CME

Hemifacial Microsomia: Clinical Features and

Pictographic Representations of the OMENS
Classification System
Alexander J. Gougoutas, M.D.
Davinder J. Singh, M.D.
David W. Low, M.D.
Scott P. Bartlett, M.D.
Philadelphia, Pa.; and Phoenix, Ariz.
Learning Objectives: After reviewing this article, the participant should be able to:
1. Describe the fundamental malformations defining hemifacial microsomia. 2.
Distinguish hemifacial microsomia from other congenital craniofacial anomalies
sharing similar features. 3. Understand the variety of systems developed to clinically
classify the features of this disorder. 4. Describe the format of the OMENS clinical
classification system and appreciate its possible advantages and limitations.
Background: The clinical manifestations of hemifacial microsomia comprise a
spectrum that is both broad and complex. The fundamental features include
unilateral hypoplasia of the craniofacial skeleton and its overlying soft tissue. Nu-
merous schemes have been developed to classify this spectrum. One of the most
recent classification systems, the OMENS system, scores five clinical manifestations
of hemifacial microsomia according to dysmorphic severity on a scale from 0 to 3:
orbital asymmetry, mandibular hypoplasia, ear deformity, nerve dysfunction, and
soft-tissue deficiency.
Methods: The authors describe the diverse features of hemifacial microsomia and
the numerous attempts at its clinical classification, with particular emphasis on the
OMENS system.
Results: With the possible exception of the OMENS scheme, the various systems
developed to classify the clinical features of hemifacial microsomia fail to possess
the flexibility and versatility needed to categorize all potential phenotypes of this
complex disorder.
Conclusions: The OMENS system represents the most comprehensive, versatile,
objective, and easily adaptable attempt at clinical classification of hemifacial mi-
crosomia to date. The authors propose a concise clinical evaluation form using a
modified version of the system to promote the use of the OMENS system, to aid in
the evaluation of hemifacial microsomia patients, and to assist in data sharing
among academic institutions. (Plast. Reconstr. Surg. 120: 112e, 2007.)

H
emifacial microsomia, a term popularized
by Gorlin and Pindborg,1 refers to a broad
spectrum of congenital malformations result-
ing from the variable dysmorphogenesis of cranio-
facial structures either derived from or intimately
related to the first and second brachial arches. Since
its earliest descriptions by Canton2 and Von Arlt3 in
1861 and 1881, respectively, a variety of names have
From the Division of Plastic Surgery, Department of Surgery,
University of Pennsylvania; Children’s Hospital of Phila-
delphia; Edwin and Fannie Gray Hall Center for Human
Appearance; and Barrow Craniofacial Center, Barrow Neu-
rological Institute.
Received for publication April 18, 2006; accepted August
31, 2006.
Copyright ©2007 by the American Society of Plastic Surgeons
DOI: 10.1097/01.prs.0000287383.35963.5e
been adopted for this anomaly. These include
craniofacial microsomia,4 first and second brachial
arch syndrome,5,6 otomandibular dysostosis,7,8 au-
riculobranchiogenic dysplasia,9 intrauterine facial
necrosis,10 lateral facial dysplasia,11 hemignathia and
microtia syndrome,6 necrotic facial dysplasia,12 oto-
mandibular-facial dysmorphogenesis,13 mandibular
laterognathism,14 oculoauriculovertebral spectrum,15
and facioauriculovertebral malformation complex.16
This extensive list attests to the difficulty of satisfacto-
rily labeling the breadth of malformations defin-
ing this syndrome. Indeed, as Longacre et al. note:
“The prominent feature of these dysplasias is their
variability.”17 For purposes of this article, we have
continued to use the most common descriptor of
this deformity, hemifacial microsomia.
In the simplest of terms, hemifacial microso-
mia manifests primarily as unilateral hypoplasia of

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Volume 120, Number 7 • Hemifacial Microsomia
the craniofacial skeleton and its overlying soft tis-
sue. Although bilateral hypoplasia has been noted
in 5 to 30 percent of cases,11,18 when present, it is
generally asymmetric.4,19 Although many studies
have advocated a right-sided20 –22 and male20,23,24
predominance, others have found equivalent
right/left and gender distributions.25,26 With an
incidence estimated at one in 3000,3,5,22,27 one in
5642,28 and one in 26,000,3 hemifacial microsomia
represents one of the most common congenital
malformations of the head and neck, second only
to cleft lip– cleft palate.29,30 Although the majority
of cases represent sporadic occurrences, reports of
successive generations demonstrating similar phe-
notypes have prompted consideration of various
modes of genetic inheritance.
