The post–syrinx syndrome: stable central myelopathy and collapsed or absent syrinx

Abstract

Among 168 cases with neurologic findings of cervicothoracic syringomyelia and MRI findings of Chiari 1 malformation and/or underdevelopment of the posterior cranial fossa, 15 patients (9.1%) had collapsed, flat syrinxes and 14 patients (8.3%) did not have syrinxes. Both groups of patients had clinical findings of central myelopathy that had been stable for at least 3 years. Magnetic resonance imaging detected atrophy of the cervical spinal cord in both groups and spontaneous communications between the syrinx and the subarachnoid space in 3 patients of the group with collapsed syrinxes. Analysis of these results and review of the literature suggest that patients with clinical signs of syringomyelia and Chiari 1 malformation or underdeveloped posterior fossa, but with small or absent syringomyelitic cavities, have the "postsyrinx" state as a result of spontaneous collapse of distended syrinxes.

Time course of syringomyelia resolution following decompression of Chiari malformation Type I

“…In the current study relief of distension of the spinal cord rather than complete disappearance of syrinx fluid was the key to clinical improvement because patients with a small residual fluid-filled cavity within the spinal cord were not at greater risk of persistent symptoms than patients with complete disappearance of the syrinx. 2 The rate of syrinx narrowing after surgery could not be predicted by the size of the syrinx, age of the patient, or duration of symptoms before surgery. …”

Epidural anesthesia for Cesarean delivery in a patient with post-traumatic cervical syringomyelia

“…A prevalence as high as 130 per 100,000 has been reported recently in some regions of the Russian Federation. 7,8 It is estimated that 1-9% of patients with spinal cord trauma have the likelihood of developing symptomatic syringomyelia within 30 years of trauma. 5 Although a few cases of peripartum management of patients with syringomyelia have been reported in the literature, these reports are described in the context of congenital form and not PTS.…”

Identifying Myelopathy Caused by Thoracic Syringomyelia: A Case Report

“…Because delay of progression of symptoms is the primary outcome of surgery, duration of symptoms 6,18 and pre-operative level of function 15,16 signifi cantly infl uence the functional outcome of each surgical case. Th is further illustrates the need for prompt recognition of myelopathy and appropriate referral and intervention for the underlying condition.…”

“…Syringomyelia is an intramedullary lesion in which a fl uid-fi lled cyst, also known as a syrinx, forms within the spinal cord 1,[5][6][7][8][9][10][11] . Th e pathophysiology of syringomyelia is widely debated although it is generally accepted that fl uid pressure imbalance leads to the fl ow of fl uid into the syrinx 12,13 .…”

“…Surgical intervention is typically recommended to treat this condition, although a successful procedure oft en is limited to providing minimal neurological improvement or simply halting the deterioration 6,[15][16][17][18] . Because delay of progression of symptoms is the primary outcome of surgery, duration of symptoms 6,18 and pre-operative level of function 15,16 signifi cantly infl uence the functional outcome of each surgical case.…”