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Evaluation of spinal ultrasound in spinal dysraphism.

AIMS: The aims of this study were to evaluate the role of spinal ultrasound in detecting occult spinal dysraphism (OSD) in neonates and infants, and to determine the degree of agreement between ultrasound and magnetic resonance imaging (MRI) findings. MATERIALS AND METHODS: Eighty-five consecutive infants had spinal ultrasound over 31 months. Of these, 15 patients (age 1 day-7 months, mean 40 days; nine male) had follow-up MRI. Ultrasound and MRI findings were correlated retrospectively. RESULTS: Six out of 15 (40%) ultrasound examinations showed full agreement with MRI, seven of 15 (47%) had partial agreement, and two of 15 (13%) had no agreement. In the present series ultrasound failed to visualize: four of four dorsal dermal sinuses, three of four fatty filum terminales, one of one terminal lipoma, two of four partial sacral agenesis, three of four hydromyelia and one of 10 low-lying cords. CONCLUSION: Agreement between ultrasound and MRI was good, particularly for the detection of low-lying cord (90%). Therefore we recommend ultrasound as a first-line screening test for OSD. If ultrasound is abnormal, equivocal or technically limited, MRI is advised for full assessment.

Female↗

Malformations of the vertebral bodies and the ribs associated to spinal dysraphism without spina bifida in a Pekingese dog.

A Pekingese dog with hemivertebrae, rib malformations and spinal cord dysraphism without spina bifida was presented. Two types of hemivertebrae were seen: bilateral and unilateral. Thoracic hemivertebrae were associated with fused ribs and rudimentary ribs. Spinal dysraphism consisted of polycavitary syringomyelia in the dorsal and lateral funiculi, hydromyelia and anomalies of the dorsal median septum and median fissure, associated to lumbar and sacral hemivertebrae. Cauda equina agenesia was also present. To conclude, the dog showed two malformations from different embryonic origins. Vertebral and rib malformations are of mesodermic origin and spinal dysraphism is of ectodermic origin. A possible common mechanism responsible for both anomalies is discussed.

Animals↗

Computed tomography in spinal dysraphism.

Computed tomography of the spine has been used to evaluate patients with spinal dysraphism. With this technique, the nature of soft-tissue masses can often be correctly diagnosed and the underlying bony anomalies clarified.

Adult↗

Magnetic resonance evaluation of pediatric spinal dysraphism.

Magnetic resonance (MR) scans of 22 children with spinal dysraphism were reviewed. MR scans of the cervicothoracic spine in 12 cases revealed 10 Chiari II malformations, 1 Chiari III malformations, and 4 syringohydromyelias. Twelve MR scans of the lumbosacral region were performed demonstrating 11 tethered cords, 6 myelomeningoceles, 4 lipomyeloschises, 2 diastematomyelias, 2 syringohydromyelias, and 2 dermal sinus tracts. MR is effective in defining the relationship of the cerebellar tonsils and brainstem to the upper cervical spinal canal and in evaluating the extent of syringohydromyelia and the degree of cord expansion. The ability to image the spinal cord and associated paraspinal lesions directly in the sagittal and coronal planes with excellent tissue characterization allows MR to accurately diagnose and define the myriad abnormalities associated with pediatric dysraphism.

Adolescent↗

Prevalence of primary tethered cord syndrome associated with occult spinal dysraphism in primary school children in Turkey.

The prevalence and associated factors of primary tethered cord syndrome (PTCS) in primary school children were investigated. A cross-sectional study was performed in four demographically different primary schools in Turkey. Demographic, familial and physical data were collected from 5,499 children based on enuresis as a predominant symptom and dermatologic and orthopedic signs as clues of occult spinal dysraphism. Statistical analysis and input of the data were carried out with the SPSS package program 10.00, and logistic regression analysis was used to identify discriminating factors between enuretic children with or without neurologic signs. Of 5,499 analyzed children, 422 (7.7%) had enuresis nocturna, and 19.9% of 422 children had also daytime incontinence. Sixteen of these 422 enuretic children (3.8%) had several dermatologic signs. Five of them had spina bifida on plain radiographies, and 4 of them had cord tethering on lumbar MRI. Fifteen of 422 enuretic children (3.7%) had gait disturbances and orthopedic anomalies without cutaneous manifestations. Six of 15 children had spina bifida on plain graphies and 2 of them had tethered cord syndrome on MRI. The general prevalence of PTCS was found to be 0.1% of 5,499 analyzed children and 1.4% of enuretic children. A good outcome after untethering was found in 83.0% in this series. Practitioners should be aware of these clues of occult spinal dysraphism and resort to further radiologic and neurosurgical assessment. Early surgical intervention may halt the progression of the neurologic deficits and stabilize or reverse symptoms.

Adolescent↗

A report of children with spinal dysraphism managed conservatively.