PHYSICAL CHARACTERISTICS
The phenotypic heterogeneity of hemifacial mi-
crosomia precludes the development of agreed on,
minimally diagnostic criteria.23,31 There is, however,
consistency in the craniofacial regions affected,
namely, the external/middle ear, mandible, and
contiguous bones of the facial skeleton along with
their overlying musculature, cranial nerves, and con-
nective tissue. Although each of these regions may
display varying degrees of hypoplasia, when all are
severely affected, a very characteristic facial appear-
ance is assumed.
The mandible has long been considered the
“cornerstone” of hemifacial microsomia19,26 and is
always involved to a degree. Mandibular hypopla-
sia may range from mild flattening of the condylar
head to complete agenesis of the condyle, ascend-
ing ramus, and glenoid fossa. The variable hyp-
oplasia of its component structures results in an
array of temporomandibular joint abnormalities,
ranging from mild malpositioning with aberrant
cranial base articulation to complete obliteration.
The mandibular body may be reduced in all di-
mensions, frequently with an increase in the size
of the gonial angle.32 Variable hypoplasia of the
ipsilateral zygomatico-orbital region is a common
finding, occasionally resulting in orbital dystopia.
Maxillary hypoplasia in combination with man-
dibular deficiency frequently results in dental mal-
occlusion and, depending on the severity of the
maxillomandibular deficiency, an upward occlu-
sal cant to the affected side. Because of their prox-
imity, secondary involvement of skeletal structures
not directly derived from, though closely related
to first and second brachial arch derivatives, is
inevitable.33 Thus, involvement of the squamous
and tympanic portions of the temporal bone,
styloid and mastoid processes, and the pterygoid
process of the sphenoid bone should be expected.
The natural course of skeletal asymmetry seen
in hemifacial microsomia patients has long been
a subject of debate. Kaban and colleagues19,34 have
advanced the view that restricted growth potential
of the affected hemimandible inhibits ipsilateral
vertical maxillary development. This inhibition, in
concert with normal development of contralateral
facial structures, results in a progressive mandib-
ular asymmetry and ultimately in secondary de-
formation of the initially unaffected facial skele-
ton. This assertion was supported by the findings
of Kearns and colleagues in a retrospective analysis
of 67 patients with hemifacial microsomia.35 Other
authors have disputed these views and feel that
development of the affected side of the face par-
allels that of the unaffected side and results in a
nonprogressive mandibular asymmetry.36
Variable deficiency of craniofacial soft tissues
contributes greatly to the phenotypic spectrum of
hemifacial microsomia. This deficiency, a combi-
nation of cutaneous and subcutaneous connective
and neuromuscular tissue, is most evident in the
region of the external ear and eye and the tem-
poral, malar, and masseteric regions of the face.
Periocular abnormality may range from mild in-
ferior displacement of the lateral canthus and/or
palpebral fissure to microphthalmia/anophthal-
mia. Colobomas of the iris or upper lid with ab-
sence of the eyelashes may also be noted.31 Lack of
subcutaneous/cutaneous tissue bulk contributes
to a characteristic temporal hollowing and malar
flattening that is best appreciated when viewed
from a submental perspective. This characteristic
appearance may be accentuated by muscular hy-
poplasia involving the four muscles of mastication:
masseter, temporalis, medial, and lateral ptery-
goids. Masticatory muscle function on the affected
side may likewise be impaired. Impairment of the
lateral pterygoid (responsible for protrusion of
the mandible), in combination with severe ipsi-
lateral maxillomandibular hypoplasia, further dis-
rupts dental occlusion and contributes to devia-
tion of the chin to the affected side. Macrostomia,
or clefting through the oral commissure, and
hypoplasia of the parotid gland may also be
present.37 Neuromuscular hypoplasia may also be
manifested in the muscles of facial expression.
