A Proposed New Classification of Craniofacial Anomalies

LINTON A. WHITAKER, M.D. HERMINE PASHAYAN, M.D. JOSEPH REICHMAN, M.D.

for The American Cleft Palate Association Committee on Nomenclature and Classification of Craniofacial Anomalies

Introduction In the fall of 1976, Kenneth R. Bzoch, President of the American Cleft Palate Association, requested the formation of an Ad Hoc Committee for Reclassification of Craniofacial Anomalies. The impetus for this was the rapid growth of new knowledge and understanding about the malformations that had occurred as a result of the development of craniofacial surgery during the previous few years. The original committee consisted of the following people: Linton A. Whitaker, M.D., Chairman; Howard Aduss, D.D.S.; Asa Berlin, Ph.D.; Mutaz Habal, M.D.; and Joseph Reichman, M.D. Each of these people helped in the early formulation of ideas about the new classification. Subsequently, Malcolm Johnson, D.D.S., and Hermine Pashayan, M.D. were added to the committee. The paper, as finally developed and edited, was written by Drs. Whitaker, Reichman, and Pashayan and was completed in the spring of 1980. Since it was thought to be desirable to present the information at the International Cleft Palate Meeting in Acapulco in May, 1981, publication has been withheld until following that meeting. KEY WORDS: craniofacial anomalies, classification of anomalies, malformations

Malformations of the craniofacial complex cian requires simplicity with clinical applica- abound with great variation. Previously, fa- bility. Such a system should be broadly based cial malformations were usually categorized both etiologically and anatomically and by two means: 1) pathogenic mechanisms and should, ideally, combine treatment goals and 2) anatomic conditions. These two areas were principles. , often mutually exclusive. The confusion in A review of some of the more widely used nomenclature arises because the names of syn- classification systems is given below. Anoma- dromes (Le., Crouzon's, Treacher-Collins, lies of the face have most often been classified etc.) do not fulfill the criteria necessary for according to a major structure involved, i.e. either the basic scientists or the clinician. lip and palate, eye, nose, mandibular, and They arose serendipitously or by isolated ob- maxillary abnormalities. servations with no effort to fit them into a system as a whole. Because of such occur- Pathogenetic Classification rences, there is now the need for a compre- hensive system that will allow the classifica- I. Lie aAnp PaAuratE tion of new syndrome entities without the use Of all facial deformities, classification of of eponyms. In formulating a new system, it cleft lip and palate has received the most is desirable to satisfy both the basic scientist attention. Superb reviews of classification sys- and the clinician. tems have been done by Millard (1976) and The basic scientist desires an embryologic Berlin (1971). While the earlier classification or biochemical classification, while the clini- systems of Davis and Ritchie (1922) and Veau 161 162 Cleft Palate Journal, July 1981, Vol. 18 No. 3

