Spinal Arteriovenous Malformation Associated with Parkes Weber Syndrome: Report of Two Cases and Literature Review.

ABSTRACT

OBJECTIVE: To present 2 cases of Parkes Weber syndrome (PWS) with spinal arteriovenous malformation (AVM) and discuss the radiologic features and clinical treatment with literature review. METHODS: Clinical data on 2 patients with PWS with spinal AVM was acquired in a prospective follow-up investigation. Clinical manifestations, radiographic features, procedural results, and follow-up outcome were collected and reviewed together with a literature review. RESULTS: The first patient presented with limb weakness and urinary dysfunction and the second with repetitive subarachnoid hemorrhage followed by paraplegia. Limb hypertrophy, skin ulceration, and extensive microfistulas in the affected limb were observed in both patients. Spinal AVM was confirmed by digital subtraction angiography and endovascular embolization was performed. The first patient experienced limb amputation at 6-year follow-up as a result of chronic ulceration and the second did not have neurologic improvement. After literature review, 15 cases (male/female ratio, 510; mean age, 22±10.4 years) were included. The presentations comprised subarachnoid hemorrhage in 6, radicular pain in 5, myelopathy in 4, and asymptomatic in 1. Embolization was performed in 9 cases, solitary surgery in 2, and combined therapy in 4. Among 10 cases with known follow-up results, 6 achieved neurologic recovery after surgery and 1 died after solitary surgery. CONCLUSIONS: Awareness of the association between spinal AVM and PWS is essential for radiographic screening of spinal lesions with myelopathy or intracranial subarachnoid hemorrhage. Clinical therapeutic strategy should be multidisciplinary and individualized on the basis of vasculature and lesion behavior.