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

Abstract

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 KTS who underwent successful surgical decompression of a thoraco-lumbar extradural cyst, highlighting the importance of reevaluating surgical interventions in KTS patients. A 15-year-old girl diagnosed with KTS 4 days postnatally, was referred to our clinic due to chronic back pain and spastic paraparesis. These symptoms were attributed to a compressive extradural thoraco-lumbar cyst. Diagnostic evaluations confirmed the presence of the cyst, leading to the decision to proceed with surgical intervention. 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 postoperatively, with no significant complications reported during the surgery. With this case, the authors question the fear of surgical intervention in KTS 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.