Surgical removal of a compressive thoracic epidural vascular malformation in a patient with Klippel-Trénaunay syndrome: illustrative case.

BACKGROUND

Klippel-Trénaunay syndrome (KTS) is a combined capillary-lymphatic-venous malformation disorder traditionally associated with high surgical morbidity. Although rare, pathologic involvement of the spinal cord has been reported in the literature. However, the safety of surgical intervention remains unclear. We report a case of successful decompression of a thoracic epidural lesion in an individual with KTS who presented with spastic paraparesis.

OBSERVATIONS

The patient is a 38-year-old male, diagnosed with KTS as an infant, who presented with spastic paraparesis secondary to a thoracic epidural lesion. He underwent laminectomies and resection of the lesion with subsequent improvement of his symptoms and without significant postoperative morbidity. Histopathology confirmed the lesion to be a benign vascular malformation.

LESSONS

Currently, the literature regarding management of symptomatic vascular lesions in individuals with KTS supports nonoperative management, due to the increased risk of operative morbidity associated with this syndrome. This case presents evidence for safe and appropriate surgical management of a thoracic epidural vascular malformation in a patient with KTS in the setting of progressive neurological decline, establishing a role for neurosurgical intervention in this high-risk population when no conservative management portends further neurological deterioration.