(1931) were based upon "operative" (ana- In the International Classification, several tomic) findings, recent advances in under- problems are apparent: standing the embryology of cleft lip and pal- 1. Numbering of groups may cause confu- ate laid the foundations of a more complete sion with the classifications of Veau and classification. These advances evolved into the Davis and Ritchie. (1972) present systems: 2. The term "anterior palate" is used A. International Classification: rather than "primary palate" (Kerna- Classification of the lip, alveolus, and pal- han and Stark, 1955) or prepalate ate (based on embryological principles) (Ber- (ACPA) (1968) lin, 1971): 3. Median clefts are listed under facial Group 1: Clefts of the anterior (primary) clefts rather than clefts of the lip. palate 4. The lip is not truly an embryologic part a. Lip: right and/or left of the "primary" palate. b. Alveolus: right and/or left It seems that the classifications of Kernahan Group 2: Clefts of the anterior and posterior and Stark (1955) and of the American Cleft (primary and secondary) palates: Palate Association (Haskins et al., 1962) are a. Lip: right and/or left the best. Both are relatively simple and incor- b. Alveolus: right and/or left porate three major areas: c. Hard palate: right and/or left 1. Clefts anterior to the incisive foramen Group 3: Clefts of the posterior (secondary) 2. Clefts posterior to the incisive foramen palate 3. Combinations of both types of clefts a. Hard palate: right and/or left While there is no agreement as to which b. Soft palate: median system is best, a more visual mode of catego- (For further subdivisions, the terms "total" rization was presented by Kernahan (1973) in and "partial" should be used.) the form of a "Y." This was modified by Rare Facial Clefts Based Upon Topograph- Elsahy (1973) and Millard (1976): . ical Findings a) Cleft: area stippled a. Median clefts of the upper lip with or b) Submucosal muscle and bony cleft: hor- without hypoplasia or aplasia of pre- izontal lines maxilla c) Nasal deformity marked with horizontal b. Oblique clefts (oro-orbital) lines proportionate to severity c. Transverse clefts (oro-auricular) d. Clefts of the lower lip, nose, and other II. Face anp SKULL very rare clefts. B. Kernahan and Stark (1955): Classification ~The most extensive overall compilation of using the incisive foramen as a boundary facial malformation with regard to patho- marker: , physiology is that proposed by Pruzansky 1. Clefts of the primary palate: lip and (1975): Sociogenic and iatrogenic factors premaxilla - Intrauterine mechanical factors 2. Clefts of the secondary palate: hard and h Focal necrosis soft palate posterior to incisive foramen. C0 Morphokinetic arrest Further description as to left, right, com- iB Primary malformations of the skull plete, and incomplete are added. i C. American Cleft Palate Association (Harkins, with secondary effects on the brain Primary malformations of the CNS et al. 1962): O 1. Prepalate: unilateral, bilateral, and me- with secondary deformities of the skull dian lip clefts; also alveolar clefts 7. Chromosomal abnormalities 2. Palate: all palatal clefts to incisive fora- 8. Environmental factors men 9. Inborn errors of metabolism 3. Prepalate and palate: unilateral and bi- 10. Syndromes of unknown etiology lateral complete clefts; incomplete clefts Sociogenic and iatrogenic factors are not of lip and palate relevant to the present classification. The 4. Rare clefts other than prepalatal or pal- ramifications of intrauterine mechanical fac- atal types. tors are uncertain. There is experimental data Whitaker et al., Crassrication or Cr Anomalies 163

(Trasler, et al., 1956) demonstrating that am- e. vomer and cartilagenous nasal sep- niotic sac puncture constricts the embryo and tum may cause cleft palate by mechanical inter- f. premaxillary bone ference. Furthermore, amniotic strands have g. anterior primary palantine triangle been found to be attached to the head and 2. Lateral brachial arch segments (2) mouth of human fetuses (Torpin, 1968). Am- Skeletal elements of the face derived from the niotic strands have also accounted for ampu- brachial arches: tations of digits. . a. temporal bone, in part, and ossicles The role of focal necrosis as a causal mech- b. zygomatic arch anism has been pursued in recent years. Pos- c. maxillary bone willo (1973, 1975b) has demonstrated an ex- d. mandible perimental model for hemifacial microsomia e. hard palate by the induction of hemorrhage of the prim- The face can then be divided into three itive stem of the stapedial artery. The extra- horizontal planes. Malformations may affect vascular blood clot results in the sequential one horizontal segment solely or predomi- processes of focal tissue death, repair, and nantly. The upper (frontal) horizontal seg- redifferentiation. It is felt that the primitive ment derives solely from the frontal (fronto- pattern of morphodifferentiation observed in nasal) process. The middle (maxillary) seg- the affected neonate is a result of varying ment is derived from the maxillary process of degrees of primary damage followed by a the first brachial arch and the prolabium greater or lesser degree of "catch-up" remod- (from the frontonasal process). The third hor- eling. A similar model (Poswillo, 1975a) is izontal segment, the lower (mandibular) seg- postulated for mandibulofacial dysostosis but ment is derived from the mandibular process with a different pathogenic mechanism. of the first brachial arch. There is early destruction of neural crest cells In studying median facial anomalies, the au- of the facial and auditory primordia which thor classifies five types of tissue defic1ency migrate to the first and second brachial disorders which he terms holoprosencephaly arches. (DeMeyer, 1964):