Facial palsies have been estimated to occur in 22
to 45 percent of patients26,38 – 40 and have been
attributed to a variety of neural and muscular
abnormalities.32
Given the common embryologic origin of por-
tions of the external/middle ear and mandible, it
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 Plastic and Reconstructive Surgery • December 2007
is not surprising that auricular and/or preauric-
ular malformations are fundamental if not man-
datory features of this syndrome.41 When present
as isolated findings, auricular malformations such
as microtia or preauricular malformations such as
skin tags or sinuses may represent the mildest form
of hemifacial microsomia.5,41 Whether isolated
dysplasias or merely one element of the overall
phenotype, the ear malformations seen in hemi-
facial microsomia are as diverse as those demon-
strated by the syndrome’s other component fea-
tures. Hypoplasia of the external ear ranges from
mild effacement of auricular architecture to com-
plete auricular agenesis and external auditory ca-
nal atresia. In severe cases, a primitive, caudoven-
trally located auricular remnant (synotia) may be
the only observable evidence of external ear de-
velopment. Occasionally, in the severest of cases,
no remnant is observable. Variable hypoplasia of
middle ear structures is also a common feature.
External and middle ear dysplasia may result in
hearing loss, predominantly conductive in nature,
in up to 75 percent of patients.40
A vast array of associated extracraniofacial anom-
alies has been reported in the hemifacial microso-
mia literature, including skeletal, cardiac, renal, gas-
trointestinal, and pulmonary malformations. An
increasing incidence of extracraniofacial anomalies
appears to parallel increasing facial malformation
severity.23,25 The constellation of hemifacial hypopla-
sia, epibulbar lipodermoids, and vertebral anoma-
lies, including fused and/or hemivertebrae, defines
the Goldenhar syndrome.27 Once considered a vari-
ant of hemifacial microsomia, the Goldenhar syn-
drome is now widely considered to be part of
the hemifacial microsomia continuum.42– 44 Other
craniofacial malformations share many features with
hemifacial microsomia, including the Treacher Col-
lins syndrome.45 This malformation, though dem-
onstrating bifacial hypoplasia, can be distinguished
from hemifacial microsomia on the basis of its he-
redity, colobomas of the outer eyelids, antimongol-
oid slant to the palpebral fissures, and general lack
of facial nerve involvement.5,17,20,46 The micrognathia
that defines the Pierre Robin sequence47 may also be
confused with hemifacial microsomia. The micro-
gnathia of Pierre Robin is, however, generally sym-
metric and, in nonsyndromic, deformational cases,
frequently self-correcting.48 Ear, midface, and or-
bital anomalies are also generally not part of the
sequence.
As is evident, the array of craniofacial struc-
tures, and the varying degree to which they are
affected, defines a phenotypic spectrum of hemi-
facial microsomia that is vastly heterogeneous.
114e
This heterogeneity, the description of which is
largely subjective, poses a formidable barrier to
clinical classification. Over the past 40 years, how-
ever, this challenge has been repeatedly addressed
through numerous classification schemes unique
in both focus and intention.
CLINICAL CLASSIFICATION SYSTEMS
Several classification systems focus on one or
two fundamental anatomical features of the syn-
drome. In a classic article published in the late
1960s, Pruzansky segregated the mandibular anom-
alies of hemifacial microsomia into three grades
(types I through III) of increasing hypoplasia based
largely on the morphology of the ramus and
condyle.13 The assumed normal and unaffected con-
tralateral hemimandible formed the basis of com-
parison in all types. It should be noted that the
Pruzansky classification (as later modified by Kaban
and colleagues34) is replicated exactly, with a minor
modification in nomenclature, in the mandibular
portion of the OMENS classification system.26 It is
thus recommended that the pictographic represen-
tation of the OMENS classification system provided
at the end of this article be reviewed during the
following discussion of Pruzansky’s original classifi-
cation and the subsequent modifications by Kaban
et al.
According to Pruzansky’s original classifica-
tion, a type I mandible is defined as retaining
normal morphologic characteristics of the ramus
and condyle but diminished in size. A type II man-
dible demonstrates significant architectural and
size distortion of the ramus, condyle, and sigmoid
notch. Finally, a type III mandible shows gross
distortion or complete agenesis of the ramus. Ka-
ban and colleagues34 later subdivided the type II
mandible into two categories reflecting the archi-
tecture and function of the temporomandibular
joint. According to their modification, a type IIA
mandible demonstrates acceptable glenoid fossa
anatomy and position with respect to unaffected
side and a type IIB mandible demonstrates tem-
poromandibular joint malpositioning.
