Sporadic Blepharophimosis Syndrome: A Case Report.

Dr.Manjunath Kamath; Dr. Shylesh Dabke; Dr.Gurudutt Kamath; Dr.Rajesh Nayak; Dr.Ajay Kamath; Dr.Susan D�souza

Volume : V, Issue : V, May - 2016

Sporadic Blepharophimosis Syndrome: A Case Report.

Dr. Manjunath Kamath, Dr. Shylesh Dabke, Dr. Gurudutt Kamath, Dr. Rajesh Nayak, Dr. Ajay Kamath, Dr. Susan D Souza

Abstract :

 Blepharophimosis syndrome is a rare congenital eyelid malformation characterized by shortened horizontal palpeal fissures (blepharophimosis), impaired function of levator palpeae superioris of upper lid (ptosis), a vertical skin fold arising from the lower eyelid that inserts medially in the upper lid (epicanthus inversus) and an increased inner canthal distance (telecanthus).Here we are reporting a case of blepharophimosis syndrome in a 8 year old female patient. She presented with drooping of eyelids which was constant throughout the day. On examination found to have chin lift, bilateral blepharoptosis with a oad and flat nasal idge, epicanthus inversus, telecanthus, and blepharophimosis. Based on the constellation of these findings, a diagnosis of BPES was reached for the girl. Patient then was planned for a two stage procedure. In the first sitting we undertook lateral canthotomy and medial canthoplasty ( Modified Mustarde technique ).Ptosis was corrected with bilateral Frontalis Sling surgery in the second sitting. Patient was followed up weekly & showed clinical improvement with minimal scarring