An example of morphokinetic arrest is that . i- Cyclopia Ethmocephaly of hypertelorism. It is conceivable that the Nn Cebocephaly angulation between the orbits might remain So

in the fetal state because of a midline aber- ~ With median cleft lip (premaxillary age- ration. nesis) Craniostenosis is an example of a primary 5. With median philtrum-premaxilla an- malformation of the skull which may affect * lage the brain. Premature closure of sutures is When there is a normal or an excess amount associated with increased intracranial pres- of tissue in the midline structure, DeMeyer sure or facial deformity. v (1967) favors the term median cleft face syn- DeMeyer, et al. (1964) believe that the drome and describes seven features of this "face predicts the brain", i.e., some facial entity.

malformations predict brain malformations. i- Orbital hypertelorism U-shaped frontal hairline They assessed facial malformations based on N

embryological types of classifications. yo Cranium bifidum occultum

The face can be separated, embryologically, B Median cleft of upper lip Median cleft of premaxilla into horizontal and vertical planes. The ver- n

tical segments are: J Median cleft of palate

1. Frontonasal segment (frontonasal pro- ~ Primary telecanthus cess) In conclusion, DeMeyer states that, when Median plane facial skeletal elements derived hypotelorism is present, there is almost cer- from the frontonasal prominence: tainly brain damage. When hypertelorism is

p frontal bones associated with one of the other malforma- crista galli 0 tions, the probability of mental retardation is

a ethmoid bone low but increases when the hypertelorism is

A nasal bones extreme and is the sole facial anomaly. 164 Cleft Palate Journal, July 1981, Vol. 18 No. 3

When the clinician looks at these systems, complete absence of tissue. A broad classifi- there are many problems. While he is capable cation is one proposed by Tessier (1976) uti- of assessing etiology of these disorders, he has lizing a clockface analogy from 0 to 14 (see virtually no information with regard to clini- Table 1). The point of reference for these cal classification. The first classification of clefts is the orbit with the clefts found in two rare craniofacial clefts was by Morian (1887). different hemispheres. Those of the lower lid He described three types of clefts. Recently, region are facial, while those of the upper lid there has been renewed interest in the rare are cranial. Clefts 0 through 4 have extensions clefts. The American Cleft Palate Association downward to involve the maxilla and fit into classification (Harkins et al., 1962) was men- the usual cleft lip and palate classifications. tioned earlier. This was then modified by Boo- Their superior extensions are the more severe Chai (1970) who subdivided oro-ocular clefts major craniofacial anomalies. Cleft 0, (Figures into Type I (coursing from lateral to cupids 1 and 2), with its cranial extension number bow to medial canthus) and Type II (from 14, is a true midline abnormality frequently lateral to cupids bow to mid-lower lid or resulting in unilobed brain or holoprosence- lateral canthus). phaly, incompatible with life. Hypotelorism In 1966, Karfik proposed a detailed classi- is usual with underdevelopment of tissue in fication of rare clefts. Most recently, Tessier the midline though, conversely, hypertelorism (1976) formulated a classification system may occur. This cleft and 1 through 3, along based upon his extensive personal experience. with their cranial extensions 13 through 11, This system uses the orbit as the frame of are centric (Figures 3, 4, and 5). The impor- reference and the clefts are based around this axis. TABLE 1. A Modification of Tessier's Cleft _ Classification System

