Koning G G, Rensma P L, van Milligen de Wit A W M, van Laarhoven C J H M
Department of Surgery, Saint Elisabeth Hospital, Tilburg, The Netherlands.
Case Rep Gastroenterol. 2008 May 24;2(2):175-80. doi: 10.1159/000129705.
The authors present a woman suffering from McKittrick-Wheelock syndrome (MKWS) with a giant rectal villous adenoma. MKWS is a rare disorder caused by fluid and electrolyte hypersecretion from a rectal tumor. The most frequently reported tumors are villous adenomas. Symptoms of dehydration with severe hyponatremia, hypokalemia, metabolic acidosis and acute renal failure are typical in MKWS. Several options for operation have been reported, such as a transsacral approach (according to Kraske), transanal endoscopic microsurgery (TEM) or total mesorectal excision (TME). In this case we report an alternative surgical approach: in-one-continuity transanal mucosectomy and transabdominal TME with a handsewn colonic-anal anastomosis.
A 54-year-old woman had a history of hospital admissions because of repeated bouts of dehydration with electrolyte disorders since 2004. At admission she presented with prerenal azotemia, hyponatremia and severe hypokalemia in combination with watery stools. At colonoscopy an 8-cm villous adenoma was seen in the rectum. Dehydration and electrolyte disturbances were treated by appropriate intravenous fluid administration. An in-one-continuity anal mucosectomy and complete rectal excision were performed and restored by a handmade colonic-anal anastomosis. Postoperative recovery was uneventful.
MKWS can be a difficult problem to assess in both gastroenterological and nephrological ways. Patients may develop severe complications which require surgical intervention in some cases. In-one-continuity transanal mucosectomy and rectum excision with a handmade colonic-anal anastomosis seemed to be a new and solid surgical therapeutic option in this case.
作者报告了一名患有麦基特里克 - 惠洛克综合征（MKWS）并伴有巨大直肠绒毛状腺瘤的女性。MKWS是一种由直肠肿瘤导致液体和电解质分泌过多引起的罕见疾病。最常报告的肿瘤是绒毛状腺瘤。脱水伴严重低钠血症、低钾血症、代谢性酸中毒和急性肾衰竭是MKWS的典型症状。已报道了几种手术选择，如经骶骨入路（根据克拉斯克法）、经肛门内镜显微手术（TEM）或全直肠系膜切除术（TME）。在本病例中，我们报告一种替代手术方法：连续性经肛门黏膜切除术和经腹TME并手工缝合结肠 - 肛管吻合术。
一名54岁女性自2004年起因反复出现伴有电解质紊乱的脱水发作而多次住院。入院时，她表现为肾前性氮质血症、低钠血症和严重低钾血症，同时伴有水样便。结肠镜检查发现直肠有一个8厘米的绒毛状腺瘤。通过适当的静脉补液治疗脱水和电解质紊乱。进行了连续性经肛门黏膜切除术和全直肠切除术，并通过手工制作的结肠 - 肛管吻合术进行修复。术后恢复顺利。
MKWS在胃肠病学和肾脏病学方面都可能是一个难以评估的问题。患者可能会出现严重并发症，在某些情况下需要手术干预。连续性经肛门黏膜切除术和直肠切除术并手工制作结肠 - 肛管吻合术在本病例中似乎是一种新的可靠的手术治疗选择。
