To report the clinical and pathological features and surgical treatment of the linear nevus sebaceous syndrome.

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.

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.

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. (Chin J Ophthalmol, 2020, 56: 846-852)

Nevus, sebaceous of jadassohn; Ophthalmologic surgical procedures; Reconstructive surgical procedures; Eyelid diseases; Conjunctival diseases; Linear nevus sebaceous syndrome