Purpose To better-characterize congenital anophthalmos and microphthal
mos in order to distinguish which patients need surgical treatment. Ma
terials and methods A retrospective study of 42 cases with congenital
anophthalmos and microphthalmos over a 16 years period was performed.
Seven anophthalmos, 20 microphthalmic globes with Mo associated colobo
matous orbital cyst and 15 microphthalmic globes associated with colob
omatous orbital cyst were observed. Complete history, pediatricial and
ophthalmological examination, electrophysiological feature, oculo-cer
ebral imagery and karyotype on each of the patients were reviewed. Res
ults Among all patients, lack of development of the lids was observed
in 45% of cases. In our group of anophthalmos, 100% had micro-orbit. I
n our group of microphthalmic globes with no associated colobomatous o
rbital cyst, 30% had micro-orbit and in our group of microphthalmic gl
obes associated with colobomatous orbital cyst, 6% had micro-orbit. 75
% of patients had ocular anomalies and 39% had systemic anomalies, mos
tly on the face. Aetiology were found in 36% of cases. Visual evoked p
otentials and retinal electric feature were useful to better determine
visual function. Conclusion Expandable orbital prothesis would appear
to be the most effective therapy for certain cases of anophtalmos and
microphthalmos with micro-orbit.