Mouriaux F, Audo I, Defoort-Dhellemmes S, Labalette P, Guilbert F, Constantinides G, Pellerin P
Service d'Ophtalmologie, Hôpital Claude Huriez, CHRU, Lille.
J Fr Ophtalmol. 1997;20(8):583-91.
To better characterize congenital anophthalmos and microphthalmos in order to distinguish which patients need surgical treatment.
A retrospective study of 42 cases with congenital anophthalmos and microphthalmos over a 16 years period was performed. Seven anophthalmos, 20 microphthalmic globes with no associated colobomatous orbital cyst and 15 microphthalmic globes associated with colobomatous orbital cyst were observed. Complete history, pediatrical and ophthalmological examination, electrophysiological feature, oculo-cerebral imagery and karyotype on each of the patients were reviewed.
Among all patients, lack of development of the lids was observed in 45% of cases. In our group of anophthalmos, 100% had micro-orbit. In our group of microphthalmic globes with no associated colobomatous orbital cyst, 30% had micro-orbit and in our group of microphthalmic globes associated with colobomatous orbital cyst, 6% had micro-orbit. 75% of patients had ocular anomalies and 39% had systemic anomalies, mostly on the face. Aetiology were found in 36% of cases. Visual evoked potentials and retinal electric feature were useful to better determine visual function.
Expandable orbital prosthesis would appear to be the most effective therapy for certain cases of anophthalmos and microphthalmos with micro-orbit.
更好地描述先天性无眼球和小眼球，以便区分哪些患者需要手术治疗。
对42例先天性无眼球和小眼球患者进行了为期16年的回顾性研究。观察了7例无眼球、20例无相关眶裂囊肿的小眼球以及15例伴有眶裂囊肿的小眼球。回顾了每位患者的完整病史、儿科和眼科检查、电生理特征、眼脑影像学检查和核型。
在所有患者中，45%的病例观察到眼睑发育不全。在我们的无眼球组中，100%有小眼眶。在我们的无相关眶裂囊肿的小眼球组中，30%有小眼眶，在我们的伴有眶裂囊肿的小眼球组中，6%有小眼眶。75%的患者有眼部异常，39%有全身异常，主要在面部。36%的病例发现了病因。视觉诱发电位和视网膜电特征有助于更好地确定视觉功能。
对于某些伴有小眼眶的先天性无眼球和小眼球病例，可扩张性眼眶假体似乎是最有效的治疗方法。
