Congenital Hemifacial Hyperplasia: An Embryologic Hypothesis and Case Report

RicHaArDp A. M.D. M. HaskeLt Newman, M.D. R. Buroi, PH.D. DonaLp P. Conpit, M.D.

Congenital hyperplasia is a developmental disorder characterized by marked unilateral asymmetry. The structural disproportions from one side to the other far exceed variations commonly seen in the normal population. Classically, as first described in 1836, the over- growth of tissue is restricted to one-half of the body, including the extremities. In other cases the hyperplastic enlargement involves segmental areas of the body, such as the hemifacial skeleton and an extremity. A case report is presented, and the clinical characteristics of 192 cases of congenital hyperplasia in the literature are briefly re- viewed. Use of the word hyperplasia to describe the condition (rather than hypertrophy) is encouraged. The authors propose a new em- bryologic hypothesis: asymmetrical development of the neural fold and hyperplasia of the neural crest cells are said to form the basis for the disorder. The rewards, risks, and limitations of reconstructive surgery over a 10-year period are discussed.

"If it is in her moments of abnormality that covering the facial skeleton are larger on Nature reveals her secrets, as Goethe once re- the left side than on the right. The ribs of marked, then the condition of hemihypertro- the thorax and the halves of the vertebral phy assumes an added piquancy of interest." bodies are larger on the right side than on A. Gesell (1927) the left. The bones of the right upper ex- tremity are longer and have greater di- Asymmetry is common and one of the ameter than those on the contralateral side more unusual and interesting errors of (Meckel, 1822; Updegraff, 1930; Haring, human reproduction. Consider the subtle 1961). Such imperfections of human anat- imperfections of human form that are omy are of little daily concern. They, in present in the average patient. The breasts, fact, often provide character and enhance pelvis, and genitalia are rarely symmetri- natural beauty (Caltagirone and Kostek1, cal. The skull and bone of the face, the 1955). _ cerebral hemispheres, and the soft tissues In patients affected by congenital hy- perplasia, asymmetry is more marked than The authors are affiliated with the University of in the normal population. The altered Michigan Medical Center. Drs. Pollock, Newman, and structural form caused by this condition is Condit are with the Section of Plastic Surgery, De- usually subtle at birth. With rapid growth, partment of Surgery. Dr. Burdi is with The Center for Human Growth and Development, Department the involved areas of the body may take of Anatomy & Cell Biology. on grotesque dimensions, leading to al- Address reprint requests to: M. H. Newman, M.D., tered form and function. The disorder may Section of Plastic Surgery, Box 22, C7028 Outpatient involve one-half of the body structure Building, University of Michigan, 1405 East Ann (Beck, 1836) or be limited to the hemlface Street, Ann Arbor, Michigan, 48109. This study was supported in part by National In- (Friedrich, 1863). stitutes of Health Research Grant DE 03610-17. This report has four objectives: (1) Re- 173 174 Cleft Journal, July 1985, Vol. 22 No. 3

FIGURE 1. Hyperplasia of the terminates at the midline and usually involves the anterior two-thirds of the tongue. constructive surgery in patients with con- The ipsilateral maxillary alveolar ridge was en- genital hyperplasia has seldom been re- larged. Excisional biopsy of the mass of the right corded. We present our surgical experience cheek confirmed the presence of fatty tissue over the past 10 years with one patient and without evidence of lymphangioma or vascular malformation. The limbs appeared symmetri- discuss the three basic problems facing the cal, and there was no evidence of abdominal reconstructive surgeon wishing to under- organomegaly. Congenital hemifacial hyper- take surgery in patients with congenital plasia was suspected. hemifacial hyperplasia. (2) Congenital At 8 years of age, the patient complained of asymmetry has frequently been described progressive diplopia that was enhanced when as congenital Aypertrophy. The authors em- gazing to the right. Orbital dystopia with hy- phasize the importance (based on histo- pertrophy of the bony orbit, elevation of the pathology) of using the term congenital right globe, and ectropion of the right lower hyperplasia. (3) Prior to this report, the eyelid associated with mild gaze strabismus were clinical characteristics of congenital hyper- noted. Clinical studies also revealed an over- growth of membranous bone and soft tissue of plasia have not been well tabulated. Infor- the right face and a downward slope of the oc- mation available from 192 cases in the lit- clusal plane to the right (Fig. 2). Enlargement erature are therefore briefly reviewed. (4) of the first rib was noted on chest radiographs Early theories regarding the embryology (Fig. 3). of congenital hyperplasia failed to ac- By late adolescence the patient complained of knowledge the role of the neural tube as severe malocclusion, and partial correction of the developmental template for body size the osseus contour defect and operative cor- and symmetry. The authors offer a new rection of the malocclusion (Figs. 4 and 5) were embryologic hypothesis to explain the accomplished by wedge Le Forte I maxillary clinical characteristics seen in this and other ostectomy, simultaneous bilateral vertical ra- case reports. mus osteotomy of the mandible, and osseus contouring of the zygoma, bony orbit, and right Case Report mandibular body (Figs. 6, 7, and 8). A white female was delivered vaginally without Despite significant improvement in the con- forceps at term weighing 9 lbs following an tour of the facial bone and establishment of a uneventful pregnancy. Physical examination was functional horizontal occlusal relationship, the unremarkable except for the presence of a right persistant soft tissue enlargement and facial cheek mass which clinically suggested a lym- sagging limited the desired cosmetic improve- phangioma. An incisional biopsy of the mass in ment. At age 13, a lower cheiloplasty, par- the neonatal period revealed adipose tissue. tial , and rhytidectomy were per- When the patient was 5 years of age, enlarge- formed and resulted in a slight improvement. ment of the right tongue to the midline was A suspected right facial paralysis was not con- noted (Fig. 1). The tongue deviated to the left. firmed electromyographically, and the distor-

