Facial Clefts

CME

Common Craniofacial Anomalies: Facial Cleftsand EncephalocelesJeremy 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 pathogenesisof facial clefting and encephaloceles. 2. Recognize and classify facial clefts and encephaloceles. 3. Understand thedifferent treatment plans for reconstruction of facial clefts and encephaloceles.

The wide variety of craniofacial malformations makesclassification difficult. A simple classification system allowsan overview of the current understanding of the causes,assessments, and treatments of the most frequently en-countered craniofacial anomalies. Facial clefts and en-cephaloceles are reviewed with respect to their diversecauses, pathogenesis, anatomical features, and treat-ments. Approaches to the surgical treatment of these con-ditions are reviewed. (Plast. Reconstr. Surg. 112: 606,2003.)

The wide variety of craniofacial malforma-tions makes them difficult to classify. Gorlin etal.1,2 stated that our limited understanding ofthe embryological features and causes of mal-formations restricts efforts at classification. In1981, the Committee on Nomenclature andClassification of Craniofacial Anomalies of theAmerican Cleft Palate Association3 groupedcraniofacial disorders according to their di-verse causes, anatomical features, and treat-ment. The committee proposed a practical sim-ple classification system in which fivecategories of deformity are identified, as fol-lows: I, facial clefts/encephaloceles and dysos-tosis; II, atrophy/hypoplasia; III, neoplasia/hyperplasia; IV, craniosynostosis; and V,unclassified. This simple classification systemallows an overview of our current understand-ing of the causes, assessment, and treatment ofthe most frequently encountered craniofacialanomalies. Deformities involving craniofacialclefting and encephaloceles are reviewed and

discussed, as is the surgical correction of thesecraniofacial anomalies.

CRANIOFACIAL EMBRYOLOGICAL DEVELOPMENT

Normal embryological development of thehuman face and cranium was described bySperber.4 After union of the male and femalegametes, the formed zygote rapidly divides toform the morula and subsequently the blasto-cyst. 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 cutane-ous and neural portions by approximately day20. At that stage, the neural portion of theblastocyst is called the neural plate; with mid-line folding of this neural plate, the neuraltube is formed.

Of particular importance to the embryolog-ical development of craniofacial structures areneural crest cells. The neural crest ectomesen-chymal tissue arises from the crests of the neu-ral fold and forms a separate pluripotentialtissue layer. Neural crest ectomesenchyme hasgreat migratory propensities and is the majorsource of connective tissue throughout thebody, because translocated neural crest cellsdifferentiate into cartilage, bone, ligaments,muscles, and arteries. Any disruption in theorderly migration and differentiation of thesecells can have severe consequences, manifestedby congenital defects.5

From the Department of Plastic and Reconstructive Surgery, University of Texas Southwestern Medical Center. Received for publication May13, 2002; revised October 8, 2002.

DOI: 10.1097/01.PRS.0000070971.95846.9C

606

The first 12 weeks of gestation representthe crucial period of organogenesis, and it isduring this period that the majority of con-genital craniofacial anomalies are estab-lished.4,5 The earliest signs of the future faceappear at approximately day 23 or 24 of em-bryonic life, as paired mandibular processesof the first visceral arch. Next, the medialnasal processes combine with the interveningforebrain to form the frontonasal process,which is destined to become the foreheadand the dorsum of the nose. The lateral nasalfolds separate the olfactory pits from thegradually developing eye region. By the endof the fifth embryonic week, the maxillaryand mandibular processes have begun to in-crease in size but have not yet fused; it is notuntil the sixth week that definitive jaws areformed. By the end of the eighth week, theface assumes most of the characteristics thatmake it recognizable as human. The face isderived from five facial prominences thatsurround the future mouth, namely, the sin-gle frontonasal process and the paired max-illary and mandibular processes.

The grooves between these facial promi-nences usually disappear by day 46 or 47 ofgestation, as the processes meet their equiva-lents from the contralateral side and fuse inthe midline. Any persisting groove betweenmeeting or adjoining processes results in a con-genital facial cleft. Slavkin et al.6 demonstratedthat this process of fusion was attributable inpart to the ability of preprogrammed cells todifferentiate independently and allow normaldevelopment.

