By Dr. Sonali Ashok Bhosale (Junior Resident ,Dr V.M.Government Medical College, Solapur )

1. GOLDENHAR SYNDROME-
A CASE REPORT
By Dr. Sonali Ashok Bhosale
(Junior resident ,Dr V.M.Government medical
college, solapur )

2. INTRODUCTION
 Goldenhar syndrome is a birth defect
resulting from the maldevelopment of the first
two branchial arches with incomplete
development of the ear, nose, soft palate, lip
and mandible.
 It is unilateral in 70-80% of the cases
 Prevalence rate is 1-9/per 100000 and
 Incidence rate is 1 per 25000-45000 births
 Male is more commonly affected than the
female (ratio 2:1).

3.  Ocular anomalies occur in about 50% of
the cases.
 Epibulbar dermoid and lipodermoid are
the most common.
 Limbal dermoid or lipodermoid are
mainly located in the inferotemporal
region of the eye.
 Coloboma of the upper eyelid may be
present.

4.  Extraocular defects are reported in 65%
of the cases and include
 Pre-auricular tags, microtia, anotia,
conductive hearing loss.
 Vertebral anomalies are combination of
hemivertebra, fused ribs, kyphosis and
scoliosis.
 Additional features such as cardiac,
genito-urinary and pulmonary systems
can also be affected.

5. Case report
 An 8 years old male child
reported at the outpatient
department of
Ophthalmology,
DrVMgovernment
medical college,
solapur,on 15 march
2016 with complains of
-swelling of the lower and
outer part of eye
-yellowish pink swelling in
the outer part of the right
eye
-associated with
preauricular tags,
hemifacial asymmetry.

6.  Ocular examination revealed
-a smooth and soft mass in
the temporal side of right eye
-posterior limit of which could
not be seen.
-From above downwards it
was extending from the
superior to the inferior fornix.

7. On palpation -It was found to be soft,
nontender and its temperature was not
raised
-It could be moved over the underlying
structure and conjunctiva over it could
also be moved.
 His visual acuity was 6/6 in both eyes.
 The rest of anterior segment was normal
 Fundoscopic examination was found
normal.
 Left eye was normal.

8.  The clinical diagnosis was
Dermolipoma, which was confirmed by a
histological examination of an excised
portion.

9. ENT examination revealed preauricular
tags present in both ears

10.  No other abnormalities were detected.
 Systemic examination like cardiovascular,
pulmonary and genito-urinary systems was
done but no abnormalities were detected.
 The laboratory investigations were within
normal limits.
 Electrocardiography was normal,
 X-ray chest (postero anterior view) showed
nothing abnormality detected,
 X-ray of the vertebral column was normal.

11.  Complete excision of
the lipodermoid was
achieved.

12. Conclusion
 This case has been presented to
increase the awareness of this rare
entity.
 To highlight the clinical findings and
association with various systemic
conditions.
 Multidisciplinary treatment approach
and long term follow up is required to
monitor the growth and development of
such patients.