Pollock, Newman, Burdi, and Condit, CONGENITAL HEMIFACIAL HYPERPLASIA 175

tion of facial sagging was presumed to reflect tissue mass. A temporalis fascial sling suspen- sion partially corrected the problem (Fig. 9). Since age 16, the patient has shown minimal progression of the hyperplastic process (Fig. 10). Her occlusion has remained stable (Fig. 11).

NOMENCLATURE

Until 1947 the histology of congenital hyperplasia was presumed to be an en- largement of individual cells. Sutton biop- sied the facial musculature of a 6-month- old child with congenital hemifacial hy- perplasia. The individual muscle fibers were not enlarged when inspected under light microscopy. A more sophisticated histologic survey of the organs of a 13-year- old male with generalized involvement failed to reveal cellular enlargement in any of the organs examined. Facial skin, fat, blood vessels, muscle, nerve, and bone from the patient in this report were also studied by light micros- copy. The cells were normal in size, shape, and distribution. The histopathology is an increase in the number of cells (hyperpla- sia) rather than an increase in cell size (hy- FIGURE 2. The patient is pictured in mid-ado- pertrophy). lescence with marked facial asymmetry caused by an Like Geiser et al (1970), the authors overgrowth of the mandible, maxilla, and zygoma, as conclude that use of the term congenital well as hyperplasia of the parotid gland and masseter muscle. Note the enlargement of the right upper lip hemifacial hyperplasia is more histologi- and aberrent occlusal plane. cally precise and clinically appropriate

FIGURE 3. A chest ra- diograph revealed a hyper- plastic right rib without other abnormality.

176 Cleft Palate Journal, July 1985, Vol. 22 No. 3

FIGURE 4. This view demonstrates the downward slope of the occlusal plane. Note the diastemata of the maxillary and mandibular teeth.

FIGURE 5. Panoramic radiographs document ab- normalities of the occlusal plane, enlargement of the right mandible and maxilla, and the presence of macro- dontia.

FIGURE 6. This illustration summarizes the sur- - gical procedures the patient underwent at age 11. Orthodontic procedures are not depicted. A, a pre- operative view is shown. B and C depict the osteo- tomies and bone excision accomplished using power driven burrs. D demonstrates preservation of the in- ferior alveolar nerve during osteotomy and contour- ing of the right mandible.

