Syringomyelia Treatment & Management

Updated: Jul 08, 2016
  • Author: Hassan Ahmad Hassan Al-Shatoury, MD, PhD, MHPE; Chief Editor: Selim R Benbadis, MD  more...
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Treatment

Medical Care

No medical treatment is known for patients with syringomyelia. However, a chronic, stable clinical course is common. Identifying the underlying cause of syrinx formation is very important. Surgical treatment most likely will be necessary.

Neurorehabilitative care facilitates preservation of remaining neurological functions and prevents complications of quadriparesis such as infection and decubitus ulcers.

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Surgical Care

A variety of surgical treatments have been proposed for syringomyelia and are discussed in the sections that follow.

Suboccipital and cervical decompression

This operation includes suboccipital craniectomy; laminectomy of C1, C2, and sometimes C3; and duraplasty. [12, 13]

Some authors report microsurgical lysis of any adhesions, opening of the fourth ventricular outlet, and plugging of the obex (later steps are based on Gardner's hydrodynamic theory).

Laminectomy and syringotomy (dorsolateral myelotomy)

After decompression, the syrinx is drained into the subarachnoid space through a longitudinal incision in the dorsal root entry zone (between the lateral and posterior columns), usually at the level of C2-C3.

Incision in the dorsal root entry area has the minimum risk of increasing neurological deficit.

Shunts

The following types of shunts may be indicated:

  • Ventriculoperitoneal shunt - Indicated if ventriculomegaly and increased intracranial pressure are present
  • Lumboperitoneal shunt - Placed infrequently because of increased risk of herniation through the foramen magnum
  • Syringosubarachnoid dorsal root entry zone shunt
  • Syringoperitoneal shunt

Percutaneous needling

This technique is advocated as a possible mode of therapy; however, rapid refilling of the hydromyelic cavity from the ventricular system follows aspiration of fluid at the time of surgery. Moreover, a needle track seems unlikely to remain open.

Terminal ventriculostomy

The terminal ventricle is the dilated portion of the central canal that extends below the tip of the conus medullaris into the filum terminale. A laminectomy is performed over the caudal limit of the fluid sac, and the filum is opened.

This procedure is suitable only in patients with symptoms of syrinx without Chiari malformation. It is inappropriate in cases in which the hydromyelic cavity does not extend into the lumbar portion of the spinal cord or into the filum terminale.

Neuroendoscopic surgery

This technique is particularly useful in evaluating and treating multiple septate syrinxes.

A fibroscope inserted through a small myelotomy allows inspection of the intramedullary cavity. Septa are fenestrated, either mechanically or by laser. Fluid from the cavity is then shunted into the subarachnoid space.

Surgical untethering in select cases with posttraumatic tethering associated with syringomyelia [14]

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