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.

Foot Macrodactyly Associated with Klippel-Trenaunay Syndrome

Klippel-Trenaunay syndrome is a rare congenital malformation which may be associated with macrodactyly. The main problem is the need for custom-made shoes. We describe the case of a female newborn affected by Klippel-Trenaunay syndrome, with a larger and longer right foot; the difference increased progressively and, at 5 years of age, the right foot was 50% larger and 38% longer than the left one. Due to the progression of the deformity, reduction surgery was advised to reduce the foot’s width. Resection of the second ray and 2nd cuneiform was performed. The result was excellent and there were no complications during a 10-year follow-up period with a decrease of width and length difference to 10% and 4%, respectively, in comparison to the contralateral foot. There were no gait anomalies. Abnormal foot width and length represented the main problems and guided the surgical strategy. Second ray resection was effective, without complications and with a good long-term functional outcome. Possibility of wearing fashionable conventional shoes without insoles was achieved to the patient’s satisfaction

Breast Hemangioma: Unique Presentation In a Patient with Klippel-Trenaunay-Syndrome

Background: Klippel–Trénaunay-Syndrome (KTS) is characterized by triad of varicose veins, port wine stain and soft tissue or bony hypertrophy and the diagnosis of KTS can be made if any two of these three features are present. Hemangiomas in various location e.g. skull, brain, epidural and vertebral hemangioma, mediastinal, colonic hemangioma, intraneural/intramuscular hemangiomas are reported with KTS. Case Presentation: Benign vascular tumors may rarely develop malignant transformation as Bugarin-Estrada et al reported breast angiosarcoma in a patient diagnosed as Klippel-Trenaunay-Syndrome. We reported a case of a 40-year-old female with known case of Klipple-Trenaunay-Syndrome with left leg varicosities, cutaneous nevus as well as unfortunate development of deep venous thrombosis and markedly enlarged right breast hemangioma. Due to low incidence or lack of early detection of breast hemangioma, its diagnosis is challenging. Conclusion: The history of patient and multi-modality imaging utilization can help in early and accurate diagnosis of diseases leading to better prognosis.