A systematic review of McKittrick–Wheelock syndrome

Abstract

Introduction McKittrick–Wheelock syndrome describes the condition of extreme electrolyte and fluid depletion caused by large distal colorectal tumours, usually the benign villous adenoma. Patients generally present critically unwell with severe hyponatraemia, hypokalaemia and/or acute kidney injury. Methods A structured literature review was undertaken to discover what is known about this condition, which is almost universally described as rare. Important features of the syndrome were identified, including common presenting symptoms, blood results, tumour location and size. Findings Our literature search identified 257 cases reported across all languages. The most remarkable features were the long duration of symptoms (median 24 months) and the significant electrolyte derangements (median sodium of 122mmol/l and median potassium of 2.7mmol/l at initial presentation). Five key recommendations are made to improve diagnosis, including aggressive fluid resuscitation to match rectal losses and surgical intervention on the index admission. The advantages and disadvantages of different treatment options are discussed, including minimally invasive alternatives to traditional resectional surgery. Conclusions McKittrick–Wheelock syndrome describes a normally benign condition that can cause patients to become critically unwell and so it behoves all clinicians to be aware of it. By publishing recommendations based on a comprehensive literature review, we aim to improve diagnosis and management of this life threatening condition.

McKittrick-Wheelock Syndrome: A Case Series

“…MKWS is a rare and life-threatening disease characterized by the triad of chronic mucous diarrhea; renal function impairment with hydroelectrolyte imbalance; and a giant left-sided tumor, often a large rectal adenoma [8]. However, as pointed out in a recent systematic review by Orchard et al [1] that identified 257 reported cases, the syndrome may have likely gone unrecognized, mainly due to the fact that it presents as a digestive disease with an overwhelming impact on renal function. All 3 patients presented with the classic symptomatology for MKWS.…”

“…Additionally, endoscopic resection has shown poor results and high recurrence rates [2,16]. The majority of reported MKWS cases (64.8%) underwent resectional surgery (i.e., anterior resection, abdominoperineal resection, Hartmann procedure, or sigmoid colectomy); these all resulted in full resolution of symptoms and electrolyte abnormalities and are likely to still be the approach of choice [1]. A sphincter-preserving oncologic resection for ultralow tumors may be the best recommendation for MKWS patients with the age of > 55 years and with the tumor size of > 4.5 cm, due to their high malignant potential.…”

McKittrick-Wheelock Syndrome: A Rare Cause of Chronic Diarrhea

“…Most patients present with chronic diarrhea and symptoms due to electrolyte imbalances, such as lethargy, muscle cramps, seizures, paresthesia, cramps, ileus, vomiting, hypotension, cardiac arrhythmias, and electrocardiographic changes [4][5][6][7][8]. The incidence and prevalence of MWS are difficult to estimate as some of the cases of MWS have also been reported as electrolyte depletion syndrome [9].…”

“…MWS is commonly seen in association with villous adenomas, but there have been few reported cases of tubulovillous adenomas with similar presentation as also seen in our case. However, there is no data available on the prevalence of tubulovillous adenoma in association with MWS currently [ 8 , 9 , 19 , 20 ].…”

A case of rectal villous tumor with shortness of breath in a 70‐year‐old woman—Uncommon symptom of McKittrick‐Wheelock syndrome

“…Subsequently, her symptom, renal failure, and electrolyte abnormalities improved. Common symptoms of MWS are general fatigue, weakness, and loss of consciousness caused by hyponatremia 1 . Shortness of breath is a rare symptom of MWS, and it was thought to be related to mainly respiratory muscle disorders due to hypokalemia.…”