Blue Rubber Bleb Nevus Syndrome with Long-Term Follow-Up: A Case Report and Review of the Literature

Hideaki Nakajima,Hiromu Miyake,Akinori Sekioka,Koji Fukumoto,Naoto Urushihara,Yutaka Yamada,Masaya Yamoto,Akiyoshi Nomura,Hiroshi Nouso

doi:10.1155/2018/8087659

Hideaki Nakajima, Hiromu Miyake + Show 7 more

Open Access

https://doi.org/10.1155/2018/8087659

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Abstract

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease in which venous malformations (VMs) involve any body organ, most commonly the skin and the gastrointestinal (GI) tract. Treatment of BRBNS aims at preserving the GI tract as much as possible. Although there are several dozen case reports about BRBNS that describe short-term clinical courses, a few provide an account of long-term clinical course. Here, we report a case of BRBNS in a girl that required multiple abdominal surgeries due to the GI VMs and a recurrence at an interval of 14 years. The preferred approach for gastrointestinal VMs involves the complete resection of all lesions without residual VMs. It is important to bear in mind the possibility of delayed recurrence of GI VMs after surgical or endoscopic treatment.

Highlights

Blue rubber bleb nevus syndrome (BRBNS) is a rare condition, characterized by multiple venous malformations (VMs) of the skin and gastrointestinal (GI) tract [1, 2]
We report the case of BRBNS with GI lesions involving long-term follow-up and multiple surgical treatments
We provide a brief review of the literature regarding longterm follow-up of GI lesions of BRBNS treated endoscopically or surgically

Summary

Introduction

Blue rubber bleb nevus syndrome (BRBNS) is a rare condition, characterized by multiple venous malformations (VMs) of the skin and gastrointestinal (GI) tract [1, 2]. The GI lesions could cause abundant bleeding and lead to severe anemia. If GI bleeding becomes uncontrollable by conservative treatment, endoscopic or surgical intervention is required. Short-term postoperative courses were mostly uneventful; some cases had prolonged courses owing to residual lesions or recurrence. We report the case of BRBNS with GI lesions involving long-term follow-up and multiple surgical treatments. We provide a brief review of the literature regarding longterm follow-up of GI lesions of BRBNS treated endoscopically or surgically

Case Presentation

Discussion

Conflicts of Interest