1214. Spinal arteriovenous malformation and subarachnoid hemorrhage associated with Parkes Weber Syndrome: a case report and literature review
Authors: DAVID MAURICIO SOLANO VARELA; ALBERTO DAZA OVALLE; HUMBERTO MADRIÑAN; FRANCISCO PEREZ; WILLIAM CORTES LOZANO; WILLIAM RIVEROS (Bogota D.C, Colombia)
Parkes weber syndrome (PWS) is an uncommon congenital vascular malformation (VM) disorder characterized by capillary malformation, AVM, soft-tissue and/or bone hypertrophy. This entity rarely affects CNS, however, SAH without intracranial malformations could be a sign of rupture of spinal high-flow AVM into subarachnoid space. There is not stablished treatment in the literature. The aim of this paper is to describe a case report and perform literature review.
A 21-year-old male with PWS complaining by sudden headache and lumbar pain. CT showed SAH and cerebral arteriography was negative for vascular lesions. Lumbosacral spine MRI showed a lesion suggestive of VM with confirmatory spinal arteriography for intramedullary AVM. We performed a systematic review (Pubmed/Medline, Cochrane and Embase) and XX publications fulfilled the eligibility criteria.
Due to intramedullary AVM originated in the anterior spinal artery for conus medullaris at L4 level with significant extension was performed onyx embolization in two stages, achieving partial occlusion in the first procedure. Later, a new episode of SAH was diagnosed and second onyx embolization was performed with near total occlusion achieved obviating the need of surgical treat. The patient was discharged at 4 days without complications. The patient was followed for 1 year with MRI with improvement of the symptoms and without sequelae.
PWS is a rare disorder affecting CNS, however, the clinical presentation could include neurological manifestations as subarachnoid hemorrhage and diagnosis criteria incorporate AVM along the neuroaxis. Sometimes genetic tests are required for definitive diagnosis due to several similar entities. There are no guidelines about treatment, however, operative management is the main stain, endovascular therapy is recommended as first stage and surgical resection in selected cases. The prognosis is usually positive.