Surgical treatment of an adolescent with Blue rubber bleb nevus syndrome: a case report and review

О. В. Щербакова, A. O. Mager, Artur S. Gabarayev, S.M. Bataev
In: Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care · 2025 · vol. 15(3) , pp. 389–398 · doi:10.17816/psaic1928 · W4415202200
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Abstract

Blue rubber bleb nevus syndrome (Bean syndrome) is a rare congenital venous malformation most frequently localized in the skin and gastrointestinal tract. Gastrointestinal involvement may manifest with recurrent intestinal bleeding, chronic anemia, and abdominal pain. This article presents a clinical case of a rare congenital disease in an adolescent and the use of a hybrid surgical approach for vascular malformations at multiple sites. The patient, a boy, was born with a vascular lesion in the area of the anterior fontanelle. In early childhood, he underwent neurosurgery for a cerebral angioma. At the age of 13, he developed abdominal pain and was diagnosed with severe anemia, requiring blood transfusion and iron supplementation. Due to persistent anemia, further evaluation was conducted at his local healthcare facility. Fibrocolonoscopy revealed and removed a vascular lesion in the transverse colon. Repeat fibrocolonoscopy demonstrated recurrent venous malformations and new vascular lesions. To clarify the diagnosis and determine further management, the patient was referred to the Federal Scientific and Clinical Center for Children and Adolescents of the Federal Medical-Biological Agency of Russia. Based on the history, clinical examination, and diagnostic findings, Bean syndrome was suspected. A combined (hybrid) surgical procedure was performed. At the first stage, diagnostic laparoscopy revealed multiple venous malformations in the parietal peritoneum and small intestine. At the second stage, under laparoscopic control of the intestinal wall at the sites of hemangiomas, endoscopic submucosal resection of the largest venous malformation in the sigmoid colon was performed. At the final stage of the hybrid operation, wedge resection of the jejunum with a vascular lesion was carried out. Histological examination confirmed venous malformations of the small and large intestines. The patient was referred to a specialized department for targeted sirolimus therapy. This case demonstrates the potential of combined surgical intervention in pediatric patients with multiple vascular malformations. A careful analysis of clinical and anamnestic data, together with laboratory and instrumental findings, made it possible to suspect this rare syndromic condition and to plan a staged hybrid laparo-endoscopic intervention.

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