Pollock, Newman, Burdi, and Condit, CONGENITAL HEMIFACIAL HYPERPLASIA 177

cross-sectional enlargement of the extrem- ities (Peabody, 1936; Bryan et al, 1958; Macewen, 1967). Anomalies of the rib cage, vertebrae, and pelvis are common. Multi- ple anomalies are found in scattered case descriptions (Table 2). Patients exhibit clinically or at autopsy: enlargement of the spleen, liver, kidney, or adrenal glands (Ringrose, 1965; Gorlin, 1962; Geormaneanu et al, 1983). The fre- quency of benign and malignant tumors of the viscera is increased particularly in those organs commonly affected by hy- perplasia (Geormaneanu et al, 1983; Geiser et al, 1970; Schwartzman et al, 1942; Hutchinson, 1915; Harwood and O'Flynne, 1935; Roth, 1938; MacNab, 1952; Riedel, 1952; Benson et al, 1963; Fraumeni and Miller, 1967). In two studies the brain was enlarged on the side of the skeletal in- volvement (Ward and Lerner, 1947; Geiser et al, 1970). Microscopic study of central nervous system tissue has not otherwise been described. FIGURE 7. This radiograph (frontal view) was taken following osteotomies of the maxilla, mandible, and zygoma depicted in Figure 5. SEGMENTAL (INCLUDING HEMIFACIAL) HYyPERPLASIA than other terms utilized to describe the In its less classic but more frequent and condition. intriguing form, congenital hyperplasia involves segmental areas of the body (Ta- HEmIBODY HYPERPLASIA ble 1). In particular, the hemiface and its In its classic form (Beck, 1836), the soft tissue are affected (Friedrich, 1863; overgrowth of tissue involves one-half of Sutton, 1949). Exaggerated growth of the the body, including the hemiface, trunk, mandible, maxilla, or zygoma has been re- and upper and lower extremities (Table ported frequently. Enlargement of the 1). Ipsilateral musculoskeletal changes are frontal, parietal, and temporal bones, and not infrequent and include lengthening and the skull base has also been described (Ward and Lerner, 1947; Stafne and Lov- estedt, 1962; Prasad, 1971; Hanley et al, 1968). The bone overgrowth is usually three dimensional. Enlargement of the foramina of the fa- cial skeleton accompanies the hyperplasia of facial bone (Ringrose et al, 1965; Bjork- lund, 1955). Stafne and Lovestedt (1962) emphasized that foraminal enlargement serves to differentiate congenital hemifa- cial hyperplasia from other conditions which cause overgrowth of the jaws (Table 3). The vessels and nerves traversing the FIGURE 8. This panoramic radiograph reveals the jaws and dentition following surgery and prior foramina and canals of involved bone are to orthodontic treatment. markedly enlarged.

178 Cleft Palate Journal, July 1985, Vol. 22 No. 3

FIGURE 9. The patient is seen at age 13, fol- FIGURE 10. The patient has persistent asym- lowing surgery of the facial skeleton and soft tissue, metry at age 17, predominantly due to soft tissue en- which included rhytidectomy and temporalis sling. largement, including parotid tissue and masseter muscle.

Dental abnormalities, particularly of the more noticeable when permanent denti- deciduous teeth, are often subtle and tion is established. The permanent teeth readily overlooked (Stafne, 1952). Deady may be enlarged anywhere along the den- et al (1969) have emphasized that the en- tal arch, but the canines are frequently the larged, more medially located deciduous largest. The bicuspids and first molars tend teeth mature earlier and erupt prema- to be the next most frequently enlarged turely. The roots of the erupted teeth are (Trapp et al, 1981; Deady et al, 1969; enlarged and distorted. The eruption of Rowe, 1962; Stafne, 1952; and Hanley, involved deciduous teeth precedes by sev- 1968). eral months the shedding of their normal The gingiva and buccal mucosa may be- counterparts. come thickened, even nodular. If the Greatest alveolar bone growth tends to tongue is involved, the anterior two-thirds occur distally, away from the midline has the greatest overgrowth. Enlargement (Bjorklund, 1955). Distal teeth may be re- precisely terminates at the midline (Fig. 1). tained within the hyperplastic bone, (Sut- The unilateral increase in size causes the ton, 1949; Prasad, 1971; Deady et al, 1969; tongue to deviate toward the normal side. Rowe, 1962), and the space created by such Enlarged papillae, particularly the circum- unerupted teeth may contribute to mal- vallate, project beyond the surface. En- occlusion. With progressive hyperplastic largement of the ipsilateral tonsil has oc- growth, the alveolar processes of the jaws casionally been reported (Sutton, 1949; become thickened, which leads in some in- Trapp et al, 1981). The palate may be wide stances to exostoses that may prevent nor- and more arched on the involved side, as mal occlusive contact. Sutton (1949) and Bergman (1973) re- Distortions of crown shape and size are ported. Pollock, Newman, Burdi, and Condit, CONGENITAL HEMIFACIAL HYPERPLASIA 179

FIGURE 11. This pho- tograph depicts the current state of dentition. Note the persistence of a slight lower- jaw diastema and asymme- tries of the teeth. The occlu- sal plane is much improved, and function is excellent.

