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  • Updated 08.19.2020
  • Released 12.05.2001
  • Expires For CME 08.19.2023

Syringomyelia

Introduction

Overview

In this article, the author reviews the early and late manifestations of syringobulbia, the differential diagnosis of syringomyelia and hydromyelia, and the 90% chance of long-term stabilization or improvement with surgical treatment in Chiari-related syringomyelia. Syringomyelia and associated conditions, such as arachnoiditis and the Chiari I anomaly, are now readily diagnosed by MRI. An associated Chiari anomaly may be overlooked by MRI in the supine position. Misdiagnosis may occur if sagittal imaging alone is used because sagittal MRI might overestimate the degree of tonsillar ectopia in Chiari I malformation. To distinguish central canal dilatations from syringomyelia, cine MRI will demonstrate no flow signal inside the dilatation and no obstruction of CSF flow adjacent to it, unlike in syringomyelia. In posttraumatic syringomyelia, a disturbance in the removal of K ions in tissue surrounding the syrinx cavity may contribute to water accumulation in the injured spinal cord, leading to syrinx formation or exacerbation of the underlying pathology.

Key points

• MRI is the diagnostic modality of choice for syringomyelia and associated conditions, such as Chiari I anomaly or arachnoiditis, and for the differential diagnosis of syringomyelia and hydromyelia.

• Preventive measures for the development of syringomyelia include the careful use of epidural anesthesia and the avoidance of a traumatic or prolonged labor and maneuvers that may induce “craniospinal” dissociation, such as Valsalva maneuver.

• Pain is a frequent and disabling manifestation of syringomyelia and a persistent symptom in a significant percentage of patients despite successful drainage of the syrinx.

• Chiari I patients may suffer from nonsuboccipital headaches, not triggered by the Valsalva maneuver.

• Some patients show progressive neurologic deterioration and disabling pain despite proper surgical drainage of the syrinx and correction of the associated craniovertebral anomaly.

• In children with Chiari I malformation and syringomyelia, the presence of comorbidities at birth has a negative impact on the result of surgical procedures.

Historical note and terminology

Ollivier d’Angers first coined the name "syringomyelia" in 1824 (51), but the condition was first recognized by Stephanus in 1545 (66). Schultze first described its clinical picture (62); its relation to Chiari malformation was first outlined by John Cleland (13). Abbe performed the first reported surgical drainage of a syrinx in 1891 (01), although claims were made that Horsley aspirated a syrinx in 1890 (53). The “hydrodynamic” theory of syringomyelia was proposed by Gardner (18) and modified by Williams' “craniospinal pressure dissociation” theory (74).

The term “syringomyelia” is used to indicate the presence of a fluid-filled cavity within the spinal cord. "Hydromyelia" refers to dilatation of the central canal of the spinal cord: cavities partially or completely lined by ependymal cells. Syringomyelia indicates cavities that lie outside the central canal and do not have ependymal lining but may have partial connection with the central canal. “Communicating” syringomyelia refers to cavities with a direct communication with the fourth ventricle through the obex and is usually associated with hindbrain malformations. “Noncommunicating” syringomyelia refers to cavities without communication with the fourth ventricle and is usually secondary to trauma and tumors of the spinal cord but is also associated to hindbrain malformations. The famous golfer Robert “Bobby” Jones suffered from syringomyelia and it has been speculated that the well-known cellist Jacqueline du Pret might have suffered from syringomyelia rather than multiple sclerosis.

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