Case report

Congenital cystic eye with multiple dermal appendages: a case report

Pankaj Gupta^{* 1} , Krishna Pal Singh Malik^{* 2} and Ruchi Goel^{* 3}

¹Senior Resident, Department of Ophthalmology Vardhman Mahavir Medical College New Delhi 110029 India

²Consultant and Head, Department of Ophthalmology Vardhman Mahavir Medical College New Delhi 110029 India

³Senior Resident, Department of Ophthalmology Vardhman Mahavir Medical College New Delhi 110029 India

author email corresponding author email* Contributed equally

BMC Ophthalmology 2003, 3:7doi:10.1186/1471-2415-3-7

Published:	3 July 2003

Abstract

Background

A partial or complete failure in the involution of the primary optic vesicle resulting in the formation of a cyst is an extremely rare anomaly known as congenital cystic eye. The primary optic vesicle is formed but instead of the anterior part of the vesicle involuting to lie in apposition with the posterior part, a cyst persists at birth and replaces the eye.

Case Presentation

We report a case of congenital cystic eye associated with multiple dermal appendages in a 1-day-old female child. This condition presented at birth as a large orbital mass in the left orbit that bulged forwards and stretched the eyelids. No globe or any other ocular structures were identified in the orbit. Multiple dermal appendages were present in the adjacent part of the face below the left orbit and on the upper part of the neck.

Conclusions

Congenital cystic eye is an extremely rare condition and with only 28 previous cases reported in the literature. We present the second case of congenital cystic eye with multiple dermal appendages of the face and neck.