Ocular motility disturbances (Duane retraction syndrome and double elevator palsy) with congenital heart disease, a rare association with Goldenhar syndrome--a case report.
This report is a case of a 4 year old male child who was admitted for meningitis. On clinical examination he was diagnosed as a case of oculo-suriculo-vertebral dysplasia with congenital heart disease, i.e., tetralogy of Fallots besides his presenting picture of meningitis. During his ophthalmic checkup for the conspicuous presence of epibulbar dermoid, he was discovered to have upper lid coloboma, double elevator palsy, and Grade 1 Duane retraction syndrome in his right eye while the pathognomic feature, a dermolipoma, was present in the left eye. The oculo-auriculo-vertebral dysplasia, as described by Goldenhar, is a disease complex of structures developed from the Ist and IInd branchial arch. The important feature of this case is the bilateral involvement of the disease complex over and above the presence of ocular motility disorders--Duane retraction syndrome and double elevator palsy.
Verma
M J
MJ
University College of Medical Sciences, Delhi.
Faridi
M M
MM
D002675:Child, Preschool; D003103:Coloboma; D003315:Cornea / Q000002:abnormalities; D005134:Eye Neoplasms; D006053:Goldenhar Syndrome; D006330:Heart Defects, Congenital; D006801:Humans; D008067:Lipoma; D008297:Male; D015835:Ocular Motility Disorders