Other skeletal classification systems include
those of Harvold and colleagues49 and Lauritzen et
al.50 Both of these systems define five groups of
skeletal deficiencies. The former of these schemas
focuses specifically on the mandible and mastica-
tory muscle function, whereas the latter, intended
to assist in surgical planning, includes deficiencies
of the zygoma and/or orbit. Most recently, Huis-
inga-Fischer and colleagues51 developed a com-
puted tomography– based system for describing
the skeletal malformations of hemifacial microso-
Volume 120, Number 7 • Hemifacial Microsomia
mia. This system consists of a mandibular defor-
mity scoring system that grades mandibular hyp-
oplasia and a cranial deformity scoring system that
grades the hypoplasia of other facial bones. These
two scoring systems are combined to produce a
comprehensive craniofacial deformity scoring sys-
tem reflecting the overall skeletal phenotype. The
craniofacial deformity scoring system results in the
assignment of a single numeric value to the overall
craniofacial deformity, which may not be useful
when planning surgery. To date, it has not been
adopted by many.
Other classification schemes have focused on
alternative single features of the syndrome. One
such system developed by Marx52 and later mod-
ified by Meurman53 focuses exclusively on the ex-
ternal ear and divides malformations into three
grades of increasing severity. These three grades,
similar to Pruzansky’s mandibular grading, range
from mild effacement of auricular architecture to
nearly complete auricular aplasia.
Broader, more complex, and occasionally neb-
ulous classification systems have also been devel-
oped that encompass multiple features of the
hemifacial microsomia spectrum. One such mul-
tiple feature system, developed by Longacre et al.
in 1963,17 divided 44 patients into two groups dis-
playing either unilateral or bilateral facial micro-
somia. These two groups were then further sub-
divided into four classes of increasing facial
deformity. The facial characteristics that defined
each class were not specified. In his 1965 evalua-
tion, Grabb5 segregated 102 patients into one of
six groups defined by varying combinations of
skeletal and soft-tissue deficiencies. Converse and
colleagues46 likewise developed a mixed feature
classification system comprising four groups in
their description of 15 patients exhibiting bilateral
facial microsomia. Rollnick and colleagues23 have
also developed a mixed feature classification com-
prised of five groups, each with microtia as the
fundamental feature. In a unique multiple feature
analysis, Edgerton and Marsh28 defined four groups
based on the “dominant dysplasia” (mandibular, soft
tissue, auricular, composite) exhibited. One final
mixed feature classification system of note, devel-
oped by David and colleagues,21 is modeled after the
tumor, node, metastasis grading system of malignant
tumors.54 This skeletal, auricular, and soft-tissue clas-
sification system was used to independently analyze
skeletal, auricular, and soft-tissue malformations in
47 patients.
Although adequately tailored to their limited
sample populations, many—arguably all— of the
aforementioned classification systems fail to pos-
ses the versatility needed to easily and accurately
categorize any potential hemifacial microsomia
phenotype. Given the complexity and diversity of
the hemifacial microsomia spectrum, a satisfactory
classification system should be broad enough to
accurately and comprehensively facilitate the cat-
egorization of the syndrome’s multiple features in
a succinct, coherent, accessible and, as much as
possible, objective manner. Such a classification
system would standardize, as much as possible, the
evaluation of hemifacial microsomia patients. It
would facilitate clinical analysis of large popula-
tions both within and between institutions and
guide surgical planning and possibly even shed
light on this syndrome’s elusive pathogenesis. To
date, the OMENS classification of hemifacial
microsomia26 appears to best satisfy these criteria.
THE OMENS CLASSIFICATION
In the words of Cohen: “The OMENS classi-
fication of Hemifacial Microsomia. . .is a welcome
addition to the literature on the subject.”44 This
system, developed by Vento and colleagues in a
1991 study of 154 hemifacial microsomia patients,
substratifies each of five anatomical manifesta-
tions of hemifacial microsomia according to dys-
morphic severity on a scale from 0 to 3. The five
manifestations each constitute one letter of the
acronym: orbital asymmetry, mandibular hypopla-
sia, ear deformity, nerve dysfunction, and soft-tis-
sue deficiency. Scoring was done on the basis of
conventional radiographs including posterior/an-
terior, lateral, submental, and panoramic views,
and physical examination and photographs. The
pictographic representation of the OMENS clas-
sification system provided at the end of this article
may aid in the visualization of the system’s sub-
tleties. It is once again recommended that this
representation be referenced during the follow-
ing discussion of the system’s five categories.
