Case Report

McKittrick-Wheelock syndrome: a cause of severe hydro-Electrolyte disorders in ED^B

A 72-year-old woman presented with a history of 1 year of diarrhea and, in the last 3 weeks, weakness and drowsiness. Initial laboratory findings were hyponatremia (sodium, 117 mmol/L), hypokalemia (potassium, 2.57 mmol/L), hypochloremia (64 mmol/L), and azotemia (urea, 434 mg/ dL and creatinine, 4.36 mg/dL). The patient had a Full recovery after hydration and correction of all electrolyte disorders. In the investigation of the cause of diarrhea, we found a polypoid lesion 17 cm in length, revealing a secretory tubulovillous adenoma in the histologic evaluation. Here we describe a rare case of the McKittrick-Wheelock syndrome with a dramatic presentation in the emergency department.

Adenomatous polyps are neoplasms with malignant po- tential, located mainly at the level of the sigmoid and rectum. Most patients with colonic adenomatous polyps present with mild Gastrointestinal symptoms or are asymptomatic, colono- scopic exploration being the procedure of choice for the diagnosis [1]. In rare cases, patients with villous adenomas exhibit secretory diarrhea with considerable loss of fluids and electrolytes. The most dramatic presentation with diarrhea, dehydration, acute renal failure, and electrolytic disorders is a Rare condition known as McKittrick-Wheelock syndrome [2]. Here we describe a case of this rare syndrome with presentation in the emergency department (ED).

A 72-year-old white woman from Sa~o Paulo, Brazil, was brought to the ED with severe watery diarrhea, dehydration, limb weakness, and sleepiness. The patient had a 1-year history of chronic diarrhea.

On examination, she was sleepy, confused, dehydrated, and with tachypnea. Her temperature was 35.48C, her pulse 134 beats per minute, her respiratory rate was 24 breaths per minute, and her blood pressure 80/50 mm Hg. Her skin was cool and clammy, and she appeared ill. Examination of the heart revealed a rapid rhythm; the sounds and point of maximal impulse were normal. The breath sounds and abdomen exami- nation were normal. The rectal examination showed a mass.

The electrocardiogram on admission showed alterations suggestive of hypokalemia (ST-segment reduction and U waves) (Fig. 1).

^B This study is supported by the Faculty of Medicine of the University of Sa~o Paulo, Brazil.

The abnormal findings of laboratory examinations on admission were severe hyponatremia (117 mmol/L), hypo- kalemia (2.57 mmol/L), hypochloremia (64 mmol/L), and severe acute renal failure (Table 1). Other laboratory data were normal, including thyroid and Hepatic function.

Colonoscopy revealed a villous, reddish-surfaced, frag- ile-consistency polyp, 17 cm from the anus (Fig. 2). A secreting villous adenoma structure without malignant areas was diagnosed on histopathologic examination.

The patient had a full recovery after hydration and correction of all electrolyte disorders (Table 1).

In the 1950s, McKittrick and Wheelock [2] were the first ones to describe the aggressive villous adenomatous polyp of the colorectal area that has a course with dehydration, electrolyte disorders, and acute renal failure.

The villous adenomas of the colon cause secretory diarrhea associated with severe electrolyte and fluid depletion syndrome (McKittrick-Wheelock syndrome). Large rectal villous adenomas (4-10 cm) can cause fluid depletion of 0.5 to 3 L every 24 hours and hypokalemia [2]. Prostaglandin E2 has been implicated as a possible secretagogue compound in the pathogenesis of this syndrome, and indomethacin and other prostaglandin inhibitors have been used with apparent benefit in controlling the volume of rectal effluent in patients with secretory villous adenomas [3,4].

The villous adenomas of the colon decrease the Serum potassium concentration because there is excessive deple- tion of this ion. The patients can present mainly with neuromuscular and cardiovascular symptoms, evolving to flaccid quadriplegia, areflexia, Respiratory insufficiency, arrhythmias, and sudden cardiac death.

The hypokalemia causes an ST-segment depression, reduction in T waves, and the presence of U waves in the electrocardiogram (Fig. 1). The treatment is based on oral potassium replacement and, in more serious symptomatic forms, by parenteral therapy.

