An Uncommon Cause for a Common Scenario

Cavernous hemangiomas are among the rarest causes of gastrointestinal bleeding. Here, we report a case of hematochezia resulting from an extensive cavernous hemangioma involving the rectum. We also highlight the difficult therapeutic challenges that can be posed by these lesions.

Case Report

A 20-year-old woman with complaints of hematochezia of two days duration was evaluated. The patient had a history of cavernous hemangioma of the buttocks, left hip, and posterior thigh since the age of 3. Her hemangioma had been treated with approximately 40 percutaneous transcatheter procedures with both sclerosing agents and coil embolizations. These treatments were complicated by damage to the patient’s left sciatic nerve and by subsequent left lower extremity paralysis.

Upon presentation to the hospital, the patient was hemodynamically stable and her hemoglobin was normal. On physical examination, it was noted that she had some bluish skin discoloration on the buttocks, as well as maroon blood on digital rectal examination. She subsequently underwent a colonoscopy that showed several polypoid, red, vascular-appearing lesions approximately 10 cm from the anal verge (Figure 1). No active bleeding was noted. An endoscopic ultrasound confirmed an extensive network of blood vessels (Figure 2). Computed tomography scan of the abdomen revealed a low-density 3.4-cm × 3.2-cm mass abutting the left wall of the rectum and extending from the soft tissues of the left pelvic floor to the soft tissues of the left hip (Figure 3). An angiogram confirmed the vascular nature of the lesion but did not find any major arterial feeders to the hemangioma amenable to embolization.

Figure 1.

Colonoscopy showing several vascular-appearing lesions approximately 10 cm from the anal verge. Some lesions were polypoid and red (A), whereas other lesions had a bluish hue (B).

Figure 2.

Endoscopic ultrasound showing extensive network of blood vessels.

Figure 3.

Computed tomography scan of the abdomen and pelvis demonstrating an ill-defined, low-density 3.4-cm × 3.2-cm mass abutting the left wall of the rectum. The lesion extended from the soft tissues of the left pelvic floor to the soft tissues of the left hip (A and B) and also showed some phlebolith formation (B).

The patient’s case and potential treatment options were discussed with specialists from interventional radiology, surgery, and gastroenterology. It was agreed that, unfortunately, treatment would be very difficult given that her hemangioma had progressed in size despite 40 prior embolizations. Different surgical options, including a left hemicolectomy with colo-anal anastomosis; an abdominoperineal resection with colostomy; or mucosectomy procedures, were contemplated and discussed with the patient. It was decided that any surgical procedure would be very risky and bloody given the extent of hypervascularity in the area. Therefore, it was decided to follow her expectantly and to proceed with surgery only in the case of significant, recurrent, or life-threatening bleeding. The patient experienced no further bleeding episodes during the 5 days she was hospitalized, and she was discharged. In a follow-up visit 1 year later, the patient reported only intermittent minor bleeding episodes with no significant progression of symptoms and decided to continue to be followed expectantly.

Discussion

Described as early as the 1800s, hemangiomas have been known to be among the rarest causes of gastrointestinal bleeding.^1,2 Hemangiomas may be classified as capillary, cavernous, or mixed types³ and are thought to be benign masses that have been present since birth.

Colonic hemangiomas usually present with occult bleeding or with slow painless hematochezia. However, massive gastrointestinal hemorrhages have also been known to occur, and a case report of cavernous hemangiomas presenting as bowel obstruction has also been reported.⁴ Most colonic hemangiomas occur in the left colon, though some may occur in the right colon.⁵

The diagnosis is best established by endoscopic visualization of a blood-filled hemangioma that has an appearance of plum-red nodules or vascular congestion. Abdominal radiographs may show phlebolith formation. It has been proposed that phlebolith formation may be related to thrombosis in a cavernous hemangioma due to perivascular inflammation and stasis of blood flow.^5-7 Computed tomography scans and magnetic resonance imaging can also be used for diagnosis and evaluation of the extent of the lesion. Computed tomography scans may reveal thickened colonic wall and pelvic phleboliths. Magnetic resonance imaging may show colon hemangiomas as bright heterogeneous signal intensity on T2 images.⁴

Small hemangiomas that are solitary can potentially be treated by endoscopic means. Amano and associates reported successful treatment of a 33-mm × 22-mm × 14-mm pedunculated, solitary cavernous hemangioma using endoscopic polypectomy.⁸ However, this is not effective on larger lesions. Embolization of the blood vessels has variable success and may not be effective in some cases. Surgical resection is generally required for larger lesions.^9,10 Among the possible surgical approaches, abdominoperineal resection has been used when the perineum is involved and low anterior resection has been used when there is no perineal involvement.^11,12 Several sphincter-saving mucosal resection techniques have also been proposed.¹³ Some have suggested that removal of the involved engorged friable rectal mucosa and use of a colo-anal sleeve anastomosis may control bleeding from rectal cavernous hemangiomas.^13,14 The type of surgery performed should depend upon the surgeon’s expertise and the extent and location of the lesion.

Our case presentation demonstrates a rare occasion of a very large hemangioma involving a significant portion of the body. Its large size made surgical resection difficult and risky, and the presence of multiple collateral vessels with limited arterial supply made embolization an option of limited value. Large hemangioma lesions represent a difficult therapeutic challenge.