection. The aim of vault remodel­
ing is to correct the deformity with a degree of overcorrection.
In contrast, the goals of suturectomy are to allow the growing
brain to remodel the skull (often with the assistance of helmet
therapy).46,47 Inevitably bony gaps will be created, and these
are treated with either split calvarial graft when possible or,
as the authors do, with bone shavings (curved Safescraper;
Meta, Reggio Emilia, Italy) (Fig. 32.8).
Stabilization of repositioned bone segments is obtained
using interposition bone grafts, sutures, and resorbable plates
and screws. The objective is to create a stable construct but
not to restrict brain growth and subsequent skull expansion.
Titanium or metallic plates are not utilized in infants undergo­
ing cranial remodeling for fear of transcranial migration and
growth restriction48–50 Limited incision and endoscopic tech­
niques for suturectomy use different, smaller incisions and no
fixation.
Sagittal synostosis
The treatment of sagittal synostosis has undergone significant
evolution in the past 20 years. There remains a significant
amount of disagreement about how to surgically correct these
patients.51 Traditionally, open vault remodeling was used to
reshape the scaphocephalic head. In 1998, Jimenez and Barone
reported the use of endoscopic strip craniectomy.52 This pro­
cedure reduced the soft-tissue exposure using the endoscopic
approach. The sagittal suture is removed and barrel stave
osteotomies are made in the parietal skull to accomplish
transverse widening. Around the same time in the late 1990s,
spring mediated cranioplasty (SMC) was first reported by
Lauritzen.53 This procedure involves using wire to create
challenge because no non-invasive exam to date provides an
excellent corollary to an invasive intracranial measurement of
ICP.34 A fundoscopic exam revealing papilledema is sugges­
tive of elevated ICP, but approximately 80% of infant patients
with elevated ICP do not have papilledema.35 There are endo­
cranial indications on CT scan such as copper beaten pattern,
thumbprinting, and scalloping, as well as blunted gyri that
suggest elevated ICP (see Fig. 32.2). Several technologies have
attempted to bridge the gap but remain unproven. In nonsyn­
dromic patients, the incidence of elevated ICP is thought to
be between 10 and 20%.36
Timing of surgery depends upon technique. For vault
remodeling procedures such as fronto-orbital advancement
and posterior vault remodeling, most surgeons agree that
between the ages of 6 and 9 months is ideal. The brain is
growing rapidly which will provide internal molding force
postoperatively, the cranial bone has matured to allow for
hardware but is still malleable for shaping, and the anesthetic
risk is lower. Newer techniques such as spring-mediated
cranioplasty or strip suturectomy are better performed at
between 3 and 4 months of age. The timing of surgery is
believed to be a balance between decreasing the neurocogni­
tive impact (early surgery) and decreasing the need for
secondary/revisional surgery (later surgery).
Surgical technique
Cranial vault remodeling procedures require a high level of
specialization and are best performed in a pediatric hospital
with specialists in pediatric anesthesia, critical care, and
neurosurgery. Traditionally the chief cause of mortality with
cranial vault remodeling was related to blood loss. This has
lessened with appropriate recognition and maneuvers by
both the surgical and anesthesia teams. There is a rare risk of
air embolism, which is assessed by cardiopulmonary status
and end-tidal CO2 recordings.37 Invasive hemodynamic moni­
toring (arterial line) is required. Cross-matched blood products
should be available and in the room before the start of the
case.
Blood loss and its sequelae are the most important consid­
eration during cranial vault operations. Blood loss must be
reduced, hemodynamics must be carefully monitored, and
resuscitation (often with blood) must be appropriate. Efforts
to limit blood loss are individual to surgeons but include
hemostatic peri-incisional (“blocking”) sutures, Raney clips,
Colorado-tip electrocautery, and epinephrine containing
tumescent.38 Most routine blood loss occurs during the skin
incision, so these steps may help to limit this. These techniques
cause a variable amount of alopecia, which must be recog­
nized.39 Other sources of routine blood loss are exposed soft
tissue, bone perforating venous emissaries, and osteotomies.
