녹내장이 발생한 Sturge-Weber 증후군 환자의임상양상과 수술성적
Clinical Features and Surgical Outcomes of Sturge-Weber Syndrome with Glaucoma
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목적: Sturge-Weber 증후군 환자의 안과, 피부과, 신경과적 임상양상과 동반된 녹내장의 수술적 치료의 효과 및 합병증에 대해 알아보고자 하였다. 대상과 방법: Sturge-Weber 증후군에 동반된 녹내장으로 진단받고 섬유주절제술 후 1년 이상 경과관찰이 가능하였던 총 8명 10안을대상으로 안과적, 피부과적, 신경학적 소견과 섬유주절제술 후 수술성공률 및 합병증을 후향적으로 분석하였다. 결과: Sturge-Weber 증후군 환자들의 수술 시 평균 나이는 12.6 ± 13.0세였으며, 수술 후 평균 경과관찰 기간은 71.6 ± 81.8개월이었다. 안면혈관종(8명), 경련(6명), 두개내 병변(6명), 발달 지연(4명), 결막 및 상공막 혈관종(4안) 및 맥락막혈관종(4안) 등 다양한 전신적임상양상이 관찰되었다. 10안 중 8안(80%)이 수술 성공을 보였으며 2안은 안압 상승으로 재수술을 시행하였다. 맥락막혈관종이 수술전 확인되었던 4안 중 2안에서 수술 후 장액망막박리를 동반한 맥락막박리가 발생하였다. 결론: Sturge-Weber 증후군은 전신적 질환의 동반이 많으므로 다각적인 협진이 필요하며 동반된 녹내장의 치료로 섬유주절제술은효과적이고 비교적 안전한 수술방법으로 생각한다. 섬유주절제술 후 장액망막박리의 위험을 고려하여, 수술 전 정밀한 안저검사를통해 맥락막혈관종의 존재여부를 확인해야 한다.
Abstract
Purpose: To report clinical manifestations including neurocutaneous and ocular findings and to evaluate outcomes of trabeculectomy in patients with Sturge-Weber syndrome. Methods: The medical records of 10 eyes of 8 glaucoma patients with Sturge-Weber syndrome who were followed up for at least 1 year after trabeculectomy were reviewed retrospectively. We analyzed neurocutaneous and ocular findings, cumulative surgical success rates, and complications in patients with Sturge-Weber syndrome. Results: The mean patient age at the time of surgery was 12.6 ± 13.0 years and mean follow-up period was 71.6 ± 81.8months. All patients showed various clinical findings including facial hemangioma (8 patients), seizure (6 patients), intracranial lesion (6 patients), developmental delay (4 patients), conjunctival/episcleral hemangioma (4 eyes), and choroidal hemangioma (4 eyes). Postoperative success was achieved in 8 out of 10 eyes (80.0%). Postoperatively, serous retinal detachment occurred in 2 out of 4 eyes with preoperative diffuse choroidal hemangioma. Conclusions: Management of glaucoma associated with Sturge-Weber syndrome requires multidisciplinary treatment because of systemic involvement. Trabeculectomy appears to be an effective and relatively safe surgical option for glaucoma associated with Sturge-Weber syndrome. However, serious complications such as serous retinal detachment should be considered when planning trabeculectomy for patients with diffuse choroidal hemangioma.
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