A Proposed New Classification System A. Basic Considerations Based on extensive recent experience in the 1. The point of reference is the orbit with the clefts treatment of craniofacial anomalies, a new, found in two different hemispheres. practical, and simple classification system is a. Those of the lower lid are classified as facial suggested here. clefts b. Those of the upper lid are classified as cranial All craniofacial anomalies may be classified clefts into five categories based on etiology, anat- c. Combined or craniofacial clefts may occur omy, and current treatment principles: 2. This system describes both the surface and under- I. Clefts lying bony anatomy. Centric 3. The extent of involvement of soft and bony tissue Acentric is variable. II. Synostoses B. The Classification Symmetric CENTRIC Asymmetric III. Atrophy Facial Clefts Corresponding Cranial Extension IV. Neoplasia-hyperplasia of Facial Clefts V. Unclassified No. 0 No. 14 All may vary in their manifestation from No. 1 No. 13 subtle to extreme. ' No. 2 No. 12 No. 3 No. 11 ACENTRIC Clefts Clefts may be subclassified in many ways. Facial Clefts Cranial Clefts They all result in regions of tissue deficiency No. 4 No. 10 which require shifts of tissue for correction. No. 5 No. 9 Usually, both bone and soft tissue are in- No. 6 volved. All gradations of hypoplasia may oc- No. 7 cur from the most minimal to wide defects of No. 8 Whitaker et al., Crassirication or Cr AnxnomauiEs 165

FIGURE 1. Centric cleft in 0 position with holopro- FIGURE 2. Centric cleft in 0 position with orbital sencephaly, orbital hypotelorism, absent midline struc- hypertelorism and cranial extension in #14 position. tures, and nasofrontal encephalocele in #14 position. Figures 1 and 2 show contrasting orbit displacement and variations of clefts located in the same position. tance of this distinction is that structures lat- through the zygomaticofrontal suture (Fig- eral to this, including the cranium, orbits, and ures 7 and 8). All result in hypoplasia in these zygoma-maxilla, are displaced laterally by the regions, though, with hemifacial microsomia, pathologic process and must be repositioned the etiology is different from that of Treacher- medially along with adding tissue to achieve Collins Syndrome (Poswillo, 19752). Clefts 9, correction. The lower extensions of clefts 1, 2, 10, and 11 are associated with deformities of 3, and 4 result in cleft lip and palate with the superior orbit-anterior cranial base and clefts of the maxilla and alveolus. Extension are rare (Figures 9 and 10). onto the mandible occurs rarely with associ- Treatment mechanisms involve reposition- ated deformities such as notching of the lower ing or reconstruction of structures that have lip, bifid tongue, and deformities of the chin, been displaced by the clefts. Number 0 and neck, and hyoid bone. 14 is often incompatible with life and, there- The other facial clefts, 4 through 8 and fore, not treated. Orbital hypotelorism may their cranial counterparts 9 through 11, are be present as a component of these clefts. acentric and require addition of bone or soft When it is not associated with holoprosence- tissue. Four in its upward extension goes into phaly and a true midline cleft, a similar de- the orbit lateral to the nasolacrimal region formity generally occurs with synostosis. (Figure 6) but medial to the infraorbital When the latter occurs, it is usually compati- nerve. Five is a cleft of the maxilla and zyg- ble with life and may appear similar clini- oma extending from lateral to the infraorbital cally, but no cleft of the lip or nose is present. nerve into the region of the canine teeth and It is, in fact, a masquerade of the true 0,14 resulting in a true isolated facial cleft. This cleft. It is important to distinguish between cleft is extremely rare. the two as treatment and timing vary. The Clefts 6, 7, and 8 are associated with hem- synostoses are preferentially treated in the ifacial microsomia if unilateral and with first six months of life by removing or reposi- Treacher-Collins Syndrome if bilateral. Six tioning abnormal structures. Clefts may be extends through the zygoma at about the treated, if severe or extreme, in the first few zygomaticomaxillary suture, 7 through the months of life by adding tissue or rearranging zygomaticotemporal suture region, and 8 it. The majority, however, are treated after

166 Cleft Palate Journal, July 1981, Vol. 18 No. 3 __

FIGURE 3. Centric cleft in #1 position with orbital hypertelorism, notched ala nasi, and laterally displaced left nasal bone. Cranial extension in #13 position.

