Adult intussusception with perforation caused by hemangioma in the distal ileum

The “Endothelialized Muscularis Mucosae”: A Case Report Describing a Large Cavernous Hemangioma at the Terminal Ileum and a New Histologic Clue for Preoperative Diagnosis from Endoscopic Biopsy

“…Intestinal hemangiomas are very rare, with multiple histologic variations accounting for a combined incidence of only 0.05% of all gastrointestinal tract (GI) tumors [ 3 ]. They are more likely to be found in the jejunum [ 4 ], in contrast with most other GI tumors, only 3–6% of which reside in the small bowel [ 1 ].…”

“…They are more likely to be found in the jejunum [ 4 ], in contrast with most other GI tumors, only 3–6% of which reside in the small bowel [ 1 ]. A literature search for cavernous hemangiomas of the terminal ileum revealed only one prior case, which described a 5 cm cavernous hemangioma that was associated with perforation located near the origin of an ileocecal intussusception [ 3 ].…”

“…In our patient, the blood noted in the abdominal cavity had likely been causing her sharp abdominal pain secondary to peritoneal irritation. Intestinal hemangiomas have also been associated with complications such as intussusception and perforation [ 3 , 11 ], or platelet sequestration leading to thrombocytopenia [ 4 ]. In some cases, these complications can be life threatening.…”

“…When they are visualized on endoscopy, features of a cavernous hemangioma may include a purple or blue color, a nodular gross appearance, and hypervascularity, possibly with friability or visible bleeding [ 7 , 8 , 12 ]. Adjacent features may include mucosal congestion and submucosal dilated varicosities [ 3 , 12 ]. Other diagnostic modalities, such as radionucleotide imaging or angiography showing hypervascularity, or barium studies demonstrating a nodular luminal defect which is compressible upon air insufflation, may help localize the lesion but cannot render a specific diagnosis [ 11 , 12 ].…”

“…Cavernous hemangiomas of the gastrointestinal tract are quite rare and, until now, difficult to diagnose preoperatively [ 1 ] due to a dearth of specific histologic description pertaining to small superficial biopsies such as those obtained endoscopically [ 2 , 3 ]. We report a unique case of a 4 cm transmural cavernous hemangioma in the terminal ileum with literature review and describe a new histologic finding—the “endothelialized muscularis mucosae,” which was discovered upon review of the endoscopic biopsy and could potentially facilitate preoperative diagnosis of these lesions from endoscopic biopsies in the future.…”