Waugh syndrome: a report of 7 patients and review of the published reports

BACKGROUND AND OBJECTIVES: Waugh syndrome (WS) is the association of intussusception and intestinal malrotation. The association is rarely reported in the literature though intussusception is a commonly encountered problem in pediatric patients as a cause of intestinal obstruction. We present our experience in 7 patients with a review of published reports. DESIGN AND SETTING: Retrospective analysis of 7 patients with the diagnosis of Waugh syndrome who were treated at our department between February 1982 to December 2012. PATIENTS AND METHODS: Seven patients with Waugh syndrome presented to our unit during the period February 1982 to December 2012. The clinical findings and management are presented and discussed. RESULTS: Seven patients (three males and four females) presented with intussusception in association with malrotation. The age range was from 4 to 11 months; the patients had bilious vomiting and blood in the stool; the diagnosis was confirmed by ultrasound (2), Ba enema (2) and intraoperatively (3). All required operative intervention; either manual reduction or bowel resection and Ladd procedure; one patient died of sepsis; recurrence of obstruction was seen in another patient while the rest did well postoperatively. CONCLUSION: The relationship between intestinal malrotation and intussusceptions may be more frequent than is reported; failure of non-operative management of intussusception may be due to this association and hence brings the attention to its existence. A prospective study is needed to look for intestinal malrotation in patient with intussusceptions who undergo abdominal sonographic examination to determine the true incidence of this association. The anomaly is suspected by presence of a reversed anatomic relationship of the superior mesenteric artery and vein and in such cases to perform an upper gastrointestinal contrast study to define the exact location of the duodenojejuonal (DJ).