FIGURE 4. Centric cleft in #2 position with orbital hypertelorism, bifid nasal bones, and nasal and lip de- formities.

FIGURE 5. Centric cleft in #3 position with orbital hypertelorism, absent nasolacrimal drainage system, and lip and nose distortions. Cranial extension in positions 11 and 12 with forehead distortions.

Whitaker et al., Crassirication or Cr AnomaLiEs 167

with the deformities in the lateral orbital region. In Treacher-Collins Syndrome, a tis- sue hypoplasia syndrome involving clefts 6, 7, and 8, lateral canthal displacement, and co- lobomas are frequent. In clefts 9, 10, and 11, globe abnormalities and cranial base deform- ities as well as eyebrow deformities may occur.

Synostoses

Symmetric Symmetrical synostoses result from closures of the metopic suture, the coronal suture, the sagittal suture, or one of the posterior sutures. Closure of the posterior sutures rarely requires FIGURE 6. Acentric cleft in #4 position with hypo- plasia of left zygoma, extension downward to the lip with surgery about the face. Bilateral coronal su- subcutaneous cleft and notching of the eyelid lateral to ture synostosis is associated with the craniofa- the lacrimal puncta. cial dysostosis syndromes, including Apert's and Crouzon's, with abnormalities of the forehead, the orbits, and the midface, includ- the first year of life. Procedures involve major ing the maxilla (Figures 11 and 12). Nasal shifts of the orbit and are complex, prolonged protrusion and high arched palatal deformi- operations. This is particularly true with up- ties usually occur. Associated with these syn- per extensions of clefts 1, 2, and 3 and their ostoses are abnormalities of the anterior cra- cranial components 13, 12, and 11. Clefts 1, nial base, generally vertical positioning, and 2, and 3 in their inferior extension are closed shallow orbits. Upper forehead protrusion, at the usual times for cleft lip and palate. lower forehead retrusion, shallow orbits with Clefts 4 and 5 may be treated early, if extreme, protrusion of the globe or exorbitism, and and later if not so severe. Clefts 6, 7, and 8 zygomaticomaxillary hypoplasia with Class II are treated with established treatment prin- malocclusion are found. Low set ears are fre- ciples and time considerations for hemifacial quent. Many variations in the synostoses oc- microsomia and Treacher-Collins Syndrome cur. If there are associated hand abnormali- usually later in childhood. Clefts 9, 10, and ties, Apert's Syndrome is designated (Figure 11 are also treated early if extreme, but later 13). Rieger's Syndrome (Figure 14) is associ- if they are not. ated with acentric pupils. Telecanthus is fre- Associated with clefts 10 through 14, en- quent in all of the syndromes, and a minimal cephalocele may occur. Clefts of the nostril grade of orbital hypertelorism is often found. may occur with 1, 2, or 3. Numbers 4 and 5 This occurs as a result of lack of forward may result in a cleft of the lip as well as the growth of the skull with compensatory hori- zygoma-maxilla and a coloboma of the eyelid. zontal growth in coronal synostosis. Hemifacial microsomia corresponds to these If the lower portion of the metopic or the clefts, but is etiologically based on Poswillo's sagittal suture closes prematurely, there is work (1973, 1974, 1975b), the result of a symmetrical lack of lateral rotation of the vascular accident in utero, resulting in under- orbits and orbital hypotelorism. Often there development or hypoplasia of the mandible- is a triangulation defect of the skull anteriorly zygoma-maxilla and orbit. This varies in se- with scaphycephaly (Figure 15). verity from extremely subtle to severe. Ear Etiologically, these structural changes are problems are associated in their subtle forms important in the light of current treatment with minor displacements and in the more methods. Craniectomies with release of the severe forms with extreme displacement or coronal suture and the metopic suture are absence of the ear. Temporomandibular joint performed in the first six months of life. At problems sometimes occur, and lateral can- the same time, the orbits, forehead, and nose thal displacement is common in association may be repositioned. The zygomaticomaxil-

