An unusual cluster of Waugh syndrome as a cause of intestinal obstruction in children - A case series.

Abstract

Introduction and importanceThe association of intussusception with intestinal malrotation is a rare phenomenon named Waugh syndrome by Brereton et. It is a rare disease entity with less than 100 cases described so far. A cluster presentation is something unusual for rare diseases.Case presentationAll four patients have presented with features suggestive of bowel obstruction as all of them have had colicky abdominal pain. Three patients have vomiting as an additional complaint, so as the diarrhea. Fever has also been reported in one patient. Patients have presented in 6 months period, non-consecutively. Abdominal pain, diarrhea and vomiting are alarming in the pediatric population. When each case presented, an immediate resuscitation was performed including IV lines, nasogastric suctioning, electrolytes correction, and blood and FFP transfusion as necessary. Abdominal ultrasonography was performed revealing target sign. After optimization for surgery, manual reduction of the intussusception was done followed by a Ladd's procedure for the malrotation was performed for all 4 patients with excellent outcomes.Clinical discussionWith the surgery, after case optimization, remains the standard of care; the presence of cluster presentation for Waugh syndrome could suggest that some environmental factors might be implicated and further observations can be carried out to look more into such phenomenon.ConclusionWaugh syndrome, a combined presentation of intestinal malrotation and intussusception, is a rare syndrome that can present in children. Optimization for surgery followed by a 2-step surgical approach is the usual treatment with excellent outcomes described.