Careful avascular dissection, bone wax, and low operative
times can reduce blood loss from these factors. After crani­
otomy, the dura may be covered with hemostatic agents such
as Surgicel and Floseal. Unexpected and catastrophic bleeding
most often occurs if a dural sinus (sagittal, occipital, and
transverse sinuses) is injured. This must be treated with rapid
surgical hemostasis and resuscitation. Medical adjuncts, spe­
cifically anti-fibrinolytic agents, have been used in craniosyn­
ostosis surgery. The most common are aminocaproic acid and

747
Surgical technique
more bone vascularized than some total cranial vault proce­
dures, but may not produce as much correction of CI as other
procedures and does not address orbital deformities. There
are numerous other forms of vault remodeling that differ in
the pattern and rearrangement of cranial bone segments to
accomplish correction.
In the authors’ institution the algorithm for the treatment
of sagittal synostosis was previously based on the deformity
but used only conventional vault reconstruction.59 Their
current algorithm is to use spring-mediated cranioplasty in
patients under 4 months of age and after that to use staged
vault remodeling (Figs. 32.11 & 32.12). In a meta-analysis
of studies comparing techniques, the authors found that
conventional vault remodeling produced a small but sta­
tistically significant better correction of cephalic index than
strip suturectomy. We found the vault remodeling produced
an equivalent correction to spring-mediated cranioplasty.
Perioperative measures such as operative time, blood loss,
hospital stay, and cost were significantly lower for strip
suturectomy and spring cranioplasty though.60 Spring-
mediated cranioplasty is discussed below in detail.
A
B
Fig. 32.8  Harvest of particulate bone graft. (A) Safescraper device. (B) Particulate bone inlayed into bony gaps. (C) Graft harvested from frontal bone. (D) Particulate
shavings collected.
C
D
springs that slowly cause bitemporal widening. This tech­
nique has been adopted in numerous centers, and most sur­
geons agree that it is not efficacious if applied after 4 months
of age.54–56 This technique necessitates a second operation to
remove the springs.
Conventional vault remodeling is still used, particularly
in patients treated at an older age (>8 months). Some sur­
geons advocate for single stage total vault reconstruction,57
while other surgeons (the authors included) prefer a staged
approach – posterior and then anterior reconstruction +/−
fronto-orbital reconstruction as necessary (Figs. 32.9 & 32.10).
One well-described technique for cranial vault reconstruction
is the pi procedure.58 This involves two parallel parasagittal
ostectomies of the two parietal bones, connected with a
transverse ostectomy, located behind the coronal suture,
and extending to the temporal region (resembling the Greek
symbol for pi). The frontal bone and the remaining fused
sagittal suture are approximated with sutures or plates to
produce fronto-occipital shortening. This shortening produces
the desired biparietal/bifrontal widening. The pi procedure
has the advantage of being one stage and possibly keeping

748
SECTION II
CHAPTER 32 • Nonsyndromic craniosynostosis
growth overall.62 A clinical example of this can be seen in
Fig. 32.14.
Other modalities have been used to treat patients with
metopic synostosis. These include endoscopic strip craniec­
tomy followed by helmeting,47 spring-mediated cranioplasty,63
and distraction osteogenesis.64 No large case series or long-
term data for these patients exists, so their efficacy remains
unknown. In their series, Jimenez and Barone reported on 50
infants with metopic synostosis treated with suturectomy and
showed it to be only 43% effective.47 In addition to new tech­
niques, there continues to be significant disagreement about
which patients require surgical correction.65
Unicoronal synostosis
In unicoronal synostosis (UCS), the patient is asymmetric
with an ipsilateral retrusive forehead and supraorbital rim, as
well as an ipsilateral orbit that is too tall. Contralaterally the
forehead may have a compensatory bulge. Therefore, the
primary goals of treatment on the ipsilateral side are to
advance the forehead and supraorbital rim anteriorly (Fig.
32.15). Similar to metopic synostosis, the most effective
method for immediate and reliable correction of the UCS
deformity remains fronto-orbital advancement. A bifrontal or
unifrontal advancement may be performed, depending on the
Metopic synostosis
The primary objective in surgically correcting metopic synos­
tosis involves anterior cranial vault expansion that corrects
bitemporal constriction and triangular head appearance.29
The intent is to round the forehead shape and to widen and
advance the supraorbits improving hypotelorism and orbital
retrusion.
