Wide local excision of perianal Paget’s disease with gluteal flap reconstruction: an interdisciplinary approach

Daniel J. Borsuk, George Melich, Jeremy Sugrue, Jed Calata, Iris A. Seitz, John J. Park, Leela M. Prasad, Slawomir J. Marecik


Perianal Paget’s disease (PPD) is an extremely rare condition characterized as intraepithelial adenocarcinoma of unclear etiology. It can be either primary or secondary. The disease typically presents as an eczema-like, erythematous, and painful skin lesion that is associated with pruritus. It is usually misdiagnosed as a common anorectal problem. Surgical excision is the preferred treatment of PPD, with the specific technique being dependent upon disease invasiveness. The treatment may involve reconstructive surgery. A 61-year-old female with a history of rectal pain and intermittent pruritus for the past two years presented with large painful lesions in her perianal area including the anal verge, diagnosed as primary PPD. After excluding other malignancies elsewhere, a laparoscopic ileostomy followed by a wide local excision (WLE) of the PPD was performed by a colorectal team. Reconstruction of the defect with gluteal advancement flaps was performed by the plastic surgeon. The patient recovered uneventfully. Her surgical site showed healing without flap compromise, widely open anal opening, and full sphincter control at the three-month follow-up exam. The patient returned to normal function after ileostomy closure. WLE with bilateral V-Y gluteal flap advancement is a feasible treatment for primary PPD.