CAUSES OF CRANIOFACIAL ANOMALIES

Identification of the genetic bases for mostcraniofacial syndromes has exploded in thepast decade, and our knowledge continues toexpand. For conditions without an identifiedgenetic pattern of inheritance, four generalcategories of environmental “cleftogens” havebeen identified to date, as follows.

• Radiation. Large doses of radiation havebeen associated with microcephaly.

• Infections. The children of mothers withtoxoplasmosis, rubella, or cytomegalovirus in-fections exhibit increased frequencies of facialclefts.

• Maternal idiosyncrasies. Mothers of childrenwith cleft lips and palates have been noted toexhibit a higher-than-normal incidence of phe-nylketonuria, and the oculoauriculovertebral

spectrum has been observed with unusual fre-quency among infants with diabetic mothers.7Many studies have suggested maternal factorssuch as age, weight, and general health as poten-tial causes of malformations.

• Chemicals. Vitamin deficiencies are associ-ated with increased incidences of cleft lips andpalates, which may be reduced with vitamin-supplementation diets for the mothers. Vita-min A, its derivatives, and related compoundssuch as isotretinoin (Accutane; Hoffman-LaRoche, Inc., Nutley, N.J.) have been implicatedin the development of facial clefting and hemi-facial microsomia. Maternal smoking has beendemonstrated to be associated withcraniosynostosis.8

Other drugs are strongly suspected to becontributors in craniofacial syndromes. Gard-ner et al.9 observed associations between cra-niosynostosis and exposure to chlorphenira-mine, chlordiazepoxide, and nitrofurantoinbut no associations with hydantoin, valproicacid, or cocaine.

FACIAL CLEFTS

Anatomical Classification

The broad spectrum of anomalies attribut-able to facial clefting makes classification diffi-cult. In 1976, Tessier10 described an anatomicalclassification system in which a number is as-signed to each malformation on the basis of itsposition relative to the sagittal midline. Thissystem has become internationally acceptedand allows concise effective communicationamong clinicians (Fig. 1).

For orientation, the orbit is divided into twohemispheres; the lower lid, cheek, and lip dem-onstrate facial clefts, and the upper lid andcranium demonstrate cranial clefts. Accordingto Tessier’s scheme, clefts of the bones and softtissues do not always coincide and frequentlyseveral different clefts coexist. This classifica-tion system remains in wide use today becauseof its accuracy and because it is relatively easyto learn and allows communication with otherclinicians. David et al.11 demonstrated a com-plete series of these craniofacial clefts in three-dimensional computed tomographic scans.Clefting may present as a tissue deficiency or atissue excess. The tissue-deficiency disorders,namely, arhinencephaly and holoprosen-cephaly, are secondary to failure of embryonicprosencephalon cleavage and of the normallongitudinal split into cerebral hemispheres.

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The tissue-excess deformities result from fail-ure of complete tissue development and rangefrom a slight midline notch of the upper lip tosevere orbital hypertelorism.

Embryological Classification

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

logical events. They envisioned the craniofacialskeleton developing along a helical course sym-bolized by the letter S (Fig. 2).

Their scheme uses “focal fetal dysplasia” inpreference to “cleft” to describe an arrest inskin, muscle, or bone development and namesthe dysplastic anomaly on the basis of the areasinvolved. For malformations characterized by

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

608 PLASTIC AND RECONSTRUCTIVE SURGERY, August 2003

dysostoses, an additional distinction is madebetween transformation defects and develop-mental arrests that occur before fusion of thefacial processes.12

In the frontosphenoidal area, dysplasia maymanifest as a Tessier no. 9 cleft, a cloverleaf skullor kleeblattschädel deformity, or less often pla-giocephaly. Frontal dysplasia is associated withorbital hypertelorism and nasal dysplasia, a wid-ow’s peak, and dystopia of the eyebrow. This areacorresponds to Tessier no. 10 and 11 clefts. In-terfrontal dysplasia is associated with bone de-fects with or without encephaloceles, as observedwith Tessier no. 0 to 14 clefts. Nasal aplasia withproboscis corresponds to the ethmocephaly orcebocephaly described by DeMyer et al.13 Nasos-chizis (Tessier no. 1 cleft) involves deformity ofone-half of the nose, with a normal septum andnasal cavity.

