Pathology and Management of Orbital Cysts seen in Microphthalmos

Abstract

 

Purpose:

 

Microphthalmos with orbital cyst (MOC) represents a rare colobomatous disorder due to failure of invagination of the optic vesicle in the early gestational period. We report the presentation, radiography, histology, and treatment outcomes in 5 patients with MOC.

 

Methods:

 

Records of 5 patients evaluated for MOC were reviewed.

 

Results:

 

4 patients were male and 1 female with a median age of 8.3 years (range 2 months - 40 years). 4 patients presented with original disease, while 1 patient had recurrence after cyst drainage at an outside institution. The diagnosis in all patients was confirmed by exam, orbital CT/MRI/US, and histologic evaluation following orbitotomy and surgical excision in all cases except one lost to follow-up (Fig 1, 2). Reconstruction of the resultant anophthalmic socket included dermis fat grafting in children and alloplastic orbital implant in adults.

 

Conclusions:

 

MOC is a rare childhood diagnosis with a mean age at presentation of 5.4 years. Orbital imaging through CT or MRI is crucial to establish the diagnosis. The treatment depends on the severity of presentation, and ranges from observation or fitting with a cosmetic scleral shell for mild cases, to enucleation with cyst excision and orbital reconstruction in more severe cases. Reconstruction of the anophthalmic socket in children should attempt to maximize orbital volume either with a dermis fat graft or largest orbital implant that can safely be positioned without risk of extrusion. Cyst drainage should not be a definitive treatment and may lead to recurrence of ocular signs and symptoms as was the case in one of our patients.  

 

Keywords: orbit • development • pathology: human