Background:
Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare condition which usually manifests as multiple hemangioma-like skin and gastrointestinal lesions. The latter often present with chronic bleeding. There is no consensus regarding the optimal management of such patients. Although rare, complications such as intestinal intussusception might occur, demanding surgical treatment. Postoperative complications such as coagulation disorders can increase morbidity and should be timely addressed. This is the first report of a life-threatening postoperative disseminated intravascular coagulation in such patients. The main objectives of this case report are to present diagnostic and treatment features of this condition and, more importantly, address the optimal management of postoperative disseminated intravascular coagulation.
Case presentation:
Twenty-five year-old female pregnant patient presents to the emergency department with colicky pain and oligohydramnios. After C-section, persistent symptoms and further investigation led to the diagnosis of intestinal intussusception. After surgical management she showed clinical and laboratory signs of disseminated intravascular coagulation (DIVC), which was corrected with transfusional therapy and intraperitoneal clot evacuation. After optimal management, she was discharged home. Sirolimus was initiated further improving her condition.
Conclusion:
This rare presentation of acute intestinal intussusception in a patient with Blue Rubber Bleb Nevus Syndrome was further complicated with postoperative coagulation disorder. Prompt surgical evaluation is essential especially when complications are suspected. Operative treatment might be necessary in the emergent setting. Close monitoring of infectious and coagulation parameters is essential in the postoperative period, and aggressive treatment should be timely initiated when disseminated intravascular coagulation is suspected.