Facial Clefts

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CME
Common Craniofacial Anomalies: Facial Clefts
and Encephaloceles
Jeremy A. Hunt, F.R.A.C.S., and P. Craig Hobar, M.D.
Dallas, Texas

Learning Objectives: After studying this article, the participant should be able to: 1. Understand the cause and pathogenesis
of facial clefting and encephaloceles. 2. Recognize and classify facial clefts and encephaloceles. 3. Understand the
different treatment plans for reconstruction of facial clefts and encephaloceles.
The wide variety of craniofacial malformations makes
classification difficult. A simple classification system allows
an overview of the current understanding of the causes,
assessments, and treatments of the most frequently encountered craniofacial anomalies. Facial clefts and encephaloceles are reviewed with respect to their diverse
causes, pathogenesis, anatomical features, and treatments. Approaches to the surgical treatment of these conditions are reviewed. (Plast. Reconstr. Surg. 112: 606,
2003.)

discussed, as is the surgical correction of these
craniofacial anomalies.
CRANIOFACIAL EMBRYOLOGICAL DEVELOPMENT

Normal embryological development of the
human face and cranium was described by
Sperber.4 After union of the male and female
gametes, the formed zygote rapidly divides to
form the morula and subsequently the blastocyst. The primary germ layers of the embryo,
namely, the ectoderm and the endoderm,
form in the inner cell mass of the blastocyst,
with the ectoderm differentiating into cutaneous and neural portions by approximately day
20. At that stage, the neural portion of the
blastocyst is called the neural plate; with midline folding of this neural plate, the neural
tube is formed.
Of particular importance to the embryological development of craniofacial structures are
neural crest cells. The neural crest ectomesenchymal tissue arises from the crests of the neural fold and forms a separate pluripotential
tissue layer. Neural crest ectomesenchyme has
great migratory propensities and is the major
source of connective tissue throughout the
body, because translocated neural crest cells
differentiate into cartilage, bone, ligaments,
muscles, and arteries. Any disruption in the
orderly migration and differentiation of these
cells can have severe consequences, manifested
by congenital defects.5

The wide variety of craniofacial malformations makes them difficult to classify. Gorlin et
al.1,2 stated that our limited understanding of
the embryological features and causes of malformations restricts efforts at classification. In
1981, the Committee on Nomenclature and
Classification of Craniofacial Anomalies of the
American Cleft Palate Association3 grouped
craniofacial disorders according to their diverse causes, anatomical features, and treatment. The committee proposed a practical simple classification system in which five
categories of deformity are identified, as follows: I, facial clefts/encephaloceles and dysostosis; II, atrophy/hypoplasia; III, neoplasia/
hyperplasia; IV, craniosynostosis; and V,
unclassified. This simple classification system
allows an overview of our current understanding of the causes, assessment, and treatment of
the most frequently encountered craniofacial
anomalies. Deformities involving craniofacial
clefting and encephaloceles are reviewed and

From the Department of Plastic and Reconstructive Surgery, University of Texas Southwestern Medical Center. Received for publication May
13, 2002; revised October 8, 2002.
DOI: 10.1097/01.PRS.0000070971.95846.9C

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The first 12 weeks of gestation represent
the crucial period of organogenesis, and it is
during this period that the majority of congenital craniofacial anomalies are established.4,5 The earliest signs of the future face
appear at approximately day 23 or 24 of embryonic life, as paired mandibular processes
of the first visceral arch. Next, the medial
nasal processes combine with the intervening
forebrain to form the frontonasal process,
which is destined to become the forehead
and the dorsum of the nose. The lateral nasal
folds separate the olfactory pits from the
gradually developing eye region. By the end
of the fifth embryonic week, the maxillary
and mandibular processes have begun to increase in size but have not yet fused; it is not
until the sixth week that definitive jaws are
formed. By the end of the eighth week, the
face assumes most of the characteristics that
make it recognizable as human. The face is
derived from five facial prominences that
surround the future mouth, namely, the single frontonasal process and the paired maxillary and mandibular processes.
The grooves between these facial prominences usually disappear by day 46 or 47 of
gestation, as the processes meet their equivalents from the contralateral side and fuse in
the midline. Any persisting groove between
meeting or adjoining processes results in a congenital facial cleft. Slavkin et al.6 demonstrated
that this process of fusion was attributable in
part to the ability of preprogrammed cells to
differentiate independently and allow normal
development.
CAUSES

