Eradication of angiomas by nylon loop snare ligation under total enteroscopy, in disseminated intravascular coagulation associated with blue rubber bleb nevus syndrome

Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital disease characterized by multiple systemic venous malformations on the skin and internal organs, including the gastrointestinal (GI) tract [1]. The GI lesions sometimes cause severe bleeding episodes, which require urgent care. In addition, BRBNS is complicated by focal and systemic disseminated intravascular coagulation, leading to hemorrhagic diathesis, which makes physicians decide against surgical procedures or endoscopic sclerotherapy [2].

A 57-year-old man who had previously been diagnosed with BRBNS complicated by disseminated intravascular coagulation, complained of repeated melena. Enhanced computed tomography revealed multiple vascular malformations in the intestine ([Fig. 1]), and capsule endoscopy (PillCam SB3; Covidien Japan, Tokyo, Japan) demonstrated numerous angiomas in the intestine ([Fig. 2]). He received conservative therapy with transfusions, iron supplementation, and antihyperfibrinolytic treatment for disseminated intravascular coagulation, including direct oral anticoagulants, without improvement. Apixaban was also ineffective in controlling GI bleeding and anemia, and the patient was referred to our department.

Fig. 1 Dynamic contrast-enhanced computed tomography (portal-venous phase) in a patient with blue rubber bleb nevus syndrome complicated by disseminated intravascular coagulation, showing multiple enhanced nodules in the intestine (yellow arrowheads).

Fig. 2 Capsule endoscopy identified jejunal angiomas. The surface of the angiomas is erosive, and abnormally dilated vessels are visible.

Because apixaban prevented intravascular consumptive coagulopathy that might increase the risk of bleeding paradoxically, we decided to perform nylon loop snare ligation (PolyLoop; Olympus, Tokyo, Japan) [3] [4] under anticoagulant therapy with apixaban. The patient underwent retrograde and anterograde double-balloon enteroscopy (DBE) (EN-450T5/W; Fujifilm, Tokyo, Japan) to achieve total enteroscopy [5] and loop ligation for every possible lesion (two and eight lesions; retrograde and anterograde, respectively) ([Video 1]). Subsequent capsule endoscopy revealed residual lesions in the jejunum. Therefore, we performed additional anterograde DBE (ligation for three lesions) and accomplished radical removal of potential bleeding sources in the small bowel ([Fig. 3]). In the series of endoscopic interventions, we experienced no complications, including that of severe bleeding. During the 2-year follow-up, the patient did not complain of anemia or melena.

Video 1 Nylon loop snare ligation of small-bowel venous malformations in a patient with blue rubber bleb nevus syndrome complicated by disseminated intravascular coagulation.

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Fig. 3 Anterograde double-balloon enteroscopy identified the jejunal angioma that was a possible cause of gastrointestinal bleeding. a The surface of the angioma appears hyperemic and erosive. b The same location 3 weeks after ligation. The angioma has completely disappeared (yellow arrow).

Nylon loop snare ligation facilitated by DBE is a curative and safe tool to treat small-bowel lesions in BRBNS under anticoagulant therapy.

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Competing interests

The authors declare that they have no conflict of interest.

Acknowledgments

We thank Jane Charbonneau, DVM, from Edanz (https://jp.edanz.com/ac) for editing a draft of this manuscript.

• References

• 1 Bean W. Vascular spiders and related lesions of the skin. AMA Arch Derm 1959; 80: 131
• 2 Yamada S, Arahata M, Morishita E. et al. Blue rubber bleb nevus syndrome complicated by enhanced-fibrinolytic-type DIC: a case report. Ann Vasc Dis 2021; 14: 252-255
• 3 Jin J, Pan J, Zhu L. Therapy for hemangiomas of blue rubber bleb nevus syndrome in the small intestine with single balloon endoscopy. Dig Endosc 2015; 27: 781-781
• 4 Campos-Murguía A, Zamora-Nava LE. An endoscopic multimodal approach in a patient with blue rubber bleb nevus syndrome and acute bleeding. Endoscopy 2021; 53: E338-E339
• 5 Yamamoto H, Sekine Y, Sato Y. et al. Total enteroscopy with a nonsurgical steerable double-balloon method. Gastrointest Endosc 2001; 53: 216-220

Publication History

Article published online:
28 October 2022