Advanced Eye Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Rom J Ophthalmol. 2022 Apr-Jun;66(2):125-131. doi: 10.22336/rjo.2022.25.
To report the cases of five children with unilateral advanced glaucoma in isolated congenital ectropion uveae (CEU) with ipsilateral ptosis and myopia. This is an ambispective observational case series. After diagnosing one patient with CEU and glaucoma, consecutive patients presenting with unilateral ptosis, congenital iris anomaly, and glaucoma between 2014 to 2020, and had completed a minimum one-year postoperative follow-up, were analyzed. Of the 1421 newly registered pediatric glaucoma patients in the period under review, five children were diagnosed with CEU. All patients presented with gradual painless diminution of vision in the left eye in early adolescence. The left eye of all patients had peculiar clinical features: mild congenital ptosis, high iris insertion, crypt-less smooth iris surface, congenital ectropion uveae, pigments over anterior lens capsule, high myopia, advanced glaucomatous optic disc cupping, and very high intraocular pressure (IOP), which was > 45 mmHg in all cases. The right eye showed signs of angle dysgenesis with mild anterior iris insertion and numerous fine iris processes. Antiglaucoma medications and angle surgery failed to control the IOP, and all children required glaucoma filtration surgery, resulting in reasonable IOP control. Despite the older age, postoperative strict amblyopia treatment resulted in significant improvement in vision. Although ectropion uveae and ptosis have been present since birth, unilaterality, and the asymptomatic nature of the disease led to the late presentation with irreversible damage. Early surgical management and amblyopia therapy are the cornerstones of management. CEU = Congenital ectropion uvea, CIES = Congenital Iris Ectropion Syndrome, ASD = Anterior segment dysgenesis syndrome, BCVA = Best-corrected visual acuity, IOP = Intraocular pressure.
报告 5 例孤立性先天性葡萄膜外翻伴同侧上睑下垂和近视的单侧进行性青光眼患儿的病例。这是一项前瞻性观察性病例系列研究。在诊断出 1 例 CEU 合并青光眼患者后，对 2014 年至 2020 年间连续出现单侧上睑下垂、先天性虹膜异常和青光眼、并完成至少 1 年术后随访的患者进行了分析。在回顾期内新登记的 1421 例儿童青光眼患者中，有 5 例被诊断为 CEU。所有患者均在青少年早期出现左眼无痛性进行性视力下降。所有患者的左眼均具有独特的临床特征：轻度先天性上睑下垂、虹膜高位插入、无虹膜陷窝、先天性葡萄膜外翻、前晶状体囊色素沉着、高度近视、晚期青光眼性视盘凹陷和极高的眼压（IOP），所有病例均>45mmHg。右眼表现为前房角发育不良，虹膜轻度插入和大量细小虹膜嵴。抗青光眼药物和前房角手术均未能控制眼压，所有患儿均需行青光眼滤过手术，从而使眼压得到合理控制。尽管年龄较大，但术后严格的弱视治疗使视力显著提高。尽管葡萄膜外翻和上睑下垂自出生即存在，但由于疾病的单侧性和无症状性，导致就诊较晚，出现不可逆转的损害。早期手术治疗和弱视治疗是治疗的基石。CEU=先天性葡萄膜外翻，CIES=先天性虹膜外翻综合征，ASD=前节发育不良综合征，BCVA=最佳矫正视力，IOP=眼压。