OF

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FACIAL CLEFTS AND ENCEPHALOCELES

CRANIOFACIAL ANOMALIES

Identification of the genetic bases for most
craniofacial syndromes has exploded in the
past decade, and our knowledge continues to
expand. For conditions without an identified
genetic pattern of inheritance, four general
categories of environmental “cleftogens” have
been identified to date, as follows.
• Radiation. Large doses of radiation have
been associated with microcephaly.
• Infections. The children of mothers with
toxoplasmosis, rubella, or cytomegalovirus infections exhibit increased frequencies of facial
clefts.
• Maternal idiosyncrasies. Mothers of children
with cleft lips and palates have been noted to
exhibit a higher-than-normal incidence of phenylketonuria, and the oculoauriculovertebral

spectrum has been observed with unusual frequency among infants with diabetic mothers.7
Many studies have suggested maternal factors
such as age, weight, and general health as potential causes of malformations.
• Chemicals. Vitamin deficiencies are associated with increased incidences of cleft lips and
palates, which may be reduced with vitaminsupplementation diets for the mothers. Vitamin A, its derivatives, and related compounds
such as isotretinoin (Accutane; Hoffman-La
Roche, Inc., Nutley, N.J.) have been implicated
in the development of facial clefting and hemifacial microsomia. Maternal smoking has been
demonstrated to be associated with
craniosynostosis.8
Other drugs are strongly suspected to be
contributors in craniofacial syndromes. Gardner et al.9 observed associations between craniosynostosis and exposure to chlorpheniramine, chlordiazepoxide, and nitrofurantoin
but no associations with hydantoin, valproic
acid, or cocaine.
FACIAL CLEFTS
Anatomical Classification

The broad spectrum of anomalies attributable to facial clefting makes classification difficult. In 1976, Tessier10 described an anatomical
classification system in which a number is assigned to each malformation on the basis of its
position relative to the sagittal midline. This
system has become internationally accepted
and allows concise effective communication
among clinicians (Fig. 1).
For orientation, the orbit is divided into two
hemispheres; the lower lid, cheek, and lip demonstrate facial clefts, and the upper lid and
cranium demonstrate cranial clefts. According
to Tessier’s scheme, clefts of the bones and soft
tissues do not always coincide and frequently
several different clefts coexist. This classification system remains in wide use today because
of its accuracy and because it is relatively easy
to learn and allows communication with other
clinicians. David et al.11 demonstrated a complete series of these craniofacial clefts in threedimensional computed tomographic scans.
Clefting may present as a tissue deficiency or a
tissue excess. The tissue-deficiency disorders,
namely, arhinencephaly and holoprosencephaly, are secondary to failure of embryonic
prosencephalon cleavage and of the normal
longitudinal split into cerebral hemispheres.

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PLASTIC AND RECONSTRUCTIVE SURGERY,

August 2003

FIG. 1. Tessier’s classification of craniofacial clefts (reprinted from Coady, M. S. E., Moore, M. H., and Wallis, K. Amniotic
band syndrome: The association between rare facial clefts and limb ring constriction. Plast. Reconstr. Surg. 101: 640, 1998).

