Wilkie's syndrome causing persistent vomiting post-colectomy.

INTRODUCTION: Wilkie's syndrome occurs when the superior mesenteric artery (SMA) compresses the third part of the duodenum causing proximal dilatation of small bowel. It is due to loss of the fat pad that sits between the duodenum and SMA and therefore is most commonly seen in people who have had significant weight loss. PRESENTATION OF CASE: A 79 year old man presented with anaemia and weight loss and an ascending colon malignancy was found. He was built up nutritionally and subsequently underwent a right hemicolectomy. Post operatively he had persistent nausea and vomiting and duodenal compression by the SMA was identified on CT imaging. He was managed initially with parenteral nutrition then gradually enteral feeding was introduced via a nasojejunal tube. Symptoms resolved with weight gain and he was discharged well. DISCUSSION: This condition often presents insidiously with intermittent nausea and vomiting. It infrequently occurs with a more acute onset in surgical patients. It has a classic appearance on CT imaging and is usually managed with conservative treatment although surgical options are feasible if this fails.
CONCLUSION: Wilkie's syndrome is an interesting and infrequent cause of small bowel obstruction following colorectal surgery. A high index of suspicion is required.

Introduction

Wilkie's syndrome is an interesting phenomenon that occurs when the superior mesenteric artery (SMA) compresses the third part of the duodenum. It is eponymous with Wilkie, who published the first case series of 75 patients in 1927 and is also known as SMA syndrome and Cast syndrome. The SMA branches off the aorta at the level of L1 and passes anteriorly and inferiorly traversing over the duodenum, left renal vein and the uncinate process of the pancreas. It was first described in 1842 by Rokitansky and occurs due to loss of the fat pad that is normally between the duodenum and SMA. The presence of the fat pad normally creates an angle of more than 35° between the SMA and the aorta however this is decreased to less than 25° in Wilkie's syndrome with resultant vascular compression of the third part of the duodenum (Fig. 1).

Fig. 1

CT image showing compression of the duodenum (D) between the aorta (A) and the superior mesenteric artery.

Radiological confirmation of SMA syndrome (by contrast CT, fluoroscopy and or MR angiography) classically demonstrates the decreased aortomesenteric angle with dilatation of the second part of the duodenum and compression of the third part. The aortomesenteric distance is less than 8 mm. On fluoroscopy, improvement in duodenal transit may be demonstrated with the patient in the left lateral decubitus position.

Presentation of case

A 79 year old man was admitted electively for surgery after investigations for weight loss and symptomatic anaemia revealed a hepatic flexure tumour. He had a complex surgical history including an open appendicectomy, open cholecystectomy, partial gastrectomy (for peptic ulcer disease in 1975), colonic resection and subsequent stoma reversal. The patient was given three weeks of oral nutritional supplements and gained 2 kg prior to admission. An open right hemicolectomy was performed following 2 h of dissection to divide extensive adhesions. Due to the complexity of the operation and the patients weight loss a defunctioning ileostomy was fashioned. Post-operatively he developed an intra-abdominal collection, which was successfully drained radiologically. Despite initial recovery and improvement in inflammatory markers following drainage the patient had persistent nausea and vomiting two weeks post operatively. This required parenteral feeding and frequent aspiration of small bowel content from a nasogastric tube. There was an associated 14% weight loss and electrolyte disturbances. CT and contrast studies revealed distension of the second part of the duodenum with compression of the third part of the duodenum by the SMA. Based on the clinical and radiological findings, a diagnosis of Wilkie's syndrome was suspected. Conservative management was continued with parenteral feeding and careful management of electrolyte abnormalities. Enteral feeding was gradually introduced, with feeding initially via an endoscopically sited nasojejunal tube. The patient gained 5 kg and symptoms resolved, corroborating with our diagnosis. He was discharged home well.

Discussion

Wilkie's syndrome is rare and much of the literature pertains to case reports and small case series. It is most frequently described in patients who have undergone spinal correction surgery, thereby distorting the vascular anatomy and decreasing the aortomesenteric angle, and patients who have illnesses such as malignancy, malabsorption syndromes, and other disorders associated with extreme weight loss. Interestingly a recent case series identified mental and behavioural disorders as an important and frequent co-morbidity. Patients may present insidiously with a longstanding history of intermittent nausea, vomiting and epigastric pain, which classically is relieved by lying in the left lateral decubitus position. Alternatively, acute onset may occur in association with rapid weight loss, particularly in surgical patients.

Conservative management is attempted initially through high calorie intake via parenteral feeding, decompression of the small bowel with a nasogastric tube, fluid resuscitation and correction of electrolyte abnormalities. Enteral feeding may be introduced with a nasojejunal tube placed distal to the obstruction.

If conservative management fails there are surgical options which can be considered. “Strongs procedure” was the original procedure described and involves dividing the ligament of Treitz, then mobilising and repositioning the duodenum so it does not lie between the aorta and SMA. Gastrojejunostomy and duodenojejunostomy are alternative options, the duodenum is often divided during the latter. These procedures have more recently been performed laparoscopically and the anastomses formed using an endoscopic gastrointestinal anastomotic (GIA) stapler. They have been reported as safe and feasible techniques. Experience is limited due to the sporadic presentation of the disease and the infrequent number of operations associated with it however outcomes have been favourable with limited complications in these small studies.

Conclusion

Wilkie's syndrome is rarely seen following colorectal surgery therefore a high index of suspicion is necessary. Radiological findings confirm diagnosis and conservative treatment is usually successful.

Conflict of interest

The authors declare no conflict of interest.

Funding

The authors did not receive any sources of funding for this research.

Ethical approval

Written informed consent was obtained from the patient for publication of this case report and accompanying image. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Author contributions

Collection of data and writing were done by Naomi Fearon. Helen Mohan helped in writing and editing the manuscript. The study was designed and edited by Des Winter.