1231 Radical Vulvectomy and Reconstruction for Severe, Refractory Crohn's Disease of the Vulva: A Case Report

Abstract

Abstract Vulval Crohn’s disease (VCD) is rare and can arise from direct infiltration, through fistulae or as non-contiguous lesions, so called metastatic disease. We present a case of a 32-year-old female with severe, refractory vulval Crohn’s disease (VCD), requiring radical vulvectomy and reconstruction. She had a subtotal colectomy for Crohn’s disease 8 years previously, and for one year had experienced vulval swelling and pain associated with perianal fistulae for which she underwent an abdomino-perineal resection. Though she achieved long term remission of her gastrointestinal disease, she continued to experience severe vulval symptoms with recurrent infections and abscesses despite treatment with immunomodulators and biologics over a period of 10 years. She then underwent a radical vulvectomy with bilateral gluteal fold flap reconstruction and skin grafting. She made an uncomplicated post-operative recovery. Histopathology showed active non-caseating granulomatous vulvitis. At 1 year follow up, there were no further disease flares, with significant improvement of her pain. Further plastic surgery is being considered. Our case demonstrates the value of surgical management in refractory VCD to improve symptoms, function and quality of life. Though medical therapy with immunosuppressive agents is recommended as first-line treatment, the course of VCD is unpredictable and often relapses on treatment cessation. VCD may persist for long after remission of gastrointestinal disease and may require more aggressive surgery using a multispecialty approach even in the context of active infective disease.