Tethered cord syndrome in occult spinal dysraphism

Cornette, Luc; Verpoorten, Carla; Lagae, Lieven; Calenbergh, Frank Van; Plets, Chris; Vereecken, R.L.; Casaer, Paul

doi:10.1212/wnl.50.6.1761

Cited by 80 publications

(33 citation statements)

References 1 publication

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“…The surgery is safe and effective, with good long-term neurological results. While most neurosurgeons have recently been operating on spinal lipomas even in the absence of symptoms [1, 2, 3, 4, 6, 7, 8, 9, 10, 28, 34, 47], it is still far from conclusive that prophylactic surgery for asymptomatic conus lipomas is more effective than conservative management [9, 13, 15, 48, 49]. Due to the obvious ethical considerations, the natural history of this clinical condition has not been established with prospective, randomized trials.…”

Section: Discussionmentioning

confidence: 99%

Spinal Lipomas in Children

et al. 2000

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Spinal cord lipomas are a common cause of cord tethering that can lead to progressive neurological defects. The role of prophylactic surgery for spinal lipomas has recently been questioned. Between 1985 and 1999, 59 children underwent a total of 69 surgical procedures at the Birmingham Children’s Hospital in Birmingham, UK. The spinal lipomas were classified into: 18 terminal, 17 transitional, 6 dorsal and 18 filum lipomas – including 12 who had a typical thickened filum terminale. At the first operation, 19 patients (32%) were asymptomatic, and 40 patients (68%) presented with symptoms. Surgical indications in the asymptomatic group included the presence of a dermal sinus tract or syrinx. Prophylactic surgery was undertaken in selected cases. The mean total follow-up for the group since the first surgical procedure was 61.8 months (range: 7.0–203.0 months). In the asymptomatic group, 26% of the patients had late neurological deterioration. Of the 14 patients with asymptomatic conus lipomas, 3 (21%) developed sphincter dysfunction and motor problems at long-term follow-up. In the symptomatic group, 68% improved, 20% remained unchanged, and 12% had late neurological deterioration. None of the 18 patients with symptomatic filum lipoma deteriorated postoperatively. However, 39% had bladder dysfunction, 54% had neuro-orthopaedic deformity, and only 15% returned to overall normal function at latest follow-up. Of the 27 patients with symptomatic conus lipomas, 67% improved, 15% remained stable, and 18% had late neurological deterioration. However, 74% had bladder dysfunction, 67% had neuro-orthopaedic deformity, and 45% had motor problems at long-term follow-up. Spinal lipomas can cause progressive neurological deficits irrespective of spinal untethering surgery. This study demonstrates that filum and conus lipomas have similar clinical presentation, but differ in their outcome following surgery. Filum lipomas are ‘benign’, for which surgery is safe and effective. Conus lipomas are more difficult to manage. When asymptomatic, our results suggest that prophylactic surgery does provide some protection from future neurological deterioration. When symptomatic, conus lipoma surgery is effective in stopping further deterioration. Improvement in neurological function can occur, but few patients return to normal overall function, and pre-existing sphincter dysfunction is not significantly altered by surgery.

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Section: Discussionmentioning

confidence: 99%

Spinal Lipomas in Children

et al. 2000

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“…Nevertheless, there is some literature which sheds light on this topic. Cornette et al[ 37] reported a group of 22 consecutive newborns diagnosed with occult spinal dysraphism associated with low conus. They were treated with an algorithm that reserved surgical untethering only for patients with upper motor neuron dysfunction.…”

Section: Diagnosismentioning

confidence: 99%

“…There does not appear to be a difference in motor improvement rates between pediatric and adult series. Early intervention after symptom development appears to result in a much greater recovery of neurological function, with many patients making full recoveries[ 37, 58]. …”

Section: Surgical Outcomementioning

confidence: 99%

“…The arguments for prophylactic untethering are that: (1) a certain (undefined) percentage of these patients will go on to develop symptomatic TCS; (2) one cannot guarantee reversibility of symptoms, even with prompt surgery, and (3) the morbidity for filum sectioning is reasonably low. For these reasons, some authors advocate strongly for prophylactic untethering[ 54, 79, 80], while others remain skeptical[ 37, 81]. Although widely practiced, the beneficial effect of prophylactic untethering in asymptomatic patients remains to be proven by controlled, prospective studies.…”

Section: Miscellaneous Topicsmentioning

confidence: 99%

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Tethered Cord Syndrome: An Updated Review

2007

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Tethered cord syndrome (TCS) is a diverse clinical entity characterized by symptoms and signs which are caused by excessive tension on the spinal cord. The majority of cases are related to spinal dysraphism. TCS can present in any age group, and presentations differ according to the underlying pathologic condition and age, with pain, cutaneous signs, orthopedic deformities and neurological deficits being the most common. Surgical untethering is indicated in patients with progressive or new onset symptomatology attributable to TCS. The surgical strategy aims to release the tethering structure and thus the chronic tension on the cord. Early operative intervention is associated with improved outcomes. Pain relief is accomplished in almost all cases. Realistic surgical goals include relief of pain and stabilization of neurological function, although improvement in function is often seen. Cord untethering can also halt the progression of scoliosis. The benefits of surgery are debated in asymptomatic patients and patients with normal imaging.

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“…In the development process of the central nervous system, the distal spinal cord is abnormally attached to the surrounding tissues, and tethering of the spinal cord interferes with the normal regression process causing damage to the spinal cord as it is stretched and placed under abnormal tension. The release of a tethered spinal cord by sectioning the tethering tissues is a straightforward surgical procedure that can prevent, arrest, or ameliorate neurological deficits [13, 14]. Laboratory studies in animals and observations in human patients show that there is a link between the degree of oxidative metabolic impairment and the severity of neurological deficits in TCS.…”

Section: Discussionmentioning

confidence: 99%

Study on the Pathomorphology of Myelodysplasia

et al. 2008

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Objective: To study the clinical pathomorphology of myelodysplasia and research its relationship with the neurological defect of myelodysplasia. Methods: 184 patients with myelodysplasia were diagnosed and evaluated depending on patient history, physical examination, imaging, voiding cystourethrogram and urodynamic tests. 182 patients underwent surgical management. The intraspinal anomalies and their relationships with the spinal cords were observed and recorded during operations. The pathological tissues which were resected during the operations were examined postoperatively. Results: Myelodysplasia was divided into 6 types depending on the intraspinal pathomorphology: type I (tethered spinal cord with morbid terminal filum), type II (tethered spinal cord with fibrous adhesion), type III (lipoma on the spinal cord), type IV (intraspinal cyst), type V (split cord malformations), and type VI (defect of spinal cords and/or nerve roots). Conclusions: Theneurological defect of myelodysplasia results from its intraspinal anomaly and the morphological change of the spinal cord. It should be actively diagnosed and surgically treated to improve the prognosis.

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Tethered cord syndrome in occult spinal dysraphism

Cited by 80 publications

References 1 publication

Spinal Lipomas in Children

Spinal Lipomas in Children

Tethered Cord Syndrome: An Updated Review

Study on the Pathomorphology of Myelodysplasia

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