This investigation is a retrospective study of 12 patients with spinal dysraphism. There were 5 males and 7 females in the cohort. Their ages ranged from 4 to 13 years at the time of their last assessment. Initial presentation included one or more of the following--a blemish on the back in the midline (lump, hairy patch, sinus and/or angioma), abnormal neurological signs and symptoms in one or both legs, and in one case dribbling of urine. Investigations performed included plain X-rays of the spine, CT scans alone or in combination with a myelogram and MR scans. Findings included thickened filum terminale, low lying cord, lipoma, syringomyelia, diastematomyelia, spina bifida occulta and sacral agenesis. The children were followed up for between 2 and 10 years. During the period of observation, none developed new symptoms or signs, and there was no progression of existing neurological deficits. This preliminary report suggests that there is no justification for prophylactic surgery in this group of patients.

Adolescent↗

Spinal dysraphism in the cervical and dorsal regions in childhood.

Spinal dysraphism entirely confined to the cervical or dorsal levels is uncommon. In a large series affecting all levels, only 26 such cases that required operation were found. These are described in order to determine their particular features which include a high incidence of expanding lesions (dermoid cyst and neurenteric cyst) and are therefore associated with a much higher risk of morbidity than dysraphism, at lower levels.

Back↗

Occult spinal dysraphism and its association with hip dysplasia in females.

We examined the pelvic radiographs of two groups of patients (more than 12 years of age) from six medical centres. Hip dysplasia was considered to be present if Shenton's line was broken and more than one third of the femoral head was revealed to be uncovered in an antero-posterior radiograph of the pelvis. Patients with hip dysplasia due to teratological or neurological causes were excluded. There were 291 patients with treated or untreated hip dysplasia in the dysplastic group. The control group of 415 individuals was collected from consecutive outpatients (with a pre-set standardised female/male ratio) for whom an antero-posterior radiograph of the pelvis had been made in one of two medical centres and which did not disclose any abnormality of the hip joints. The aim of the study was to assess the co-existence of hip dysplasia and occult spinal dysraphism. Radiographs of all patients were examined, and any partial or complete defect of the posterior vertebral arch was recorded. In the dysplastic group, a defect was recorded in 23% (67/291) radiographs and in the control group in 12% (48/415). In both groups, L5 and S1 were the most commonly recorded sites with a defect. In the dysplastic group, a defect was recorded in 56/190 females and in the control group in 30/302 females. In males, there was no significant difference between the recorded findings in the two groups. In females with hip dysplasia, occult spinal dysraphism seems to be fairly common.

Adult↗

Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification.

Our purpose was to review the neuroradiological features of spinal dysraphism and to correlate them with clinical findings and up-to-date embryological theory. We also aimed to formulate a working classification which might prove useful in clinical practice. We reviewed series of 986 children referred to our Spina Bifida Centre in the past 24 years. There were 353 children with open spinal (OSD) and 633 with closed (skin-covered) spinal (CSD) dysraphism. By far the most common open abnormality was myelomeningocele, and all patients with OSD had a Chiari II malformation. CSD was categorised clinically, depending on the presence of a subcutaneous mass in the back. CSD with a mass mainly consisted of lipomas with dural defects and meningoceles, and accounted for 18.8 % of CSD. CSD without a mass were simple (tight filum terminale, intradural lipoma) or complex (split cord malformations, caudal regression). Our suggested classification is easy to use and to remember and takes into account clinical and MRI features; we have found it useful and reliable when making a preoperative neuroradiological diagnosis in clinical practice.

Child, Preschool↗

The incidence of occult spinal dysraphism and the onset of neurovesical dysfunction in children with anorectal anomalies.

The urological malformations associated with anorectal anomalies (ARA) are not only anatomical, but also functional, the latter being related to congenital neurovesical dysfunction (NVD). The true incidence of spinal dysraphism (SD) in these children is still unclear and is probably underestimated. The concept of caudal regression could explain its association with the anorectal anomalies. Because of awareness of the late onset of neurovesical dysfunction and/or orthopaedic symptoms in some of our patients, in 1991 we started to screen with magnetic resonance imaging (MRI) the spinal cord of all patients with ARA. Eighteen (44%) out of 41 patients without neurological or orthopaedic symptoms and 7 (78%) of 9 children with neurological or orthopaedics symptoms screened by MRI showed pathological findings. The overall incidence of spinal dysraphism in ARA was 50%, without any great difference with respect to the type of the anomaly (high, low, cloacal). The pathological MRI findings encountered were: fibrolipoma (with or without a thickened filum terminale), tethering of the cord and syringomyelia, and sac anomalies. In order to check the onset of NVD in these children, we performed urodynamic studies with external sphincter electromyography in 24. Grouped by age: 14 were between 5 and 18 months and ten were 4 to 13 years old. Ten patients (71%) out of the first group and 3 (30%) out of the second had a normal urodynamic pattern. A total of 11 children had pathological findings; of these, 4 infants had a hyperreflexic bladder (one with detrusor-sphincter dyssynergia) suggesting upper motor neuron (UMN) lesion. Of the 7 older patients, two had UMN and 3 lower motor neuron (LMN) lesion.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Occult spinal dysraphism: neuroradiological study.