The tissue-excess deformities result from failure of complete tissue development and range
from a slight midline notch of the upper lip to
severe orbital hypertelorism.
Embryological Classification

Van der Meulen et al.12 attempted to correlate clinical features of disorders with embryo-

logical events. They envisioned the craniofacial
skeleton developing along a helical course symbolized by the letter S (Fig. 2).
Their scheme uses “focal fetal dysplasia” in
preference to “cleft” to describe an arrest in
skin, muscle, or bone development and names
the dysplastic anomaly on the basis of the areas
involved. For malformations characterized by

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FACIAL CLEFTS AND ENCEPHALOCELES

609

FIG. 2. Van der Meulen’s classification of craniofacial dysplasias. D., dysplasia (reprinted from van der Meulen, J. C., Mazzola,
R., Vermey-Keers, C., Stricker, M., and Raphael, B. A morphogenetic classification of craniofacial malformations. Plast. Reconstr.
Surg. 71: 560, 1983).

dysostoses, an additional distinction is made
between transformation defects and developmental arrests that occur before fusion of the
facial processes.12
In the frontosphenoidal area, dysplasia may
manifest as a Tessier no. 9 cleft, a cloverleaf skull
or kleeblattschädel deformity, or less often plagiocephaly. Frontal dysplasia is associated with
orbital hypertelorism and nasal dysplasia, a widow’s peak, and dystopia of the eyebrow. This area
corresponds to Tessier no. 10 and 11 clefts. Interfrontal dysplasia is associated with bone defects with or without encephaloceles, as observed
with Tessier no. 0 to 14 clefts. Nasal aplasia with
proboscis corresponds to the ethmocephaly or
cebocephaly described by DeMyer et al.13 Nasoschizis (Tessier no. 1 cleft) involves deformity of
one-half of the nose, with a normal septum and
nasal cavity.
The cleft lip produced with medial nasomaxillary dysplasia is usually observed in combination with other deformities. When associated

with lateral nasomaxillary dysplasia, it is known
as the Morian I, Tessier no. 3, or naso-ocular
cleft. The median (Tessier no. 4, Morian II)
and lateral (Tessier no. 5, Morian III) oroocular clefts are dysplasias of the orbit and
maxilla that spare the nose. The deformity
known as maxillozygomatic dysplasia is equivalent to a Tessier no. 6 cleft or incomplete
Treacher-Collins syndrome. The complete
form of Treacher-Collins syndrome is characterized by zygotemporoauromandibular dysplasia and corresponds to Tessier no. 6, 7, and
8 clefts. Temporoauromandibular dysplasia is
also known as auromandibular dysostosis,
hemifacial microsomia, or first and second
branchial arch syndrome.
Maxillomandibular dysplasia is a failure of
the maxillary and mandibular processes to
fuse, resulting in macrostomia. Mandibular
dysplasias, exemplified by the Pierre Robin syndrome, are associated with micrognathia, glossoptosis, and respiratory distress.

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PLASTIC AND RECONSTRUCTIVE SURGERY,

Holoprosencephaly

Pathogenesis of Facial Clefts

The holoprosencephaly malformation represents a hypoplastic Tessier no. 14 cleft in association with a tissue deficiency or a tissue excess. 12,13 Cohen and Sulik 14 presented a
modern analytical review of the holoprosencephalic disorders, in which the central nervous
system findings and craniofacial anatomical
features were discussed, syndromes and associated anomalies were updated, and the differential diagnosis was reviewed.
Elias et al.15 reviewed holoprosencephaly
and midline facial anomalies in an attempt to
redefine their classification and treatment.
They noted that true holoprosencephaly encompasses a series of midline defects of the
brain and face, with a spectrum of expression.
In most cases, holoprosencephaly is associated
with severe malformations of the brain that are
incompatible with life. At the other end of the
spectrum are patients with midline facial defects and normal or nearly normal brain development. With computed tomographic findings
and a period of observation, patients who
could benefit from surgical treatment can be
selected.