The , particularly the lower lip, may scribed (Gorlin and Meskin, 1962; Henry be twice as large on the affected side (Sut- et al, 1972; Geormaneanu et al, 1983). ton, 1949; Rowe, 1962; Hanley et al, 1968; There have been isolated reports of thick- Prasad, 1971). Enlargement of the upper ened hair and facial skin, with enlarge- lip displaces the philtrum toward the un- ment of sebaceous glands, or the presence involved hemiface (Updegraff, 1930). The of nevi or telangiectasia (Adams, 1894; mass of the ipsilateral parotid and the Muller et al, 1980). Bryan (1958) and Rin- masseter muscle is increased. grose et al (1965) have described patients The ipsilateral pinna and pupil may be whose ipsilateral scalp hair had a different larger (Reister, 1964), but an increase in texture and variant color. the size of the inner ear or globe of the In several reports of segmental hyper- eye has not been reported (Trapp et al, plasia, the trunk is spared, and the ex- 1981; Deady et al, 1969; and Werner, tremities or hemiface and limb are the sole 1905). Iris heterochromia has been de- areas of involvement (Table 1). Very rarely,

TABLE 1. Data from 192 Cases of Congenital TABLE 2. Associated Conditions in Generalized Hyperplasia in the World Literature Hemihyperplasia Location of Hyperplasia N -% Chest /Thorax Urogenital Entire Side (N = 84) 43.7 Pectus carinum Hydrocele Hemiface, trunk, extremities Enlarged ribs with Cryptorchidism Right 46 wide intercostal Hypospadias, Left 38 spaces phimosis Segmental (N = 101) 52.6 Ipsilateral breast Enlarged penis, scro- Hemiface 34 hypertrophy tum, or labia Hemiface and trunk J Supernumerary Extremities Hemiface, ipsilateral extremities 22 nipple Polydactyly, syndac- Hemiface and lower extremity 6 Congenital heart tyly, camptodactyly Trunk and extremities 10 disease Lobster claw defor- Trunk and lower extremity 3 Scoliosis mity Upper and lower extremities 20 Abdomen Genu varum A single extremity 3 Umbilical hernia Club foot Crossed (N = 7) 3.7 Neoplasms/hyperpla- Hemiface and opposite lower sia of the organ extremity 5 systems Trunk and opposite lower Kidney, adrenals, or extremity 2 liver

180 Cleft Palate Journal, July 1985, Vol. 22 No. 3

TABLE 3. The Differential Diagnosis of been clinically reported, and karyotypes of Congenital Hyperplasia is Aided by the Presence children with congenital hyperplasia are of Enlarged Foramina in the Facial Skeleton routinely normal (Wiedemann et al, 1964;

Overgrowth of Bone and Soft Tissue with Gerloczy and Shuler, 1968; and Nyhan and Foraminal Enlargement Sakati, 1976). Aberrancy or enlargement Congenital hyperplasia on both sides of the body, which would Overgrowth of Bone or Soft Tissue Without support the concept of chromosomal mo- Foraminal Enlargement saicism, is possibly documented in only Fibrous dysplasia seven cases in the world literature (Table Paget's disease 1). More critically, prior theorists failed to Neurofibromatosis consider the high probability that many of Tuberous sclerosis the structures enlarged in congenital hy- Arterial venous malformations or perplasia owe their morphogenic initia- hemangiomas, (including Klippel- Trenaunay syndrome) tion to (1) the nervous sysem and (2) neural Malignancy crest cell differentiation and migration. Consider recent embryologic research that has focused on neural tube develop- case reports have described crossed in- ment and on the formation (migration, in- volvement (Table 1); in each such instance teraction, and cytodifferentiation) and fate the patient has had a Wilm's tumor (Frau- of neural crest cells (Weston, 1970; John- meni et al, 1967; Miller et al, 1964). ston, 1966; Johnston and Listgarten, 1972; Noden, 1978, 1980; and LeDouarin, 1980). ETIOLOGY: AN EMBRYOLOGIC At 18 days of gestation, the neural groove HYPOTHESIS deepens and bilateral neural folds are In 1921, Gesell suggested that some formed. Neural crest cells appear shortly forms of congenital hyperplasia are based after the neural folds unite to form the on a chromosomal abnormality. Fankheu- neural tube (21 to 28 days of gestation). ser and Humphrey (1959) linked the dis- The crest cells give rise to precursor cells order in the experimental axolotl to dip- and thus, a variety of cells including mus- loid, triploid, and tetraploid chromosomal cle, bone, and connective tissue. Within the aberrations. Several reports in the litera- head and neck area, neural crest cell dif- ture suggest the possibility of autosomal ferentiation is best illustrated by tooth and dominant inheritance (Fraumeni et al, craniofacial skeletal formation. 1967; Scott, 1935; Herman, 1969; Burch- Based on this current knowledge, the field and Escobar, 1980). authors offer an hypothesis concerning the Gesell proposed a second hypothesis in early pathogenesis of congenital hyperpla- 1927. He suggested that congenital hyper- sia. The hypothesis holds that one neural plasia is related to imperfect twinning, the fold is larger than the other (Fig. 12). The result of an unequal first division of the enlarged half of the neural tube gives rise zygote into two daughter cells. Earlier in to an enlarged ipsilateral brain and spinal the 1900s Spemann performed a classic set cord. The neural crest cells (which remain of experiments which supported the Ge- after the neural tube closes and separates sell hypothesis (Spemann, 1921). Triton from the skin of the embryo) are propor- eggs were constricted around their equa- tionately more numerous on the involved tor prior to and during early stages of side. The increased number of crest cells blastocyst formation. One member of the persists throughout much of the prenatal conjoined twins produced in this manner and formative postnatal growth periods of was demonstrably larger than the other. life and leads to unilateral overgrowth of Early embryologic proposals inade- crest-cell-derived bone, muscle, and soft quately explain the clinical uniqueness of tissues (Fig. 10). Neural tube morphogen- congenital hyperplasia, particularly the esis takes place in a cephalocaudal gra- presence of unilateral enlargement of the dient. It is reasonable to expect that some face and limbs. The combination of twin- tube segments may be involved more often ning and hyperplasia (Gesell, 1927) has not than others. Therefore, the tissues of the

