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

Abstract

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.

Introduction

Colorectal adenomas are premalignant lesions and are pathologically classified as tubular, tubulovillous, or villous adenomas. Villous adenomas comprise around 5% of adenomas with a 15%–40% estimated risk of malignancy also influenced by size of the adenoma.¹ About 3% of large (>3–4 cm) villous adenomas have secretory activity.^2–4 The epithelial composition of secretory adenomas shows mucin-secreting goblet cells.^5–7

The McKittrick Wheelock Syndrome (MKWS), first described in 1954, 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.⁶ Whereas a normal bowel absorbs sodium and water and secretes potassium, bowels affected by large villous secretory adenoma secretes water, sodium, and potassium. The large size allows more surface area for secretion, and the distal location of these lesions means a minimal area of normal colonic mucosa remaining to allow absorption.⁵

The mechanism behind these symptoms is suggested to elevate levels of cyclic adenosine monophosphate and prostaglandin E2 (PGE2), which increase the loss of fluid and electrolytes from plasma to the intestinal lumen.^5–8

A decrease in PGE2 levels is found after treatment with indomethacin and somatostatin.^8,9 Definite treatment consists of endoscopic or surgical treatment to stop secretory diarrhea with all conceivable consequences and to prevent carcinoma formation.

A few reports describe minimal invasive techniques to treat MKWS.^10,11 Minimal invasive techniques reduce costs and may have less morbidity than major transabdominal procedures. Transanal endoscopic microsurgery (TEM) has been described as a local excision technique to solve this syndrome.¹¹ Single-site surgical ports are nowadays used as another option to the classical TEM rectoscope and this is called transanal minimally invasive surgery (TAMIS). This technique is likely to have a shorter learning curve. Moreover, a single port is more readily available in most centers in contrast to a rectoscope. TAMIS has already proven to be a feasible and safe modification without compromising anorectal functioning.^12,13 In this case report, we describe our experience with TAMIS in patients with MKWS.

Materials and Methods

From a prospective database in our center (a tertiary referral center for TAMIS and laparoscopic colorectal surgery), patients having MKWS were analyzed. This database includes clinical and pathological features of all transanal surgical procedures (TEM or TAMIS) accomplished between December 1996 and July 2015 (n = 1029).

Procedures were performed by surgeons who are extensively experienced in TEM and quite experienced in TAMIS. TAMIS is performed using the single-site laparoscopic access system (SSL, Ethicon) in combination with a 30° laparoscope. A single enema was given preoperatively. Preoperative antibiotics (cefazoline/metronidazole) were administered. All patients underwent this procedure under general anesthesia in the lithotomy position. A pneumorectum of 12–15 mmHg was determined using carbon dioxide insufflation. Dissection of the lesion was accomplished full thickness, in most cases in one piece, which allowed accurate pathological evaluation of the specimen. The defect in the rectal wall was closed with a running V-loc suture (Medtronic).

Results

Three patients were found to have MKWS and details are shown in Table 1. The first patient, an 84-year-old female, was admitted to hospital with secretory diarrhea, severe dehydration, and electrolyte disorders. Rectoscopy showed an adenoma 0–12 cm from the anal verge. She underwent a TAMIS (Fig. 1) and was discharged after 2 days. Pathology analysis revealed a villous adenoma, with a surface area of 224 cm². During follow-up, the patient complained about variable defecation and rectal examination showed a stenosis. This stenosis was treated three times by dilatation of the rectum. Colonoscopy 13 months later showed no stenosis or recurrence.

FIG. 1.

Macroscopic pathology of villous adenoma.

Table 1.

Clinical Details of Patients with McKittrick Wheelock Syndrome Treated with Transanal Minimally Invasive Surgery

Patient	Gender	Age	ASA	Distance from anal verge (cm)	Circumference (%)	Size (cm)	Pathology	Follow-up (MO)
I	F	84	2	0–12	100	16 × 14	VA LGD	13
II	M	67	1	0–15	100	4 × 6	VA LGD+HGD CA (pT1)	54
III	M	78	2	5–14	100	11 × 9	VA LGD	20

ASA, American Society of Anesthesiologists; CA, carcinoma; F, female; HGD, high-grade dysplasia; LGD, low-grade dysplasia; M, male; MO, months; VA, villous adenoma.

