Congenital ectropion uveae (CEU) is a rare, nonprogressive anomaly characterized by the proliferation of the iris pigment epithelium on the anterior surface of the iris, often associated with glaucoma. Due to its rarity and complexity, standardized glaucoma surgical management is limited. To our knowledge, the application of glaucoma drainage devices in CEU is rarely documented. Here, we report a case of Ahmed glaucoma valve (AGV) implantation for unilateral CEU associated with glaucoma. A 26-year-old female initially presented with blunt ocular trauma with an intraocular pressure (IOP) of 37 mm Hg in the right eye. After starting glaucoma medication, IOP promptly decreased to 21 mm Hg. However, the patient was subsequently lost to follow-up. A definitive diagnosis was not made due to the limited understanding of CEU at the first visit. Nearly 3 years later, the patient was referred to our hospital again with decreased vision for 6 months and mild distending pain in the right eye. The best-corrected visual acuity was 20/25, IOP was 51 mm Hg, and the cup-to-disc ratio was 0.8. Slit-lamp examination of the right eye revealed 360° ectropion uveae, extending around the pupil to the mid-periphery of the iris, which was an unaltered condition since the first visit. Thus, the patient was diagnosed with CEU and unilateral glaucoma. The AGV implantation surgery with mitomycin C was performed in the right eye. The best-corrected visual acuity of the right eye improved to 20/20. IOP stabilized without medications during the entire period of follow-up for 3 years. Although CEU is rare, ophthalmologists should remain vigilant to avoid missed diagnoses due to its high association with glaucoma. AGV implantation with mitomycin C may be considered an effective surgical management for adult patients with late-onset glaucoma secondary to CEU.

Anterior segment dysgenesis (ASD) encompasses a range of congenital disorders affecting the development of the eye's anterior segment, often leading to significant visual impairment and glaucoma. Although numerous studies have focused on the genetic basis of ASD, few have comprehensively compared the clinical features across ASD subtypes. This study aims to address this gap by describing and comparing the clinical characteristics and ultrasound biomicroscopy (UBM) imaging features of various ASD subtypes in a Chinese tertiary medical center. A retrospective review of medical records was conducted for hospitalized patients diagnosed with ASD in the Eye & ENT Hospital, Fudan University between January 2013 and December 2022. Patients were enrolled consecutively. Demographic data, clinical manifestations, and UBM images of various ASD subtypes were analyzed. A total of 1,031 patients were included in the analysis. Primary congenital glaucoma (PCG) was the most prevalent, affecting 396 patients (625 eyes), whereas congenital ectropion uveae (CEU) was the rarest, affecting 8 patients (13 eyes). All subtypes exhibited a spectrum of characteristic clinical manifestations. UBM imaging revealed distinct anterior segment abnormalities characteristic of each subtype. Trabeculodysgenesis was implicated in glaucoma pathogenesis across multiple ASDs, including PCG, aniridia, Axenfeld-Rieger syndrome, Peters anomaly, CEU, iris coloboma, and microcornea, whereas persistent fetal vasculature and congenital fibrovascular pupillary membrane exhibited completely closed angles in glaucomatous eyes. Our study provides comprehensive insights into the clinical features and UBM characteristics of ASD subtypes, enhancing diagnostic accuracy and guiding appropriate interventions.

