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Video case report| Volume 5, ISSUE 9, P412-414, September 2020

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Blue rubber bleb nevus syndrome in a 10-year-old child treated with loop ligation facilitated by double-balloon enteroscopy

Open AccessPublished:June 23, 2020DOI:https://doi.org/10.1016/j.vgie.2020.05.009
Blue rubber bleb nevus syndrome in a 10-year-old child treated with loop ligation facilitated by double-balloon enteroscopy
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      Blue rubber bleb nevus syndrome is an extremely rare systemic vascular disorder (with only 200 cases published to date), characterized by multiple cutaneous and GI venous malformations.
      • Beck P.L.
      • Aspinall A.I.
      • Kilvert V.M.
      • et al.
      Blue rubber bleb nevus syndrome.
      Gastrointest Endosc. 2002; 56: 598-600
      Patients present with fatigue, iron-deficiency anemia, and occult or overt obscure GI bleeding. Patients are usually managed conservatively with iron supplementation and/or blood transfusions. However, endoscopic (argon plasma coagulation, sclerotherapy, polypectomy, and ligation), radiologic, and surgical approaches are preferred for severe cases.
      • Wu C.
      • Luo R.
      • Li X.
      • et al.
      Endoscopic management of blue rubber bleb nevus syndrome in the colon with hemostatic clamp and snare.
      Endoscopy. 2017; 49: E149-E150
      ,
      • Malafaia M.C.
      • Heissat S.
      • Jacob P.
      • et al.
      Blue rubber bleb nevus syndrome: endoscopic treatment with sclerotherapy during double-balloon enteroscopy in a 9-year-old boy.
      Endoscopy. 2019; 51: E98-E100
      Systemic therapy with sirolimus appears promising.
      • Wong X.L.
      • Phan K.
      • Rodriguez Bandera A.I.
      • et al.
      Sirolimus in blue rubber bleb naevus syndrome: a systematic review.
      J Paediatr Child Health. 2019; 55: 152-155
      A 10-year-old girl with iron-deficiency anemia and multiple cutaneous lesions was diagnosed with blue rubber bleb nevus syndrome at a local hospital (Fig. 1). The patient was investigated with a small-bowel capsule endoscopy that revealed vascular lesions throughout the small bowel (Fig. 2). Consequently, the patient was referred to our institution for further management and consideration of small-bowel endotherapy owing to blood transfusion dependence and rectal bleeding.
      Figure thumbnail gr1
      Figure 1Cutaneous lesions.
      Figure thumbnail gr2
      Figure 2Small-bowel lesion seen in capsule.
      An anterograde double-balloon enteroscopy was performed with the patient under general anesthesia. Two 20-mm vascular lesions were identified in the gastric body (Fig. 3). A loop ligating device (PolyLoop; Olympus, Tokyo, Japan) was applied around the base of each lesion, tightened, and completely detached (Fig. 4; Video 1, available online at www.VideoGIE.org). After loop ligation was deployed, deflation of the lumen facilitated optimal device use and successful treatment. No further vascular malformations were found in the duodenum, jejunum, and proximal ileum. Although transfusion requirements decreased over the following 6 months, a follow-up retrograde double-balloon enteroscopy was performed because of persistent anemia. Six lesions were identified: 2 in the transverse colon, 1 in the cecum, and 3 in the distal ileum. Loop ligation was used for the 2 transverse colonic (Figs. 5 and 6) and ileal lesions (Fig. 7). The 2 remaining lesions were too flat, and loop ligation was not technically feasible. Protruding, raised lesions are usually most suited for treatment by this technique, whereas loop ligation of flat lesions has a high failure rate because application of the loop device is challenging. No immediate, early, or late adverse events occurred. At 1-year follow-up, guided by the patient’s clinical response (ie, anemia and transfusion requirements), no further GI investigation or intervention has been deemed necessary.
      Figure thumbnail gr3
      Figure 3Gastric lesion.
      Figure thumbnail gr4
      Figure 4Loop ligating devise applied around gastric lesion.
      Figure thumbnail gr5
      Figure 5Colonic lesion.
      Figure thumbnail gr6
      Figure 6Loop ligation of colonic lesion.
      Figure thumbnail gr7
      Figure 7Small-bowel lesion.
      Double-balloon enteroscopy–facilitated loop ligation appears to be a safe and minimally invasive option in patients with blue rubber bleb nevus syndrome, reducing blood transfusion dependence.

      Disclosure

      Drs Despott and Murino receive research support from Aquiliant Medical and Fujifilm and educational support from Olympus and Pentax Medical. All other authors disclosed no financial relationships.

      Supplementary data

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        Video 1

        Double-balloon enteroscopy facilitated loop ligation for the management of blue rubber bleb nevus syndrome lesions in the stomach, small bowel, and colon.

      References

        • Beck P.L.
        • Aspinall A.I.
        • Kilvert V.M.
        • et al.
        Blue rubber bleb nevus syndrome.
        Gastrointest Endosc. 2002; 56: 598-600
        View in Article
        • Wu C.
        • Luo R.
        • Li X.
        • et al.
        Endoscopic management of blue rubber bleb nevus syndrome in the colon with hemostatic clamp and snare.
        Endoscopy. 2017; 49: E149-E150
        View in Article
        • Malafaia M.C.
        • Heissat S.
        • Jacob P.
        • et al.
        Blue rubber bleb nevus syndrome: endoscopic treatment with sclerotherapy during double-balloon enteroscopy in a 9-year-old boy.
        Endoscopy. 2019; 51: E98-E100
        View in Article
        • Wong X.L.
        • Phan K.
        • Rodriguez Bandera A.I.
        • et al.
        Sirolimus in blue rubber bleb naevus syndrome: a systematic review.
        J Paediatr Child Health. 2019; 55: 152-155
        View in Article

      Article info

      Publication history

      Published online: June 23, 2020

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      DOI: https://doi.org/10.1016/j.vgie.2020.05.009

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      © 2020 American Society for Gastrointestinal Endoscopy. Published by Elsevier Inc.

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