先天性葡萄膜外翻

先天性葡萄膜外翻是一种罕见的眼科疾病,以虹膜基质前表面存在虹膜色素上皮、房角发育异常、进行性青光眼为特征.一般为非进行性,最主要的并发症是发展为先天性或青少年型青光眼.此病与神经嵴发育异常相关,可与某些遗传病伴发.较为特殊的临床表现是环绕瞳孔的虹膜色素膜样外翻,终止于虹膜的中周部.边界清晰,外翻的程度各方向不尽相同.几乎所有患者在确诊后都行滤过性手术治疗.     

先天性葡萄膜外翻与青光眼

先天性葡萄膜外翻为虹膜色素上皮自瞳孔缘向虹膜基质表面过度增生,表现为虹膜表面色素增多,常伴发青光眼,因临床中较为少见,常被误诊、误治,造成视力不可逆性损伤,因此本文对先天性葡萄膜外翻及伴发青光眼的病因、临床特点、发病机制及治疗进展进行综述。     

先天性葡萄膜外翻一例

患儿,女,5岁,因左眼视物模糊1个月至天津市眼科医院就诊.患儿为第一胎,足月顺产,其母孕期无异常,父母非近亲结婚,无家族性遗传病史.患儿自出生后即出现左眼上睑下垂,于16个月前就诊于北京某医院,诊断为左眼先天性青光眼、左眼上睑下垂,行左眼小梁切开术,11个月前行上睑下垂额肌瓣悬吊术.     

先天性葡萄膜外翻与青光眼

目的 探讨先天性葡萄膜外翻的临床特点及其与青光眼的关系.方法 回顾性系列病例研究.收集2002年9月至2008年5月间共11例(16只眼)先天性葡萄膜外翻患者的临床资料,回顾性分析其临床特点和治疗效果.结果 11例(16只眼)先天性葡萄膜外翻患者中,有10例(14只眼)合并青光眼,1例(2只眼)诊断为高眼压症.有4例(5只眼)合并先天性上睑下垂,2例(3只眼)合并眼球突出,1例(1只眼)合并先天性白内障,1例(2只眼)合并先天性晶状体半脱位,1例(2只眼)合并先天性小角膜,3例合并并指畸形,1例合并Rieger综合征.9例(12只眼)青光眼患者在接受复合式小梁切除术后,10只眼的眼压得到有效控制.结论 先天性葡萄膜外翻是一种罕见的先天性异常,常伴随其他眼部畸形和全身异常;其发生青光眼的机制与虹膜前房角的发育异常有关.对先天性葡萄膜外翻合并青光眼的患者应尽早发现,早期干预和治疗.     

先天性葡萄膜外翻继发性青光眼一例

患者女性,18岁。因右眼青光眼、小梁切除术后半年、眼压失控5个月于2011年5月到南昌爱尔眼科医院就诊。患者自2009年底开始不明原因出现右眼视力逐渐下降（当时发现右眼瞳孔异常＂变大＂）,偶伴眼胀,未予诊治。于2010年12月因右眼视物不见在其他医院就诊,诊断为双眼原发性开角型青光眼（右眼晚期,左眼早期）,并行右眼小梁切除术,     

先天性晶体葡萄膜缺损1例

患者，苏XX，男，12岁．因自幼右眼视物不清外斜视．视力逐渐下降．于1992年5月20日来我院眼科就诊，既往健康，足月顺产．第一胎．患者父母非近亲结婚，母亲在孕期无特殊患病史，家族同胞中无类似眼气全身检查未见异常．眼部情况：视力．右眼眼前手动，左眼1，2，双眼压Tn，左眼外服及眼底均正逢．右眼外科25度，眼球各方位活动不受限右眼校在眼间小，结困无充血，角膜过明，前民深浅可，房水情，下方＊的担损，区又如染形尖端向下相对应过目状体缺如．充分四大吐孔后，可见晶状体下部呈半月形征状闻报，且有点扶混浊，缺损处悬韧带亦缺…     

