Surgical management of a thoraco-lumbar extradural cyst in a pediatric patient with Klippel-Trenaunay Syndrome: A case report

Abstract

Introduction and Importance: Klippel-Trenaunay Syndrome (KTS) is a congenital disorder characterized by the abnormal development of blood vessels, soft tissues, bones, and the lymphatic system. The syndrome is rare, with few cases reported worldwide, especially those describing an association between KTS and spinal extradural meningeal cysts (SEMC). This report highlights a rare case of a pediatric patient with Klippel-Trenaunay Syndrome who underwent successful surgical decompression of a thoracolumbar extradural cyst, highlighting the importance of reevaluating surgical interventions in KTS patients. Case Presentation: A 15-year-old girl diagnosed with Klippel-Trenaunay Syndrome four days postnatally, was referred to our clinic due to chronic back pain and spastic paraparesis. These symptoms were attributed to a compressive extradural thoracolumbar cyst. Diagnostic evaluations confirmed the presence of the cyst, leading to the decision to proceed with surgical intervention. Clinical Discussion: The surgical approach involved a laminoplasty at T11-T12-L1, resulting in the total removal of the cystic lesion. The patient exhibited a complete resolution of symptoms post-operatively, with no significant complications reported during the surgery. Conclusion: With this case, we question the fear of surgical intervention in Klippel-Trenaunay Syndrome patients, which is often avoided due to concerns of high-risk complications like excessive bleeding or poor wound healing, and hint at a possible association between KTS and Extradural Meningeal Cysts.