线状皮脂腺痣综合征眼部临床特征及手术整复效果分析

Ocular manifestations and surgical treatment of the linear nevus sebaceous syndrome

目的探讨线状皮脂腺痣综合征(LNSS)的临床病理学特征及手术整复效果。方法回顾性系列病例研究。收集2009年4月至2018年2月在首都医科大学附属北京同仁医院北京同仁眼科中心就诊的11例(14只眼)LNSS患者的临床资料,分析总结性别、年龄、临床表现、受累部位、组织病理学特点、手术方式和整复效果。结果11例患者中男性6例,女性5例;就诊年龄6个月至7岁,平均年龄为2.8岁。皮脂腺痣累及头部9例,累及面部5例,累及颈部3例。最常见的眼部表现为眼睑缺损(13只眼)和眼表肿物(9只眼)。手术整复患者9例(11只眼),均行眼睑缺损修复重建术,其中4例(4只眼)联合角结膜病灶切除术,5例(5只眼)联合眼睑肿物切除眼睑重建术,3例(5只眼)联合睑球粘连分离结膜囊重建术。6例(6只眼)眼睑及角结膜肿物行组织病理学检查,结果均为复合迷芽瘤。9例手术整复患者均取得满意的眼部外观。结论线状皮脂腺痣综合征有典型的皮肤病变特点和眼部表现,其眼表肿物均属复合迷芽瘤,切除肿物并矫治眼睑畸形,可达到较好的外观整复效果。

Objective To report the clinical and pathological features and surgical treatment of the linear nevus sebaceous syndrome.Methods It was a retrospective case series study.The clinical records of 11 patients(14 eyes)who were diagnosed as linear nevus sebaceous syndrome between April 2009 and February 2018 at Beijing Tongren Hospital were analyzed.Data collected included sex,age,clinical manifestations,disease site,pathological features,surgical treatment and therapeutic effects.Results There were six males and five females,aged 6 months to 7 years(mean,2.8 years).The nevus sebaceous was located at the head(9 patients),face(5 patients)and neck(3 patients).The most common ocular manifestations were blepharocoloboma(13 eyes)and external ocular mass(9 eyes).Surgical treatment was performed in 9 patients(11 eyes),including lid reconstruction in all these eyes,corneal and conjunctival mass resection in four patients(4 eyes),eyelid tumor resection in 5 patients(5 eyes),and symblepharon separation and conjunctival sac reconstruction in 3 patients(5 eyes).Pathological examinations of the external ocular,corneal and conjunctival masses(6 eyes)confirmed the diagnosis of complex choristoma.All operated patients had improved appearance.Conclusions Linear nevus sebaceous syndrome has characteristic cutaneous lesions and ocular manifestations.Histopathologically,all epibulbar lesions prove to be complex choristomas.The appearance of patients with linear nevus sebaceous syndrome can be significantly improved by oculoplastic surgery.