View 2019 Abstracts

Our study describes the natural history and the management course of the largest case series of patients with microphthalmia associated cysts. We provide recommendations for timing and indications for surgical treatment based on our outcomes.

Retrospective case series of patients attending the Ocularistica Italiana, Rome over a twenty-year period. Patients were identified from a clinical database. Medical records, clinical photographs and MRI scans were utilized to assess the cosmetic and anatomical outcomes of the management of microphthalmia with cyst.

68 patients (71 sockets with microphthalmia associated cysts) presented to a specialist microphthalmia-anophthalmia clinic in Rome over a 20-year period. The median age of presentation is 1 year old (range 9 days – 30 years). 50% of patients were under the age of 1 year at presentation.

The cysts were either detected clinically or were diagnosed on the baseline MRI. They were located in the inferior quadrant (49%), posteriorly (15%), and in the superior quadrant (13%). A deviated microphthalmic eye usually heralded the presence of a cyst.
55% of microphthalmic cysts were removed surgically. The principle reasons for surgical intervention were 1) prosthetic shells no longer remained centralized or stable. 2) Inability to fit a prosthetic 3) distortion of the socket, conjunctival tissues and lid. Four sockets had primary placement of a ball implant at the time of cyst excision. However all these patients required subsequent removal of the implant because of its resultant eccentric position. Five dermis fat grafts were used to replace the orbital volume as secondary procedures at least six months after cyst excision to allow the orbital tissues to settle.
The 32 cysts, which were left in situ, did not impede satisfactory prosthetic fitting and provided orbital volume. Six were seen to spontaneously resolve or contract with time leading to orbital volume loss. The mean follow up was 7.5 years (1 month – 28 years).

Cysts associated with microphthalmia may present clinically or be detected on imaging but they do provide useful volume enhancement for a growing orbit. However, we do recommend removal if it impedes the cosmetic rehabilitation of the socket and periocular tissues. Around half the patients in this series required removal of cyst. We found that the secondary placement of dermis fat graft to replace orbital volume following excision of the cysts provides the best, stable reconstruction for the socket and support for the optimal cosmetic prosthesis.