The second patient, a 67-year-old male, complained about secretory diarrhea with electrolyte disorders on blood testing. An impressive bulky tumor in the rectum was discovered on colonoscopy. An endoscopic mucosal resection (EMR) was attempted twice, but incompletely because of the bulky tumor. Pathology analysis showed villous adenoma with high-grade dysplasia and intramucosal carcinoma. This patient was referred and we performed a TAMIS. The remaining tumor was removed in one piece that had a surface area of 24 cm². Postoperative course was uneventful, and the patient was discharged after 2 days.

A low-grade pT1R0 carcinoma was seen on final pathology analysis. Magnetic resonance imaging (MRI) showed no lymph nodes and abdominal and chest computed tomography (CT) showed no metastases. A strict follow-up was indicated. After 1 year, a local recurrence 4 cm ab ano was seen by rectoscopy. Pathology analysis showed high-grade dysplasia suspected for carcinoma. Pelvic MRI and abdominal and chest CT showed no recurrence or metastases. An abdominal-perineal resection was performed (pT1N1R0). After 52 months of follow-up, no metastases were detected.

The third patient, a 78-year-old male, had an extensive course: this patient reported 3 years of secretory diarrhea years with a frequency of 15 times a day. At the emergency department, weakness was seen with electrolyte disturbance and prerenal failure. After resuscitation, we performed further analyses: feces specimens and ultrasonography of the abdomen showed no abnormalities. CT of the abdomen described a mass in the rectum without pathological lymph nodes. Colonoscopy showed a giant villous circular adenoma 5–14 cm ab ano. Pathology analysis showed a villous adenoma with low-grade dysplasia.

A TAMIS was performed but because of high mucus and fluid production, no proper overview could be obtained. This together with opening of the peritoneum prompted us to convert to a laparoscopic Hartmann procedure. Final pathology analysis showed a villous adenoma with low-grade dysplasia having a surface area of 99 cm². Besides this technical difficulty, the patient recovered uneventful and was discharged after 15 days.

Discussion

The typical triad of MKWS includes dehydration, prerenal failure, and severe electrolytic abnormalities in the setting of chronic diarrhea caused by an underlying hypersecretory villous adenoma. The syndrome could have detrimental outcomes, especially in large adenomas with a distal location. Our cases, particularly in the third patient, highlight the importance of being aware of MKWS and the need to include this diagnosis in the work-up of patients with secretory diarrhea.

Missed diagnosis will lead to recurrent episodes of depletion syndrome and could result in life-threatening situations. Definite endoscopic or surgical treatment could be life saving. The most adequate management of giant rectal villous adenomas remains a discussion. The adenomas are basically benign and require treatment options with low morbidity and low mortality. In contrast, the higher risk of malignancy demands a radical excision of the lesion. Several procedures have been described ranging from endoscopic to major transabdominal resections: each of these approaches has its advantages and disadvantages, and the most applicable procedure should be selected with regard to the size of the adenoma, distance from the anal verge, imaging, pathology, availability of equipment, surgeons' experience, and patient's preference.

Table 2 shows all procedures performed in the treatment of patients with MWKS, as described in the literature.^{2,4,7,10,11,14–25} Surprisingly, almost all are major transabdominal procedures. Laparoscopic low anterior resection is the most frequently applied procedure in the treatment of MKWS.^{2,15,19–22,25} These procedures are associated with mortality and morbidity such as surgical site infection, anastomotic leakage, fecal incontinence, and sexual and urinary dysfunction.^26–28 Moreover, a permanent colostomy is sometimes required, which may influence the patient's quality of life.^29,30

Table 2.

