Idiopathic Intrasyringal Hemorrhage: A Case Report and Review of the Literature

Abstract

Abstract INTRODUCTION Intrasyringal hemorrhages, also known as Gower's hemorrhages, are exceedingly rare entities. There are sporadic case reports of intrasyringal hemorrhages arising from intramedullary tumors, trauma, anticoagulation, or syringomyelic cavities secondary to Chiari malformations. Management is typically tailored to the underlying pathology. Given the paucity of literature, the clinical course and management modalities remain poorly defined. METHODS We present the case of a 19-yr-old male with a complex pasat medical history significant for Pierre Robin sequence, cervicomeddulary dysfunction with posterior fossa decompression and occipital-cervical fusion, hydrocephalus with fourth ventricular outlet fenestration, ventriculoperitoneal shunt conversion to ventriculoatrial shunt, and complex syringomyelia with thoracic syringo-subarachnoid shunt. Our patient presented to us with 3 wk of acute decompensation with complete loss of lower extremity function and a mid-thoracic sensory level. An magnetic resonance imaging (MRI) demonstrated an extensive dilation of his known syrinx with associated accumulation of heterogeneous blood products within the cavity encapsulating the syringo-subarachnoid shunt. RESULTS We performed a prompt revision thoracic laminectomy to identify the patient's existing syringo-subarachnoid shunt. Once the shunt was identified we performed a myelotomy overlying the existing shunt, and immediately noted high pressure chronic hematoma exiting from the myelotomy site. We placed a new syringo-subrachnoid shunt and gently advanced the catheter superiorly while gently irrigating the syrinx cavity. We continued to irrigate the cavity until the blood products cleared and fluid resembling cerebrospinal fluid returned from the cavity. The postoperative MRI demonstrated overall decreased size of the syrinx with less intrasyringal hemorrhage. The patient had a slight improvement in his thoracic sensory level at short-term follow-up. CONCLUSION Intrasyringal hemorrhages are exceedingly rare pathologic entity which may cause significant dilation of an existing syrinx even in the presence of a syringo-subrachnoid shunt. Exam features are variable but may demonstrate an acute and drastic progression. Prompt surgical intervention may improve functional neurologic prognosis.