先天性葡萄膜外翻继发青光眼一例

患者,女,20岁,因“右眼视力下降2年,加重5 d”于吉林大学第二医院眼科中心门诊就诊。患者2年前无明显诱因下出现视力下降,2019年6月7日自觉右眼视力下降明显,伴右眼胀痛,于2019年6月11日就诊。既往史:该患者出生后自觉右侧面部肥大,14岁时发现鼻部软组织肥厚,2016年于北京某医院行鼻部软组织切除术,具体情况不详(见图1)。否认眼部其他疾病及眼部外伤史。家族史无异常。眼科检查:视力:右眼颞侧光感,矫正不应;左眼0.1,-4.00,DS=1.0。眼压:右眼58 mmHg(1 mmHg=0.133 kPa),左眼12 mmHg。右眼轻度上睑下垂,遮盖上方角膜缘3~4 mm(见图2)。球结膜混合充血,角膜上皮轻度水肿,前房加深,瞳孔欠圆、散大,直径6.0 mm。     

房水的葡萄膜巩膜排出通道

房水的葡萄膜巩膜排出通道是房水排出途径的重要组成部分，随着拟前列腺素药物在抗青光眼治疗中的应用，其临床重要性重新引起了人们的注意。对有关房水的葡萄膜巩膜排出通道的研究进行综述，重点介绍房水葡萄膜巩膜排出的测量技术和方法，正常值，生理学基础，药理学基础（主要包括胆碱能类药物、肾上腺素、前列腺素及其衍生物对房水葡萄膜巩膜排出的影响和机制）。     

葡萄膜巩膜房水排出通道的研究进展

尽管在３０年前,有学者就提出葡萄膜巩膜房水流产的概念,但是由于研究手段的限制,在相当一段时间内有关这一房水引流途径的几乎处于停满足状态。近几年来,随着新的研究手段的应用,许多研究结果使我们加了对这一房水流出途径的认识。从而对青光眼房水排出障碍,眼压升高机制增加了新的认识。     

先天性葡萄膜外翻伴有青光眼

本病罕见,综合各家所见具有以下特征:(1)单眼发病;(2)虹膜可见先天性葡萄膜外翻和周边部虹膜实质萎缩。先天性葡萄膜外翻为非进行性的,瞳孔反应良好,虹膜纹理清晰,易与后天性者相鉴别;(3)房角特征:虹膜向前附着,显示房角发育不良;(4)多     

Congenital ectropion uveae with glaucoma

Congenital ectropion uveae (CEU) is a rare, nonprogressive anomaly characterized by the presence of iris pigment epithelium on the anterior surface of the iris stroma, often associated with neurofibromatosis and occasionally with other ocular anomalies. We present eight patients with unilateral CEU. Seven patients had glaucoma in the involved eye, while the eighth was a 10-week-old infant. In the two patients with bilateral glaucoma, the second eye was similar to the first, but without CEU. Three patients had neurofibromatosis, two had facial hemihypertrophy, one had Rieger's anomaly, one had Prader-Willi syndrome, and one had no systemic anomalies. Two had initially been misdiagnosed as having a large pupil in the involved eye and one as having a Horner's syndrome in the uninvolved eye. The finding of CEU in an infant warrants continued observation for the development of glaucoma and disorders of neural crest origin.     

Congenital ectropion uveae and glaucoma

Congenital ectropion uveae is a rare condition which may be present in one or both eyes. If the patient is followed glaucoma will always be found to be present. Associated features which have been described are ptosis, Rieger's anomaly, Prader Willi syndrome, facial hemiatrophy and neurofibromatosis. This paper describes a patient followed for 18 years who had bilateral congenital ectropion uveae, bilateral ptosis, asthma and late onset of a dental defect.     

CONGENITAL ECTROPION UVEAE AND GLAUCOMA

Congenital ectropion uveae is a rare condition which may be present in one or both eyes. If the patient is followed glaucoma will always be found to be present. Associated features which have been described are ptosis, Rieger's anomaly, Prader Willi syndrome, facial hemiatrophy and neurofibromatosis. This paper describes a patient followed for 18 years who had bilateral congenital ectropion uveae, bilateral ptosis, asthma and late onset of a dental defect.     

Age-related medial ectropion of the lower eyelid

Background : A simplified procedure is proposed for the repair of medial lower eyelid age-related ectropion. Methods : A posterior horizontal incision is made in the medial half of the lower eyelid at the inferior border of tarsus. The lower eyelid retractors are exposed and then plicated to the tarsus without excision of posterior lamellae or the use of everting sutures. The lid is then shortened horizontally with excision of a pentagonal section or lateral tarsal strip procedure. Results : The procedure was performed in six patients successfully without complication. Conclusions : This is an effective method for repair of lower lid medial age-related ectropion.