A new perspective in oculoplastic surgical management of symptomatic distichiasis in lymphedema-distichiasis syndrome

Abstract

Lymphedema-distichiasis syndrome (LDS) is an autosomal dominant condition associated with FOXC2 mutations. Patients with distichiasis are mostly symptomatic, and efforts to deal with their ocular complaints comprise of electrolysis, cryotherapy and a variety of surgical techniques. We describe an enhanced surgical technique for a case of symptomatic distichiasis of the right eye with scarred, irregular eyelid margins secondary to initial cryotherapy, whereby the distorted tarsus was excised to remove the aberrant hair follicles, the levator palpebrae superioris was released to extend the upper lid and prevent lagophthalmos and a mucous membrane graft was used to cover the exposed portion of the tarsal plate. At 14 months follow up, the lid cosmesis and position remained satisfactory, with no infection or rejection of the mucous membrane graft. Therefore, this surgical technique provides a sound option for symptomatic distichiasis, where cryotherapy can cause lid irregularity and keratinization.

Keywords: Distichiasis; cryotherapy; lid split; mucous membrane graft; tarsal plate excision.