Prior Reports of Patients with McKittrick Wheelock Syndrome Treated with Endoscopic or Surgical Treatment

Author	Sex, age	Distance from anal verge (cm)	Pathology	Operation
Miles et al.²	M, 68	10–20	TVA LGD	LAR
Dagan and Reissman⁷	F, 52	0–22	TVA, LGD/HGD	Laparoscopic PS+DLI
Winstanley et al.²³	M, 58	0–9	VA, LGD	Proctectomy
Koning et al.¹⁶	F, 54	0–8	VA, LGD	TM+TME+TLI
Learney et al.¹⁷	F, 65	2–12	VA	Laparoscopic APR
Popescu et al.⁴	M, 74	ND	VA, LGD/HGD	APR
Podesta et al.²²	M, 72	0–15	VA, HGD	Laparoscopic LAR+DLI
	M, 83	ND	VA, HGD	TE
Raphael et al.²⁰	F, 58	5–16	TVA, LGD/HGD, CA (pT1)	LAR+DLI
Hashash et al.¹⁴	M, 62	0–30	VA, HGD	PS+DLI
Barendse et al.¹¹	F, 83	ND	VA, LGD	EMR+TEM
	M, 66	0–10	VA, HGD, CA (pT1)	EMR+TAMIS
Bruno et al.¹⁸	F, 70	0–11	VA, CA (pT1N1)	Intersphincteric RR+DLI
Tuta et al.²⁴	M, 55	7–16	VA+CA	RA+CS
Choi et al.²¹	M, 59	7-ND	TVA+CA	Laparoscopic LAR
Khalife et al.¹⁹	F, 69	0–10	VA	LAR
Sanchez Garcia et al.¹⁵	F, 77	ND	VA	LAR+CS
Ohara et al.¹⁰	F, 66	ND	VA+CA	ESD
Targarona et al.²⁵	F, 63	0–10	VA	Laparoscopic LAR
	F, 69	1–11	VA	Laparoscopic PS
	F, 69	10–20	VA	Laparoscopic LAR

APR, abdominal-perineal resection; CA, carcinoma; CS, colostomy; DLI, diverting loop ileostomy; EMR, endoscopic mucosal resection; ESD, endoscopic submucosal dissection; F, female; HGD, high-grade dysplasia; LAR, low anterior resection; LGD, low-grade dysplasia; M, male; ND, not described; PS, proctosigmoidectomy; RA, rectal amputation; RR, rectal resection; TAMIS, transanal minimal invasive surgery; TE, transanal excision; TEM, transanal endoscopic microsurgery; TLI, temporary loop ileostomy; TM, transanal mucosectomy; TME, total mesorectal excision; TVA, tubulovillous adenoma; VA, villous adenoma.

In our opinion, a minimal invasive procedure such as TEM or TAMIS is the preferred treatment option in patients with rectal adenomas and intramucosal adenocarcinoma.^31–34 In case of T1 adenocarcinomas, complete total mesorectal excision or intensive follow-up should be discussed with the patient, as in those cases recurrence rates may be higher than smaller tumors.³⁵

In MKWS, these giant tumors are challenging and TAMIS can be extremely difficult. In experienced hands, this procedure might be more successful. Perioperative complications are rarely seen during TAMIS. Development of strictures is a frequently encountered complication after TAMIS for these large tumors (case 1). Most of them can be easily treated with transanal dilatation, or repeat TAMIS, wherein the fibrotic tissue is excised. In rare cases, a major transabdominal surgery is necessary. Another major advantage of TAMIS is the resection of the specimen in one piece. In all our cases, this could be accomplished, and proper pathology analysis can be performed. This is of utmost importance, as these large tumors may harbor an invasive carcinoma.

Endoscopic resection cannot always provide an en bloc resection because most of the large villous adenomas are covering 100% of the circumference as we showed in our cases.³⁶ After one initial attempt, EMR for large rectal adenomas appears to be less effective but safer than TEM.^37,38 Endoscopic submucosal dissection (ESD) is another endoscopic option, it provides a submucosal dissection and accurate staging in case of malignancy. However, ESD compared with EMR is technically more challenging and, therefore, has still not gained popularity.^39,40

Complete cure can be expected with (laparoscopic) abdominal procedures. These invasive procedures could have, in contrast to TAMIS, high morbidity (wound infection, anastomotic leakage, fecal incontinence, and urinary and sexual dysfunction) and sometimes the need of a stoma.^26–30

In conclusion, 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. In this report, we showed that TAMIS is feasible and safe for the treatment of MKWS, and in our clinic it is the surgical technique of choice. Experience with TAMIS may improve results in technically demanding cases.

Footnotes

Disclosure Statement

No competing financial interests exist.

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