方法：回顾性分析2000-01/2012-12在天津医科大学眼科中心接受治疗的先天性白内障合并小角膜患者11例18眼的临床资料，术前检查包括角膜直径(<9.5mm)、眼部异常和全身异常，术后随访至少1a，评价术后视力和并发症等。

结果：患者11例中有先天性白内障家族史4例，18眼中合并虹膜缺损6眼，眼球震颤18眼，原始玻璃体动脉残留1眼。对11例18眼患者施行三种手术方案：(1)行囊外白内障摘除术(extra capsular extraction of cataract， ECCE)者5眼；(2)Ⅰ期行ECCE，Ⅱ期行人工晶状体植入术(posterior chamber intraocular lens，PC-IOL)者4眼；(3)Ⅰ期行ECCE联合PC-IOL植入者9眼。术后患者视力均有明显改善，18眼术后最佳矫正视力0.145±0.0958(0.01～0.20)。ECCE术中并发症主要为后囊破裂1眼，术后并发症主要为后发性白内障10眼，继发性青光眼2眼。

结论：先天性白内障合并小角膜常伴发虹膜、脉络膜缺损，眼球震颤等合并症，人工晶状体的植入能明显提高患者视力，但术中应注重防范角膜损伤、后囊破裂等并发症，术后加强管理，特别是眼压的监测，以极早防治青光眼等并发症。

METHODS: The clinical materials of 18 eyes of 11 patients with microcorneas who underwent surgery for congenital cataracts from January 2000 to December 2012 were retrospectively analyzed. Preoperative examination including corneal diameter(<9.5mm), ocular abnormalities and systemic anomalies. The visual acuity and various complications were followed up for 1a.

RESULTS: The morphologic types of cataract were family history of congenital cataract(4 cases)among 11 patients. Other preoperative ocular abnormalities included aniridia(6 eyes), nystagmus(18 eyes)and primary vitreous artery residues(1 eye). Totally 11 patients(18 eyes)had been performed the following three different types of operation: 1)extra capsular extraction of cataract(ECCE)was performed in 5 eyes; 2)ECCE for the first step and posterior chamber intraocular lens(PC-IOL)for the second step was performed in 4 eyes; 3)ECCE and PC-IOL was performed in 9 eyes. Visual acuity was achieved after surgery, the best-corrected visual acuity(BCVA)was 0.145±0.0958(0.01-0.20). ECCE intraoperative complication was posterior capsule rupture(1 eye). The main postoperative complications were posterior capsular opacification(10 eyes)and secondary glaucoma(2 eyes).

CONCLUSION: The main complication in congenital cataracts with microcornea including aniridia, choroidal defect and nystagmus. IOL implantation can significantly improve visual acuity. Patients should pay attention to prevention corneal injury, posterior capsular rupture and etc. However, adequate management of postoperative complications, especially posterior capsular opacification and glaucoma is required.