Pollock, Newman, Burdi, and Condit, CONGENITAL HEMIFACIAL HYPERPLASIA 181

Neural plate

Ectoderm Neural folds A

Surface ectoderm ...... ------...... Neural crest ...... ----- ..... Doce Neural groove nnnnnn ------......

Neural tube

RIGHT SIDE ...... NORMAL ------HYPERPLASIA ------ooooooooooooooo ------Neural crest ...... ooooooooo ...... veneered. my are bd nes or ......

Neural

Neural

crest Q cells Ectomesenchymal cells

[ A

Schwann cells ‘ Melanoblast

Ectomesenchymal cells

"A \ Fibroblasts ‘ | I Chondroblasts Odontoblasts Neuroblast

/ Osteoblasts

Sensory Sympathetic Motor - Chromaffin Cementoblasts neurons neurons neurons cells

FIGURE 12. This illustration depicts the early pathogenesis of congenital hyperplasia as proposed by the authors. A and B: The neural tube and its precursor are unilaterally hyperplastic, leading to enlarged ipsilateral central nerve structures. B, C, and D: The neural crest cells are proportionately more numerous

on the involved side, creating an overgrowth of crest-cell derived bone, cartilage, muscle, nerve, and soft tissue. The hyperplasia may be segmental, such that certain areas of the body may be involved while others

are spared (Table 1). The insert provides a longitudinal view.

cephalic region would be expected to be and ipsilateral neural crest cells. Such affected clinically more often than the tis- speculation seems reasonable since the

sues of other more caudal areas. clinical manifestation of the disorder is

clearly and most often a one-sided phe-

nomenon. _ DISCUSSION There are three basic problems facing

The hypothesis proposed in this report the reconstructive surgeon: progressive

to explain the etiology of congenital hy- tissue growth, patient need, and airway perplasia honors the basic premise that the management. The structural overgrowth neural tube is the developmental template typically develops at a rate proportional to for the early establishment of size and the growth of the unaffected side, which proportionality of the body. Although creates the major problem in the rehabil- speculative, the theory implicates asym- itation of the patient with congenital hy- metrical development of the neural fold perplasia. Dental, soft tissue, maxillofacial,