The orbit category reflects both orbital size and
position, the latter of which, when abnormal, is
marked with an arrow indicating superior or inferior
displacement. Scoring within the mandible category
is done so on the basis of radiographs and uses the
systems of Pruzansky13 and Murray et al.19,34 dis-
cussed previously. Scoring of external ear anomalies
uses the systems of Marx and Meurman,52,53 with the
addition of the grade 0 category meant to reflect the
absence of any observable external ear malforma-
tion. Scoring within the facial nerve category groups
the zygomatic and temporal branches into one
group and the buccal, marginal mandibular, and
cervical branches into another, thus dividing the
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 Plastic and Reconstructive Surgery • December 2007

face into upper and lower halves. Categories are also
reserved for no nerve involvement and panhemifa-
cial paralysis. Finally, scoring of soft-tissue deficien-
cies uses a modified version of the system developed
by Murray and colleagues30 and grades subcutane-
ous/muscular deficiency as either absent, mild,
moderate, or severe. Classification for each side of
the face is done separately in cases of bifacial mi-
crosomia.
An early commentary by Cohen44 criticized
the OMENS system for neglecting the inclusion
of significant extracraniofacial anomalies. This
was answered by a modification to the system by
Horgan et al. in 199525 that allows for the op-

Fig. 1. Modified OMENS (⫹) classification of hemifacial microsomia form.

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Volume 120, Number 7 • Hemifacial Microsomia
tional addition of a plus sign [OMENS (⫹)] to
denote the presence of associated, extracranio-
facial anomalies. Additional criticism has cen-
tered around the system’s definition of orbital
dystopia. It has been suggested by Cousley and
Calvert55 that this definition needs to be further
refined so as to clarify the amount of radio-
Fig. 2. Modified OMENS (⫹) classification of hemifacial microsomia form, continued.
graphic evidence needed to classify the orbit as
abnormal in both size and position (O3 designa-
tion). A final suggestion, also by Cousley,56 calls for
the expansion of the auricular category to include
both middle ear and preauricular defects.
Despite these criticisms, the OMENS system
represents a very accessible, flexible, comprehen-
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 Plastic and Reconstructive Surgery • December 2007
sive, and largely objective means of classifying the
range of abnormalities constituting the spectrum
of hemifacial microsomia. The grading systems
within each category encompass the full range of
dysplastic severity, defining each anatomical mal-
formation in a very simple and reproducible man-
ner. The use of a numeric classification also serves
to objectify, within limits, the many inherently sub-
jective features of this disorder and in doing so
aides in the analysis of this population within and
between institutions.
Unfortunately, Vento and colleagues’ original
study of 154 patients26 and a study of 65 patients
conducted by Poon et al.57 are, to our knowledge,
the only studies to date that classify populations of
hemifacial microsomia patients according to the
OMENS scheme. Furthermore, Vento and col-
leagues’ original study is the only study to date that
correlates the degree of mandibular hypoplasia
with the other four fundamental features of the
classification system (orbital, auricular, facial nerve,
and soft-tissue morphology). Their elegant study
found a positive correlation between mandibular
hypoplasia and each of the other anatomical fea-
tures of the acronym—a finding that underscores
the fundamentality of mandibular abnormality to
the syndrome.
Further studies that classify large hemifacial mi-
crosomia populations according to the OMENS
scheme are needed. Such studies, as Vento and col-
leagues suggest,26 would allow for independent anal-
ysis of hemifacial microsomia’s numerous anatomi-
cal features and may reveal possible relationships
between the various craniofacial and extracraniofa-
cial features of this diverse and complex syndrome.
Although many photographic and radiographic
examples of various OMENS classifications were in-
cluded in the original article by Vento et al., many of
the 22 possible subcategories lacked visual depic-
tions. We have therefore included in this continuing
medical education article a concise hemifacial mi-
crosomia evaluation form containing clinical illus-
trations depicting each grade of severity within the
five OMENS categories. Illustrations are composite
sketches based on clinical examples provided by the
hemifacial microsomia population evaluated be-
tween 1972 and 2005 by the Department of Plastic
Surgery at the Children’s Hospital of Philadelphia.
