Hemi facial microsomia /orthodontic courses by Indian dental academy

Hemifacial Microsomia: VariableHemifacial Microsomia: Variable
findings in 4 casesfindings in 4 cases
 INDIAN DENTAL ACADEMYINDIAN DENTAL ACADEMY
 Leader in continuing DentalLeader in continuing Dental
EducationEducation
www.indiandentalacademy.comwww.indiandentalacademy.com

INTRODUCTIONINTRODUCTION
 Hemifacial microsomia is the second most commonHemifacial microsomia is the second most common
congenital facial anomaly after cleft lip /palate with acongenital facial anomaly after cleft lip /palate with a
reported incidence of about 1 in 5600 live births.reported incidence of about 1 in 5600 live births.
 It is a condition in which the tissue on one side of the faceIt is a condition in which the tissue on one side of the face
is underdeveloped, affecting primarily the aural, oral andis underdeveloped, affecting primarily the aural, oral and
mandibular areas. Children may have a mild form thatmandibular areas. Children may have a mild form that
includes mild facial asymmetry and ear deformity to aincludes mild facial asymmetry and ear deformity to a
more severe form that includes additional eye, vertebral,more severe form that includes additional eye, vertebral,
cardiac, and renal malformations (Goldenhar syndrome).cardiac, and renal malformations (Goldenhar syndrome).
 In some cases both sides of the face can be affected andIn some cases both sides of the face can be affected and
may involve the skull, as well as the face & in some raremay involve the skull, as well as the face & in some rare
cases facial nerve paralysis.cases facial nerve paralysis.

Case 1Case 1
 A 40 year old femaleA 40 year old female
patient came to the OPDpatient came to the OPD
of our college with theof our college with the
chief complaint of painchief complaint of pain
in all her teeth since 3in all her teeth since 3
months.months.

CASE 2CASE 2
 A 6 year old femaleA 6 year old female
patient came to thepatient came to the
OPD with her fatherOPD with her father
complaining ofcomplaining of
asymmetry of her faceasymmetry of her face
since birth.since birth.

CASE 3CASE 3
 A 5 year old maleA 5 year old male
of our college with chiefof our college with chief
complaint of decayedcomplaint of decayed
tooth in left lower backtooth in left lower back
tooth region as told bytooth region as told by
the father of the patient.the father of the patient.

CASE 4CASE 4
 A 7 year old maleA 7 year old male
of our college with chiefof our college with chief
complaint of pain in hiscomplaint of pain in his
left lower back toothleft lower back tooth
since 3 months.since 3 months.

COMPARATIVE FINDINGSCOMPARATIVE FINDINGS
S.NoS.No FEATURESFEATURES CASE 1CASE 1 CASE 2CASE 2 CASE 3CASE 3 CASE 4CASE 4
1.1. UNDERDEVELOPM-UNDERDEVELOPM-
-ENT OF FACIAL-ENT OF FACIAL
STRUCTURESSTRUCTURES
++ ++ ++ ++
2.2. SMALL ORSMALL OR
MISSHAPEN EARSMISSHAPEN EARS ++ ++ ++ ++
3.3. PREAURICULARPREAURICULAR
SKIN TAGSSKIN TAGS __ ++ ++ __
4.4. MACROSTOMIAMACROSTOMIA
__ ++ ++ __

5.5. INTRAORALINTRAORAL
PROBLEMSPROBLEMS __ ++ ++ ++
6.6. CRANIAL NERVECRANIAL NERVE
INVOLVEMENTINVOLVEMENT __ ++ __ ++
7.7. EYE DEFECTSEYE DEFECTS
__ ++ __ __
8.8. VERTEBRALVERTEBRAL
DEFECTSDEFECTS __ __ __ ++

9.9. HEARING LOSSHEARING LOSS
++ __ __ ++
10.10. MISSING TEETHMISSING TEETH
__ __ __ ++
11.11. BILATERALBILATERAL
INVOLVEMENTINVOLVEMENT __ __ ++ __
12.12. OMENSOMENS
CLASSIFICATIONCLASSIFICATION
OO00MM00EE33NN00SS11 OO00MM11EE11NN1212
SS22 OO00MM11EE11NN00SS22 OO00MM11EE33NN77
33SS11

DISCUSSIONDISCUSSION
 Hemifacial microsomia was first recorded by German
physician Carl Von Arlt in 1845 and Goldenhar in 1952
defined the syndrome more clearly.
 Hemifacial microsomia (HM) is also known as Goldenhar-
Gorlin syndrome, first and second branchial arch syndrome,
and oculo-auriculo-vertebral dysplasia.
 Males appear to be more frequently affected than females
and the right side is affected more often than the left side
(3:2).

