Extra-mammary Paget's disease of the perianal region: a review of the literature emphasizing the operative management technique.

The perianal skin is a common area for extra-mammary Paget's disease development. The unique clinical, histopathological, and immunohistochemical features which this medical phenomenon demonstrates, along with its rarity and frequent association with synchronous or metachronous carcinomas, present us with a treatment challenge. In order to organize the surgical treatment, it is important to determine whether the disease is localized exclusively to the perianal skin or associated with metastasis or anorectal carcinomas. Despite several controversies concerning its optimal therapeutic management, wide local excision of the skin and subcutaneous tissue in the perianal region is generally recommended for the treatment of the non-invasive form of the disease. Such an aggressive operative management usually results in a large perianal tissue defect, which can not be primarily suppressed without resultant tension and possible complications, requiring a special technique for its coverage. Various techniques have been described in the literature for the treatment of these defects, often associated with unfavourable long term results, i.e. split-thickness skin grafts and vacuum-assisted closure devices. More recently several authors have reported favourable results using various transposition or rotation local skin flaps, myocutaneous flaps of the gluteal and thigh muscles, and V-Y island flaps to cover these areas of tissue loss. In this article we present a short review of the literature concerning perianal Paget's disease with special attention to its management and a demonstration of the operative technique we prefer on patients with perianal non-invasive Paget's disease, i.e. wide local excision with a 2 cm margin in the anal mucosa and use of U and V-Y shaped perianal fatty-cutaneous island flaps for reconstruction by covering the bilateral anal skin defects.