Because of the frequency of associated macrostomia,
estimated to occur in 23 to 35 percent3,58 of the
hemifacial microsomia population, we have modi-
fied the original OMENS classification and included
illustrations of both complete and incomplete
Tessier no. 7 clefts, which we feel should be a com-
ponent of initial patient evaluation. We have also
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included a field for the documentation of miscella-
neous extracraniofacial anomalies [OMENS (⫹)]
(Figs. 1 and 2).
Despite the addition of the aforementioned
modifications we recognize that the OMENS system
is arguably incomplete. For example, the system
does not include a category defining the extent of
malar/midface skeletal deficiency. In addition, the
nerve category does not allow for the subcategori-
zation of minor single-branch paresis (e.g., marginal
mandibular). It is hoped that the miscellaneous field
included in our modified OMENS (⫹) evaluation
form will be used by the clinician to elaborate on
these subtleties until a more inclusive, evidence-
based system is developed.
Currently in our craniofacial unit, we use the
Hellenic Craniofacial Center Database59 for the
management of craniofacial data. This system, de-
veloped by Drs. Alexander Stratoudakis and Platon
Alexiadés, is remarkable in its breadth and is used by
most craniofacial centers in the United Kingdom.
We have modified this wonderfully comprehensive
electronic database to include the OMENS classifi-
cation, complete with the pictographic examples
presented in this article. It is our hope that the clin-
ical evaluation form provided in this continuing
medical education article will promote the use of the
OMENS classification system in the evaluation of
future hemifacial microsomia patients and facilitate
the cataloguing of clinical data in charts and/or
electronic databases.
Scott P. Bartlett, M.D.
Division of Plastic Surgery
Department of Surgery
University of Pennsylvania
10 Penn Tower
3400 Spruce Street
Philadelphia, Pa. 19104
scott.bartlett@uphs.upenn.edu
DISCLOSURE
None of the authors has a financial interest in any of
the products, devices, or drugs mentioned in this article.
REFERENCES
1. Gorlin, R. J., and Pindborg, J. J. Syndromes of the Head and Neck.
New York: McGraw-Hill, 1964. Pp. 641–646.
2. Canton, E. Arrest of development of the left ramus of the
lower jaw, combined with malformation of the external ear.
Trans. Pathol. Soc. Lond. 12: 237, 1861.
3. Gorlin, R. J., Cohen, M. M., and Hennekam, R. C. M. Syn-
dromes of the Head and Neck. New York: Oxford University
Press, 2001. Pp. 790–798.
4. Converse, J. M., McCarthy, J. G., Coccaro, P. J., et al. Clinical
aspects of craniofacial microsomia. In J. M. Converse, J. G.
McCarthy, and D. Wood-Smith (Eds.), Symposium on Diagnosis
Volume 120, Number 7 • Hemifacial Microsomia
and Treatment of Craniofacial Anomalies. St. Louis: Mosby, 1979.
Pp. 461–462.
5. Grabb, W. C. The first and second brachial arch syndrome.
Plast. Reconstr. Surg. 36: 485, 1965.
6. Stark, R. B., and Saunders, D. E. The first brachial arch
syndrome, the oral-mandibular-auricular syndrome. Plast.
Reconstr. Surg. 29: 229, 1962.
7. Francois, J. Heredity in Ophthalmology. St. Louis: Mosby, 1961.
Pp. 122–123.
8. Obwegeser, H. L. Correction of the skeletal anomalies of
otomandibular dysostosis. J. Maxillofac. Surg. 2: 73, 1974.
9. Caronni, E. P. Embryogenesis and classification of branchial
auricular dysplasia. Transactions of the Fifth International Con-
gress of Plastic and Reconstructive Surgery, Melbourne, Australia,
1971.
10. Walker, D. G. Malformations of the Face. Baltimore: Williams &
Wilkins, 1961. Pp. 67–68.
11. Ross, R. B. Lateral facial dysplasia (first and second brachial
arch syndrome, hemifacial microsomia). Birth Defects 11: 51,
1975.
12. Keith, A. Three demonstrations on congenital malforma-
tions of the face, head and foot. Br. J. Surg. 28: 173, 1940.
13. Pruzansky, S. Not all dwarfed mandibles are alike. Birth Defects
1: 120, 1969.
14. Dingman, R. O., and Grabb, W. C. Mandibular laterogna-
thism. Plast. Reconstr. Surg. 31: 563, 1963.
15. Gorlin, R. J., Cohen, M. M., and Levin, L. S. Syndromes of the
Head and Neck. New York: Oxford University Press, 1990. Pp.
