Microphthalmia, Dermal Aplasia, and Sclerocornea Syndrome: Endoscopic Cyclophotocoagulation in the Management of Congenital Glaucoma.

Journal Article (Journal Article)

PURPOSE:To report on the use of endoscopic cyclophotocoagulation (ECP) to treat congenital glaucoma in a triple X female with microphthalmia, dermal aplasia, and sclerocornea (MIDAS) syndrome. OBSERVATIONS:The patient demonstrated linear streaks on the face and neck consistent with dermal aplasia. The corneas were scleralized with ectatic areas of corneal thinning, and the eyes were microphthalmic. Ultrasound biomicroscopy demonstrated congenital aphakia and iris stumps. The patient had elevated intraocular pressure (IOP) that responded to topical glaucoma therapy in the right but not the left eye. Intraoperative endoscopy of the posterior segment revealed multiple hypopigmented chorioretinal lacunae surrounding a pale, cupped optic nerve. ECP of the ciliary processes in the left eye led to marked improvement in IOP. CONCLUSIONS AND IMPORTANCE:Patients with MIDAS syndrome can develop congenital glaucoma secondary to angle dysgenesis. This is the first case report to demonstrate the safe and effective use of ECP to treat elevated IOP in a patient with MIDAS.

Full Text

Duke Authors

Cited Authors

  • Thompson, AC; Thompson, MO; Lim, ME; Freedman, SF; Enyedi, LB

Published Date

  • January 2018

Published In

Volume / Issue

  • 27 / 1

Start / End Page

  • e7 - e10

PubMed ID

  • 29088057

Electronic International Standard Serial Number (EISSN)

  • 1536-481X

International Standard Serial Number (ISSN)

  • 1057-0829

Digital Object Identifier (DOI)

  • 10.1097/ijg.0000000000000812


  • eng