Gastrointestinal (GI) hemangiomas are relatively rare benign vascular tumors. Although GI hemangiomas can occur anywhere along the GI tract, they most frequently occur in the small bowel, followed by the colon and rectum¹⁾. Most patients with GI hemangiomas experience GI bleeding, which may require blood transfusion, with melena and/or hematochezia. GI hemangioma is not usually a highly suspected cause of GI bleeding in children because of its rarity. Herein, we report on 3 cases of jejunal, rectal, and multiple GI hemangiomas, presenting with GI bleeding in children.

Most patients with GI hemangioma present with melena and hematochezia as major complaints, both of which can be the cause of life-threatening anemia. In addition, patients may complain by various symptoms such as abdominal discomfort, abdominal pain, dizziness, nausea, vomiting, fatigue, malaise, and general weakness. GI hemangioma is most frequently found in the small bowel, and followed by the colon and rectum²⁾. GI hemangioma may manifest as part of Maffucci syndrome, Klippel-Trénaunay syndrome, disseminated neonatal hemangiomatosis, and blue rubber bleb nevus syndrome¹⁾. GI hemangioma can be histologically classified as capillary, cavernous, and mixed types. Of these types, cavernous hemangioma is the most common. In terms of clinical manifestations, capillary hemangioma is usually solitary and tends to induce anemia by minor chronic bleeding, whereas cavernous hemangioma can result in hematemesis and melena due to acute excessive bleeding. In one of the 3 cases reported here, blue rubber bleb nevus syndrome was diagnosed, accompanied by cutaneous hemangioma and multiple GI hemangiomas. Histologically, all cases were of the cavernous type of hemangioma.

The diagnostic tools that can be used irrespective of patient age and anatomical features include imaging tests such as CT, magnetic resonance imaging, abdominal ultrasonography, scintigraphy, and angiography. Esophagogastroduodenoscopy can be used depending on anatomical features of the bleeding focus, for example, when upper GI bleeding in the stomach or duodenum is suspected. Naturally, lower GI bleeding in the colon or rectum is suspected, colonoscopy can be used. Generally, experienced pediatricians may perform esophagogastroduodenoscopy or colonoscopy without severe side effects. In addition, these procedures can be used on neonates and infants who do not have specific contraindications³⁾. When the bleeding focus is suspected to be located in the small bowel, scintigraphy or capsule endoscopy can be performed. Scintigraphy may be useful when the bleeding focus is not detected by endoscopy or angiography. However, this method does have limitations if there is no active bleeding site or an intermittent bleeding site⁴⁾. Capsule endoscopy has been reported to be useful in the diagnosis of small bowel hemangioma in young infants⁵⁾. However, capsule endoscopy is not considered feasible for young infants who can barely swallow capsules and have a high risk of developing obstruction. Accordingly, the optimal diagnostic method has not yet been established for neonates and infants.

For the treatment of GI hemangioma, it is preferable that patients are observed and followed up if there are no overt symptoms or the patient has small, single lesions with mild symptoms. However, conservative treatment such as red blood cell transfusion or administration of iron supplements can be used if necessary. For medical care, corticosteroids, antiangiogenic agents, and interferon alpha can be administered. Corticosteroids have been the drug of choice for GI hemangioma for several decades. Corticosteroids are known to be suppressed the expression of vascular endothelial growth factor (VEGF)-A. interferon alpha have been reported to be an effective treatment for diffuse hemangiomatosis. Recently, enteroscopic excision was considered for cases of small hemangioma⁶⁾.

Surgical intervention should be considered for the patients who do not respond to medical care. This includes patients with large lesions and comorbidities, with multiple lesions with symptoms, or with severe GI bleeding⁷⁾. The surgical method is dependent on the location of the lesions. For the lesions in the stomach, wedge resection, antrectomy, subtotal gastrectomy, or total gastrectomy can be performed. For the small bowel lesions, segmental resection can be performed⁸⁾. Lesions in the colon can be treated with ileocecectomy, right hemicolectomy, left hemicolectomy, or segmental resection. Finally, lesions in the rectum can be treated with low anterior resection.

In the case of blue rubber bleb nevus syndrome, a careful approach is required for selecting the appropriate surgical treatment modality because surgical outcomes may be limited and recurrence is possible⁹⁾. Recent technological advances such as thermocoagulation have facilitated the management of patients with blue rubber bleb nevus syndrome. Recently, the success of pharmacologic treatment has been reported¹⁰⁾. Corticosteroids, antiangiogenic agents, and interferon alpha can be administered for blue rubber bleb nevus syndrome. Patients with blue rubber bleb nevus syndrome have recurrent massive GI bleeding despite corticosteroids, antiangiogenic agents and interferon alpha therapy, low-dose sirolimus may be useful. Sirolimus is thought to act by blocking the VEGF-mediated pathwasys.

In conclusion, we have reported on 1 case of small bowel hemangioma, 1 case of rectal hemangioma, and 1 case of blue rubber bleb nevus syndrome. A better knowledge and understanding of GI hemangioma should help reduce the delay in diagnosis and avoid inappropriate management. Although rare, hemangioma should be considered as a differential diagnosis for cases of GI bleeding.