Evisceration in patients with Sturge-Weber syndrome

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...

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Bibliographic Details
Published in:Orbit (Amsterdam) 2017-01, Vol.36 (1), p.48-51
Main Authors: Merritt, Helen, Pfeiffer, Margaret L., Phillips, Margaret E., Richani, Karina
Format: Article
Language:English
Subjects:
Blindness - surgery
Choroidal hemangioma enucleation
evisceration
Eye Evisceration
Eye Pain - surgery
Eye, Artificial
Humans
Male
Middle Aged
Orbital Implants
Prosthesis Implantation
Sturge-Weber syndrome
Sturge-Weber Syndrome - diagnosis
Sturge-Weber Syndrome - surgery
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Summary: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.
ISSN:0167-6830
1744-5108
DOI:10.1080/01676830.2017.1279643