목적: 안뇌신증후군 환자에서 선천백내장의 임상 양상 및 수술 결과를 알아보고자 하였다. 대상과 방법: 안뇌신증후군과 연관된 선천백내장으로 진단 받고 수술을 시행 받은 후 1년 이상 추적 관찰이 가능했던 10명 20안에 대해 후향적 의무기록 분석을 통해 나이, 성별, 백내장의 종류, 수술 방법 및 시기, 수술 전후 시력 및 수술 후 안압, 동반된 안과 질환 등을 조사하였다. 결과: 10명의 환자는 모두 남자였고 초진 시 양안에 선천백내장이 있었다. 백내장 진단 시 평균 연령은 2.5개월(2.5±1.4)이었고 평균 4.9년(4.9±4.2)동안 추적 관찰하였다. 백내장의 형태는 9명 18안에서 핵백내장이었고, 1명 2안에서 피질백내장이었다. 대상 안 모두에 서 생후 1년 이내에 양안 수정체관류흡입술, 후낭절제술, 앞유리체절제술을 시행하였다. 이 중에서 9명 18안에서 생후 6개월 이내에 수술을 시행하였고, 3명 6안에서 생후 2개월 이내에 수술을 시행하였다. 또한 3명 6안에서 이차적 인공수정체삽입술을 생후 평균 34개월 (34±13.2)에 시행하였다. 수술 후 최대교정시력은 광각유부터 0.15까지 측정되었다. 생후 6개월 이전에 수술한 9명 18안의 수술 후 시력 은 1명을 제외하고는 중등도의 고정주시 및 따라보기 이상이었고, 각각 생후 9개월 및 12개월에 수술한 1명 2안의 수술 후 시력은 안전수동이었다. 또한 백내장 이외에 2명 3안에서 녹내장, 1명 2안에서 각막혼탁 그리고 3명에서 사시가 발생하였다. 결론: 안뇌신증후군 환자에서 선천백내장 수술을 한 경우는 전반적으로 시력 개선 정도가 낮았고, 특히 진단 및 수술이 늦었던 환자에서는 시력 개선 정도가 더욱 낮았다. 따라서 선천백내장의 최대한 빠른 진단 및 수술이 필요하다. 또한 녹내장, 각막혼탁, 사시 등의 안과 질환이 동반될 수 있으므로 이에 대한 지속적인 경과 관찰이 중요하다.

Purpose: To describe the clinical features and surgical outcomes of congenital cataracts in patients with oculocerebrorenal syndrome. Methods: We retrospectively analyzed the age, sex, types of cataracts, operation method and time, perioperative visual acuity, postoperative intraocular pressure, and associated ocular disease in 10 patients (20 eyes) that had been diagnosed with congenital cataracts associated with oculocerebrorenal syndrome and undergone cataract surgery with a minimum follow-up period of 1 year. Results: All 10 patients were male and had bilateral cataracts detected on their first full ophthalmic examination. The mean age at diagnosis was 2.5 (2.5±1.4) months, and the mean duration of the follow-up period was 4.9 (4.9±4.2) years. The types of cataracts were nuclear sclerosis in 18 eyes of nine patients and cortical opacity in two eyes of one patient. All patients underwent irrigation and aspiration of the lenses, posterior capsulectomy and anterior vitrectomy in both eyes before the age of 1 year. Nine patients (18 eyes) underwent cataract extraction before the age of 6 months, and three patients (6 eyes) underwent the surgery before the age of 2 months. Secondary intraocular lens implantation was done in six eyes of three patients at the age of 34 (34±13.2) months. Postoperative best corrected visual acuity ranged from light perception to 0.15. Out of the nine patients who underwent cataract extraction before the age of 6 months, seven patients could fix and follow objects moderately. The visual acuity of patient who underwent cataract extraction of each eye at the age of 9 months and 12 months was hand motion. In addition to cataracts, glaucoma occurred in three eyes of two patients, corneal opacity in two eyes of one patient and strabismus in three patients. Conclusions: Visual acuity after cataract surgery was poor in patients with oculocerebrorenal syndrome, especially when the diagnosis and surgery was delayed. Earlier identification and surgical removal of cataracts is recommended. Patients should be monitored regularly for other ocular diseases such as glaucoma, corneal opacity and strabismus.

Purpose: To describe the clinical features and surgical outcomes of congenital cataracts in patients with oculocerebrorenal syndrome. Methods: We retrospectively analyzed the age, sex, types of cataracts, operation method and time, perioperative visual acuity, postoperative intraocular pressure, and associated ocular disease in 10 patients (20 eyes) that had been diagnosed with congenital cataracts associated with oculocerebrorenal syndrome and undergone cataract surgery with a minimum follow-up period of 1 year. Results: All 10 patients were male and had bilateral cataracts detected on their first full ophthalmic examination. The mean age at diagnosis was 2.5 (2.5±1.4) months, and the mean duration of the follow-up period was 4.9 (4.9±4.2) years. The types of cataracts were nuclear sclerosis in 18 eyes of nine patients and cortical opacity in two eyes of one patient. All patients underwent irrigation and aspiration of the lenses, posterior capsulectomy and anterior vitrectomy in both eyes before the age of 1 year. Nine patients (18 eyes) underwent cataract extraction before the age of 6 months, and three patients (6 eyes) underwent the surgery before the age of 2 months. Secondary intraocular lens implantation was done in six eyes of three patients at the age of 34 (34±13.2) months. Postoperative best corrected visual acuity ranged from light perception to 0.15. Out of the nine patients who underwent cataract extraction before the age of 6 months, seven patients could fix and follow objects moderately. The visual acuity of patient who underwent cataract extraction of each eye at the age of 9 months and 12 months was hand motion. In addition to cataracts, glaucoma occurred in three eyes of two patients, corneal opacity in two eyes of one patient and strabismus in three patients. Conclusions: Visual acuity after cataract surgery was poor in patients with oculocerebrorenal syndrome, especially when the diagnosis and surgery was delayed. Earlier identification and surgical removal of cataracts is recommended. Patients should be monitored regularly for other ocular diseases such as glaucoma, corneal opacity and strabismus.