Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 16, 2022; 10(32): 11929-11935
Published online Nov 16, 2022. doi: 10.12998/wjcc.v10.i32.11929
Blue rubber bleb nevus syndrome complicated with disseminated intravascular coagulation and intestinal obstruction: A case report
Jian-Hua Zhai, Shi-Xin Li, Ge Jin, Yuan-Yuan Zhang, Wei-Long Zhong, Yan-Fen Chai, Bang-Mao Wang
Jian-Hua Zhai, Shi-Xin Li, Yan-Fen Chai, Department of Emergency Medicine, Tianjin Medical University General Hospital, Tianjin 300052, China
Jian-Hua Zhai, Ge Jin, Wei-Long Zhong, Bang-Mao Wang, Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, Tianjin 300052, China
Yuan-Yuan Zhang, Department of Radiology, Tianjin Medical University General Hospital, Tianjin 300052, China
Author contributions: Zhai JH and Li SX were the patient’s attending doctors; Zhai JH reviewed the literature and contributed to manuscript drafting; Li S collected the clinical data; Jin G and Zhong WL collected the enteroscopy images; Zhang YY analyzed and interpreted the imaging findings; Chai YF and Wang BM were responsible for the revision of the manuscript; All authors issued final approval for the version to be submitted.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All of the authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Bang-Mao Wang, MD, PhD, Chief Doctor, Chief Physician, Department of Gastroenterology and Hepatology, Tianjin Medical University General Hospital, No. 154 Anshan Road, Heping District, Tianjin 300052, China. mwang02@tmu.edu.cn
Received: June 24, 2022
Peer-review started: June 24, 2022
First decision: August 21, 2022
Revised: September 16, 2022
Accepted: September 23, 2022
Article in press: September 23, 2022
Published online: November 16, 2022
Processing time: 137 Days and 1.3 Hours
Abstract
BACKGROUND

Blue rubber bleb nevus syndrome is a rare vascular malformation syndrome with unclear etiopathogenesis and noncurative treatments. It is characterized by multiple vascular malformations of the skin, gastrointestinal tract, and other visceral organs. The most common symptoms are intermittent gastrointestinal bleeding and secondary iron deficiency anemia, thus requiring repeated blood transfusions and hospitalizations. It is easily missed and misdiagnosed, and there is no specific treatment.

CASE SUMMARY

We report a case of blue rubber bleb nevus syndrome combined with disseminated intravascular coagulation and efficacy of treatment with argon plasma coagulation under enteroscopy and sirolimus. A 56-year-old female patient was admitted to the hospital with 3-year history of fatigue and dizziness that had aggravated over the past 10 d with melena. The patient had a history of repeated melena and multiple venous hemangiomas from childhood. After treatment with argon plasma coagulation combined with sirolimus for nearly 8 wk, the patient’s serum hemoglobin increased to 100 g/L. At the 12-mo follow-up, the patient was well with stable hemoglobin (102 g/L) and no recurrent intestinal bleeding.

CONCLUSION

Argon plasma coagulation and sirolimus may be an efficacious and safe treatment for blue rubber bleb nevus syndrome, which currently has no recommended treatments.

Keywords: GI bleeding, Disseminated intravascular coagulation, Argon plasma coagulation, Sirolimus

Core Tip: We present a case of blue rubber bleb nevus syndrome (BRBNS) complicated with disseminated intravascular coagulation and intestinal obstruction. BRBNS is a rare disease characterized by multiple vascular malformations of the skin, gastrointestinal tract, and other visceral organs. It is easily missed and misdiagnosed, and there is no unified treatment. We treated the patient with antifibrinolytic medication, sirolimus and argon plasma coagulation under enteroscopy. We followed up the patient for 12 mo, she was well with stable hemoglobin level and the size of skin hemangioma became smaller. In a patient with obscure, recurrent intestinal bleeding a diagnosis of BRBNS should be considered.