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 KTS who underwent successful surgical decompression of a thoraco-lumbar extradural cyst, highlighting the importance of reevaluating surgical interventions in KTS patients. Case presentation: 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. 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 postoperatively, with no significant complications reported during the surgery. Conclusion: 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.
ABSTRACT
INTRODUCTION: Cavernomas are rare vascular lesions that can occur anywhere along the neuraxis. However, they are most commonly found in the cerebral hemispheres. Spinal cavernomas are more uncommon and intradural extramedullary cavernomas are the most uncommon as they constitute only 3 % of spinal cavernomas. PRESENTATION: A 36-year-old female presented to our neurosurgical clinic with a history of back pain radiating to the left side of the chest with left lower extremity paresthesia and ataxia without urinary disturbance. Neurological exam showed left-sided hypoesthesia below the T9 dermatome in addition to increased patellar and Achilles reflexes on the left side. MRI showed a homogeneous intradural extramedullary mass which was hyperintense on T1 and hypointense on T2 and it was surgically resected. Pathological examination confirmed the diagnosis of intradural extramedullary cavernoma. DISCUSSION: Intradural extramedullary cavernomas are extremely rare lesions that arise within the area located between the inner surface of the dura and the pial surface of the spinal cord. Up until 2022 only 40 cases of intradural extramedullary cavernomas were reported in the literature. MRI is the preferred imaging modality and pathology is the golden standard for diagnosis. Surgical resection showed very promising results and it is considered the golden standard for treating this condition. However, surgery should be performed urgently to give an optimal outcome. CONCLUSION: Clinicians should consider this condition in their differential diagnoses when faced with progressive spinal root compression symptoms, sudden onset myelopathy, or progressive subarachnoid hemorrhage.