The cleft lip produced with medial nasomax-illary dysplasia is usually observed in combina-tion with other deformities. When associated

with lateral nasomaxillary dysplasia, it is knownas the Morian I, Tessier no. 3, or naso-ocularcleft. The median (Tessier no. 4, Morian II)and lateral (Tessier no. 5, Morian III) oro-ocular clefts are dysplasias of the orbit andmaxilla that spare the nose. The deformityknown as maxillozygomatic dysplasia is equiva-lent to a Tessier no. 6 cleft or incompleteTreacher-Collins syndrome. The completeform of Treacher-Collins syndrome is charac-terized by zygotemporoauromandibular dys-plasia and corresponds to Tessier no. 6, 7, and8 clefts. Temporoauromandibular dysplasia isalso known as auromandibular dysostosis,hemifacial microsomia, or first and secondbranchial arch syndrome.

Maxillomandibular dysplasia is a failure ofthe maxillary and mandibular processes tofuse, resulting in macrostomia. Mandibulardysplasias, exemplified by the Pierre Robin syn-drome, are associated with micrognathia, glos-soptosis, and respiratory distress.

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).


Holoprosencephaly

The holoprosencephaly malformation repre-sents a hypoplastic Tessier no. 14 cleft in asso-ciation with a tissue deficiency or a tissue ex-cess.12,13 Cohen and Sulik14 presented amodern analytical review of the holoprosence-phalic disorders, in which the central nervoussystem findings and craniofacial anatomicalfeatures were discussed, syndromes and associ-ated anomalies were updated, and the differ-ential diagnosis was reviewed.

Elias et al.15 reviewed holoprosencephalyand midline facial anomalies in an attempt toredefine their classification and treatment.They noted that true holoprosencephaly en-compasses a series of midline defects of thebrain and face, with a spectrum of expression.In most cases, holoprosencephaly is associatedwith severe malformations of the brain that areincompatible with life. At the other end of thespectrum are patients with midline facial de-fects and normal or nearly normal brain devel-opment. With computed tomographic findingsand a period of observation, patients whocould benefit from surgical treatment can beselected.

Pathogenesis of Facial Clefts

There are two leading theories of facial cleftformation. The classic theory, championed byDursy16 and His,17 holds that clefts are causedby the failure of fusion of the facial processes.18

In this theory, the face forms as the fingerlikeends of the maxillary processes meet and coa-lesce with the united paired globular processesbeneath the nasal pits. Once epithelial contactis established, mesenchymal penetration com-pletes the fusion and the lip and hard palateare formed. When the sequence is disturbed,clefting occurs.

The mesodermal penetration theory was de-scribed by Pohlmann19 and Veau20 and waslater advocated by Stark and Saunders.21,22 Pro-ponents of the mesodermal penetration theorythink that free-end facial processes do not existand the face consists of a bilaminar ectodermalmembrane, with epithelial seams demarcatingthe major processes. The mesenchyme mi-grates into this double wall of ectoderm, pen-etrates it, and smoothes out the seams. If mes-enchymal penetration fails, then theunsupported epithelial wall dehisces and acleft is produced. The severity of the cleft is

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


inversely proportional to the success of meso-dermal penetration, with different degrees ofincomplete and complete clefts (Figs. 3 and 4).

Treatment of Facial Clefts

By far the most common craniofacial anom-aly is cleft lip/palate, followed by isolated cleftpalate as a distant second. The incidence ofrare clefts is estimated to be 1.4 to 4.9 cases per100,000 live births.23 Reconstruction initiallyfocuses on soft-tissue closure,23 with excision ofall scar within the cleft until normal tissue isreached, followed by meticulous, layered, soft-tissue closure. Because of underlying bony hypopla-sia, skeletal reconstruction is often necessary, al-though it is delayed until the child is older.23

Van der Meulen24 provided a thorough reviewof the pathological features, causes, and recon-struction of oblique facial clefts. He agreed withTessier’s principle of combining skeletal and soft-tissue realignment in one major surgical proce-dure. Resnick and Kawamoto,25 Galante andDado,26 and Fuente del Campo27 provided spe-cific recommendations for the treatment of un-usual facial clefts, on the basis of their respectiveexperiences with Tessier no. 4, 5, and 8 clefts.

