The horizontal sacrum as an indicator of the tethered spinal cord in spina bifida aperta and occulta

2007 ◽  
Vol 23 (2) ◽  
pp. 1-4 ◽  
Author(s):  
R. Shane Tubbs ◽  
Cuong J. Bui ◽  
Marios Loukas ◽  
Mohammadali M. Shoja ◽  
W. Jerry Oakes

Object The authors report on symptomatic patients with myelomeningocele (MMC) and lipomyelomeningocele (LMMC) who were found to have changes in their lumbosacral angle (LSA) corresponding to the onset of symptoms indicative of a tethered spinal cord. Methods The authors review data obtained in these two cohorts of patients and compare the LSAs measured in the perinatal period with those seen when the patients presented with symptoms of a tethered spinal cord. Results Children with LMMC, roughly one third of studied cases, were symptomatic due to a tethered spinal cord at their most recent follow-up. In children in whom the MMC was the closed form at birth, 20 of 30 had symptoms that could be indicative of a tethered spinal cord at their most recent follow-up. The LSA was altered in both groups with symptoms. Conclusions Signs and symptoms indicative of a tethered spinal cord appear to correspond to increases in the LSA.

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 In case 1, the fistula was connected to the dural sac on the conus medullaris, and the tethered spinal cord was retracted caudally.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case 2, the tubular structure was connected to the dural sac on the thoracic spinal cord, and the tethered spinal cord was retracted dorsally.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.


2019 ◽  
Vol 7 (3) ◽  
pp. e000894
Author(s):  
Clément Musso ◽  
Camille Bismuth ◽  
Laurent Cauzinille

An 8-month-old male Malinois dog was presented for progressive chronic pelvic limbs ataxia, paraparesis, arched back since ambulation associated with mild urinary and faecal incontinence. Clinical evaluation revealed a dorsocaudal thoracic spine non-healing wound with a fistula from which a clear fluid leaked. Neurological examination was compatible with a T3-L3 spinal cord lesion. CT myelography showed a T11-T12 spina bifida associated with a meningomyelocele with a fistula tract to the skin (spina bifida aperta). An MRI revealed a syringohydromyelia cranially and caudally to the meningomyelocele. Surgical correction involved removal of the fistula up to the dura mater and closure. A 4 and 12 months clinical and MRI follow up revealed a good locomotion improvement with residual mild ataxia without incontinence. MRI showed no relapse of the meningomyelocele but persistent although reduced syringohydromyelia.


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.


Author(s):  
Anna Clebone

Myelomeningocele, also known as spina bifida aperta (often shortened to the nonspecific name “spina bifida”) is a congenital disorder of the spine. In infants with a myelomeningocele, the neural tube has not closed, and the vertebral arches have not fused during development, leading to spinal cord and meningeal herniation through the skin. Because of the high potential for injury and infection of the exposed spinal cord, which could lead to lifetime disability, these lesions are typically repaired within 24 to 48 hours after birth. A myelomeningocele occurs before day 28 of human fetal development and is an abnormality in which the posterior neural tube closes incompletely. The outcome is a vertebral column deformity, through which the meningeal-lined sac herniates. After the bony defect is created, the hypothesized mechanism of meningeal herniation is that the pulsations of cerebrospinal fluid act progressively to balloon out the spinal cord. If the sac is filled with spinal nerves or the spinal cord, it is known as a myelomeningocele; if the sac is empty, it is called a meningocele.


1998 ◽  
Vol 88 (6) ◽  
pp. 958-961 ◽  
Author(s):  
Bermans J. Iskandar ◽  
Benjamin B. Fulmer ◽  
Mark N. Hadley ◽  
W. Jerry Oakes

Object. The management of tethered spinal cord syndrome with onset of symptomatology occurring in adulthood remains controversial, although the necessity of early surgery in the pediatric tethered cord syndrome population is well established. To ascertain the results of surgery in adult patients with this anomaly, the authors undertook a retrospective review of 34 cases. Methods. The authors studied the hospital records of 34 consecutive patients who presented in adulthood with tethered cord syndrome and conducted follow-up phone interviews with 28 of them. The population consisted of 12 men and 22 women, ranging in age from 18 to 70 years (mean 34 years). The most common presenting feature was pain, followed by weakness and incontinence. All patients underwent surgery. The most common operative findings were tight filum terminale, split cord malformation, and lipomyelomeningocele, paralleling those observed in pediatric studies. Long-term surgical results and patient outcome ratings were encouraging. After a mean clinical follow-up period of 4 years, significant improvement occurred in 22 of 27 patients presenting with pain, 13 of 27 patients with motor or sensory dysfunction, and 11 of 18 patients with bowel and bladder disturbance. In addition, telephone interviews were obtained after a period of 8.6 years. Twenty-two (79%) of 28 patients called the operation a long-term success; 21 (75%) of 28 patients believed that they had significant postoperative improvement (and not just stabilization) in pain and/or neurological function. Surgical complications were generally minor. Nineteen (86%) of 22 employed patients returned to work after surgery. Two (33%) of six patients who were not employed before surgery worked full time postoperatively. Only two of the 28 patients interviewed had received Workers' Compensation benefits; both of these had good outcomes and returned to work. Conclusions. Tethered spinal cord syndrome in adults is an uncommon entity that can become symptomatic. Although surgery in adults involves greater risk of neurological injury than in children, it is a low-risk procedure with encouraging results. Because neurological deficits are generally irreversible, early surgery is recommended.


1975 ◽  
Vol 20 (3) ◽  
pp. 129-132 ◽  
Author(s):  
A. J. Dougall ◽  
J. C. Grant ◽  
J. O'Connor

The further progress of the survivors in 1969, of a group of 150 babies born with spina bifida cystica in the period 1960–66 is described. Children with a meningocele continued to do well. Those with a myelomeningocele are divided into 2 groups. There were 4 late deaths and a considerable degree of handicap in the group of 23 in whom the spinal cord was exposed on the surface as a plaque at birth. There were 2 late deaths and a much lower incidence of handicap in the group of 25 in whom only ectopic nervous tissue was found in the sac at birth. The incidence and treatment of hydrocephalus is described. Changes in the attitude to the treatment of a neonate with a myelomeningocele are discussed.


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