641–649.
16. Jongbloet, P. H. Goldenhar syndrome and overlapping dys-
plasias in vitro fertilisation and ovopathy. J. Med. Genet. 24:
616, 1987.
17. Longacre, J. J., De Stephano, G. A., and Holmstrand, K. E.
The surgical management of first and second brachial arch
syndromes. Plast. Reconstr. Surg. 31: 507, 1963.
18. Posnick, J. C. Craniofacial and Maxillofacial Surgery in Children
and Young Adults, 1st Ed. Philadelphia: Saunders, 2000. Pp.
419–445.
19. Kaban, J. C., Mulliken, J. B., and Murray, J. E. Three-dimen-
sional approach to analysis and treatment of hemifacial mi-
crosomia. Cleft Palate J. 18: 90, 1981.
20. Coccaro, P. J., Becker, M. H., and Converse, J. M. Clinical and
radiographic variation in hemifacial microsomia. Birth Defects
11: 314, 1975.
21. David, D. J., Mahatumarat, C., and Cooter, R. D. Hemifacial
microsomia: A multisystem classification. Plast. Reconstr. Surg.
80: 525, 1987.
22. Poswillo, D. E. Otomandibular deformity: Pathogenesis as a
guide to reconstruction. J. Maxillofac. Surg. 2: 64, 1974.
23. Rollnick, B. R., Kaye, C. I., Nagatoshi, K., et al. Oculoauricu-
lovertebral dysplasia and variants: Phenotypic characteristics
of 294 patients. Am. J. Med. Genet. 29: 361, 1987.
24. Wilson, G. N. Cranial defects in Goldenhar’s syndrome.
Am. J. Med. Genet. 14: 435, 1983.
25. Horgan, J. E., Padwa, B. L., LaBrie, R. A., et al. OMENS-plus:
Analysis of craniofacial and extracraniofacial anomalies in
hemifacial microsomia. Cleft Palate Craniofac. J. 32: 405, 1995.
26. Vento, A. R., LaBrie, R. A., and Mulliken, J. B. The
O.M.E.N.S. classification of hemifacial microsomia. Cleft Pal-
ate Craniofac. J. 28: 68, 1991.
27. Gorlin, R. J., Jue, K. L., Jacobsen, U., et al. Oculoauriculo-
vertebral dysplasia. J. Pediatr. 63: 991, 1963.
28. Edgerton, M. T., and Marsh, J. L. Surgical treatment of
hemifacial microsomia: First and second brachial arch syn-
drome. Plast. Reconstr. Surg. 59: 653, 1977.
29. Christiansen, R. L., and Evans, C. A. Habilitation of severe
craniofacial anomalies: The challenge of new surgical pro-
cedures. Cleft Palate J. 12: 167, 1975.
30. Murray, J. E., Kaban, L. B., and Mulliken, J. B. Analysis and
treatment of hemifacial microsomia. Plast. Reconstr. Surg. 74:
186, 1984.
31. Cohen, M. M. Variability versus “incidental findings” in the
first and second brachial arch syndrome: Unilateral variants
with anophthalmia. Birth Defects 7: 103, 1989.
32. McCarthy, J. G. Craniofacial microsomia: A primary and
secondary surgical treatment plan. Clin. Plast. Surg. 24: 459,
1997.
33. Converse, J. M., Coccaro, P. J., and Becker, M. On hemifacial
microsomia. Plast. Reconstr. Surg. 51: 268, 1973.
34. Kaban, L. B., Moses, M. H., and Mulliken, J. B. Surgical
correction of hemifacial microsomia in the growing child.
Plast. Reconstr. Surg. 82: 9, 1988.
35. Kearns, G. J., Padwa, B. L., Mulliken, J. B., et al. Progression
of facial asymmetry in hemifacial microsomia. Plast. Reconstr.
Surg. 105: 492, 2000.
36. Polley, J. W., Figueroa, A. A., Liou, E. J., et al. Longitudinal
analysis of mandibular asymmetry in hemifacial microsomia.
Plast. Reconstr. Surg. 99: 330, 1997.
37. Whitaker, L. A., and Bartlett, S. P. Craniofacial anomalies. In
M. J. Jurkiewicz, T. J. Krizek, S. J. Mathes, and S. Ariyan
(Eds.), Plastic Surgery Principles and Practice, Vol. 1, 1st Ed. St.
