Pelvic splenosis and endometriosis in adolescent with hereditary spherocytosis

The patient is a 16-year-old with a history of hereditary spherocytosis. At age 12, she underwent a laparoscopic partial converted to total splenectomy for intraoperative bleeding. The spleen was placed in a retrieval bag and removed via morcellation. She first reported abdominal pain at age 8, with dysmenorrhea after menarche at age 13. Her dysmenorrhea was managed with a progesterone IUD. Due to worsening pain, she underwent MRI which showed multiple lobulated structures throughout the pelvis and ventral abdomen, suggestive of endometriomas versus splenules. Laparoscopy revealed dozens of black-blue nodules coating the anterior abdominal wall and multiple multi-centimeter nodules attached to the sigmoid colon, descending colon, jejunum, and ovarian fossae, with vesicular lesions deep in pelvis. Abdominal wall and pelvic lesions were excised; removal of the nodules adherent to the colon and small bowel was deferred given inadequate bowel preparation. Frozen section and final histopathology confirmed splenosis throughout the abdomen and pelvis, with presence of endometriosis in right uterosacral ligament and ovarian fossa. Future surgery is planned to excise remaining splenules. This patient's case demonstrates that splenosis and endometriosis can exist simultaneously and are difficult to differentiate from one another symptomatically, on imaging, and even under direct visualization. Gynecologic surgeons should include splenosis in the differential diagnosis of pelvic nodular masses, particularly in patients with a history of splenectomy.