Objective: To investigate the clinical characteristics and treatment outcomes of glaucoma secondary to congenital ectropion uveae (CEU) using penetrating Schlemm's canaloplasty. Methods: This was a retrospective case series study. Medical records of patients diagnosed with glaucoma secondary to CEU and undergoing penetrating Schlemm's canaloplasty at the Eye Hospital of Wenzhou Medical University between August 2020 and December 2021 were collected. Clinical characteristics including the extent and location of iris ectropion, type of glaucoma, were analyzed. Follow-up visits were conducted at 1, 3, 6 months, and 1 year postoperatively. Visual acuity, intraocular pressure (IOP), anterior segment and fundus condition, filtering bleb morphology, use of IOP-lowering medications, ultrasound biomicroscopy results, and other indicators were analyzed to summarize surgical outcomes. Results: Six cases (6 eyes) of glaucoma secondary to CEU were included, all unilateral, with 3 left eyes and 3 right eyes; median age was 10.0 (5.3, 28.8) years; including 3 males and 3 females. Preoperative IOP was (31.7±10.0) mmHg (1 mmHg=0.133 kPa), and the preoperative number of IOP-lowering medications used was 2.0 (2.0, 3.2). The extent of iris ectropion in the 6 cases ranged from 270 ° to 360 °, with peripheral anterior synechiae corresponding to the location of iris ectropion, and angle closure with the degree of synechiae extending beyond Schwalbe's line. No surgical complications occurred in any of the 6 cases postoperatively. At 1 month postoperatively, the IOP was (16.4±3.2) mmHg, with a median of 0.0 (0.0, 1.5) medications used. At 3 months postoperatively, the IOP was (14.8±6.0) mmHg, with a median of 0.0 (0.0, 2.2) medications used. At 6 months postoperatively, the IOP was (18.1±6.1) mmHg, with a median of 0.0 (0.0, 0.5) medications used. Among them, 5 patients had a follow-up period of 1 year postoperatively, all achieving controlled IOP without the use of IOP-lowering medications, with an average IOP of (15.5±3.1) mmHg. No obvious filtering bleb formation was observed at the surgical site in all patients. Conclusions: Glaucoma secondary to CEU manifests primarily as closed-angle glaucoma, with a correspondence between the closure range of anterior iris adhesions in the angle and the extent of iris ectropion. Penetrating Schlemm's canaloplasty demonstrates favorable and stable efficacy for its treatment. 目的： 探讨先天性葡萄膜外翻继发性青光眼的临床特征和采用穿透性Schlemm管成形术治疗的效果。 方法： 回顾性病例系列研究。收集2020年8月至2021年12月于温州医科大学附属眼视光医院确诊为先天性葡萄膜外翻继发性青光眼且行穿透性Schlemm管成形术患者的病历资料，分析虹膜外翻范围和位置、青光眼类型等临床特征，于术后1、3、6个月和1年随访，分析患者的视力、眼压、眼前节和眼底情况、滤过泡形态、降眼压药物使用情况、超声活体显微镜检查结果等指标，总结手术效果。 结果： 纳入先天性葡萄膜外翻继发性青光眼患者6例（6只眼），均为单眼患病，左眼和右眼各3只；年龄为10.0（5.3，28.8）岁；其中男性3例，女性3例；术前眼压为（31.7±10.0）mmHg（1 mmHg=0.133 kPa），术前降眼压药物使用数量为2.0（2.0，3.2）种。6例患者虹膜外翻范围为270~360 °，周边虹膜前粘连范围与虹膜外翻位置基本相同，房角关闭且房角粘连程度超过Schwalbe线。6例患者术后均未出现手术相关并发症，术后1个月眼压为（16.4±3.2）mmHg，降眼压药物使用数量为0.0（0.0，1.5）种；术后3个月眼压为（14.8±6.0）mmHg，降眼压药物使用数量为0.0（0.0，2.2）种；术后6个月眼压为（18.1±6.1）mmHg，降眼压药物使用数量为0.0（0.0，0.5）种。其中5例患者术后随访时间满1年，均在未使用降眼压药物情况下，眼压得到控制［（15.5±3.1）mmHg］。所有患者患眼手术部位均未见明显滤过泡形成。 结论： 先天性葡萄膜外翻继发性青光眼表现为闭角型青光眼的临床特征，房角虹膜前粘连关闭范围与虹膜外翻范围存在对应关系，类型主要属于闭角型青光眼。穿透性Schlemm管成形术对其治疗的效果较好且稳定。.

