Post-traumatic syringomyelia: a review

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Abstract

More than a quarter of spinal cord injured patients develop syringes and many of these patients suffer progressive neurological deficits as a result of cyst enlargement. The mechanism of initial cyst formation and progressive enlargement are unknown, although arachnoiditis and persisting cord compression with disturbance of cerebrospinal fluid flow appear to be important aetiological factors. Current treatment options include correction of bony deformity, decompression of the spinal cord, division of adhesions, and shunting. Long-term improvement occurs in fewer than half of patients treated. Imaging evidence of a reduction in syrinx size following treatment does not guarantee symptomatic resolution or even prevention of further neurological loss. A better understanding of the causal mechanisms of syringomyelia is required to develop more effective therapy.

Introduction

Syringomyelia is a heterogeneous collection of conditions characterized by the presence of abnormal fluid filled cavities (syringes) within the spinal cord. The aetiology of the condition remains enigmatic and treatment results are often unsatisfactory.1 Patients developing syringomyelia after suffering a spinal cord injury pose particularly difficult management problems (Fig. 1). The abundance of aetiological theories and management practices is testament to a lack of understanding of even the basic mechanisms of cyst formation and enlargement. The aim of this review is to offer guidance for diagnosis and management of patients with post-traumatic syringomyelia, whilst providing an update on current research findings that may lead to new treatment directions.

Section snippets

Definitions

Although first described in 1546 by Charles Estienne, the term syringomyelia was suggested in 1827 by Ollivier d’Angers after the Greek συριγξ (syrinx), meaning pipe, tube, or channel and μυελoς (myelos), meaning marrow.[2], [3] Later, hydromyelia was used to indicate a dilatation of the central canal, and syringomyelia referred to cystic cavities separate from the central canal.[4], [5], [6] Syringohydromyelia and hydrosyringomyelia have been used in the literature to encompass all syringes,

Epidemiology

Syringomyelia affects mainly children and young adults, presenting on average before the 29th year.[9], [14], [15] Estimates prior to widespread MRI availability indicate a prevalence of 9 per 100,000, and an incidence of 0.44 cases per year, suggesting 1500 people in Australia and 22,000 in the United States are affected.[16], [17], [18], [19], [20] Prevalences as high as 130 per 100,000 have been reported for some regions of the Russian Federation.21 Half the patients presenting with

Classification and pathology

Milhorat et al.[36], [37], [38] have classified syringes (Fig. 2) according to pathological and MRI findings into: (a) communicating central canal syringes, (b) noncommunicating central canal syringes, (c) noncommunicating extracanalicular syringes, (d) atrophic cavitations, and (e) neoplastic cavities. Communicating central canal syringes are central canal dilatations in continuity with the fourth ventricle and are often associated with hydrocephalus. They occur in children and young adults

Aetiology

Despite intensive speculation, the mechanism of post-traumatic syrinx formation and route and source of fluid flow have remained unclear. Previous investigations and theories have concentrated on canalicular syringomyelia associated with Chiari malformations, often ignoring post-traumatic cases.[16], [41], [42], [43] Early hydrodynamic theories proposed that fluid flowed into the central canal from the fourth ventricle due to arterial or respiratory pressure effects. The explanation for

Clinical presentation

Post-traumatic syringomyelia develops between 3 months and 34 years following spinal cord injury.[22], [29], [31], [37] Symptomatic progression is usually gradual, although sudden deterioration from haemorrhage into a syrinx has been described.9 Common initial symptoms include segmental pain and sensory loss.[9], [29], [31], [32], [34] Pain is dull, aching, or burning in nature, reflecting injury to spinothalamic pathways.[9], [29], [31] The pain is often at or above the level of injury, and

Diagnosis

Syringomyelia was difficult to diagnose before the development of modern neuroimaging techniques.[64], [65], [66], [67] Plain CT is unreliable for the diagnosis, primarily because of degradation of the spinal cord image by the effects of surrounding bone.[27], [34] Combined with contrast myelography, CT is capable of demonstrating subarachnoid adhesions, but 10–50% of syringes are not detected with this technique.[68], [69], [70], [71]

Currently, MRI is the imaging modality of choice for the

Natural history

Most patients demonstrate slow progression of symptoms and signs.[21], [66], [85], [86], [87] A few progress more rapidly, sometimes immediately after myelography or from haemorrhage into the syrinx from vessels in the wall of the cavity.[27], [29], [88] In small observational series, 17–50% of patients remained static without treatment over 10 years or more.[86], [87], [89], [90] There are reports of spontaneous resolution in adults and children, which may be due to decompression of the syrinx

Management

Post-traumatic syringomyelia remains difficult to manage. Despite reports of neurological recovery following surgery, 5 and 10 year follow-up studies demonstrate deterioration or stabilisation only in up to 80% of cases regardless of the method of treatment or achievement of radiological improvement.[34], [94] Surgical options include correction of deformity or compression, various shunting procedures, arachnolysis with or without duraplasty, and cord transection. Foetal spinal cord tissue

Conclusions

It is clear from the current literature that the aetiology of post-traumatic syringomyelia remains poorly understood and its treatment produces unsatisfactory results. There is a need for further research not only to understand the underlying pathophysiology, but to identify better ways of treating the condition. Improvements in MRI, animal models, and mathematical modelling can all play a role in this investigation. Currently, a combination of arachnolysis and duraplasty appears to be a

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