Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology
The patient was a 15 year old female with vascular hamartomatous malformations of the skin, stomach, small bowel and colon. The most severely affected region of skin was the right forearm, which was often painful. Past surgical history was remarkable for reductive surgery of the right forearm. The GI tract vascular malformations caused chronic GI bleeding which has resulted in 20 blood transfusions. Past surgical history was also remarkable for endoscopic band ligation of the GI vascular malformations which was unsuccessful.
Clinical Physical Exam:
Black-blue rubbery subcutaneous nodules were present on the right neck, left mandible, back of neck, upper back, soles of both feet, left fourth toe, and posterior aspect of the right upper extremity.
Hematocrit was normal during this admission
Clinical Differential Diagnosis:
Blue rubber bleb nevus syndrome
On the day before admission the patient had a Nuclear Medicine GI bleeding study which showed pooling of tracer in multiple discrete areas in the abdomen and pelvis that accumulated tracer over time. Tracer uptake was also noted in the right forearm and no GI bleeding was noted during the course of the exam.
On the day of admission the patient had an angiographic study of the mesenteric vessels and right arm which demonstrated venous malformations in the small bowel, ascending colon, and right forearm.
The patient, on the same day, had lesions in the right forearm, right supra-clavicular and left submandibular areas sclerosed using direct ethanol injection.
The patient did well post sclerosis and subsequently returned and had a repeat sclerosis of the right forearm lesions 7 weeks later in order to further shrink the lesions.
Imaging Differential Diagnosis:
Blue rubber bleb nevus syndrome
A decision was made to try to provide a curative solution to the patient's problems of recurrent GI bleeding via surgical intervention. Two weeks later, via a midline incision, 37 vascular malformations from the small bowel and colon were resected using 20 enterostomies and 2 local small bowel resections with end-to-end anastomoses. Seventeen smaller vascular malformations in the small bowel and two in the colon were directly injected and sclerosed with desiccated alcohol.
Examination of the surgical specimen revealed intestinal type tissue with extensive submucosal and muscular vascular malformations (cavernous hemangiomas) composed of cavernous spaces partially filled with blood and thrombus. Dilated lymphatic spaces were also present.
Blue Rubber Bleb Nevus Syndrome
Follow-up and Prognosis:
The patient has done very well post operatively.
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