Tissue expansion and its application in facialclefting were described by Menard et al.,28 whoreported soft-tissue closure for eight patients withfacial clefts. In summary, the goals of surgicaltreatment of facial clefts include functional cor-rection of macrostomia, soft-tissue reconstruc-tion of the eyelid to prevent globe exposure,separation of the confluent oral, nasal, and or-bital spaces, and aesthetic correction of thedeformity.

ENCEPHALOCELES

An encephalocele is a protrusion of part of thecranial contents through a defect in the skull.The mass may contain meninges (meningocele),meninges and brain (meningoencephalocele),or meninges, brain, and ventricle (meningoen-cephalocystocele).29 Encephaloceles are catego-rized according to their positions in the skull andcan be basal, sincipital, or convexity.30 The sin-cipital group can be further divided into fronto-ethmoidal, interfrontal, and associated withclefts. The frontoethmoidal group can be subdi-vided into nasofrontal, nasoethmoidal, and naso-orbital types.31 Boonvisut et al.32 reviewed themorphological features of 120 skull base defectsand classified the defects as involving either asingle opening (type I) or multiple openings(type II) between the frontal, nasal, ethmoidal,and orbital bones. The groups were further di-vided into type IA/IIA, in which the defect waslimited to two bones within the area, and typeIB/IIB, in which the defect extended laterally toinvolve adjacent structures (Fig. 5).

The presence of an encephalocele may bedetected on antenatal ultrasonograms or withelevated �-fetoprotein levels.30 The differentialdiagnosis of frontal midline masses includesencephaloceles, teratomas, gliomas, and der-moids,33,34 and high-resolution computed to-mographic scans can establish the intracranialcomponent of encephaloceles.34 In a fronto-ethmoidal or nasal encephalocele, the cranialdefect is in the anterior midline between thefrontal bone (preformed in membrane) andthe ethmoid process (preformed in cartilage).The craniofacial deformity involves hypertelor-ism, orbital dystopia, elongation of the face,and dental malocclusion, reflecting the distort-ing effects on facial bone growth of the ex-truded intracranial contents.

The pathogenesis of frontoethmoidoen-cephaloceles is as follows. Early in embryogen-esis, diverticula of dura project anteriorlythrough the fonticulus nasofrontalis (a small

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


fontanelle between the developing nasal andfrontal bones) or inferiorly through the devel-oping frontal bone into the prenasal space.These diverticula may come in contact withskin and adhere to it. Normally, the diverticularegress and the bone closes, creating the nor-mal nasofrontal suture anteriorly and, passingthrough the skull base just anterior to the cristagalli, the foramen cecum. In a frontoethmoidalencephalocele, the diverticulum does not re-cede and the bone does not close. The causesof encephaloceles are unknown but involveracial, genetic, environmental, and paternalfactors.35 Encephaloceles also occur with anumber of craniofacial syndromes.36

The worldwide incidence of encephalocelesis one case per 5000 births.37 In Western Eu-rope, North America, Australia, and Japan,back-of-the-head encephaloceles predominate;of 265 encephaloceles recorded in more than20 years, 196 were occipital. In Southeast Asiaand Russia, however, anterior encephalocelesoutnumber posterior encephaloceles in a 9.5:1ratio.37,38 The reason for this discrepancy isunknown (Figs. 6 and 7).

The principles of encephalocele treatmentinclude incision of the sac, amputation ofexcess tissue to the level of the surroundingskull, closure of the dura, and closure of theskin.39 David,40 Forcada et al.,41 and Smit etal.42 reviewed the spectrum of cranial andcerebral malformations that may occur withfrontoethmoidal encephaloceles and dis-cussed the diagnosis and treatment of thesedeformities. David40 analyzed the experienceof the Australian Craniofacial Unit from1975 to 1993 and reached the following con-clusions with respect to frontoethmoidal en-cephaloceles. (1) Early complete surgicaltreatment is indicated to allow the develop-ing brain and eyes to remodel the facial de-formity. (2) Intracranial abnormalities arecommon. (3) Frontoethmoidal encephaloce-les differ from other neural tube defects inthe absence of a familial pattern and thepeculiar geographic distribution. (4) Treat-ment with a craniofacial technique is best.(5) Established deformities can be effectivelytreated with craniofacial osteotomies. (6)Most patients exhibit abnormal intercanthal

