Spina Bifida Occulta with Tethered Spinal Cord

Spina Bifida ◽  
1999 ◽  
pp. 52-52
Author(s):  
Kuniyoshi Ohisuka
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.


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.


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.


1971 ◽  
Vol 8 (3) ◽  
pp. 232-238 ◽  
Author(s):  
A. H. Martin

The lumbar part of the spinal cords of two Manx kittens showed syringomyelia associated with spina bifida occulta. The syringomyelia may have been caused by a secondary mechanism, such as occlusion of blood vessels, that occurred after formation of the spinal cord. There was no apparent hyperplasia of neural tissue. Incomplete fusion of vertebral arches may also have been caused in part by such a vascular defect.


2007 ◽  
Vol 7 (3) ◽  
pp. 315-322 ◽  
Author(s):  
Sharad Rajpal ◽  
M. Shahriar Salamat ◽  
R. Shane Tubbs ◽  
David R. Kelly ◽  
W. Jerry Oakes ◽  
...  

Object The goal of the present study goal was to systematically confirm the previously recognized nomenclature for tethering tracts that are part of the spectrum of occult spinal dysraphic lesions. Methods The tethering tract in 20 patients with spina bifida occulta underwent histological examination with H & E staining and epithelial membrane antigen (EMA) immunolabeling, and additional selected specimens were stained with Masson trichrome. Results All tethering tracts contained fibrous connective tissue. Four tracts were lined with epithelial cells and either originated within a dermoid cyst, terminated at a skin dimple/sinus opening, or had both of these characteristics. No tethering tracts exhibited EMA positivity or meningeal elements. Although all tethering tracts originated in juxtaposition to the spinal cord, their termination sites were variable. Conclusions Based on histological findings and presumed embryological origin, the authors broadly classified tethering tracts terminating within the dura mater, epidural space, or lamina as “short tethering tracts” (STTs). The STTs occurred mostly in conjunction with split cord malformations and had a purely fibrous composition. Tethering tracts terminating superficial to the overlying lamina were classified as “long tethering tracts” (LTTs), and the authors propose that these are embryologically distinct from STTs. The LTTs were of two varieties: epithelial and nonepithelial, the former being typically associated with a skin dimple or spinal cord (epi)dermoid cyst. In fact, analysis of the data suggested that not every tethering tract terminating in or on the skin should be classified as a dermal sinus tract without histological confirmation, and because no evidence of meningeal tissue–lined tracts was detected, the use of the term “meningocele manqué” may not be appropriate.


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