INTRODUCTION
Syringomyelia refers to a cystic dilation within the spinal cord.[1] Syringomyelia can occur in association with a Chiari, arachnoiditis, posttrauma, neoplasms of the spine, and posterior fossa.[1–3] The incidence of symptomatic posttraumatic syringomyelia (PTS) ranges from 1% to 7%.[4]
Following spinal trauma, about 50% develop spinal cystic change and 21%–28% develop a syrinx over the next three decades.[1] However, only 1%–9% of patients with spinal cord injury progress to symptomatic syringomyelia.[1]
The clinical presentation includes pain, ascending sensory, and motor deficits.[5] In the current era, it is important to identify and treat such cases of PTS at the earliest, considering not only the diagnostic modalities and treatment options available but also the long-term effects including permanent neurological deficits if left untreated.
CASE REPORT
A 34-year-old male presented to us with a history of giddiness and heaviness of the head, impaired fine motor movements of both hands for 1 month. He had sustained a D12 compression fracture when he met with a road traffic accident 7 years ago and developed paraparesis with bilateral foot drop, for which he underwent instrumented stabilization of the fracture.
Clinical evaluation revealed features of myelopathy with exaggerated deep tendon reflexes across both upper and lower extremities with the presence of deltoid and pectoralis jerks, small muscle wasting of the hands, and upper motor neuron (UMN) bladder with residual bilateral foot drop. In this case, although his trauma was 7 years ago, his symptoms related to the syrinx were only present for the past 1 month. His UMN bladder could be a part of posttraumatic myelomalacia myelopathy (PPMM), as explained by Fischbein etal.[2]
His magnetic resonance imaging (MRI) of the spine [Figure 1a and b] revealed a T1 hypointense and T2 hyperintense lesion within the cord extending from the medulla up to the conus with C3-D10 level showing maximum intensity, suggestive of a holocord syrinx.
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Figure 1: (a) MRI whole spine T2W image, mid sagittal section of the patient showing holocord syrinx extending up to the medulla, (b) MRI T2W image axial section at the C1-C2 level showing syrinx. MRI: Magnetic resonance imaging, T2W: T2 weighted
A syringopleural shunt was done for the patient. D5 laminectomy and midline durotomy were done and the catheter was placed in the syrinx cavity. A left subscapular incision was made through which the other end of the catheter was placed in the pleural cavity [Figure 2a and b]. The advantage of the syringopleural shunt over other shunt procedures is the presence of continuous negative pressure in the pleural cavity, which augments the drainage of the syrinx. His postoperative period was uneventful, and he was discharged on the 4th postoperative day. His giddiness, fine movements of his hands, and bladder symptoms gradually improved over 8–10 months. Radiographic resolution of the syrinx was noted on the 1-year follow-up MRI [Figure 3a and b].
Figure 2: (a) Intraoperative image of shunt tube insertion into the syrinx, (b) Intraoperative image of pleural end of shunt tube insertion
Figure 3: (a) 1-year follow-up MR T2W sagittal image with resolving syrinx, (b) 1-year follow-up MR T2W axial image with resolving syrinx. MR: Magnetic resonance, T2W: T2 weighted
DISCUSSION
In posttraumatic syrinx formation, the inciting event is the focal cord injury.[2] PPMM represents a continuum of interrelated disease processes that can precede cyst formation, in which patients with syrinx develop features of myelopathy such as spasticity along with motor weakness.[2] This could explain the reason our patient presented with UMN bladder with syringomyelia. The presence of localized arachnoiditis plays an important role in the pathophysiology of PPMM and local cerebrospinal fluid dynamics.[2] The time of presentation can vary from 6 months to 34 years following trauma.[5,6]
To the best of our knowledge, only four cases of posttraumatic holocord syrinx, have been reported in the literature till date, the summary of the cases, patient’s symptoms, level of injury, and management are given in Table 1.
Table 1: The summary of the previously published case reports of posttraumatic holocord syrinx
Syringes will often reduce in size after cord decompression or correction of the deformity.[5] Surgical options include direct lysis of arachnoid adhesions with or without duraplasty and shunting procedures, including syringocavitary, syringo-subarachnoid, and lumboperitoneal shunts.[1,5] Authors have reported good short-term results following syringopleural shunting.[1] Syringosubarachnoid shunts can have concerns about flow reversal.[1]
Studies demonstrate that 5 or 10 years of follow-up show the stabilization of syrinx only in up to 80% of patients regardless of the method of treatment.[1,5]
However, in the existing literature, there is no satisfactory standard treatment guideline for PTS. Hence, PTS remains a neurosurgical challenge.[7]
We reiterate that early diagnosis of the posttraumatic syrinx and timely intervention along with neurorehabilitation is the key to the management of PTS.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that their name and initial will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
REFERENCES
1. Roguski M, Samdani AF, Hwang SW. Adult Syringomyelia Youman and Winn Neurological Surgery 7th ed Ch. 301 Philadelphia, PA Elsevier 2017.
2. Fischbein NJ, Dillon WP, Cobbs C, Weinstein PR. The “Presyrinx”state:Is there a reversible myelopathic condition that may precede syringomyelia?. Neurosurg Focus 2000;8:E4.
3. Koyanagi I, Iwasaki Y, Hida K, Houkin K. Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis. Surg Neurol 2005;63:350–5 discussion 355-6.
4. Krebs J, Koch HG, Hartmann K, Frotzler A. The characteristics of posttraumatic syringomyelia. Spinal Cord 2016;54:463–6.
5. Brodbelt AR, Stoodley MA. Post-traumatic syringomyelia:A review. J Clin Neurosci 2003;10:401–8.
6. Vernon JD, Silver JR, Ohry A. Post-traumatic syringomyelia. Paraplegia 1982;20:339–64.
7. Kleindienst A, Laut FM, Roeckelein V, Buchfelder M, Dodoo-Schittko F. Treatment of posttraumatic syringomyelia:Evidence from a systematic review. Acta Neurochir (Wien) 2020;162:2541–56.
