Evisceration in patients with Sturge-Weber syndrome

Orbit. 2017 Feb;36(1):48-51. doi: 10.1080/01676830.2017.1279643. Epub 2017 Feb 1.


The management of blind, painful eyes in Sturge-Weber syndrome patients poses unique challenges to the oculoplastic surgeon. Intraocular and orbital vascular malformations and calcification may theoretically lead to unexpected hemorrhage and difficulty placing an implant in a calcified scleral shell. We present two cases of patients with Sturge-Weber syndrome with blind, painful eyes who underwent evisceration with silicone implant and discuss the relevant current literature. Both of our patients had uncomplicated surgeries and post-operative courses. Our literature review reveals that both evisceration and enucleation are viable surgical options for globe removal in Sturge-Weber syndrome, yet careful preoperative planning must be undertaken to minimize risk.

Keywords: Choroidal hemangioma enucleation; Sturge–Weber syndrome; evisceration.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Blindness / surgery
  • Eye Evisceration*
  • Eye Pain / surgery
  • Eye, Artificial
  • Humans
  • Male
  • Middle Aged
  • Orbital Implants
  • Prosthesis Implantation
  • Sturge-Weber Syndrome / diagnosis
  • Sturge-Weber Syndrome / surgery*