Objective: To analyze the disease composition and primary surgical procedures in pediatric inpatients with secondary glaucoma. Methods: A retrospective case series study was conducted. Clinical data of children aged≤16 years with secondary glaucoma who were admitted to the Zhongshan Ophthalmic Center, Sun Yat-sen University, between January 1, 2017, and December 31, 2021, were included. The patients were classified according to the Childhood Glaucoma Research Network (CGRN) classification system, and their diagnoses, underlying factors, gender, age of onset, affected eye(s), age and type of initial surgery, and ophthalmic examination data were analyzed. Statistical analysis was performed using Kruskal-Wallis rank sum test and χ2 test. Results: A total of 540 patients (744 eyes) were included in this study, comprising 319 males (59.1%) and 221 females (40.9%). Unilateral disease was observed in 336 cases (62.2%), while bilateral involvement was present in 204 cases (37.8%). The age of onset was 4.0 (0.0, 9.0) years, and the median age of the first anti-glaucoma surgery was 5.0 (0.7, 10.0) years. Among them, there were 195 cases (36.1%) of secondary glaucoma associated with non-acquired ocular anomalies (SCG-O), with a median age of onset of 0.0 (0.0, 4.0) years, and 97 of these cases (49.7%) were male. secondary glaucoma associated with non-acquired systemic disease or syndrome (SCG-S) were observed in 68 cases (12.6%), with a median age of glaucoma onset of 0.1 (0.0, 4.0) years, and 47 of these cases (69.1%) were male. Secondary glaucoma associated with acquired conditions (SCG-A) accounted for 192 cases (35.6%), with a median age of onset of 9.0 (5.0, 13.0) years, and 125 of these cases (65.1%) were male. There were 85 cases (15.7%) of secondary glaucoma following cataract surgery (SCG-C), with a median age of onset of 3.0 (0.8, 7.0) years, and 50 of these cases (58.8%) were male. Male patients were predominant in SCG-S and SCG-A, with 47 cases (69.1%) and 125 cases (65.1%), respectively (χ2=9.94, 17.52; P=0.002,<0.001). Except for SCG-O, all other types of pediatric secondary glaucoma predominantly affected only one eye: SCG-S in 52 cases (76.5%), SCG-A in 128 cases (66.7%), and SCG-C in 54 cases (63.5%) (χ2=19.06, 21.33, 6.22; all P<0.05). The highest proportion of SCG-O was attributed to congenital ectropion uveae (46 cases, 23.6%). Sturge-Weber syndrome was the most common SCG-S (45 cases, 66.3%), while SCG-A mostly resulted from trauma (59 cases, 30.8%) and corticosteroid use (56 cases, 29.2%). Trabeculectomy (211 eyes, 30.8%) and glaucoma drainage device implantation (197 eyes, 28.7%) were the most frequently performed primary surgical procedures. Conclusions: SCG-O and SCG-A were found to be common types of pediatric secondary glaucoma. The age of onset and the choice of primary anti-glaucoma surgical procedures varied among different types of pediatric secondary glaucoma. However, overall, trabeculectomy and glaucoma drainage device implantation were the primary surgical procedures predominantly employed. 目的： 分析儿童继发性青光眼住院患者疾病类型构成及首次手术方式。 方法： 回顾性病例系列研究。纳入2017年1月1日至2021年12月31日于中山大学中山眼科中心住院治疗的16岁及以下儿童继发性青光眼患者的临床资料，依据儿童青光眼研究网络（CGRN）分类系统分类，并分析其诊断及继发因素、性别、发病年龄、受累眼别、首次手术年龄及类型、眼科检查资料。采用Kruskal-Wallis秩和检验和χ2检验进行统计学分析。 结果： 共纳入540例（744只眼）患者，其中男性319例（59.1%），女性221例（40.9%）。单眼发病336例（62.2%），双眼发病204例（37.8%）。发病年龄为4.0（0.0，9.0）岁，首次行抗青光眼手术的年龄为5.0（0.7，10.0）岁。其中非获得性眼部异常继发性青光眼195例（36.1%），中位发病年龄0.0（0.0，4.0）岁，男性97例（49.7%）；非获得性全身疾病或综合征继发性青光眼68例（12.6%），中位青光眼发病年龄0.1（0.0，4.0）岁，男性47例（69.1%）；获得性疾病继发性青光眼192例（35.6%），中位发病年龄9.0（5.0，13.0）岁，男性125例（65.1%）；白内障摘除手术后继发性青光眼占85例（15.7%），中位发病年龄3.0（0.8，7.0）岁，男性50例（58.8%）。非获得性全身疾病或综合征继发性青光眼和获得性疾病继发性青光眼以男性患者为主，分别为47例（69.1%）和125例（65.1%）（χ2=9.94，17.52；P=0.002，<0.001）。除非获得性眼部异常继发性青光眼外，其余类型的儿童继发性青光眼均以单眼发病为主：非获得性全身疾病或综合征继发性青光眼为52例（76.5%），获得性疾病继发性青光眼为128例（66.7%），白内障摘除手术后继发性青光眼为54例（63.5%）（χ2=19.06，21.33，6.22；均P<0.05）。非获得性眼部异常继发性青光眼以先天性葡萄膜外翻占比最高［46例（23.6%）］；非获得性全身疾病或综合征继发性青光眼以斯德奇-韦伯综合征多见［45例（66.3%）］；获得性疾病继发性青光眼多继发于外伤［59例（30.8%）］和使用糖皮质激素［56例（29.2%）］。小梁切除术［211只眼（30.8%）］和青光眼引流阀植入术［197只眼（28.7%）］为占比最高的首次手术方式。 