The role of the plastic surgeon in the treatment of Gorlin syndrome

ABSTRACT

Introduction:

Gorlin syndrome or nevoid basal cell carcinoma syndrome is a rare multisystemic disease with a potential to cause a broad spectrum of abnormalities and other cancers. It is an autosomal dominant disease with a high penetrance and large variability of expression, manifesting in all ethnic groups but more prevalent in Caucasians, and presenting at a sex ratio of 1:1.

Objective:

The aim of this study was to discuss Gorlin syndrome, which is little commented on in the literature , and is possibly being underdiagnosed and undertreated by plastic surgeons and other professionals.

Method:

A literature review was done by selecting articles about Gorlin syndrome from the Medline/PubMed database from 2009 to 2013, and a caseby-case description from the records of the Clinical Hospital of Ribeirão Preto, University of São Paulo is provided.

Conclusion:

Gorlin syndrome is a multisystemic syndrome with a wide spectrum of manifestations and a great potential for causing problems related mainly to the treatment of its three major changes or features. The plastic surgeon plays an important role in its detection, and can assist in the comprehensive treatment with proper follow-up of patients.

Keywords:
Orofacial-digital syndrome; Gorlin syndrome; Plastic surgery