Louis: Mosby, 1990. Pp. 99–136.
38. Bassila, M. K., and Goldberg, R. The association of facial palsy
and/or sensorineural hearing loss in patients with hemifacial
microsomia. Cleft Palate J. 26: 287, 1989.
39. Bergstrom, L., and Baker, B. B. Syndromes associated with
congenital facial paralysis. Otolaryngol. Head Neck Surg. 89:
336, 1981.
40. Carvalho, G. J., Song, C. S., Vargervik, K., et al. Auditory and
facial nerve dysfunction in patients with hemifacial micro-
somia. Otolaryngol. Head Neck Surg. 125: 209, 1999.
41. Rollnick, B. R., and Kaye, C. I. Hemifacial microsomia and
variants: Pedigree data. Am. J. Med. Genet. 15: 233, 1983.
42. Figueroa, A. A., and Friede, H. Craniovertebral malforma-
tions in hemifacial microsomia. J. Craniofac. Genet. Dev. Biol.
Suppl. 1: 167, 1985.
43. Cohen, M. M., Rollnick, B. R., and Kaye, C. I. Oculoauricu-
lovertebral spectrum: An updated critique. Cleft Palate J. 26:
276, 1989.
44. Cohen, M. M. A critique of the OMENS classification system
of hemifacial microsomia. Cleft Palate Craniofac. J. 28: 77,
1991.
45. Franceschetti, A., and Klein, D. The mandibulofacial dysos-
tosis: A new hereditary syndrome. Acta Ophthal. (Kbh.) 27:
143, 1949.
46. Converse, J. M., Wood-Smith, D., McCarthy, J. G., et al. Bi-
lateral facial microsomia. Plast. Reconstr. Surg. 54: 413, 1974.
47. Robin, P. La chute de la base de la langue considerée comme
une nouvelle cause de gene dans la respiration naso-phar-
yngienne. Bull. Acad. Med. (Paris) 89: 37, 1923.
48. Singh, D. J., and Bartlett, S. B. Congenital mandibular hy-
poplasia: Analysis and classification. J. Craniofac. Surg. 16:
291, 2005.
49. Chierici, G. Radiologic assessment of facial asymmetry. In
E. P. Harvold, K. Vargervik, and G. Chierici (Eds.), Treatment
of Hemifacial Microsomia, 1st Ed. New York: Alan R. Liss, 1983.
Pp. 57–87.
50. Lauritzen, C., Munro, I. R., and Ross, B. R. Classification and
treatment of hemifacial microsomia. Scand. J. Plast. Reconstr.
Surg. 19: 33, 1985.
119e
 Plastic and Reconstructive Surgery • December 2007
51. Huisinga-Fischer, C. E., Zonneveld, F. W., Vaandrager, J.
M., et al. CT-based size and shape determination of the
craniofacial skeleton: A new scoring system to assess bony
deformities in hemifacial microsomia. J. Craniofac. Surg.
12: 87, 2001.
52. Marx, H. Die Missbildungen des Ohres: Sekundare Ohrmis-
sibildungen. In a. O. L. F. Henke (Ed.), Handbuch der spez-
iellen pathologischen Anatomie and Histologie, 1st Ed. Berlin:
Springer-Verlag, 1962. Pp. 697–702.
53. Meurman, Y. Congenital microtia and meatal atresia. Arch.
Otolaryngol. 66: 443, 1957.
54. Copeland, M. M. American Joint Committee on Cancer Stag-
ing and end results reporting: Objectives and progress. Can-
cer 18: 1637, 1965.
120e
55. Cousley, R. R. J., and Calvert, M. L. Current concepts in the
understanding and management of hemifacial microsomia.
Br. J. Plast. Surg. 50: 536, 1997.
56. Cousley, R. R. J. A comparison of two classification systems
for hemifacial microsomia. Br. J. Oral Maxillofac. Surg. 31:
78, 1993.
57. Poon, C. C., Meara, J. G., and Heggie, A. A. Hemifacial
microsomia: Use of the OMENS-plus classification at the
Royal Children’s Hospital of Melbourne. Plast. Reconstr. Surg.
111: 1011, 2003.
58. Fan, W. S., Mulliken, J. B., and Padwa, B. L. An association
between hemifacial microsomia and facial clefting. J. Oral
Maxillofac. Surg. 63: 330, 2005.
59. Stratoudakis, A. Personal communication, 2006.