182 Cleft Palate Journal, July 1985, Vol. 22 No. 3 and craniofacial procedures performed in TABLE 4. Major Surgical Procedures Recorded early adolescence may be obscured by con- in the Literature tinual change. Secondary or tertiary pro- Bailey cedures may be required to maintain the Superficial parotidectomy status quo and sustain a relatively normal Rhytidectomy appearance. The stress of adolescent peer Osteotomy of the zygoma relationships may precipitate requests for Otoplasty surgical intervention more often than de- Deady, Silagi and Hutton sired by the surgeon. Vertical sliding osteotomy Induction of anesthesia may be made Kruger difficult by the hyperplasia of bone and soft Unilateral vertical mandibular osteotomy Coronoidotomy tissue. The increased height of the ipsilat- Mohnac eral mandible, altered range of motion of Anterior and posterior maxillary osteotomies the temporomandibular joint, the pres- Contouring of the mandible ence of exostoses in the floor of the mouth, . Reid, (Hinds, Mohnac and the hypertrophy of tonsilar tissue on Contouring of the inferior border the involved side may obstruct visibility of the mandible during endotracheal intubation (Trapp, Unilateral vertical mandibular osteotomy 1981). Bilateral posterior maxillary osteotomies Six percent of patients reported in the Bilateral vertical mandibular osteotomies world literature (11 of 192 cases) have Schudardt undergone major surgical procedures Mandibular osteotomies Stafne and Lovestedt (Table 4). The uncommon use of surgery Reduction (R) maxillary tuberosity appears to reflect the difficulty with air- Sutton way management and to a greater degree Excision of excess fatty tissue the basic problem, i.e., progression of the Ligation of the ext. maxillary artery hyperplasia throughout late adolescence Reduction cheiloplasty and early adulthood. Nevertheless, the Elevation (L) angle of mouth limited success with our patient from a Trapp, Lee, Troutman, Simon surgical and orthodontic point of view has (R) Mandibular condylectomy been encouraging. Without surgical inter- Gingivectomy and vention, the involved facial structure would Updegraff have taken on grotesque dimensions, with Excision inferior border, body (R) mandible, with excision and inferior advancement of facial altered form and function. Although the skin patient still carries stigmata of the disor- der following surgery, she has maintained a viable position within her family and so- 1985). Infraorbital incisions offer addi- cial unit. ' tional exposure of the orbital skeleton. Craniofacial and maxillofacial surgical Sharp periosteal elevation is accom- techniques developed in the past two de- plished to provide wide exposure of the cades (Tessier, 1981; Munro, 1981) favor involved bone. Care is taken to protect the intervention in patients with congenital enlarged terminal branches of the cranial hemifacial hyperplasia. Preoperative eval- nerves, (1.e., the mental, inferior alveolar, uation by routine and cephalometric ra- infraorbital, and supraorbital nerves) dur- diographs and by computerized tomog- ing surgery. raphy (CT scan) allow surgical planning. The hyperplastic bone is contoured by The mandible is approached through an a combination of techniques, to include intraoral incision, the maxilla and zygoma osteotomy and the use of cutting burrs. In by way of the gingivobuccal sulcus, and the the maxilla, the excision traverses the mid- orbit, zygoma, and brow through a trans- face in wedge-like fashion and may extend coronal incision with degloving of the up- across the midline (Fig. 6). The zygoma is per facial skeleton (Shaw and Parsons, mobilized by standard or modified osteo- 1975; Gruss, 1982; Pollock and Dingman, tomies to permit reduction of the malar Pollock, Newman, Burdi, and Condit, CONGENITAL HEMIFACIAL HYPERPLASIA 183

