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Vascular Malformation
Joseph Junewick, MD FACR
over 6 years ago
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Gastrointestinal Hemangiomatosis

Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: MR CT
Activities:
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History

3 month old with protuberant abdomen and anemia.


Case Images


Diagnosis

Gastrointestinal Hemangiomatosis

Findings

CT – Diffuse mesenteric and enteric hemangiomatous infiltration. Note the marked enlargement of the portal venous system and marked attenuation of the abdominal aorta distal to the superior mesenteric artery.

MR – Coronal T2 images confirm the extensive hemangiomatous involvement of the bowel and mesentery. MR arteriography and venography also confirm the vascular alterations noted on CT.

Discussion

Gastrointestinal hemangiomatosis is a complex vascular malformation that occurs primarily in infancy and childhood although occasionally it is not diagnosed until adulthood. The clinical presentation is variable, although most patients present with gastrointestinal bleeding. Patients may also present with intussusception, small-bowel obstruction, perforation, or malabsorption. Cutaneous hemangiomas are often present. Gastrointestinal hemangiomatosis may be associated with blue rubber bleb nevus syndrome, Olser-Weber-Rendu, Klippel-TrĂ©naunay-Weber syndrome, Maffucci’s syndrome, Kasabach-Merritt syndrome, vonHIppel-Lindau syndrome, diffuse neonatal hemangiomatosis, and Proteus syndrome. Hemangiomatosis is manifested by diffuse infiltration of the intestinal wall, the mesentery, and, occasionally, the retroperitoneum. Solid organs in the abdomen may also be involved.

The radiographic findings of hemangiomatosis include the presence of phleboliths on abdominal radiographs, scattered submucosal small-intestine nodules on barium examination, and mural thickening with phleboliths on CT. If gastrointestinal hemangiomatosis is suspected, water or fat enteric contrast should be employed instead of positive contrast.

Numerous therapies have been used in an attempt to treat hemangiomas when complications develop during the proliferative phase. The prolonged use of systemic agents during the period of endothelial proliferation is often associated with increased side effects. The current first line of treatment is systemic administration of corticosteroids. Approximately 30% of hemangiomas will respond dramatically to corticosteroids and another 40% will have some response. Side effects of steroid therapy include severe irritability, weight gain, cushingoid appearance, growth delay, hypertension, diabetes, gastroesophageal reflux, and susceptibility to infections. Other antiangiogenesis drugs such as vincristrine and vincristine regimens (VAC) are also successful. Alpha-interferon therapy has been used in the past but is associated with irreversible neurologic spastic diplegia.

Reference

Scafidi DE, McLeary MS, Young LW. DIffuse neonatal gastrointestinal hemangiomatosis: CT findings. Pediat Radiol (1998); 28(7):512-514.

Levy AD, Abbott RM, Rohrmann CA, Frazier AA, Kende A. Gastrointestinal Hemangiomas: Imaging Findings with Pathologic Correlation in Pediatric and Adult Patients. AJR (2001); 177:1073-1081.

Donnelly LF, Adams DM, Bissett GS. Vascular Malformations and Hemangiomas: A Practical Approach in a Multidisciplinary Clinic. AJR (2000); 174:597-608



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