ETIOLOGYETIOLOGY
 SporadicSporadic
 Familial inheritanceFamilial inheritance
 Neural crest theoryNeural crest theory

Features include:
 Macrostomia
 Underdevelopment of the muscles of the face, cheek bones and
skin.
 Microtia or anotia
 Preauricular skin tags or pits
 Mouth problems such as lack of saliva, problems in tongue &
missing teeth.
 Vertebral defects
 Other problems that may occur in some but not all cases are
eye defects, deafness, cleft palate, heart, limb or kidney.
 The presence of otic hypoplasia, lateral facial cleft and
vertebral anomalies are the minimum criteria for the diagnosis
of Goldenhar syndrome.

INVESTIGATIONSINVESTIGATIONS
 Maxillary occlusal radiograph
 Panoramic radiograph
 Lateral cephalometric radiograph
 Posterior-anterior view
 Computed tomography
 Magnetic resonance imaging
 Hearing evaluation, phonics tests, laryngoscopic inspection
and vocalization analysis
 Spine radiograph
 General & systemic examination

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS
 Pierre Robin Syndrome
 Mobius syndrome
 Treacher Collins
syndrome
 Unilateral ankylosis

TREATMENTTREATMENT
 Conservative
 Surgical

ConclusionConclusion

BIBLIOGRAPHYBIBLIOGRAPHY
 EVELYN MARUKO et al. Hypodontia in Hemifacial Microsomia Cleft Palate–
Craniofacial Journal, January 2001, Vol. 38 No. 1
 Muhammad Arif Maan et al.Goldenhar syndrome: case reports with review of
literature ;Journal of Pakistan Association of Dermatologists 2008; 18: 53-55.
 M. V. V. Reddy, P. P. Reddy :Facio-auricular vertebral syndrome—a case report;
Indian Journal of Human Genetics September-December 2005 Volume 11 Issue 3
 C. Moulin-Romse et al. Treatment of hemifacial microsomia in a growing child: the
importance of co-operation between the orthodontist and the maxillofacial surgeon;
Journal of Orthodontics, Vol. 31, 2004, 190–200
 Gerard J. Carvalho et al: Auditory and Facial Nerve Dysfunction in Patients With
Hemifacial Microsomia; ARCH OTOLARYNGOL HEAD NECK SURG/VOL
125, FEB 1999
 RICHARD MONAHAN et al: Hemifacial microsomia Etiology, diagnosis and
treatment; JADA, Vol. 132, October 2001

BIBLIOGRAPHY(contd.)BIBLIOGRAPHY(contd.)
 P. Kalsotra et al: Carniofacial Microsomia Vol. 8 No. 2, April-June 2006 169 JK
SCIENCE
 Russell R. Wang et al: Hemifacial microsomia and treatment options for auricular
replacement: A review of the literature; THE JOURNAL OF PROSTHETIC
DENTISTRYVOLUME 82 NUMBER 2
 J. K. Sharma et al: GOLDENHAR-GORLIN’S SYNDROME: A CASE REPORT;
Indian Journal of Otolaryngology and Head and Neck Surgery Vol. 58, No. 1,
January-March 2006
 Kapur R et al: Hemifacial Microsomia A Case Report; J Ind Soc Pedod Prevent
Dent, Suppli 2008.
 David Healey et al: Cervical spine instability in children with Goldenhar’s
syndrome; Can J Surg, vol. 45, no. 5, October 2002
 D. H. MELLOR, JOYCE E. RICHARDSON: Goldenhar's syndrome; Archives of
Disease in Childhood, 1973, 48, 537.
 Antônio Luiz Barbosa et al: Goldenhar’s Syndrome - Case Report; Braz Dent J
(2003) 14(1): 67-70

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Hemi facial microsomia /orthodontic courses by Indian dental academy

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