Definitions Definitions syringomyelia a syrinx (fluid filled cavity) within the spinal cord that progressively expands and leads to neurologic deficits syringobulbia a syrinx within the brain stem that leads to neurologic symptoms Epidemiology Synringomyelia incidence prevalence of the disease is about 8.4 cases per 100,000 people demographic occurs more frequently in men than in women usually appears in the third or fourth decade of life Etiology Pathophysiology usually result from lesions that partially obstruct CSF flow including craniocervical junction abnormalities login to view 2 more bullets spinal cord trauma login to view 2 more bullets spinal cord tumors login to view 1 more bullet post-infectious (e.g. meningitis) login to view 1 more bullet Associated conditions developmental scoliosis 25% to 80% of cases of syringomyelia may have scoliosis login to view 1 more bullet Klippel-Feil deformity Charcot's joints occurs in < 5% with syringomyelia often involves shoulder joint Presentation Syringomyelia symptoms symptoms usually begin insidiously between adolescence and age 45 occipital headache login to view 1 more bullet back pain radicular pain neurologic deficits login to view 1 more bullet physical exam reflexes login to view 4 more bullets motor login to view 2 more bullets sensory login to view 2 more bullets deformity login to view 2 more bullets Syringobulbia symptoms related to cranial nerve involvment tongue weakness and atrophy (CN XII) sternocleidomastoid & trapezius weakness (CN XI) dysphagia and dysarthia (CN IX, CN X) facial palsy (CN VII) Imaging Radiographs relative lack of apical lordosis may indicate presence of syrinx scoliosis series for evaluation of scoliosis CT not applicable for characterization of syrinx, only for associated scoliosis MRI diagnosis of syrinx made by MRI obtain MRI with gadolinium enhancement to rule out associated tumor indications for MRI in patients with scoliosis abnormal curve (e.g. double curve, apex left) neurologic deficit infantile or juvenile age at onset male gender with atypical or large curve thoracic kyphosis >30 degrees Histopathology Gross pathology cavitation of spinal cord gray matter syrinx in continuity with or adjacent to central canal inner layer of gliotic tissue Differential Diagnosis Hydromyelia Glioependymal cysts Myelomalacia Cystic tumors Persistent central canal Treatment Nonoperative observation indications login to view 2 more bullets Operative decompression of the foramen magnum and upper cervical cord +/- shunting indications login to view 4 more bullets technique login to view 1 more bullet outcomes login to view 3 more bullets spinal fusion indications login to view 1 more bullet technique login to view 2 more bullets Complications In general, same as for idiopathic scoliosis Increased risk of neurologic deterioration including paralysis if fusion done with undiagnosed syrinx Prognosis Natural history not well defined prognosis depends on degree of spinal deformity and neurologic deficits multiple authors report resolution or improvement in syrinx size and neurologic deficits