Acquired Chiari malformation Type I and holocord syringomyelia associated with a high-flow supratentorial fistulous arteriovenous malformations: A case report and literature review

Abstract

Background: Chiari malformation Type I (CMI) is generally considered a congenital lesion and typically associated with syringomyelia. Acquired CMI or adult Chiari malformation caused by intracranial mass is extremely rare. Brain arteriovenous malformations (AVMs) are characteristically symptomatic due to seizure, intracranial hemorrhage, or neurological deficit. We report an extremely rare case of an acquired CMI and extensive syringomyelia associated with a large supratentorial AVM. Case Description: A 35-year-old woman was referred to our institute after a diagnosis of CMI and extensive syringomyelia from whole-spine magnetic resonance imaging (MRI) due to complaining of low back pain radiating to the right leg for the past 1 month. She had intermittent headache for 2 years. The patient underwent suboccipital decompression and C1 laminectomy followed by duraplasty. Two months later, she developed severe right-sided sciatic pain and complete right foot drop. Follow-up MRI revealed progressive enlargement of a syrinx cavity at the lower spinal cord and a large right parieto-occipital AVM with markedly dilated cortical draining veins and diffuse engorgement of dural venous sinuses was detected. This AVM supplied mainly by enlarged cortical branches of the right middle cerebral artery and posterior cerebral artery with multiple dural supplies. Endovascular treatment of a high-flow fistulous AVM was successfully performed with N-butyl cyanoacrylate (NBCA) through the hypertrophic branches of the right middle cerebral artery. Four months after embolization, the patient had recovered completely from the right foot drop. Further staged embolization was planned to reduce the size and flow of the AVM before stereotactic radiosurgery. However, the patient was lost to follow-up due to financial reason. One year later, she developed sudden severe headache followed by alteration of conscious due to intraventricular hemorrhage from the AVM, leading to obstructive hydrocephalus requiring cerebrospinal fluid diversion. During a period of 2 years, the patient underwent several staged embolization with NBCA and Onyx. Final cerebral angiography after embolization demonstrated a significant reduction in size and flow of the brain AVM. A control whole-spine MRI revealed a significant reduction in syrinx size. At the end of embolization, the patient had no neurological deficit. However, she had suffered from persistent central neuropathic pain at the right lower extremity. The AVM remnant was further treated by stereotactic radiosurgery. Conclusion: Increased cerebral venous hypertension secondary to a high-flow supratentorial AVM leading to posterior fossa venous hypertension may play a major role in the pathogenesis of CMI, induced the formation of syringomyelia. Endovascular treatment of brain AVM, the underlying cause of CMI, resulted in a significant reduction of the size of the syrinx. The need for cranial imaging in initial evaluation of cases with adult Chiari malformation is important.