There are two leading theories of facial cleft
formation. The classic theory, championed by
Dursy16 and His,17 holds that clefts are caused
by the failure of fusion of the facial processes.18
In this theory, the face forms as the fingerlike
ends of the maxillary processes meet and coalesce with the united paired globular processes
beneath the nasal pits. Once epithelial contact
is established, mesenchymal penetration completes the fusion and the lip and hard palate
are formed. When the sequence is disturbed,
clefting occurs.
The mesodermal penetration theory was described by Pohlmann19 and Veau20 and was
later advocated by Stark and Saunders.21,22 Proponents of the mesodermal penetration theory
think that free-end facial processes do not exist
and the face consists of a bilaminar ectodermal
membrane, with epithelial seams demarcating
the major processes. The mesenchyme migrates into this double wall of ectoderm, penetrates it, and smoothes out the seams. If mesenchymal penetration fails, then the
unsupported epithelial wall dehisces and a
cleft is produced. The severity of the cleft is

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FIG. 3. (Left) Tessier no. 0 to 14 clefts. (Right) Craniofrontal dysplasia with Tessier no. 0 to 14 clefts.

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FACIAL CLEFTS AND ENCEPHALOCELES

Tissue expansion and its application in facial
clefting were described by Menard et al.,28 who
reported soft-tissue closure for eight patients with
facial clefts. In summary, the goals of surgical
treatment of facial clefts include functional correction of macrostomia, soft-tissue reconstruction of the eyelid to prevent globe exposure,
separation of the confluent oral, nasal, and orbital spaces, and aesthetic correction of the
deformity.
ENCEPHALOCELES

FIG. 4. Four-month-old patient with a naso-ocular cleft,
representing Tessier no. 3 to 11 clefts.

inversely proportional to the success of mesodermal penetration, with different degrees of
incomplete and complete clefts (Figs. 3 and 4).
Treatment of Facial Clefts

By far the most common craniofacial anomaly is cleft lip/palate, followed by isolated cleft
palate as a distant second. The incidence of
rare clefts is estimated to be 1.4 to 4.9 cases per
100,000 live births.23 Reconstruction initially
focuses on soft-tissue closure,23 with excision of
all scar within the cleft until normal tissue is
reached, followed by meticulous, layered, softtissue closure. Because of underlying bony hypoplasia, skeletal reconstruction is often necessary, although it is delayed until the child is older.23
Van der Meulen24 provided a thorough review
of the pathological features, causes, and reconstruction of oblique facial clefts. He agreed with
Tessier’s principle of combining skeletal and softtissue realignment in one major surgical procedure. Resnick and Kawamoto,25 Galante and
Dado,26 and Fuente del Campo27 provided specific recommendations for the treatment of unusual facial clefts, on the basis of their respective
experiences with Tessier no. 4, 5, and 8 clefts.

An encephalocele is a protrusion of part of the
cranial contents through a defect in the skull.
The mass may contain meninges (meningocele),
meninges and brain (meningoencephalocele),
or meninges, brain, and ventricle (meningoencephalocystocele).29 Encephaloceles are categorized according to their positions in the skull and
can be basal, sincipital, or convexity.30 The sincipital group can be further divided into frontoethmoidal, interfrontal, and associated with
clefts. The frontoethmoidal group can be subdivided into nasofrontal, nasoethmoidal, and nasoorbital types.31 Boonvisut et al.32 reviewed the
morphological features of 120 skull base defects
and classified the defects as involving either a
single opening (type I) or multiple openings
(type II) between the frontal, nasal, ethmoidal,
and orbital bones. The groups were further divided into type IA/IIA, in which the defect was
limited to two bones within the area, and type
IB/IIB, in which the defect extended laterally to
involve adjacent structures (Fig. 5).
The presence of an encephalocele may be
detected on antenatal ultrasonograms or with
elevated ␣-fetoprotein levels.30 The differential
diagnosis of frontal midline masses includes
encephaloceles, teratomas, gliomas, and dermoids,33,34 and high-resolution computed tomographic scans can establish the intracranial
component of encephaloceles.34 In a frontoethmoidal or nasal encephalocele, the cranial
defect is in the anterior midline between the
frontal bone (preformed in membrane) and
the ethmoid process (preformed in cartilage).
The craniofacial deformity involves hypertelorism, orbital dystopia, elongation of the face,
and dental malocclusion, reflecting the distorting effects on facial bone growth of the extruded intracranial contents.
The pathogenesis of frontoethmoidoencephaloceles is as follows. Early in embryogenesis, diverticula of dura project anteriorly
through the fonticulus nasofrontalis (a small

