Blue rubber bleb nevus syndrome coexisted with intestinal intussusception: a case report

Abstract

Blue Rubber Bleb Nevus Syndrome (BRBNS) is an uncommon congenital disorder characterized by sporadic venous malformation which mainly occurs in skin and alimentary canal. Here, we report a BRBNS patient with concomitant intestinal intussusception who diagnosed by intraoperative endoscopy and ultimately managed using surgical resection. A 19-year-old boy was referred to urgent surgery for acute melena and stomachache. He had used to be a long-term iron user for undiagnosed chronic anemia and papules. Abdominal CT on admission demonstrated the presence of intestinal intussusception. The following exploratory laparotomy and intraoperative endoscopy revealed multiple gastrointestinal hemangiomas. The postoperative course was uneventful and pathological examination certified multiple cavernous hemangiomas in the resected gastrointestines.

Blue rubber bleb nevus syndrome

“…Rarely, abdominal pain, intussusception, volvulus, infarction were its complications. [ 13 , 14 ] On the morphological aspects, just like Kang Kook Choi's report, GI BRBNS could arise in diverse forms, including polylobulated, nodular, sessile, pedunculated, or ulcerated, [ 15 ] and submucosal protrusive was also its appearance in our patients. Venous malformations could distribute throughout any segment of GI tract from the oral mucosa to the anal canal and mostly located in small intestine and distal colon.…”

Postoperative disseminated intravascular coagulation in a pregnant patient with Blue Rubber Bleb Nevus Syndrome presenting with acute intestinal obstruction: Case report and literature review

“…Some authors highlight the risk of short gut syndrome in extensive resections, and recurrence [8] , [9] . For overt hemorrhages or complications such as torsion, intussusception or rupture, operative intervention is mandatory and resection of the affected segment may be required [3] , [5] , [7] , [14] .…”

Surgical Emergencies in Intestinal Venous Malformations

“…2,9 Other less common symptoms reported with small series and cases reports include abdominal pain, obstruction, intussusceptions, and volvulus. 4,5,10 The diagnosis, as stated earlier, is usually a challenge. Some patients have skin stigma or are already diagnosed with syndromes that have associated intestinal malformations, which can help us focus on the treatment.…”

“…2 In conclusion, the intestinal location of the VM can cause severe complications that require emergency surgery, such as the cases described in the literature and in our study. 4,5,10 These must be kept in mind, regardless of its low incidence. Fig.…”