Most surgeons treating metopic synostosis use some form
of frontal orbital advancement. This technique has been in
use for nearly 50 years, though it has undergone significant
refinement.61,62 The current iteration at our institution entails
splitting the fronto-orbital bar in the midline and interposing
a bone segment to increase the bitemporal and interorbital
distance (see Figs. 32.10 & 32.13). The new construct exhibits
a more obtuse endocranial angle, witnessed by advancement
and expansion of the lateral rims and temporal region. This
bandeau is held in place by sutures at the zygomaticofrontal
and nasofrontal regions and temporally with resorbable
plates. Relapse and collapse of the bandeau are prevented
with sutures, resorbable plates, and key interposition
bone grafts at the temporal region advancement and with
notched grafts in the orbit. The widening and advancement
are performed in an overcorrected manner in anticipation
of loss of bandeau growth potential and continued cranial
Fig. 32.9  (A–D) Posterior vault remodeling for sagittal
craniosynostosis. Markings for osteotomy demonstrate
tongue-in-groove pattern of pieces A1 and A2 at vertex
that will be widened and switched to the occiput. The
occipital segment “B” is radially scored and used to
create a lowered vertex. Anteriorly barrel stave osteotomies
are used to blend anterior and posterior. The postoperative
configuration can be seen.
A1
A1
A2
A2
A2
Rotate
down
180º
B
B
A
B
C
D

749
Surgical technique
of bandeau advancement is usually required on the affected
side. There are several techniques and modifications attrib­
uted to the fronto-orbital advancement, depending on the
correction sought and the severity of the deformity67 (see Fig.
32.13). Simultaneous to repositioning the orbital rim, the
ipsilateral forehead is advanced, the orbital height may be
reduced, and the contralateral forehead is recessed or con­
toured as necessary. A canthopexy may be performed to lower
the affected side lateral canthus if an upward slant is present.
Some advocate including the nasal bones in continuity with
the frontal bar and uprighting the nasal radix deviation with
a closing wedge osteotomy. Others feel the nasal root devi­
ance corrects by adolescence with the lack of continued “pull”
toward the fused suture.68
severity of the deformity.66 This approach allows correction of
the ipsilateral orbital deformity and reshaping of the ipsilat­
eral and contralateral forehead. A bilateral craniotomy is typi­
cally necessary coupled with advancement of the ipsilateral
or bilateral supraorbital bandeau (see Fig. 32.15 for a clinical
example). In general, correction is possible with an ipsilateral
bandeau movement. The bandeau is planned as a very straight
osteotomy 2 cm above the supraorbital rim. The movement is
a widening with bone graft between the two bandeau seg­
ments (typically 5 mm) to accommodate the difference in
orbital width as well as an advancement forward into an
overcorrected position, typically 6 to 7 mm. The objective of
the fronto-orbital advancement is to position the supraorbital
rim 12–13 mm anterior to the cornea. An 8–15-mm magnitude
Fig. 32.10  Fronto-orbital advancement. (A) A bicoronal incision is used to expose the frontal bones and supraorbital bandeau. The temporalis muscle may be incorporated
into the scalp flap (shown) or raised separately. (B) After removal of the frontal craniotomy, the supraorbital bandeau is removed. The bandeau is then fixated into an
anteriorly advanced position and the frontal bone is replaced. The patient’s anatomy and the surgeon’s preference dictate alterations to the width and shape of both the
frontal bone and bandeau. (C) Completed fronto-orbital advancement. (Redrawn from an illustration by Dr. David Low.)
A
B
C

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CHAPTER 32 • Nonsyndromic craniosynostosis
A
B
C
D
E
F
Fig. 32.11  Posterior cranial vault remodeling for sagittal craniosynostosis. Intraoperative depiction of similar expansion as Figure 32.9. (A, B) Patient is in the prone
position. A large posterior craniotomy has been marked. (C) The widening cuts are added to the markings – this can guide the placement of burr holes. (D) The large
craniotomy specimen on the back table. If necessary the neurosurgeon can remove this in two pieces. (E, F) The vertex has been rotated to the occiput and widened via the
tongue and groove pattern. This is fixated with resorbable plates and suture. The original occiput has been radially cut and flattened to accomodate the vertex.
