Staged pelvic exenteration followed by oblique fleur-de-lis rectus abdominis myocutaneous (OFRAM) flap and keystone flap reconstruction for extramammary Paget’s disease

Extramammary Paget’s disease (EMPD) is a rare, locally aggressive cutaneous adenocarcinoma which typically involves the anogenital region. The prognosis heavily depends on early diagnosis with definitive surgical treatment. To reduce local recurrence, wide radical resection with margin control and Moh’s micrographic surgery are conventionally recommended. We describe a case of a 65-year-old female with extensive perineal EMPD involving the vulva, vagina, ectocervix, urethra, and anal canal, extending to the sacral region. She required pelvic exenteration and skin resection with 5-cm margins. A rectus abdominis myocutaneous flap (OFRAM) was designed with a fleur-de-lis skin paddle, with the vertical limb extended in an oblique fashion. We name this the OFRAM design and it allows maximal recruitment of abdominal tissue for the reconstruction of massive pelvic defects. The oblique limb obliterated the pelvic floor defect after the pelvic exenteration, while the horizontal limbs were used to resurface the perineal defect. One week later, after formal histology confirmed oncologic clearance, a gluteal keystone flap was used to resurface the remnant posterior defect. We discuss the disease pathology and reconstructive options in such cases. Level of Evidence: Level V, therapeutic study.