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., Suja-tanond, M., et al. Morphologic study of 120 skull base defects in frontoethmoidal encepha-lomeningoceles. Plast. Reconstr. Surg. 101: 1784, 1998).


distances but normal interpupillary and lat-eral canthal measurements. (7) The frontalsinus region often requires repeat bonegrafting, and nasal bone grafts often must bereplaced as patients age. (8) Treatment ofcraniofacial clefts should be postponed untilgrowth is complete. (9) Early treatment ofpatients with basal encephaloceles is indi-cated, to prevent further damage and infec-tion. (10) Surgical treatment of extensivebasal encephaloceles is complex and proba-bly should be performed through a facialhemisection approach.

Holmes et al.43 reported their experiencewith 35 cases of frontoethmoidal encephalo-celes. The goals of treatment are (1) urgentclosure of open skin defects, to prevent in-fections and desiccation of brain tissue, (2)removal or invagination of nonfunctional ex-tracranial tissue, (3) watertight dural closure,and (4) total craniofacial reconstruction,particularly avoiding the “long-nose deformi-ty.” To correct the deformity caused by hy-pertelorism and a long midface, Holmes etal.43 lower the supraorbital bar by rotating itmedially, posteriorly, and downward in themidline, with lateral widening to correct thetrigonocephalic deformity. Successful correc-tion is dependent on (1) an understandingof the pathological anatomical features, (2)careful planning of osteotomies and bonemovements that correct the entire deformity(including trigonocephaly and the long-nosedeformity), (3) nasal reconstruction with acantilever graft, to avoid the long-nose defor-mity, (4) skin closure that removes abnormalskin, with careful attention to the positioningof scars, (5) transnasal canthoplasty to repo-sition the medial canthi, and (6) single-stagesurgical treatment, involving craniofacialand neurosurgical expertise.

P. Craig Hobar, M.D.Department of Plastic and Reconstructive SurgeryUniversity of Texas Southwestern Medical Center411 N. Washington AvenueSuite 6000, LB 13Dallas, Texas [email protected]

ACKNOWLEDGMENTS

We thank Marco A. Medina and Leesa Thompson, TheFogelson Plastic Surgery and Craniofacial Center for Chil-dren (Dallas, Texas), for invaluable assistance and contribu-tions to the article.

FIG. 6. Nasofrontal form of frontal encephalocele.

FIG. 7. Interfrontal encephalomeningocele.


REFERENCES

1. Gorlin, R. J., Pindborg, J. J., and Cohen, M. M. Syndromesof the Head and Neck, 2nd Ed. New York: McGraw-Hill,1976.

2. Gorlin, R. J. Classification of craniofacial syndromes. InJ. M. Converse and J. G. McCarthy (Eds.), Symposiumon the Diagnosis and Treatment of Craniofacial Anomalies.St. Louis, Mo.: Mosby-Year Book, 1979.

3. Whitaker, L. A., Pashayan, H., and Reichman, J. A pro-posed new classification of craniofacial anomalies.Cleft Palate J. 18: 161, 1981.

4. Sperber, G. H. Craniofacial Embryology, 4th Ed. London:Wright, 1989.

5. Johnston, M. C. Embryology of the head and neck. InJ. G. McCarthy (Ed.), Plastic Surgery, Vol. 4. Philadel-phia: Saunders, 1990. Pp. 2451–2495.

6. Slavkin, H. C., Bringas, P., Jr., Sasano, Y., and Mayo, M.Early embryonic mouse mandibular morphogenesisand cytodifferentiation in serumless, chemically de-fined medium: A model for studies of autocrineand/or paracrine regulatory factors. J. Craniofac.Genet. Dev. Biol. 9: 185, 1989.

7. Cohen, M. M., Jr., Rollnick, B. R., and Kaye, C. I. Ocu-loauriculovertebral spectrum: An updated critique.Cleft Palate J. 26: 276, 1989.

8. Kallen, K. Maternal smoking and craniosynostosis. Ter-atology 60: 146, 1999.

9. Gardner, J. S., Guyard-Boileau, B., Alderman, B. W., Fern-bach, S. K., Greene, C., and Mangione, E. J. Maternalexposure to prescription and non-prescription phar-maceuticals or drugs of abuse and risk of craniosyn-ostosis. Int. J. Epidemiol. 27: 64, 1998.