Minimally invasive techniques (distraction osteogenesis,
spring-mediated cranioplasty) have been proposed and
attempted, but these techniques have not yet matured to the
point that they produce a reliably efficacious result. Jimenez
and Barone used endoscopic suturectomy followed by helmet
therapy in a series of UCS patients and found that the success
rate was 43%.47
Bicoronal synostosis
In contrast to UCS, bicoronal synostosis involves much less
asymmetry. This entails largely a forward movement of the
supraorbital bandeau and forehead. This is accomplished via
bifrontal craniotomy with a bilateral bandeau. Current tech­
niques involve a bifrontal craniotomy and creation of a
bilateral frontal bandeau. The anterior dimension is advanced,
creating greater forehead prominence, and the supraorbital
rim is positioned ahead of the corneal zenith. The width is
narrowed by creating a midline endocranial flexion or a
median ostectomy of the repositioned frontal bones. In bicoro­
nal synostosis the height of the skull may also be increased
(turribrachycephaly), and this can be addressed by barrel
staves and greenstick fracture of the posterior parietal or
occipital bones.30
Lambdoid synostosis
Lambdoid synostosis is a very rare diagnosis. If the deformity
is present but mild, patients may be treated with an open
broad synostectomy with barrel staves and outfracture of the
ipsilateral occipital bone. The correction is prone to relapse
because of infant back sleeping, therefore semi-rigid (resorb­
able) fixation must be used to avoid this. Older infants and
children with more severe dysmorphology require treatment
by cranial vault remodeling. We recommend a switch cranio­
plasty with occipital bar advancement33,69 (Fig. 32.16). The
posterior vault bone flap is hemisected, and each half is
transposed to the opposite side and rotated 90–180° to achieve
a best fit. These are fixed anteriorly with resorbable plates and
bone gaps are filled with bone shavings. A number of other
posterior vault reconstruction procedures have also been
described to correct lambdoid synostosis.70
Endoscopic strip craniectomies and molding therapy
have been advocated by some in young infants. In a small
number of patients there was reported improvement in head
circumference and head shape symmetry, but this data is
limited.71,72
Emerging considerations
Spring-mediated cranioplasty
Spring-mediated cranioplasty (SMC) uses simple springs to
apply force across an osteotomy. It is similar to distraction
osteogenesis in pushing apart two bony segments, but once
in place the spring is not manipulated as a distractor is. This
concept was first shown to be experimentally effective in rabbits
by Persing et al.73 Lauritzen et al. were the first to demonstrate
this technique clinically.53 SMC has been demonstrated for
numerous craniosynostosis diagnoses including metopic,63,74
bicoronal,63 sagittal,63 and multisuture synostoses.75

751
Surgical technique
technique is now used routinely at the authors’ institution,
and in their series they demonstrated similar findings.56 The
authors’ patients were on average 4.6 months old. The opera­
tive time for placement was 90 minutes with an average blood
loss of 85 mL. Importantly, the average hospital stay is only
2.2 days. It is important to recognize that this technique
requires a second surgery for spring removal, and in the
authors’ series this operation required 45 minutes with a
blood loss of 45 mL and a hospital stay of 0.9 days. Addition­
ally, the authors’ systematic review of the comparative litera­
ture showed that for SMC operative time is halved, blood loss
SMC has gained widest usage for sagittal synostosis in
nonsyndromic cases, and, in many institutions, this has now
become the standard treatment in patients who present before
4 months of age (see Fig. 32.12). The literature now contains
comparative data from several groups54,55,76–79 as well as sys­
tematic reviews.56,60 To date, the largest series, by David et al.,
reports on 75 patients. This study demonstrated equivalent
correction of cephalic index (CI) as calvarial vault remodeling
while showing improved perioperative markers such as blood
loss, operative time, transfusion requirement, and length of
hospital stay with a nearly four-year follow-up period. This
Fig. 32.12  Treatment of sagittal
craniosynostosis with spring-mediated
cranioplasty. (A) A typical spring before it
has been implanted. (B) Cephalogram
demonstrating spring placement.
(C) Patient with scaphocephaly
preoperatively. (D) Patient postoperatively
with excellent head shape correction.