168 Cleft Palate Journal, July 1981, Vol. 18 No. 3

FIGURE 7. Symmetrical acentric clefts in positions 6, 7, and 8 with severe zygomatic hypoplasia and secondary pseudocoloboma of the lower eyelids. Mandibular abnormality with short ramus, obtuse gonial angle, long vertical chin, and microtia also present. Mandibulofacial dysostosis. lary region may be treated using the excess side, downward displacement of the ear, and bone from the craniectomy. upward shift of the mouth. Variations include The spectrum of anomalies in these syn- protrusion of the forehead on the opposite dromes varies, as it does in with others, from side and shifts of the opposite orbit. Com- extremely subtle to severe. monly associated is a contralateral flattening of the occipitoparietal region (Figures 16 and Asymmetric 17). Unilateral closure of the coronal or lamb- doid suture results in asymmetric deformities with orbital displacement, zygomaticomaxil- Atrophy-Hypoplasia lary, and forehead or occipitoparietal prob- lems. This is isolated craniofacial dysostosis or A multitude of anomalies occur in this plagiocephaly. Commonly, the deformities re- category. Romberg's Syndrome or coup-de- sult in forehead flattening on the involved sabre is an example of an important form of side, verticalization and shortening of the an- atrophy (Figure 18). Depending on the age of terior cranial base, and retrusion of the su- onset, there is skin, subcutaneous tissue, and praorbital and lateral orbital regions and of ' underlying muscle and bony atrophy with the zygoma on the involved side. In addition, inhibition of growth and deformity of the there may be a slight shift of the nose to that face. There may be displacement of the orbit, ear, nose, mouth, as well as of the forehead, zygomaticomaxillary region, and mandible. Its expression varies from subtle, if it occurs later in life when growth is nearly complete, to extremely severe if onset is early in life. Absence of the sphenoid wing or anterior cranial base unilaterally with exophthalmos in association with neurofibromatosis is an example of a mixed atrophy-hyperplasia syn- drome. There is hyperplasia associated with

FIGURE 8. Asymmetrical acentric cleft in right po- sitions 6 and 7. Zygomatic-maxillary hypoplasia with inferior displacement of the right lateral canthus, hypo- plasia of the right mandibular ramus, and microtia.

Whitaker et al., Crassirication or Cr AnomauEs 169

"aX FIGURE 9. Acentric cleft in positions 9 and 10. Lack FIGURE 10. Acentric cleft in positions 10 and 11 of complete orbit rotation with orbital hypertelorism and with absence of left ocular globe, excess interorbital coloboma of the right upper eyelid. Also, in this instance, distance, and absence of left nostril. fibro-fatty-glial tissue in upper eyelid.

Neoplasia-Hyperplasia the tumor and aplasia of the orbit. With the Lymphangioma occurring early in life re- brain against the ocular globe, there is result- sults in hyperplasia of all adjacent tissues, ant exorbitism (Figure 19). including skin, subcutaneous tissue, muscle, Other atrophy-hypoplasia syndromes are: and underlying bone. Hemangiomas create mandibular hypoplasia; isolated atrophy as a similar problems, but there is less uniformity result of trauma, particularly if it occurred of tissue hyperplasia. Fibrous dysplasia (Fig- early in life; and scleroderma, which results ure 21) generally develops later in life and is in atrophy and hypoplasia. Hypoplasia of the treated at a different time. Mandibular prog- maxilla occurs in association with clefts, mon- nathism develops prior to and during active golism, blepharonasal facial syndrome, and adolescent growth unless there is associated syndromes such as cutis laxa. There are other lymphangioma-hemangioma or other tumors. rare syndromes such as whistling face deform- Treatment is carried out at the end of this ity with hypoplasia and globe deformities. growth period in cases of isolated hyperplasia Hallermann-Streiff Syndrome is a mixture of of the mandible. Lymphangioma, heman- skin-subcutaneous tissue hypoplasia, orbit gioma, and fibrous dysplasia are treated in and globe hypoplasia, abnormalities of bone accordance with the severity of the problem, growth, and chin deformities. Aglossia-hypo- the age of the patient, and functional and dactyly is a problem ranging from severe un- psychological needs. Tongue, lip, ear, nose, derdevelopment of the mandible to total man- and other single-organ enlargements may oc- dibular absence (Figure 20). This has oc- cur for all the reasons described along, with curred in at least one case which included others. Multiple other tumors also occur absence of the tongue and fusion of the man- Treatment is planned according to the prin- dibular ramus to the palate. The deformity ciples already outlined. was confined to the lower face. Clefts of the soft tissue of the nose, lips, ears, and lip, and Unclassified pits, or grooves may all occur. The list is Other extremely rare problems occur, but extensive. they generally fit into the first four categories.

