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Successful Trabeculectomy for Childhood Glaucoma Associated with Atypical Sturge-Weber Syndrome and Turner's Syndrome

小樑網切除術成功應用於不典型Sturge-Weber併Turner's症候群之兒童青光眼病人

摘要


目的:報告一右眼青光眼個案合併有Sturge-Weberl乏Tumer嗡症候群經小樑網切除術治療。 方法:病例報告。 結果:一位8歲的女孩自幼因為右臉酒紅色斑被診斷為Sturge-Weber。近一年來,她因為右眼視力模糊及間歇性頭痛來就診。初次檢查右眼最佳矯正視力為6/10,左眼為6/6.7。右眼眼壓為39mmHg,左眼為2lmmHg。視神經凹陷與視盤比,右眼為0.6(上標 *)0.7,左眼為0.3(上標 *)0.3。弘快速視野檢查顯示右眼有一中心旁視野缺損。由於該病人身材矮小,因此我們安排了染色體檢查,該檢查顯示病人罹患Turner's症候群。病人因為右眼青光眼而接受小樑網切除術。術後門診追蹤,病人眼壓控制良好。 結論:我們提出此一個案匐跑童青光眼合併有不典型Sturge-Weber及Tumer'症候群。在這樣的案例中,早期小樑網切除術對於減低弱視和視覺功能喪失的危險性顯得格外重要。

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並列摘要


Purpose: To present a case of Sturge-Weber syndrome combined with Turner's syndrome undergoing trabeculectomy for glaucoma in the right eye. Methods: A case report. Results: A 8-year-old female was diagnosed as Sturge-Weber syndrome since childhood due to port-wine stain over the right face. She presented to our clinic with 1-year history of diminished vision in the right eye and intermittent headache. The initial visual acuity was 6/10 in the right eye and 6/6.7 in the left eye. The intraocular pressure (IOP) was 39 mmHg in the right eye and 21 mmHg in the left eye. The cup-disc ratio was 0.6×0.7 in the right eye and 0.3×0.3 in the left eye. The frequency doubling technology (FDT) perimeter revealed a paracentral visual field defect in the right eye. Due to short stature of the patient, we also arranged chromosomal examination and the result pointed to Turner's syndrome. The patient underwent trabeculectomy in the right eye under the impression of glaucoma. She was later followed-up at the outpatient department with smooth post operative course. Conclusion: We presented a case of childhood glaucoma associated with atypical Sturge-Weber syndrome and Turner’s syndrome. Early trabeculectomy procedure is important in reducing the risk of amblyopia and visual function loss in such case.

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