10. Tessier, P. Anatomical classification of facial, craniofa-cial and latero-facial clefts. J. Maxillofac. Surg. 4: 69,1976.

11. David, D. J., Moore, M. H., and Cooter, R. D. Tessierclefts revisited with a third dimension. Cleft Palate J. 26:163, 1989.

12. Van der Meulen, J. C., Mazzola, R., Vermey-Keers, C.,Stricker, M., and Raphael, B. A morphogenetic clas-sification of craniofacial malformations. Plast. Recon-str. Surg. 71: 560, 1983.

13. DeMyer, W., Zeman, W., and Palmer, C. G. The facepredicts the brain: Diagnostic significance of medianfacial anomalies for holoprosencephaly (arhinen-cephaly). Pediatrics 34: 256, 1964.

14. Cohen, M. M., Jr., and Sulik, K. K. Perspectives on ho-loprosencephaly: II. Central nervous system, cranio-facial anatomy, syndrome commentary, diagnostic ap-proach, and experimental studies. J. Craniofac. Genet.Dev. Biol. 12: 196, 1992.

15. Elias, D. L., Kawamoto, H. K., Jr., and Wilson, L. F. Ho-loprosencephaly and midline facial anomalies: Rede-fining classification and management. Plast. Reconstr.Surg. 90: 951, 1992.

16. Dursy, E. Zur Entwicklungsgeschichte des Kopfes des Men-schen und der Hoeheren Wirbelthiere. Tübingen, Germa-ny: Verlag der H. Lauppschen-Buchhandlung, 1869.P. 99.

17. His, W. Die Entwickelung der menschlichen undtierischer Physiognomen. Arch. Anat. Entwicklungsge-sch. 384, 1892.

18. Kawamoto, H. K. The kaleidoscopic world of rarecraniofacial clefts: Order out of chaos (Tessier classi-fication). Clin. Plast. Surg. 3: 529, 1976.

19. Pohlmann, E. H. Die embryonale Metamorphose derPhysiognomie und der Mundhoehle des Katzenko-pfes. Morphol. Jahrb. Leipzig 41: 617, 1910.

20. Veau, V. Hasencharten menschlicher Keimlinge aufder Stufe 21–23 mm SSL. Z. Anat. Entwicklungsgesch.108: 459, 1938.

21. Stark, R. B. The pathogenesis of harelip and cleft pal-ate. Plast. Reconstr. Surg. 13: 20, 1954.

22. Stark, R. B., and Saunders, D. E. The first branchialsyndrome: The oral-mandibular-auricular syndrome.Plast. Reconstr. Surg. 29: 299, 1962.

23. Ozaki, W., and Kawamoto, H. K., Jr. Craniofacial cleft-ing. In K. Y. Lin, R. C. Ogle, and J. A. Jane (Eds.),Craniofacial Surgery: Science and Surgical Technique. Phil-adelphia: Saunders, 2002. Pp. 309–331.

24. van der Meulen, J. C. H. Oblique facial clefts: Pathol-ogy, etiology, and reconstruction. Plast. Reconstr. Surg.76: 212, 1985.

25. Resnick, J. I., and Kawamoto, H. K., Jr. Rare craniofacialclefts: Tessier no. 4 clefts. Plast. Reconstr. Surg. 85: 843,1990.

26. Galante, G., and Dado, D. V. The Tessier number 5cleft: A report of two cases and a review of the liter-ature. Plast. Reconstr. Surg. 88: 131, 1991.

27. Fuente del Campo, A. Surgical correction of Tessiernumber 8 cleft. Plast. Reconstr. Surg. 86: 658, 1990.

28. Menard, R. M., Moore, M. H., and David, D. J. Tissueexpansion in the reconstruction of Tessier craniofa-cial clefts: A series of 17 patients. Plast. Reconstr. Surg.103: 779, 1999.

29. Jackson, I. T., Tanner, N. S. B., and Hide, T. A. H. Fron-tonasal encephalocele: “Long nose hypertelorism.”Ann. Plast. Surg. 11: 490, 1983.