170 Cleft Palate Journal, July 1981, Vol. 18 No. 3

2 % FIGURE 11. Synostosis, symmetric. Severe, craniofacial dysostosis, classic Crouzon's syndrome with related coronal synostosis, cranial base abnormality. Severe exorbitism and midface hypoplasia.

When these problems cannot be classified, b. Nose they are best placed in this last category, c. Eye and orbit which can best be subdivided into the follow- d. Lips ing areas: e. Ears A. Multiple-Organ Involvement £. Jaws B. Single-Organ Involvement a. Tongue A. Multiple-Organ Involvement

FIGURE 12. Symmetric synostosis, craniofacial dysostosis or upper Crouzon's variation. Has associated coronal synostosis, anterior cranial base deformity, lower forehead retrusion, upper forehead protrusion, and slight midface hypoplasia.

Whitaker et al., Crasstrrcation or Cr 171

FIGURE 13. Symmetric synostosis. Craniofacial dysostosis variant, Apert's syndrome. Has associated coronal synostosis, anterior cranial base deformity, and severe midface hypoplasia with exorbitism and retromaxillism. Hand anomalies present.

These are numerous isolated occurrences the anterior cranium. The specific defects in- which cannot be systematically classified ex- volved are: absence of the nose, small nose, cept as described previously. absence of the nasal septum, absence of half of the nose, persistent frontonasal process, half B. Single-Organ Involvement nose, and proboscis. Treatment: Operative repair of the defect a. Tongue or reconstruction is indicated if either func- Macroglossia, or large tongue, may result tional or cosmetic problems are present. from a hemangioma or lymphangioma. In The nose can have a variety of character- muscular macroglossia, hypertrophy of indi- istics. It may be small, narrow, beaked, flat, vidual muscle fibers has been observed. Treat- or pear-shaped, and these may be syndrome- ment is surgical. Aglossia or hypoglossia is the specific. More specific abnormalities include: absence of or a small tongue. The tongue may transverse groove, where a horizontal depression be cleft, lobed, or fissured. These conditions or groove 1 to 3 mm wide and about 1 mm may not require treatment if tongue or oral deep is located caudal to the ala nasi; and function is not impaired. bifid nose, where a congenital median fissure is present. These may also be duplication of the b. Nose bony structure of the nose with two additional The external nose and nasal septum are nasal cavities that end blindly or connect to derived from a prolongation of the frontonasal the surface medial to the normal ones, slit-like process together with an infolding which pro- openings in the nasal columella, or nostril-like duces the nasal septum. Unequal growth of openings in the nasal columella. the two sides of the nasal septum or excessive Anterior atresia may occur when the epithe- infolding of the septal portion without fusion lial plugs, normally present from the second of the two parts can account for the majority to sixth month of intrauterine life fail to of the abnormalities of the nose and septum. absorb. Clinically, it may represent a spec- The unusual presence of dermoids of dural trum from narrow nostril(s) to bony or mem- origin can be accounted for by dural exten- branous stenosis of the nostril. The treatment sions which have become incorporated into of this condition is reconstructive surgery de- the frontonasal process as it grows out from signed to provide an epithelial lined nasal 172 Cleft Palate Journal, July 1981, Vol. 18 No. 3

FIGURE 14. Symmetric pseudosynostosis, Ricger's FIGURE 15. Symmetric synostosis of metopic suture syndrome. Midface hypoplasia without craniostenosis or with anterior triangulation defect of skull (trigonence- cranial base deformity. Has acentric pupils and car de- phaly), orbital hypotelorism, and decreased bitemporal formities as part of the syndrome. distance. Coincidental ear deformities.

