Epidural spinal cord compression in a patient with blue rubber bleb nevus syndrome

Abstract

<jats:p>Blue rubber bleb nevus syndrome (BRBNS) can present with vascular malformations throughout the body, especially in the gastrointestinal tract. Spinal cord compression from these lesions is rare, particularly in the pediatric population. The authors report a case of BRBNS involving an 18-year-old female patient who presented with back pain and an epidural thoracic mass with cord compression. She underwent an uncomplicated thoracic laminectomy and decompression, with removal of what appeared to be a venous malformation. Postoperatively her pain improved, and imaging revealed resolution of cord compression. Pathological analysis highlighted dilated venous channels with myxoid degeneration in the wall with clot, characteristic of BRBNS. The early age of presentation and location are unique based on the literature search of BRBNS. The present report highlights the multiplicity of venous malformations in BRBNS, and the management of this case.</jats:p>

Low‐dose sirolimus controls recurrent iron deficiency in a patient with blue rubber bleb nevus syndrome

“…The patient has had chronic occult blood loss and recurrent episodes of frank GI bleeding since early childhood, that led to treatment with red blood cell transfusions and intravenous iron therapy. She has multiple BRBNS lesions on the skin and along the GI tract, a large oral mucosal lesion, and a prior surgically removed lesion in the thoracic spine . She was previously treated with propranolol, with no clinical improvement in GI bleeding.…”

Síndrome de nevus azules cauchosos: reporte de un caso familiar

“…Otras posibles complicaciones diferentes a la hemorragia son las calcificaciones y, en casos menos frecuentes, la presencia de coagulopatía de consumo, trombocitopenia y, por supuesto, todas las complicaciones secundarias a la hemorragia según su volumen y tiempo de instauración (2,3) . Además de las manifestaciones en la piel, la formación de estos angiomas en otros órganos puede llevar a hemorragia digestiva y anemia ferropénica, hemorragia en el sistema nervioso central (SNC) y en algunos casos malformaciones orbitales, intususcepción, vólvulo, isquemia intestinal y compresión medular (4) . Jin y colaboradores, en una revisión de la literatura, analizaron 120 reportes de casos, donde el 87% de los pacientes presentaba compromiso de más de un órgano, 97% tenía compromiso en la piel, 76% presentó hemangiomas digestivas y 13%, compromiso del SNC (5) .…”

A case of spinal epidural venous malformation with mediastinal extension: management with combined surgery and percutaneous sclerotherapy

“…4,15,18 While spinal epidural arteriovenous malformations, fistulas, and shunts are extensively reported, spinal extradural venous malformations are rare. 8,14,23,26 To the best of our knowledge, this is the first report of a spinal epidural and paraspinal venous malformation precipitating the formation of a syrinx, which was managed in a multidisciplinary fashion with surgery and subsequent percutaneous sclerotherapy. The clinical manifestations, imaging and pathological findings, and outcomes of surgery and percutaneous interventional sclerotherapy are discussed with a review of the literature.…”