30. Simpson, D. A., David, D. J., and White, J. Cephaloceles:Treatment, outcome, and antenatal diagnosis. Neuro-surgery 15: 14, 1984.

31. Suwanwela, C., and Suwanwela, N. A morphologicalclassification of sincipital encephalomeningoceles.J. Neurosurg. 36: 201, 1972.

32. Boonvisut, S., Ladpli, S., Sujatanond, M., et al. Mor-phologic study of 120 skull base defects in frontoeth-moidal encephalomeningoceles. Plast. Reconstr. Surg.101: 1784, 1998.

33. Mazzola, R. F. Congenital malformations in the fron-tonasal area: Their pathogenesis and classification.Clin. Plast. Surg. 3: 573, 1976.

34. David, D. J., Sheffield, L., Simpson, D., and White, J.Fronto-ethmoidal meningoencephaloceles: Morphol-ogy and treatment. Br. J. Plast. Surg. 37: 271, 1984.

35. Jacob, O. J., Rosenfeld, J. V., and Watters, D. A. K. Therepair of frontal encephaloceles in Papua NewGuinea. Aust. N. Z. J. Surg. 64: 856, 1994.

36. Cohen, M. M. Craniosynostosis and syndromes with cra-niosynostosis: Incidence, genetics, penetrance, vari-ability, and new syndrome updating. Birth Defects 15:13, 1979.

37. Humphreys, R. P. Encephalocele and dermal sinuses.In W. R. Cheek, A. E. Marlin, D. G. McLone, D. H.Reigel, and M. L. Walker (Eds.), Pediatric Neurosurgery:Surgery of the Developing Nervous System, 3rd Ed. Phila-delphia: Saunders, 1994. P. 96.

38. Charoonsmith, T., and Suwanwela, C. Frontoethmoidalencephalomeningocele with special reference to plas-tic reconstruction. Clin. Plast. Surg. 1: 27, 1974.

39. Pollack, I. F. Management of encephaloceles and


craniofacial problems in the neonatal period. Neuro-surg. Clin. North Am. 9: 121, 1998.

40. David, D. J. Cephaloceles: Classification, pathology, andmanagement: A review. J. Craniofac. Surg. 4: 192, 1993.

41. Forcada, M., Montandon, D., and Rilliet, B. Frontoeth-moidal cephaloceles: Transcranial and transfacial sur-gical 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 14consecutive 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 onthe next page.


Self-Assessment Examination

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

1. REGARDING CRANIOFACIAL ORGANOGENESIS, THE CRUCIAL PERIOD OF DEVELOPMENT TAKES PLACEIN THE:A) First 48 hoursB) First weekC) First trimesterD) Second trimesterE) Third trimester

2. ENVIRONMENTAL CLEFTOGENS IDENTIFIED IN THE DEVELOPMENT OF CRANIOFACIAL ANOMALIESINCLUDE ALL OF THE FOLLOWING EXCEPT:A) RadiationB) CytomegalovirusC) IsotretinoinD) CocaineE) Vitamin deficiency

3. WHICH OF THE FOLLOWING PRINCIPLES OF RECONSTRUCTION OF FACIAL CLEFTING IS LEASTIMPORTANT?A) Excision of all scars within the cleftB) Initial soft-tissue closureC) Meticulous, layered, soft-tissue closureD) Immediate skeletal reconstructionE) Separation of oral, nasal, and orbital spaces

4. THE CRANIOFACIAL DEFORMITY OF NASAL ENCEPHALOCELE INCLUDES ALL OF THE FOLLOWINGEXCEPT:A) HypertelorismB) Orbital dystopiaC) Elongation of the faceD) Dental malocclusionE) Alveolar cleft

5. THE WORLDWIDE INCIDENCE OF ENCEPHALOCELES AMONG LIVE-BORN INFANTS IS:A) One in 1000B) One in 2000C) One in 3000D) One in 4000E) One in 5000

6. WHICH OF THE FOLLOWING IS AN INDICATION FOR AN EMERGENCY OPERATION AMONG CHILDRENWITH ENCEPHALOCELES?A) Bone defect of more than 5 cmB) Ulceration of skin with exposure of the brain or duraC) Presence of ventricle within the encephaloceleD) Concomitant presence of a facial cleftE) Elevated �-fetoprotein level

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

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Facial Clefts