Endoscopic Submucosal Dissection of a Giant Rectal Adenoma Manifesting as McKittrick–Wheelock Syndrome

Abstract

McKittrick–Wheelock syndrome (MKWS) is an uncommon clinical manifestation of large, villous, epithelial lesions of the distal colon and rectum. Excessive secretion of electrolyte-rich mucus from these lesions leads to secretory diarrhea, electrolyte disorders and acute renal failure. Several cases of MKWS have been reported since its initial description in 1954. The definitive treatment for the great majority of MKWS cases has consisted of surgical resection of the affected part of the colorectum, usually in the form of a low anterior resection or an abdominoperineal resection with the formation of an ostomy. Recent developments in endoscopic resection techniques now offer new, minimally invasive treatment alternatives for MKWS patients. We present the first reported case in the Western world of MKWS caused by a rectal adenoma with a size of 19 × 10 cm, treated through endoscopic submucosal dissection. Through the lessons learned by this case, as well as by a thorough review of the literature, we discuss this uncommon syndrome, focusing on treatment alternatives.

Zusammenfassung

Das McKittrick-Wheelock-Syndrom (MKWS) stellt eine außergewöhnliche klinische Manifestation einer großen, villösen, adenoiden Rektumläsion dar. Die erhöhte Sekretion von elektrolytreichem Schleim durch den Tumor führt zu sekretorischer Diarrhoe, Elektrolytstörungen und akutem Nierenversagen. Seit der initialen Beschreibung im Jahr 1954 wurden mehrere Fälle veröffentlicht, in denen meistens eine chirurgische Resektion des betroffenen Kolonabschnitts als definitive Therapie durchgeführt wurde. Dies erfolgte in der Regel im Sinne einer tiefen anterioren Rektumresektion oder abdominoperinealen Rektumamputation mit Stoma. Die aktuellen Entwicklungen im Bereich der endoskopischen Resektionstechniken bieten heutzutage neue, minimalinvasive Therapieoptionen für diese Patienten. Wir präsentieren den – nach unserem Wissen – ersten Fall eines MKWS mit einem 19×10cm großen Rektumadenoms, der mittels endoskopischer Submukosadissektion (ESD) in der westlichen Welt behandelt wurde. Durch die neuen Erkenntnisse aus diesem Fall, sowie eine ausführliche Literaturrecherche, möchten wir dieses außergewöhnliche Syndrom diskutieren und dabei neue Therapieoptionen beleuchten.

Schlüsselwörter

endoskopische Resektion - transanale Chirurgie - Rektumkarzinom - minimalinvasive Chirurgie - endoskopische Submukosadissektion

Keywords

endoscopic resection - transanal surgery - rectal cancer - minimally invasive surgery - endoscopic submucosal dissection

Publication History

Received: 19 July 2023

Accepted after revision: 21 August 2023

Article published online:
26 September 2023

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