McKittrick Wheelock Syndrome Treated by Transanal Minimally Invasive Surgery: A Single-Center Experience and Review of the Literature.

Introduction: McKittrick Wheelock Syndrome (MKWS) is a rare syndrome characterized by secretory diarrhea, dehydration, prerenal acute kidney injury, and severe electrolyte abnormalities caused by a large hypersecretory villous adenoma located in the rectum or rectosigmoid junction. Transabdominal (laparoscopic) procedures are the most described procedures in the treatment of MKWS. We report an alternative surgical approach, transanal minimally invasive surgery (TAMIS), to solve this syndrome.
Materials and Methods: All patients who underwent transanal endoscopic microsurgery or TAMIS were included in a prospectively collected database in our center. Between 1996 and 2015, 3 patients were found to have MKWS and treated by TAMIS. Demographics, characteristics of the adenoma, surgery-related data, and outcome were analyzed.
Results: The first patient had a villous adenoma 0-12 cm from the anal verge. After TAMIS, she complained of a stenosis that was successfully treated by dilatation. The second patient had an impressive bulky tumor in the rectum. An endoscopic mucosal resection was attempted twice, but incompletely. Pathology analysis showed villous adenoma with high-grade dysplasia and intramucosal carcinoma. One year after TAMIS, a recurrence was detected and treated by an abdominal-perineal resection. A TAMIS was performed in the third patient because of a giant villous circular adenoma, but because of high mucus and fluid production, no proper overview could be obtained. Together with opening of the peritoneum, this prompted us to convert to a laparoscopic Hartmann procedure. Besides this technical difficulty, the patient recovered uneventful.
Conclusions: MKWS is a rare syndrome and missed diagnosis could result in life-threatening situations. Different endoscopic and surgical treatments are described to solve this syndrome. TAMIS should be considered as a rectum-preserving surgical treatment option for such extensive adenomas.