结论： 儿童继发性青光眼中以非获得性眼部异常继发性青光眼和获得性疾病继发性青光眼为常见的类型；不同类型的儿童继发性青光眼发病年龄以及主要首次抗青光眼术式选择有所不同，但整体上以小梁切除术和青光眼引流阀植入术为主。. Iris ectropion syndrome (ectropion iridis or ectropion uveae) is characterized by ectropion or the presence of the pigmented epithelium of the iris on the anterior surface of the iris. Iris ectropion syndrome can be congenital or acquired. Congenital ectropion uveae (CEU, or primary iris pigment epithelial hyperplasia) was first reported by Colsman in 1869. However, he was actually describing iris flocculi. Iris flocculi are congenital smooth cyst-like benign structures arising from the pigmented epithelium at the pupillary margin.  Boleslaw Wicherkiewicz, a Polish ophthalmologist in 1891 and Spiro in 1896, are credited with reporting iris ectropion syndrome. Congenital iris ectropion is usually unilateral and nonprogressive, though bilateral cases have been reported. Progressive open-angle glaucoma may be associated in some cases due to the dysgenesis of the angle of the anterior chamber. The posterior pigment epithelium of the iris is found on the anterior stroma at birth. The iris surface is glassy smooth and devoid of crypts. The pupil is usually round and reactive. Other features are anterior insertion of the root of the iris and trabecular dysgenesis. The sphincter muscle of the iris and the stroma are not affected. Developmental glaucoma is a frequent association. Mild to moderate ptosis with good levator function may be seen. Neurofibromatosis, Prader-Willi syndrome, facial hemihypertrophy, prominent corneal nerves, asthma, dental problems (late-onset), and Rieger anomaly may be associated with congenital iris ectropion. Acquired iris ectropion can occur in many conditions and is more common than congenital ectropion uveae. Fibrovascular membrane formation on the anterior surface of the iris causes tractional forces pulling the posterior pigment epithelium of the iris to the anterior surface. This is associated with neovascularization of the iris. This membrane can form due to proliferative diabetic retinopathy, venous occlusions, and ischemic, inflammatory, and neoplastic etiologies. Unlike congenital ectropion uveae, acquired ectropion is progressive unless the underlying causes are treated.

Congenital ectropion uveae (CEU) is a rare anomaly of the embryonic development of the anterior segment of the eye. We report the case of a 5-year-old child with an undiagnosed CEU who was treated urgently for an acute angle closure attack. A 5-year-old child was referred urgently for evaluation of anisocoria with mydriasis of the right eye and severe headache. Brain imaging with contrast injection was initially performed in the pediatric emergency department and ruled out central nervous system pathology. The initial examination of the right eye revealed an intraocular pressure (IOP) of 37mmHg, corneal edema, congenital ectropion uveae, mydriasis with pupillary block, a closed angle on gonioscopy, and a clear lens. The examination of the left eye was unremarkable, with no visible CEU. The initial management consisted of medical treatment with topical glaucoma drops and miotics and acetazolamide at 10mg/kg/d. Re-evaluation under general anesthesia showed persistent mydriasis and no resolution of the pupillary block. Filtering surgery was performed in the absence of a complete response to medical treatment, allowing control of IOP without drops and complete regression of the corneal edema. CEU is a rare malformation, and pressure complications represent an insignificant proportion of pediatric glaucoma cases. The acute presentation of acute angle closure in this potentially blinding short-term setting, however, makes detection and management difficult in very young children in a great deal of pain. Only one similar case has been reported in the pediatric literature. Acute angle closure complicating CEU is exceptional and difficult to diagnose in a pediatric context. Parents of children with this predisposing condition should be informed of the need to consult urgently when clinical signs of elevated intraocular pressure appear.