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PLASTIC AND RECONSTRUCTIVE SURGERY,

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FIG. 5. Type IA and IB skull defects, representing nine subtypes. F, frontal; N, nasal; E,
ethmoidal; O, orbital; B, bilateral; C, cephalad (reprinted from Boonvisut, S., Ladpli, S., Sujatanond, M., et al. Morphologic study of 120 skull base defects in frontoethmoidal encephalomeningoceles. Plast. Reconstr. Surg. 101: 1784, 1998).

fontanelle between the developing nasal and
frontal bones) or inferiorly through the developing frontal bone into the prenasal space.
These diverticula may come in contact with
skin and adhere to it. Normally, the diverticula
regress and the bone closes, creating the normal nasofrontal suture anteriorly and, passing
through the skull base just anterior to the crista
galli, the foramen cecum. In a frontoethmoidal
encephalocele, the diverticulum does not recede and the bone does not close. The causes
of encephaloceles are unknown but involve
racial, genetic, environmental, and paternal
factors.35 Encephaloceles also occur with a
number of craniofacial syndromes.36
The worldwide incidence of encephaloceles
is one case per 5000 births.37 In Western Europe, North America, Australia, and Japan,
back-of-the-head encephaloceles predominate;
of 265 encephaloceles recorded in more than
20 years, 196 were occipital. In Southeast Asia
and Russia, however, anterior encephaloceles
outnumber posterior encephaloceles in a 9.5:1
ratio.37,38 The reason for this discrepancy is
unknown (Figs. 6 and 7).

The principles of encephalocele treatment
include incision of the sac, amputation of
excess tissue to the level of the surrounding
skull, closure of the dura, and closure of the
skin.39 David,40 Forcada et al.,41 and Smit et
al.42 reviewed the spectrum of cranial and
cerebral malformations that may occur with
frontoethmoidal encephaloceles and discussed the diagnosis and treatment of these
deformities. David40 analyzed the experience
of the Australian Craniofacial Unit from
1975 to 1993 and reached the following conclusions with respect to frontoethmoidal encephaloceles. (1) Early complete surgical
treatment is indicated to allow the developing brain and eyes to remodel the facial deformity. (2) Intracranial abnormalities are
common. (3) Frontoethmoidal encephaloceles differ from other neural tube defects in
the absence of a familial pattern and the
peculiar geographic distribution. (4) Treatment with a craniofacial technique is best.
(5) Established deformities can be effectively
treated with craniofacial osteotomies. (6)
Most patients exhibit abnormal intercanthal

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FACIAL CLEFTS AND ENCEPHALOCELES

FIG. 6. Nasofrontal form of frontal encephalocele.

distances but normal interpupillary and lateral canthal measurements. (7) The frontal
sinus region often requires repeat bone
grafting, and nasal bone grafts often must be
replaced as patients age. (8) Treatment of
craniofacial clefts should be postponed until
growth is complete. (9) Early treatment of
patients with basal encephaloceles is indicated, to prevent further damage and infection. (10) Surgical treatment of extensive
basal encephaloceles is complex and probably should be performed through a facial
hemisection approach.
Holmes et al.43 reported their experience
with 35 cases of frontoethmoidal encephaloceles. The goals of treatment are (1) urgent
closure of open skin defects, to prevent infections and desiccation of brain tissue, (2)
removal or invagination of nonfunctional extracranial tissue, (3) watertight dural closure,
and (4) total craniofacial reconstruction,
particularly avoiding the “long-nose deformity.” To correct the deformity caused by hypertelorism and a long midface, Holmes et
al.43 lower the supraorbital bar by rotating it
medially, posteriorly, and downward in the
midline, with lateral widening to correct the
trigonocephalic deformity. Successful correction is dependent on (1) an understanding
of the pathological anatomical features, (2)
careful planning of osteotomies and bone
movements that correct the entire deformity
(including trigonocephaly and the long-nose
deformity), (3) nasal reconstruction with a
cantilever graft, to avoid the long-nose deformity, (4) skin closure that removes abnormal
skin, with careful attention to the positioning
of scars, (5) transnasal canthoplasty to reposition the medial canthi, and (6) single-stage
surgical treatment, involving craniofacial
and neurosurgical expertise.
P. Craig Hobar, M.D.
Department of Plastic and Reconstructive Surgery
University of Texas Southwestern Medical Center
411 N. Washington Avenue
Suite 6000, LB 13
Dallas, Texas 75246
[email protected]
ACKNOWLEDGMENTS

