Conservative management of congenital microphthalmos with prosthesis

Abstract

Congenital microphthalmos is a rare malformation of the eye resulting from abnormalities in the development of the primary optic vesicle. Treatment for this condition aims to avoid the retardation of orbital growth and cosmetically devastating facial asymmetry. This report details the experiences of patients with microphthalmos treated conservatively with prostheses. The clinical characteristics of congenital microphthalmos were investigated, and the effects of prosthesis treatment on orbital symmetry and cosmetic outcome were analyzed.The medical records of 32 patients were reviewed for the timing of prosthesis treatment, characteristics of the orbits and eyelids, and presence of associated systemic abnormalities. Surveys inquiring about problems experienced during the prosthesis treatment were administered to ocularists and patients’ parents. Current prosthesis size was measured and compared with the primary prosthesis. Orbital symmetry and cosmetic outcome were graded in current photographs of patients.Twenty-six patients (81.3%) had unilateral microphthalmos, and six (18.7%) had bilateral microphthalmos. Mean patient age was 10.7±3.8 years. At initial prosthesis placement, patients demonstrated eyelid asymmetry (n=12), orbital bone hypoplasia (n=9), and zygomaticomaxillary hypoplasia (n=6). Orbital bone hypoplasia was associated with eyelid asymmetry (p=0.038) and zygomaticomaxillary hypoplasia (p<0.001). Patient age at the treatment initiation was significantly greater in patients with zygomaticomaxillary hypoplasia than in patients without hypoplasia (p=0.046). Cosmetic outcome with the prosthesis was fair to good in all patients. However, patients with either orbital bone hypoplasia or zygomaticomaxillary hypoplasia at primary treatment tended to have remaining orbital bone asymmetry after treatment (p<0.001) and to show poorer cosmetic outcomes (p=0.004). Additionally, patients with zygomaticomaxillary hypoplasia were more likely to have an insufficient socket after treatment (p=0.031).The management of congenital microphthalmos is challenging. However, treatment with a prosthesis can be effective for most patients, and early initiation of this treatment is crucial to avoid orbital and facial asymmetry.