Synchronous multiple small bowel intussusceptions in an adult with blue rubber bleb naevus syndrome: Report of a case and review of literature

Abstract

Background: Blue rubber bleb naevus syndrome (BRBNS), is an uncommon condition characterised by cavernous haemangiomas of skin and gastrointestinal tract. The most common complication of this syndrome is gastrointestinal bleeding. Intussusception of bowel, although a known complication, has rarely been reported.

Case presentation: We report the case of a 37-year-old man who presented with multiple intussusceptions of small bowel. He required an urgent laparotomy and bowel resections. He suffered from BRBNS. This is the first reported case of multiple synchronous intussusceptions affecting both jejunum and ileum, secondary to haemangiomas occurring in an adult with BRBNS. The underlying conditions of acute abdomen in patients with BRBNS may include intramural haemorrhage, infarction, volvulus or intussusception of bowel. Treatment options include pharmacological manipulation, bowel resection, and interventions such as sclerotherapy, angiographic embolisation, endoscopic ligation, electrocautery and laser photocoagulation for visceral lesions.

Conclusion: A high index of suspicion is required whilst dealing with acute abdomen in patients with BRBNS. Clinical trials may provide some answers as to the preference of treatment in individual cases, as the current level of evidence does not offer a clear choice of optimal treatment.

Figure 1

Cutaneous haemangiomas affecting right chest wall.

Figure 2

CT scan of abdomen showing i) multiple intussusceptions demonstrating doughnut signs (white arrows), intussusceptiens [A], intussusceptum [B], distended loop of small bowel [C] and ii) haemangioma of right quadratus lumborum muscle (dark arrow).

Figure 3

Specimen of limited right hemicolectomy showing terminal ileum [A], caecum [B] and ascending colon [C], and demonstrating multiple haemangiomas affecting mucosa and submucosa (arrows).