"Im FIGURE 16. Asymmetric synostosis, plagiocephaly or isolated craniofacial dysostosis. Unilateral synostosis of coronal suture (left in this instance) and unilateral cranial base deformity. Manifestations include retrusion of left frontotemporal region, left orbit, and left zygoma. Left FIGURE 17. Asymmetric synostosis. Isolated crani- lateral canthus and ear frequently displaced inferiorly on ofacial dysostosis variant with unilateral coronal suture involved side. Often there is contralateral occipitoparietal and cranial base abnormalities. In this instance, protru- flattening. sion of the left forehead, inferior displacement of the left globe including both canthi, flattening with retrusion of cavity wall with cosmetically acceptable nos- left zygoma, and slight retrusion of right frontal area. trils. Posterior atresia (choanal atresia) occurs with failure of the nasal cavities as they form to c. Eyes and Orbits complete excavation. An obstructing mem- Epicanthus palpebris tarsalis inversus includes brane is found that traverses the posterior excessive skin at the root of the nose, poor nasal cavity. development of the bones of the bridge of the

Whitaker et al., Crassirication or Cr 173

palate, the lip pit syndrome, or the popliteal pterygium syndrome (multiple connective tis- sue bands 0.3-5.0 mm in width). The bands extend from the white line of one lid to that of the other lid, posteriorly to the cilia, and anteriorly to the meibomian orifice.

d. Lips Paramedial lip pits or wounds originate as ves- tigeal remnants of the lateral sulci appearing in the embryonic mandible at the stage when

FIGURE 18. Atrophy. Romberg's syndrome. Atro- phy of skin, subcutaneous tissue, muscle, and bone on involved side (right).

FIGURE 19. Hypoplasia. Absence of development of anterior cranial base and sphenoid wing on left. Pulsatile exophthalmos present because of missing brain-ocular globe bone barrier.

FIGURE 20. Hypoplasia. Aglossia-hypodactyly with complete absence of tongue and central two-thirds of mandible.

nose, and retention of the fetal epicanthal fold. Simple eptcanthus is a variation of normal which disappears by puberty in Caucasians and should not be operated on prematurely for cosmetic reasons alone. S g g Filiform fusion of the eyelids may be an isolated FIGURE 21. Neoplasia-hyperplasia. Fibrous dyspla- feature or may be associated with cleft lip and sia involving right orbit and zygoma-maxilla.

174 Cleft Palate Journal, July 1981, Vol. 18 No. 3 length is from 7.5 to 12.5 mm. Pits of the cleft malformations in the face, Acta Chirurg. Plast., 8, upper lip are the result of failure of complete 163, 1966. D. A., and Starx, R. B., A new classification fusion of the premaxilla and the maxillary for cleft lip and cleft palate, Plast. Reconstr. Surg., 22, processes. They may be associated with the 435, 1955. popliteal pterygium syndrome or with cleft KErnaran, D. A., On cleft lip and palate classification, lip or palate. Plast. Reconstr. Surg., 51, 578, 1973. Mircarp, D. R., Jr., The naming and classifying of cleft, e. Ear Cleft Craft, Volume I. Boston: Little, Brown and Com- pany, 41-55, 1976. The external ear (auricle) is developed from MorIAN, R., Uber die schrage Gesichtsspalte, Arch. K/iim. six swellings called auricular hillocks. These Chir., 35, 243, 1887. 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