The possibility of hyponatremia must be considered whenever there is dysfunction of the central nervous system [5]. The main therapy consists in increasing the serum sodium concentration to correct the neurologic symptoms, and it must be done supported by insurances formulas and by characteristics of infusates (change in serum Na+=infu- sate Na+/total body water+1) not exceeding 1 mEq/h or

12 mEq/24 h [6]. The presence of symptoms and their severity determine the pace of correction. Nevertheless,

0735-6757/$ – see front matter D 2007

1083.e2 Case Report

Fig. 1 Electrocardiogram suggests hypokalemia: ST-segment reduction and U waves.

correction should be of a sufficient pace and magnitude to reverse the manifestations of hypotonicity but not be so rapid and large as to pose a risk of the development of osmotic demyelination [6].

The patient was admitted in the hospital with severe hyponatremia, hypokalemia, hypochloremia, and uremia. What is impressive in this case are the levels of urea (434 mg/L) and creatinine (4.36 mg/L) on admission; these values had been normalized after aggressive replacement of fluid and electrolytes (urea, 23 mg/dL and creatinine, 0.73 mg/dL).

The precocious diagnosis of the McKittrick-Wheelock syndrome is very important for immediate replacement of fluid and electrolyte. Even so, the surgical removal of the adenoma led to Complete recovery of the symptoms.

To our knowledge, our case is one of the most striking hydro-electrolyte disorders with high levels of creatinine and urea [2,7-13], all this totally reversible.

Although the McKittrick-Wheelock syndrome is a rare illness, it is very important to make the diagnosis in precocious phases, allowing the total reversibility of the abnormalities with adequate treatment.

Case Report 1083.e3

Fig. 2 Rectosigmoidoscopy: villous rectal adenoma. Mucous injury of the villous aspect, sessile with circumferential form, filling the lumen of the rectum.

Herlon Saraiva Martins MD Rodrigo Anto^nio Branda~o-Neto MD Andre’ Laranjeira de Carvalho MS Alfredo Nicodemo Cruz Santana MD Francisco Jose’ Bueno Aguiar MD Augusto Scalabrini-Neto MD, PHD Irineu Tadeu Velasco MD, PHD

Faculty of Medicine of the University of Sa~o Paulo

Brazil E-mail address: [email protected]

doi:10.1016/j.ajem.2007.03.002

References

1. Bond JH. Polyp guideline: diagnosis, treatment and surveillance for patients with colorectal polyps. Am J Gastroenterol 2000;95: 3053 – 63.
2. McKittrick LS, Wheelock FC. Carcinoma of the colon. 1954. Dis Colon Rectum 1997;40(12):1494 – 5 [discussion 1495-6].
3. Pugh S, Thomas GA. Patients with adenomatous polyps and carcinomas have increased colonic mucosal prostaglandin E2. Gut 1994;35:675 – 8.
4. Smelt AH, Meinders AE, Hoelman K, Noort WA, Keirse MJ. Secretory diarrhea in villous adenoma of rectum: effect of treat- ment with somatostatin and indomethacin. Prostaglandins 1992;43: 567 – 72.
5. Hans DS, Cho BS. Therapeutic approach to hyponatremia. Nephron 2002;92(Suppl. 1):9 – 13.
6. Androgue HJ, Madias NE. Hyponatremia. N Engl J Med 2000;342: 1581 – 9.
7. Kioer HW. Villous adenomas of the colon and the rectum with electrolyte disturbances. McKittrick-Wheelock’s syndrome. Ugeskr Laeger 1969;131(39):1646 – 9.
8. Tosi F, Branchini L, Armiraglio L, Scandroglio I, Massazza C. Villous adenoma of the rectum with water-electrolyte imbalance (McKittrick- Wheelock syndrome). Minerva Chir 1987;42(9):793 – 7.
9. Suarez J. McKittrick and Wheelock syndrome. Clinical case. Rev Med Chil 1994;122(2):198 – 200.
10. Older J, Older P, Colker J, Brown R. Secretory villous adenomas that cause depleting syndrome. Arch Intern Med 1999;159:879 – 80.
11. Helgesen A, Fuglsig S. Villous adenoma of the rectum with electrolyte imbalance. McKittrick-Wheelock syndrome. Ugeskr Laeger 2000; 162(32):4272 – 3.
12. Popescu A, Orban-Schiopu AM, Becheanu G, Diculescu M. McKittrick-Wheelock syndrome–a rare cause of acute renal failure. Rom J Gastroenterol 2005;14:63 – 6.
13. Lepur D, Klinar I, Mise B, Himbele J, Vranjican Z, Barsic B. McKittrick-Wheelock syndrome: a rare cause of diarrhea. Eur J Gastroenterol Hepatol 2006;18(5):557 – 9.