Year : 1970 | Volume
: 18 | Issue : 1 | Page : 37--38
Oculo-auricular dysplasta - a case report
DL Maria, KG Tehra, MD Kale
Medical College, Aurangabad, India
D L Maria
Medical College, Aurangabad
|How to cite this article:|
Maria D L, Tehra K G, Kale M D. Oculo-auricular dysplasta - a case report.Indian J Ophthalmol 1970;18:37-38
|How to cite this URL:|
Maria D L, Tehra K G, Kale M D. Oculo-auricular dysplasta - a case report. Indian J Ophthalmol [serial online] 1970 [cited 2013 May 19 ];18:37-38
Available from: http://www.ijo.in/text.asp?1970/18/1/37/35059
This rare syndrome was first described by Goldenhar  . It is characterised by epibulbar dermoids; accessary auricular appendages and aural fistulae. This is unilateral and sometimes may be associated with mandibulo facial dysostosis.
Rarely other anomalies of the eye; nervous, muscular and skeletal systems may be associated with Goldenhar's syndrome. Recently a case of Oculo auricular Cranial dysplasia, having hydrocephalous meningo - encephalocele and a-nasal deformity along with Goldenhar's syndrome has been reported by Gupta J. S  .
We have come across an adult male having bilateral auricular appandages, epibulbar dermoid and othe rare anomalies. This case is being reported because of its extreme rarity, mixed picture of Goldenhar syndrome with other rare anomalies and bilateral auricular appendages.
A twenty years old hindu male was admitted in the ophthalmology department on 12-8-1968 with cosmetic disfigurement and diminution of vision. He was moderately built with normal mental behaviour. schooling in the tenth class. The face was asymmetrical slightly deviating to the left side with slight underdeveloped mandible. There was one necessary auricular appandage on the right side and two on the left side. (Plates 1 and 2)
The left side of the chest was more prominent with scoliosis to the right side in the mid thoracic region. There was a hypopigmented patch on the right iliac region. (Plate 3)
There was bilateral microphthalmos, with dermoid cyst on the outer side of the limbus in the left eye showing fine hairs. (Plate 4)
The cyst was pigmented with keratosis. The iris on the left side showed typical coloboma while the right iris was normal. Both fundi showed coloboma of the choroid in the typical position. Vision of both the eyes was 3/60 and tension was 13 mm. Hg. The vision could not be improved with glasses.
He had five brothers and two sisters all being normal except one brother who had congenital absence of fingers of the left hand. No anomaly of any kind is present among his paternal and maternal relations as far as he could tell.
Dermoid cyst was excised on 20th August 1968.
A rare case of Oculo-Auricular dysplasia having anomalies on both sides with slight deviation of the face to the left side with under developed mandible, scoliosis and prominence of left side chest is presented.
|1||Goldenhar- Quoted by Duke Elder System of Ophthalmology Vol. III part 2. Henry Kimpton, London P. 1021, 1964.|
|2||Gupta J. S., Gupta S. G. - British Journal of Ophthalmology. Vol. 52. P 346-47. 1968.|