FIG. 7. Interfrontal encephalomeningocele.

We thank Marco A. Medina and Leesa Thompson, The
Fogelson Plastic Surgery and Craniofacial Center for Children (Dallas, Texas), for invaluable assistance and contributions to the article.

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PLASTIC AND RECONSTRUCTIVE SURGERY,

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41. Forcada, M., Montandon, D., and Rilliet, B. Frontoethmoidal cephaloceles: Transcranial and transfacial surgical treatment. J. Craniofac. Surg. 4: 203, 1993.

42. Smit, C. S., Zeeman, B. J., Smith, R. M., and de V. Cluver, P. F.
Frontoethmoidal meningoencephaloceles: A review of 14
consecutive patients. J. Craniofac. Surg. 4: 210, 1993.
43. Holmes, A. D., Meara, J. G., Kolker, A. R., Rosenfeld, J. V.,
and Klug, G. L. Frontoethmoidal encephaloceles:
Reconstruction and refinements. J. Craniofac. Surg. 12:
6, 2001.

Self-Assessment Examination follows on
the next page.

Self-Assessment Examination
Common Craniofacial Anomalies: Facial Clefts and Encephaloceles
by Jeremy A. Hunt, F.R.A.C.S., and P. Craig Hobar, M.D.
1. REGARDING CRANIOFACIAL ORGANOGENESIS, THE CRUCIAL PERIOD OF DEVELOPMENT TAKES PLACE
IN THE:
A) First 48 hours
B) First week
C) First trimester
D) Second trimester
E) Third trimester
2. ENVIRONMENTAL CLEFTOGENS IDENTIFIED IN THE DEVELOPMENT OF CRANIOFACIAL ANOMALIES
INCLUDE ALL OF THE FOLLOWING EXCEPT:
A) Radiation
B) Cytomegalovirus
C) Isotretinoin
D) Cocaine
E) Vitamin deficiency
3. WHICH OF THE FOLLOWING PRINCIPLES OF RECONSTRUCTION OF FACIAL CLEFTING IS LEAST
IMPORTANT?
A) Excision of all scars within the cleft
B) Initial soft-tissue closure
C) Meticulous, layered, soft-tissue closure
D) Immediate skeletal reconstruction
E) Separation of oral, nasal, and orbital spaces
4. THE CRANIOFACIAL DEFORMITY OF NASAL ENCEPHALOCELE INCLUDES ALL OF THE FOLLOWING
EXCEPT:
A) Hypertelorism
B) Orbital dystopia
C) Elongation of the face
D) Dental malocclusion
E) Alveolar cleft
5. THE WORLDWIDE INCIDENCE OF ENCEPHALOCELES AMONG LIVE-BORN INFANTS IS:
A) One in 1000
B) One in 2000
C) One in 3000
D) One in 4000
E) One in 5000
6. WHICH OF THE FOLLOWING IS AN INDICATION FOR AN EMERGENCY OPERATION AMONG CHILDREN
WITH ENCEPHALOCELES?
A) Bone defect of more than 5 cm
B) Ulceration of skin with exposure of the brain or dura
C) Presence of ventricle within the encephalocele
D) Concomitant presence of a facial cleft
E) Elevated ␣-fetoprotein level

To complete the examination for CME credit, turn to page 722 for instructions and the response form.

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