eminence and modification of the infero- Acta Paediatrica 1955; 44:286. lateral margins of the orbit. The mandi- BryAN RS, Lirscoms PR, CHATTERTON CC. Ortho- bular deformity is often marked, and re- paedic aspects of congenital hypertrophy. Am J Surg construction may require multiple ap- 1958; 96:654. ' BURCKHFIELD D, ESCOBAR V. Familial facial asymmetry proaches to include osteotomies of the (autosomal dominant hemihypertrophy?) Oral Surg ramus, the inferior border of the body, and 1980; 50:321. » the mental protuberance. Condylectomy CALTAGIRONE JV, KOSTECKI JL. The face in Greek for serious derangement is seldom nec- and Roman art. Plast Reconstr Surg 1955; 15:165. essary but has been reported by Trapp DrEapy MJ, SILAGI SL, HUTTON CE. Hemihypertro- phies of the face and mandible. Oral Surg 1969; (1981). 27:577. Segments of excised bone may be con- FANKHEUSER G, HUMPHREY RR. The origin of spon- toured and used as grafts to fill any osseus taneous heteroploids in the progeny of diploid, tri- defects that evolve in the reconstruction. ploid, and tetraploid axolotl females. J Exper Zool 1959; 142:379. ’ Interosseus wires and standard- or mini- FRAUMENI JF, GEISER CF, Manning MD. Wilm's tu- reconstruction plates (and screws) provide mor and congenital hemihypertrophy: report of rigid fixation of the remodeled bone. The five new cases and review of the literature. Pedi- periosteum is reapplied to the facial skel- atrics 1967; 40:886. eton, and the surgical incisions are closed FRAUMENI JF, MILLER RW. Adrenocortical neoplasms with hemihypertrophy, brain tumors, and other in multiple layers. disorders. J Pediatrics 1967; 70:129. Soft tissue redundancy is managed by FRIEDRICH N. "uber congenitale" Halbseitige simple excision, such as rhytidectomy or Kopfhypertrophie. Virchow's Arch Path Anat cheiloplasty. The clinical result of the con- 28:474, 1863. GEISER CF, BAEZ A, SCHINDLER AM, SHIH VE. Epi- touring may be compromised by the en- thelial hepatoblastomas associated with congenital largement of the masseter and hyperplasia hemihypertrophy and cystathioninuria: presenta- of the parotid gland. tion of a case. Pediatrics 1970; 46:66. Refinement of the occlusion is often re- GEORMANEANU M, IacaRU N, PorEsCcuU-MICLOSANU S, quired in the postoperative period. This is BADULESCU M. Congenital hemihypertrophy: ten- dency to association with other abnormalities and/ best accomplished by preoperative ortho- or tumors. Morphol Embryol 1983; 29:39. dontic consultations and continued eval- GERLOCZY F, SHULER D. Hemihypertrophy: inci- uation in the postoperative period. dence and chromosomal examinations. ACTA Paediatr Acad Sci Hung 1968; 9:323. GESELL A. Hemihypertrophy and twinning: Further Acknowledgement. The authors wish to acknowl- study of the nature of hemihypertrophy with re- edge the assistance of Marilyn Simpson, A.C.L.S., port of a new case. Arch Neurol Psychiatry 1921; University of Michigan Taubman Medical Library, 6:400. for historical research and the help of Ms. Linke Gib- GESELL A. Hemihypertrophy and twinning: further son for typing the manuscript. The drawings are those study of nature of hemihypertrophy with report of of Denis Lee, Director, Medical Illustration, Univer- new case. Am J Med Sci 1927; 173:542. sity of Michigan Medical Center, Ann Arbor. GORLIN RJ, MrEskIn LH. Congenital hemihypertro- phy. J Pediatr 1962; 61:870. REFERENCES GRUSS JS. Fronto-naso-orbital trauma. Clin Plast Surg 1982; 9:577. ADAMS SS. A case of hemihypertrophy (giant growth). Haney FJ, F.oyD E, PARKER D. Congenital partial Arch Pediat 1894; 2:901. hemihypertrophy of the face: report of three cases. BAILEY BJ. Congenital hemihypertrophy: surgical J Oral Surg 1968; 26:136. management of facial stygmata. In: Bernstein L, HARING OM, LEwIs JF. The etiology of congenital ed. Plastic and reconstructive surgery of the head developmental anomalies. Surg Gyn Obstet 1961; and neck, Vol 2. San Francisco: Grune & Stratton, 113:1-18. 1981:9. HARWOOD J, O'FLYNNE E. Right sided hemihypertro- BECK RT. Med Annalen von Puchelt, Chelius und phy associated with pubertas praecox. Proc Roy Soc Naegele (1836). Quoted by Ziehl in Virchow's Arch - Med 1935; 28:857. Path Anat 91:1883. HENRY M, Louis JP, HoErFEL JC, PERNOT C. He- Benson PF, VULLiamy DG, TaAUBMAN JO. Congenital mihypertrophie congénitale associée a une anom- hemihypertrophy and malignancy. Lancet 1963; alie des arcs aortiques et a diverses malformations 1:468. squelettiques, cutanées, oculaires. Ann Med de BERGMEN JA. Primary hemifacial hypertrophy. Arch NANCY 1972; 11:1111. Otolaryngol 1973; 97:490. HERMAN J, OPITZ JM. A dominantly inherited first BJORKLUND SI. Hemihypertrophy and Wilm's tumor. arch syndrome. Birth Defects 1969; 5:110. 184 Cleft Palate Journal, July 1985, Vol. 22 No.

