Abstract

OBJECTIVE:To present a case of Congenital Cranial Disinnervation Disorder with RE Superior Oblique weakness with Marcus Gun Jaw Winking METHODS:A 8yr child was brought with C/O of drooping of right eyelid and occasional inward deviation of (RE) since birth.Born to 3rd degree consanguinity.O/e CHP:right face turn & chin elevation.Facial symmetry:lost-(RE)ptosis and ocular symmetry:lost-(RE)exotropia.HCRT:(RE)7deg Exotropia and 7deg Hypertropia.ACT:(BE)moving in alternately to fixate,briskly.EOM:Limitation-(RE)levoelevation and (RE)levodepression.Marcus Gun Jaw Winking was seen.Intraop:(RE)SupOblique laxity noted with globe going up into the orbit beyond midline.InfOblique overaction secondary to SupOblique weakness was seen.Patient was managed surgically with Parks procedure;(RE)InfOblique Graded Recession and insertion 3mm away from InfRectus,along with SupOblique 10mm tucking.Postop decrease in vertical deviation achived with residual exotropia.CONCLUSION: MGJW syndrome is one of CCDD