We present a retrospective study of occult spinal dysraphism in 47 children aged 0 to 14 years, all studied with plain X-rays, 60% with CT and myelo-CT, and 40% with MR. We consider the classification and grading of these malformations, clinical, neuroradiological patterns, and indications for surgery. In the light of our findings and of the published data MR emerges as the key investigation. Only in a few cases of great anatomical complexity is it now necessary to perform CT and myelo-CT as well. A case in point is when the conus and thickened filum terminale are inextricably bound together and can no longer be considered separate structures. We propose the term "neurofibrous structure" to define the conus-thickened-filum-terminale unit when these structure are no longer distinguishable.

Adolescent↗

Magnetic resonance imaging in the pre-operative assessment of closed spinal dysraphism in children.

Magnetic resonance imaging (MRI) was used in the pre-operative assessment of closed spinal dysraphism in 61 paediatric patients. There were 25 cases of tethered cord, 6 of retethering at the site of myelomeningocele repair, 15 cases of diastematomyelia, 12 cases of lipomyelomeningocele, and 1 case each of dermal sinus, dermoid cyst and neurenteric cyst. MRI was found to have accurate correlation with surgical findings in all cases of tethered cord, and diastematomyelia. In cases of re-tethering at the site of previous myelomeningocele repair, while it was a significant advance on previous imaging techniques precise delineation of neural tissue and discrimination from post-operative fibrosis was frequently not possible. Imaging of dorsal lipomyelomeningoceles also correlated with surgical findings and provided accurate pre-operative information. However, the accuracy and pre-operative detail in cases of transitional and terminal lipomyelomeningoceles was disappointing.

Adolescent↗

The embryology of spinal dysraphism.

The theories of abnormal embryogenesis suggested as explanations for the various forms of SBA and occult spinal dysraphism appear in some instances too all encompassing, as in Gardner's hydrodynamic theory or Padget's neuroschisis theory, or too limited, as in the theory of arrested development proposed by Daniel and Strich for the ACM, or just too speculative in many cases. The observation of a completed defect does not allow one to venture backwards in development to a specific time and cause with any accuracy. Perhaps several different causes striking different tissues at different times can set up a series of aberrations that lead to morphologically similar mature anomalies. The ability of developmental processes to heal themselves, as shown experimentally, may obscure the true mechanism and timing of occurrence although the final morphological expression may be dramatic. Since the study of human embryogenesis in the experimental laboratory is ethically unacceptable although technically feasible, the elucidation of the mechanisms of these neural defects will be long in coming.

Arnold-Chiari Malformation↗

Cutaneous lesions in occult spinal dysraphism--correlation with intraspinal findings.

OBJECTS: This study was conducted to investigate the frequency and type of cutaneous stigmata in different forms of occult spinal dysraphism (OSD) and their correlation to the underlying malformation. METHODS: Fourteen different forms of spinal malformations were identified in 358 operated patients with OSD. Most frequent findings (isolated or in combinations) were spinal lipoma, split cord malformation, pathologic filum terminale, dermal sinus, meningocele manqué, myelocystocele and caudal regression. Stigmata were present in 86.3% of patients, often in various combinations. Using a binary logistic regression analysis, significant correlations with distinct malformations were found for subcutaneous lipomas, skin tags, vascular nevi, pori, hairy patches, hypertrichosis, meningoceles and "cigarette burn" marks. CONCLUSIONS: Cutaneous markers in a high percentage accompany spinal malformations. Due to the correlations of different stigmata to distinct malformations, they can aid the clinician in further diagnostic and therapeutic work.

Adolescent↗

The role of magnetic resonance imaging in the investigation of spinal dysraphism in the child with lower limb abnormality.

A review of magnetic resonance imaging (MRI) performed to exclude the presence of spinal dysraphism in children presenting with lower limb pathology is unrewarding in the absence of abnormal neurology. Over a 5-year period, 29 children ages 2 weeks to 15 years with a mean age of 6 years presenting with lower limb abnormalities were referred for MRI of the spine to exclude an occult neurologic cause for the deformities. More than one limb abnormality, for example pes cavus and limb length discrepancy, was present in 93% of the children, and 11 children had severe or recurrent talipes equinovarus deformity. Only two children (7%), both of whom had abnormal limb neurology, had abnormal MRI scans. In the absence of a demonstrable neurologic deficit in the lower limb, there appears to be no advantage in requesting MRI of the spine in children presenting with lower limb abnormality.

Child↗

Faun tail naevus: a cutaneous marker of spinal dysraphism.

We describe three cases (one male and two females) of faun tail nevi, which is one of the most important cutaneous marker of spinal dysraphism. One of the patients presented with acro-osteolysis leading to auto amputation of the toes of the left foot, which required operative intervention. This lays stress on the early recognition of lumbar paraspinal skin lesions and early treatment to avoid irreversible sequelae.

Acro-Osteolysis↗