A
B
C
D

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CHAPTER 32 • Nonsyndromic craniosynostosis
6 and 12 N – we have chosen higher forces over time. Average
spring expansile distance is 5 to 7 cm from a compressed
position to a relaxed position. Fabrication is typically per­
formed using a purpose-built wire bender. This wire is bent
to produce a gentle U-shape, and the ends are bent into 3 mm
hooks for bony attachment. The opening force of the com­
pressed spring is then verified using a digital force gauge.56,80
Surgery is done in supine position. Two small (5 cm) trans­
verse incisions are at each fontanelle. Through these incisions
the sagittal suture and a surrounding strip of cranium (1.5 cm)
are removed using an ultrasonic device (Sonopet; Stryker,
Kalamazoo, MI, USA). In general, endoscopic visualization of
the dura is not necessary. Small notches are made with a
rongeur to accommodate the footplates of the springs. Two
springs are then placed – one anterior, one posterior. If mid-
vault correction is inadequate after placement of two springs,
a third is added. Morselized bone graft is placed in the oste­
otomy particularly deep to the springs. Plain radiographs are
obtained postoperatively, and spring removal is planned for
4 months later.
Cranial vault distraction osteogenesis
Distraction osteogenesis (DO) in the craniofacial skeleton was
popularized by McCarthy for mandibular distraction.81 This
technique uses rigid hardware to slowly separate bone causing
intervening bone formation. The use of DO has expanded to
the midface and the cranium. The largest role that DO now
plays in craniosynostosis is in syndromic patients to expand
the posterior vault – posterior vault distraction osteogenesis
(PVDO) (Fig. 32.17) The advent of distraction has allowed a
new paradigm of treatment for some patients with craniosyn­
ostosis. This approach has been advocated by several
groups82–85 where PVDO is used early to expand intracranial
volume protecting the brain during its high growth period
followed by FOA for aesthetic normalization as needed. DO
has a number of purported advantages which include ability
to gain greater changes in length or volume than traditional
reconstruction as well as decreased perioperative morbidity.
A recent study from our institution demonstrated that PVDO
produces nearly double the intracranial volumetric increase
as frontal orbital advancement.86 Surgeons continue to believe
that markers of operative and perioperative morbidity are
better with PVDO compared to traditional open calvarial
vault reconstruction (CVR); however, there have only been
head-to-head studies that demonstrate equivalence. Taylor
et al. showed no difference in blood loss, operative time,
transfusion requirement, and length of stay.87
The disadvantages of DO are primarily that the bone of
very young infants is too soft and too thin to withstand the
application of hardware and the forces during distraction.
Furthermore, with metallic hardware, a second procedure is
required for removal and possible plating of the distracted
segments. A review by Losee et al. demonstrated that in the
limited literature that is available on this topic, complication
rates are substantial. In their review of 11 studies and 86
patients, they found a complication rate of 35.5% (12.5–100%).
These complications include cerebrospinal fluid (CSF) leak,
infection, wound dehiscence, and hardware failure; however,
no reports included significant morbidity or mortality.88 The
largest series of PVDO by Johnson et al. reaffirms this high
complication rate, reporting a 61% complication rate.89 Many
is four-fold lower, length of stay is halved, and cost is approxi­
mately one-third when compared to calvarial vault remodel­
ing.60 Importantly, the correction of cephalic index was found
to be equivalent.
SMC is typically used for patients up to ages 6 or 7 months.
After this time, cranial development requires a greater widen­
ing with greater forces than springs can provide. This typically
necessitates calvarial vault reconstruction or distraction osteo­
genesis with rigid semi-buried distractors. In our institution
patients currently undergo spring placement at 3–4 months of
age. Springs for this technique are usually bent in-house. An
FDA-approved stainless steel wire is used. The diameter is
typically 1.3 mm (18 gauge). Average spring force is between
Fig. 32.13  Metopic synostosis bandeau reshaping. (A) The bandeau is split in the
midline to permit widening. (B) A bone graft and a resorbable plate are typically
used to stabilize widening. (C) The temporal tabs from the zygomaticofrontal suture
(ZF) and posteriorly are reshaped to correct the abnormal geometry of the bandeau.
This is often supported by endocortical resorbable plates. (D) Additional
stabilization of the midline is achieved with bone grafts secured by suture or wire.
This may be particularly important because of thickness differences between the
bandeau of and harvested cranial bone graft. (E) The advanced position of the
bandeau is further supported by stabilizing bone grafts at the orbital roof resorbable
plates and bone grafts at the temporal tabs.
A
B
C
D
E