What is the treatment for spinal syringohydromyelia?

Treatment of Spinal Syringohydromyelia

For symptomatic syringomyelia associated with Chiari I malformation, perform posterior fossa decompression with or without duraplasty as first-line surgical treatment, and wait 6-12 months before considering additional intervention if the syrinx persists. 1, 2

Treatment Strategy Based on Etiology

The surgical approach must be tailored to the underlying cause of the syrinx, as treatment directed at normalizing CSF flow dynamics produces the best outcomes. 3

Chiari Malformation-Associated Syrinx (Most Common)

Primary Surgical Options:

• Posterior fossa decompression (PFD) alone OR PFD with duraplasty (PFDD) are both acceptable first-line treatments for symptom improvement (Grade C recommendation). 1, 2
• Surgeons may add cerebellar tonsil resection or reduction during the procedure to enhance syrinx resolution and symptom improvement (Grade C recommendation). 1, 2

Critical Timing Consideration:

• Do not rush to reoperation: Allow 6-12 months after initial surgery for syrinx improvement before considering additional neurosurgical intervention (Grade B recommendation). 1, 2, 4, 5
• Reoperation for persistent syrinx is potentially beneficial only if radiographic improvement has not occurred within this 6-12 month window. 1, 2

Important Caveat:

• Symptom resolution and syrinx resolution do not correlate directly—patients may improve clinically even without complete radiographic syrinx collapse. 1, 2, 4

Syrinx with Hydrocephalus

For communicating syrinxes (anatomically continuous with the 4th ventricle) occurring with hydrocephalus:

• Ventriculoperitoneal shunt placement is the empirical treatment of choice, achieving excellent results in the majority of cases. 6

For Chiari II malformations with hydrocephalus:

• VP shunt placement produces excellent outcomes in all treated patients. 6

Syrinx Secondary to Extramedullary Compression

For syrinxes caused by mass lesions or spinal deformity:

• Excision of the obstructive lesion at the rostral end of the syrinx results in cavity collapse or disappearance in the majority of patients. 6, 7
• Treatment must address the primary pathological problem; syrinx drainage alone without treating the underlying cause produces poor results. 7, 3

Post-Traumatic Syringomyelia

Shunting procedures are effective:

• Syringoperitoneal or syringocisternal shunt placement achieves good results in post-traumatic cases. 6

Idiopathic Syrinx (No Demonstrable Cause)

For cases without identifiable etiology:

• Syringosubarachnoid shunting is the ideal treatment, producing favorable outcomes. 7

Diagnostic Workup Before Treatment

Complete imaging is mandatory:

• Obtain MRI of the entire brain and spine to evaluate for hydrocephalus, complete extent of syringomyelia, tethered spinal cord, and Chiari malformation (Grade C recommendation). 2, 5
• Brain imaging must be included even when syrinx is detected on spine imaging, as Chiari malformation is the most common underlying cause. 5

Assess for craniocervical instability:

• Some patients require decompression AND fusion of the craniocervical junction, which should be evaluated preoperatively with appropriate imaging. 2

Special Populations

Terminal Syringohydromyelia with Occult Spinal Dysraphism

For large, symptomatic terminal syrinxes:

• Shunting procedures are necessary and effective, particularly with progressive symptoms. 8
• Complete or significant syrinx resolution occurs in the majority of shunted patients, with complete pain resolution in all patients presenting with back pain. 8

Atrophic Syrinxes with Myelomalacia

Conservative approach:

• Surgery is not indicated for the syrinx itself; operate only to relieve symptoms from the causal lesion. 6

Common Pitfalls to Avoid

• Do not perform syrinx shunting alone in Chiari malformation cases without posterior fossa decompression—this produces poor outcomes. 7
• Do not operate on asymptomatic syrinxes detected incidentally, particularly small ones in adolescents with scoliosis. 1
• Do not rely solely on radiographic improvement as the indicator of surgical success; clinical outcome is more reliable. 7
• Do not obtain routine CT imaging for initial evaluation—MRI is the gold standard. 5