scholarly journals History of the current understanding and management of tethered spinal cord

2016 ◽  
Vol 25 (1) ◽  
pp. 78-87 ◽  
Author(s):  
Sam Safavi-Abbasi ◽  
Timothy B. Mapstone ◽  
Jacob B. Archer ◽  
Christopher Wilson ◽  
Nicholas Theodore ◽  
...  

An understanding of the underlying pathophysiology of tethered cord syndrome (TCS) and modern management strategies have only developed within the past few decades. Current understanding of this entity first began with the understanding and management of spina bifida; this later led to the gradual recognition of spina bifida occulta and the symptoms associated with tethering of the filum terminale. In the 17th century, Dutch anatomists provided the first descriptions and initiated surgical management efforts for spina bifida. In the 19th century, the term “spina bifida occulta” was coined and various presentations of spinal dysraphism were appreciated. The association of urinary, cutaneous, and skeletal abnormalities with spinal dysraphism was recognized in the 20th century. Early in the 20th century, some physicians began to suspect that traction on the conus medullaris caused myelodysplasia-related symptoms and that prophylactic surgical management could prevent the occurrence of clinical manifestations. It was not, however, until later in the 20th century that the term “tethered spinal cord” and the modern management of TCS were introduced. This gradual advancement in understanding at a time before the development of modern imaging modalities illustrates how, over the centuries, anatomists, pathologists, neurologists, and surgeons used clinical examination, a high level of suspicion, and interest in the subtle and overt clinical appearances of spinal dysraphism and TCS to advance understanding of pathophysiology, clinical appearance, and treatment of this entity. With the availability of modern imaging, spinal dysraphism can now be diagnosed and treated as early as the intrauterine stage.

Author(s):  
Stephen L. Kinsman

The term “spinal dysraphism” encompasses the broadest array of the conditions known as the neural tube defects. The open neural tube defects (spina bifida aperta and cystica) include both disorders of primary and/or secondary neuralation and are best defined as myelomeningocele complex (MMC) due to their protean nervous system manifestations beyond the spinal lesion. Closed spinal dysraphisms (so-called spina bifida occulta) include lipomatous lesions, forms of tethered spinal cord, sinus tracts, and forms of split spinal cord (diastematomyelia). Both genetic and environmental etiologies have been identified. Gene-environment and gene-gene interactions are also important in the pathobiology of these conditions.


2021 ◽  
Vol 28 (1) ◽  
pp. 21-27
Author(s):  
Mark S. Dias ◽  
Ming Wang ◽  
Elias B. Rizk ◽  
Robin Bowman ◽  
Michael D. Partington ◽  
...  

OBJECTIVE The aims of this study were to review the National Spina Bifida Patient Registry (NSBPR) data set to study the rates of tethered spinal cord release (TCR) among patients with myelomeningocele and variability between centers, to compare TCR rates between males and females, and to study the relationships between TCR rates and other condition-specific characteristics. METHODS The NSBPR registry was queried to identify all patients with myelomeningocele. TCR rates were calculated over time using survival analyses; rates between centers and between males and females were compared. Cox proportional hazards models were constructed to identify relationships between TCR rates and sex, functional lesion level, ambulation status, treated hydrocephalus, and prior Chiari decompression. RESULTS Of 6339 patients with information about their operations, 1366 (21.5%) underwent TCR, with significant variability between centers. The majority (75.8%) underwent a single TCR. The annual TCR rate was linear between birth and 13 years (1.8%/year) but declined sharply from 14 to 21 years (0.7%/year). There was no period of time at which the TCR rate accelerated. There were no significant differences in TCR rates between males and females. TCR rate was not related to functional lesion level but was lower among nonambulators compared with community ambulators (p = 0.005) and among those with treated hydrocephalus (HR 0.30, p < 0.001), and higher among those having prior Chiari decompression (HR 1.71, p < 0.001). CONCLUSIONS These results extend the results of prior single-institution studies, demonstrate significant treatment variability between institutions, and challenge the traditional concept that tethering is related to spinal cord stretching due to spinal growth.


2017 ◽  
Vol 3 (1) ◽  
pp. 205511691770806 ◽  
Author(s):  
Masahiro Tamura ◽  
Takashi Oji ◽  
Satoshi Une ◽  
Makiko Mukaino ◽  
Tatsuro Bekki ◽  
...  

Case summary Two castrated male cats, aged 8 months old (case 1) and 10 months old (case 2), showed a history of progressive paraparesis, an over-reaching pelvic limb gait, urinary incontinence and a palpable dermoid fistula. In case 1, the fistula was connected to the dural sac on the conus medullaris, and the tethered spinal cord was retracted caudally. In case 2, the tubular structure was connected to the dural sac on the thoracic spinal cord, and the tethered spinal cord was retracted dorsally. Tethered cord syndrome secondary to spina bifida aperta was suspected in both cats. Excision of the fistula and release of the tethered spinal cord was performed. A histopathological examination confirmed the diagnosis of a meningomyelocele in case 1 and a meningocele in case 2. Paraparesis improved postoperatively in both cats. However, urinary incontinence in case 1 remained partially unresolved. Relevance and novel information This is the first report to describe the imaging characteristics, surgical treatments and outcomes of two different types of tethered cord syndrome with spina bifida aperta in cats. Tethered cord syndrome with spina bifida aperta needs to be included in the differential diagnosis of slowly progressive paraparesis in younger cats with or without vesicorectal failure and a palpable dermoid fistula.


Spine Surgery ◽  
2005 ◽  
pp. 1131-1145
Author(s):  
Gary W. Tye ◽  
John D. Ward ◽  
John S. Myseros

2005 ◽  
Vol 3 (5) ◽  
pp. 390-392 ◽  
Author(s):  
Christopher Uff ◽  
Robert Bradford

✓ The authors present the case of a large, posttraumatic sacral pseudomeningocele in the presence of spina bifida occulta. A pseudomeningocele in the sacral region is associated with trauma and with Marfan syndrome, but only one occurrence has been reported in association with spinal dysraphism. Trauma resulted in bleeding into the pseudomeningocele and retrograde passage of blood and fat into the ventricles. An oculomotor nerve palsy subsequently developed in the patient. The authors suspected a subarachnoid hemorrhage caused by a posterior communicating artery aneurysm, although this hypothesis was refuted on further investigation. The pseudomeningocele was drained by direct exposure of the neck and opening of the sac. Postoperatively, communicating hydrocephalus developed and the patient underwent ventriculoperitoneal shunt placement, resulting in resolution of the cranial nerve palsies. This first report of intradural bleeding from direct trauma to a pseudomeningocele illustrates the rare phenomenon of retrograde passage of blood from the sacral region to the brain. It also illustrates a possible but unlikely differential diagnosis of intraventricular blood and fat.


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