Marakhouski K, Sharafanovich E, Kolbik U, Sautin A, Nikalayeva K, Pataleta A, Sanfirau K, Svirsky A. Endoscopic treatment of blue rubber bleb nevus syndrome in a 4-year-old girl with long-term follow-up: A case report. World J Gastrointest Endosc 2021; 13(3): 90-96 [PMID: 33763189 DOI: 10.4253/wjge.v13.i3.90]
Corresponding Author of This Article
Aleh Sautin, MBBS, Pediatric Gastroenterology Fellow, Surgeon, Diagnostic Division, Republican Scientific and Practical Center of Pediatric Surgery, Nezavisimosti Ave 64A, Minsk 220013, Belarus. sautin.oleg@gmail.com
Research Domain of This Article
Pediatrics
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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: http://creativecommons.org/licenses/by-nc/4.0/
Kirill Marakhouski, Aleh Sautin, Aleh Pataleta, Kiryl Sanfirau, Diagnostic Division, Republican Scientific and Practical Center of Pediatric Surgery, Minsk 220113, Belarus
Elena Sharafanovich, Uladzislau Kolbik, Department of Elective Surgery, Republican Scientific and Practical Center of Pediatric Surgery, Minsk 220113, Belarus
Katsiaryna Nikalayeva, Aliaksandr Svirsky, Department of Pediatric Surgery, Republican Scientific and Practical Center of Pediatric Surgery, Minsk 220013, Belarus
Author contributions: Marakhouski K, Pataleta A and Sanfirau K conceived of and designed the work; Sharafanovich E contributed to conception of the work; Pataleta A and Svirsky A contributed to design of the work; Marakhouski K, Kolbik U, Nikalayeva K, Pataleta A and Svirsky A collected the literature data; Kolbik U, Sautin A and Nikalayeva K collected the patient’s clinical data; Marakhouski K, Sharafanovich E, Kolbik U, Sautin A, Nikalayeva K, Pataleta A, Sanfirau K and Svirsky A performed data analysis; Sharafanovich E and Sanfirau K interpreted the data; Marakhouski K, Sharafanovich E and Sanfirau K performed the patient’s diagnostic tests; Kolbik U and Sautin A plotted the hemoglobin dynamics; Sautin A generated a visual table for enteroscopy; Sharafanovich E provided the final diagnosis; Marakhouski K treated the patient; Marakhouski K, Sharafanovich E, Sanfirau K and Svirsky A drafted and critically revised the manuscript for important intellectual content; Kolbik U contributed to drafting of the manuscript; Sautin A performed text structuring and critical revision of the manuscript for important intellectual content; Nikalayeva K drafted the manuscript and performed text structuring and critical revision of the manuscript; all authors gave final approval for the version to be published.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest related to this case or its publication.
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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Aleh Sautin, MBBS, Pediatric Gastroenterology Fellow, Surgeon, Diagnostic Division, Republican Scientific and Practical Center of Pediatric Surgery, Nezavisimosti Ave 64A, Minsk 220013, Belarus. sautin.oleg@gmail.com
Received: November 4, 2020 Peer-review started: November 4, 2020 First decision: December 18, 2020 Revised: January 1, 2021 Accepted: January 28, 2021 Article in press: January 28, 2021 Published online: March 16, 2021 Processing time: 125 Days and 0.7 Hours
Abstract
BACKGROUND
Blue rubber bleb nevus syndrome (BRBNS) is a rare vascular disease, difficult to diagnose and choose a treatment method, especially in young children. There are several limiting factors to the use of enteroscopy for diagnostics and treatment in pediatric patients, in general. The literature on BRBNS cases is limited and presents various therapeutic approaches.
CASE SUMMARY
We present here a case of BRBNS involving a 4-year-old female, whose intestinal venous lesions were successfully treated by endoscopic sclerotherapy and aethoxysklerol foam. Skin lesions, typical for BRBNS, appeared on the 8th d of the child’s life and their number increased over the next several months. The child also experienced episodes of critical decrease in hemoglobin level (by as much as 52 g/L) for several years, requiring iron supplementation and several blood transfusions. Video capsule endoscopy revealed numerous vascular formations in the small bowel. The combined findings of gastrointestinal venous formations and skin lesions prompted BRBNS diagnosis. Single-balloon enteroscopy was used to perform sclerotherapy, with aethoxysklerol foam. A positive effect was observed within 19 mo of follow-up. We continue to monitor the patient’s hemoglobin level, every 2 wk, and it has remained satisfactory (> 120 g/L).
CONCLUSION
Endoscopic sclerotherapy can be effective in the clinical management of gastrointestinal manifestations of BRBNS in young children.
Core Tip: In blue rubber bleb nevus syndrome (BRBNS), vascular malformations can affect any organ in the body but skin and gastrointestinal tract are the most frequent. Skin venous malformations have been observed in patients with BRBNS since childhood, with number and size of lesions increasing through time. Gastrointestinal lesions also occur at an early age and provoke gastrointestinal bleeding, leading to anemia. Treatment of the clinical manifestations of BRBNS can be carried out by endoscopic, pharmacological or surgical approaches. We present here a BRBNS case in a young child, treated by sclerotherapy with aethoxysklerol foam applied during single-balloon enteroscopy.