HUTCHINSON R. Hemihypertrophy. Proc Roy Soc Med face. J Laryngol Otol 1971; 85:607. 1915-1916:9. REID R, Hinps EC, MoHNAc AM. Surgical correction JounstoN MC. A radioautographic study of the mi- of facial asymmetry associated with open bite. J Oral gration and fate of the cranial neural crest cells in Surg 1966; 24:527. the chick embryo. Ant Rec 1966; 156:143. REISTER HC, SCHERZ RC. Silver's syndrome. Am J Dis Jounsron MC, LIsTGARTEN MA. Observations on the Child 1964; 107:410. migration, interaction, and early differentiation of RIEDEL HA. Adrenogenital syndrome in a male child orofacial tissues. In: Slavkin HC, Bavetta LA, eds. due to adrenocortical tumor. Pediatrics 1952; 10:19. Developmental aspects of oral biology. New York: RINGROSE RE, JABBOUR JT, KEELE DK. Hemihyper- Academic Press, 1972:53. trophy. Pediatrics 1965; 36:434. KrucErR GO. Textbook of Oral Surgery. St. Louis: ROTH F. Zur Kenntnis und auffassung der leber mis- CV Mosby Co, 1959:430. chgeschwulste und des halbsetigen rie sen wuchses. LEDOUARIN N. Migration and differentiation of neural Frankfurt Ztschr Path 1938; 52:163. , crest cells. Current Topics in Devel Biol 1980; 16:32. RowE NH. Hemifacial hypertrophy: review of the lit- MAcNAB GH, MoNCRIEFF AA, BopIAN M. British erature and addition of four cases. Oral Surg 1962; Empire Cancer Campaign. Animal Report 1952; 15:572. 30:162. SCcHUDARDT K. Experiences with the surgical treat- MAcEwWEN GD. Congenital hemihypertrophy. Clin ment of some deformities of the jaws. In: Wallace Orthopaedics 1967; 50:147. AB, ed. Transaction Second Congress Interna- MECKEL JF. Ueber die seitliche Asymmetrie im tier- tional Society of Plastic Surgeons, London, 1959. ischen Korper. Anatomische physiologische Beo- London: ES Livingstone, 1961:73. bachtungen und untersuchungen. Halle: Renger, SCHWARTZMAN J, GROSSMAN L, DRAGUTSKY D. True 1822:147. (Cited in: Halperin G. Normal asym- total hemihypertrophy. Arch Pediatrics 1942; metry and unilateral hypertrophy. Arch Intern Med 59:637. 1931; 48:676.) ScoTt AJ. Hemihypertrophy. Report of four cases. MILLER RW, FRAUMENI JF, MANNING MD. Associa- J Pediatrics 1935; 6:650. tion of Wilm's tumor with aniridia, hemihypertro- SHAw RC, PARSONS RW. Exposure through a coronal phy and other congenital malformatins. N Engl J incision for initial treatment of facial fractures. Plast Med 1964; 18:922. Reconstr Surg 1975; 56:254. Monnac AM. Maxillary osteotomy in the manage- SPEMANN H. Die Erzeugung tierischer Chimaren durch ment of occlusal deformities. J Oral Surg 1966; heteroplastiche embryonale Transplantation 24:305. zwischen Triton cristatus und taeniatus. "Roux" MurlER JT, PickETT AB, FREDERICK FD. Facial he- Arch f Ent-mech 1921; 48:533. mihypertrophy associated with nevus unius lateris STAFNE EC, LoveEstTEDT SA. Congenital hemihyper- syndrome. Oral Surg 1980; 50:226. trophy of the face (facial giantism). Oral Surg 1962; MunNrRO IR. Craniofacial surgical techniques for aes- 15:184. thetic results in congenital and acute traumatic de- STAFNE EC. Oral roentgenographic diagnosis; uni- formities. Clin Plast Surg 1981; 8:303. lateral hyperplasia of the face (facial giantism). Ra- NODEN DM. Interactions directing the migration and diology 1952; 58:254. cytodifferentiation of avian neural crest cells. In: SUTTON LE. Hemihypertrophy of the face. Plastic Garrod D, ed. The specificity of embryological in- Reconstr Surg 1949; 4:276. teractions. London: Chapman and Hall Ltd., TEssIER P. Aesthetic aspects of bone grafting to the 1978:4. ‘ face. Clin Plast Surg 1981; 8:279-301. . NoDEN DM. The migration and cytodifferentiation TRAPP LD, LEE CM, TROUTMAN KC, SIMON JE. An- of cranial neural crest cells. In: Pratt RM, Chris- esthetic management of the pediatric patient with tiansen RL, eds. Current research trends in pre- multiple congenital anomalies including severe natal craniofacial development. New York: Elsev- hemifacial hypertrophy. Anesth Prog 1981; 28:162. ier North Holland, 1980:3. UPDEGRAFF HC. Reconstructive surgery in unilateral NYHAN WL, SAKATL NO. Genetic and Malformation progressive facial hemihypertrophy. Am J Surg Syndromes in Clinical Medicine. Chicago: Year 1930; 10:439. Book Medical Publishers, 1976:293. WARD J, LERNER HH. A review of the subject of con- PEABODY CW. Hemihypertrophy and hematrophy. J genital hemihypertrophy and a complete case re- Bone Joint Surg 1936; 18:466. port. J Pediatrics 1947; 31:403. PoLLOCK RA, DINGMAN RO. Management and re- WERNER R. Congenital habseitige gesichtshyperttro- construction of athletic injuries of the face, ante- phie. Arch Klin Chir 1905; 74:583. rior neck and upper respiratory tract. In: Schnei- WEsTON JA. The migration and differentiation of der RC, Kennedy JC, Plant ML, eds. Sports injuries: neural crest cells. Adv Morphogenesis 1970; 8:41. mechanisms, prevention & treatment. Baltimore: WIEDEMANN HR, ToLkspoRr M, HAaANsEN HG. Con- Williams & Wilkins, 1985. genital asymmetry with diploid-triploid mosaicism. Prasad U. Congenital true hemihypertrophy of the Lancet 1964; 1:1166.