Terminal Syringomyelia in Association with the Tethered Cord Syndrome

Erkan, Kaan; Ünal, Faruk; Kırış, Talat

doi:10.1097/00006123-199912000-00018

Cited by 51 publications

(38 citation statements)

References 30 publications

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“…TS is a phenomenon that has been increasingly recognized in association with spinal dysraphism [2,3,4,5], especially in TCS [1]. The pathophysiology of the problem is not clearly understood [6,][7].…”

Section: Discussionmentioning

confidence: 99%

“…A mechanism related to anomalous persistence of the ventriculus terminalis has been proposed [8]. However, the close anatomic relationship between the syrinx and dysraphism implies a local dysfunction in CSF dynamics that results in syrinx formation [1]. Because the pathophysiology of this problem is not well understood, it is also not clear whether it is indicated to surgically treat this kind of syringomyelia or whether repair of the dysraphism is sufficient.…”

Section: Discussionmentioning

confidence: 99%

“…Also, it is not well understood whether an associated syrinx needs to be treated in addition to treating the tethered cord, or whether cord untethering alone will result in resolution of the syrinx and any associated symptoms. One study [1] has addressed this problem in a series of 32 adult and pediatric patients with TCS and TS. The incidence of TS in their TCS population was 24%.…”

Section: Introductionmentioning

confidence: 99%

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Clinical Significance of Terminal Syringomyelia in Association with Pediatric Tethered Cord Syndrome

Beaumont¹,

Muszynski²,

Kaufman³

2007

Pediatr Neurosurg

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An association between terminal syringomyelia (TS) and tethered cord syndrome (TCS) has been recognized. The clinical significance of TS and the need for concurrent treatment is not known. Thirty-four patients with TCS undergoing surgery for tethered cord release were evaluated for the incidence of TS. The clinical and radiological response to untethering with and without syrinx drainage was assessed. The group incidence of TS was 29.4%. There were no significant differences in the sex, age, underlying pathology or preoperative symptoms between the TCS group (TCS, n = 24) and the TCS group with TS (TCS + TS, n = 10). In the TCS group, 37.5% of the patients were asymptomatic preoperatively, and in the TCS + TS group 50% were asymptomatic preoperatively. After surgery, none of these asymptomatic patients developed symptoms. All of the symptomatic TCS + TS patients improved clinically, 12 of 15 symptomatic TCS patients improved, 2 patients were unchanged and 1 had worsening scoliosis. Syrinx did not develop in patients lacking it preoperatively. In patients where postoperative imaging was available, preoperative syringes improved. TS is clearly associated with TCS. Tethered cord release alone may be sufficient to improve preoperative symptoms and TS may be an associated phenomenon that does not mandate separate treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical Significance of Terminal Syringomyelia in Association with Pediatric Tethered Cord Syndrome

Beaumont¹,

Muszynski²,

Kaufman³

2007

Pediatr Neurosurg

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“…Although concomitant hindbrain malformation exists with syringomyelia in the majority of cases (5), terminal syringomyelia in the lower third of the spinal cord is more commonly seen with spinal dysraphism, especially tethered cord (6,7). Syringomyelia also rarely develops in association with primary spinal intramedullary tumors (11,17) or intradural metastases (9).…”

Section: Introductionmentioning

confidence: 99%

Terminal syringomyelia mimicing intramedullary tumor: case report

Abuzayed

Dashti

Erdinçler

et al. 2009

Turkish Neurosurgery

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In this report, the authors present a 14-year-old male patient admitted with progressive paraparesis and urinary incontinence that developed in a 3-month period. Thoracolumbar MRI revealed an intramedullary lesion starting from the level of the T10 vertebra and extending to fill the sacral spinal canal. Based on the radiological findings an intramedullary tumor was suspected. Exploratory operation with right L5 hemilaminectomy was done and the intraoperative finding was syringomyelia which was punctured with releasing of the tethered cord. The patient was followed for 3 years with clinical and radiological regression of the pathology and no symptoms and sings have remained. Syringomyelia can mimic intramedullary tumors clinically and radiologically. That is why it is useful to consider exploratory hemilaminectomy to confirm diagnosis.

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“…3) Terminal syringomyelia is a cystic dilatation of the lower third of the spinal cord thought to be independent of hindbrain malformation, and is often associated with occult spinal dysraphism and especially with tethered cord. 4,5,10) Terminal syringomyelia was present in 32 of 132 patients with tethered cord syndrome, 4) and in 27% of cases of occult spinal dysraphism. 5) Terminal syringomyelia occurred in 54% of cases of meningocele manque and 38% of cases of split cord malformation.…”

Section: Introductionmentioning

confidence: 99%

Terminal Syringomyelia: Is It As Innocent As It Seems?-Case Report-

Emmez

Çağatay

Kurt

et al. 2004

Neurol. Med. Chir.(Tokyo)

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A 5-day-old newborn girl presented with cutaneous signs of hypertrichosis, skin dimple, and subcutaneous lipoma in the lumbar region. Magnetic resonance (MR) imaging of the thoracolumbar spine demonstrated spinal dermal sinus, type II split cord malformation, and tethered cord. A small terminal syringeal cavity was also present. Five months after the diagnosis, weakness in dorsal flexion of the bilateral feet and toes was detected. Surgery to untether the cord was performed. Her deficits resolved completely. Nearly 4 years later, she was admitted with weakness in dorsal flexion of the right foot and toe. MR imaging of the spine showed holocord syringomyelia, postoperative changes, and tethered cord. No Chiari malformation was seen. Syringosubarachnoid shunt placement was performed. Terminal syringomyelia is common in cases of occult spinal dysraphism, but the natural history and management remain unclear. Progression, regression, or persistence of terminal syringeal cavity can occur whether untethering is performed or not. Progression of the terminal syrinx occurred after tethered cord release and the enlargement of the cavity involved the entire spinal cord in the present case. Terminal syringomyelia requires close follow up for a long time even in asymptomatic cases. Surgical treatment should be considered in symptomatic cases.

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Terminal Syringomyelia in Association with the Tethered Cord Syndrome

Cited by 51 publications

References 30 publications

Clinical Significance of Terminal Syringomyelia in Association with Pediatric Tethered Cord Syndrome

Clinical Significance of Terminal Syringomyelia in Association with Pediatric Tethered Cord Syndrome

Terminal syringomyelia mimicing intramedullary tumor: case report

Terminal Syringomyelia: Is It As Innocent As It Seems?-Case Report-

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