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PubMed2004

Imaging in spine and spinal cord malformations.

Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis.

Humans↗

PubMed1998

The human tail.

The human tail is a congenital anomaly with a protruding lesion from the lumbosacrococcygeal region. A newborn with a tail-like structure over the coccygeal area observed since birth is presented. Lipoma accompanied by tethered spinal cord were found. In reviewing the literature from 1960 to 1997, 59 cases were described. Higher incidences of spinal dysraphism (49.15%) and tethered spinal cord (20.34%) compared with previous reports were evident. This fact plays an important role in understanding the disturbance of development and regression of human tails. A new classification according to whether the anomaly appears in combination with spinal dysraphism is proposed for clinical usage. Preoperative detailed image studies are needed to clarify the possibility of tethered spinal cord syndrome developing in the future and thus prevent it. Magnetic resonance imaging is the modality of choice if available. Long-term follow-up for possible sequelae after operation, especially in cases with spinal dysraphism, is necessary.

Animals↗

PubMed1998

Functional results following the antegrade continence enema procedure.

BACKGROUND: Forty children who underwent the antegrade continence enema (ACE) procedure for faecal soiling were studied to determine factors predictive of outcome. METHODS: There were four patient groups: (1) ambulant with spinal dysraphism (n = 13), (2) wheelchair bound with spinal dysraphism (n = 14), (3) ambulant with miscellaneous disorders (n = 11) and (4) wheelchair bound with miscellaneous disorders (n = 2). Effectiveness of the procedure was assessed using technical evaluation and quality-of-life improvement (QOLI) scores (0-5). Objective assessment included colonic transit time (CTT) and anorectal manometry. Median follow-up was 21 (range 5-37) months. RESULTS: Some 28 of 40 children achieved continence. The procedure was reversed in four of 40 children. Of the other 36 children with a functioning ACE stoma, all reported improvement in quality of life (mean QOLI score 3.5). There were no significant differences in technical evaluation score, QOLI score, CTT, manometry findings or continence between ambulant groups and the wheelchair-bound group with miscellaneous disorders. QOLI score, anorectal squeeze pressure and continence were significantly poorer in those who were wheelchair bound with spinal dysraphism. Absent squeeze pressure was associated with poor outcome. CONCLUSION: Wheelchair-bound children with spinal neuropathy have a poorer outcome following the ACE procedure. Although ACE is an effective method of promoting faecal continence, it is essential to determine the aetiology of incontinence and sphincter function before operation.

Adolescent↗

PubMed2004

[Occult spinal dysraphia].

One of the most complex and most difficult congenital anomalies is spina bifida. Peter Van Forest was the first one who noticed this anomaly in 1587, and Recklinghausen, in 1886, classified spina bifida to types and suggested surgical procedures for its management. Earlier name, spina bifida, is currently more and more replacing with a term "defect of neural tube" (NTDs), or even more, "spinal dysraphia". Anomaly can appear at any level of spinal cord (cervical, thoracal, lumbar and sacral) and posterior localization is more often than the anterior one. Contrary to the open spinal dysraphism that can be perceived immediately, closed spinal dysraphism is very deceiving anomaly, and therefore, it must be treated properly as soon as it is diagnosed. Because of its seclusion, the term usually used is "occult spinal dysraphism (OSD)". The incidence of this anomaly is unknown, but it has been reported that it is more common among female children. Etiology of OSD is also unknown, but some of its risk factors are as follows: previous pregnancy with NTD, partner with NTD, type 1 diabetes mellitus, usage of anticonvulsives, a lack of folates in mother's nutrition. Prenatal diagnose of OSD is practically impossible. Skin changes, orthopedic, urological and neurological problems, suggest considering this complex anomaly. X-ray, ECHO, MRI, as well as neuropsychological examination corroborate diagnosis. At the same time, the diagnosis (once it is confirmed) represents the indication for neurosurgical treatment.

Child↗

PubMed2000

[Sonography of the central nervous system in prematures and newborns].

UNLABELLED: Examination of the central nervous system in premature and full-term infants requires a sensible selection of the available diagnostic procedures such as ultrasound, MRI and CT to allow sufficient and accurate diagnosis and management. ULTRASOUND: Ultrasound, including the various Doppler techniques, is a very reliable, safe, noninvasive diagnostic tool that allows evaluation of even severely ill patients in intensive care units. It is especially well suited for the study of intracranial hemorrhage, hypoxic-ischemic encephalopathy, congenital malformations of the brain and lesions produced by intrauterine and acquired infections. Sonography is also very reliable in examination of spinal dysraphism and spinal cord injuries during birth. The necessity of surgical intervention (i.e. shunt implantation) as well as subsequent postoperative follow-up can be provided. MRI: In contrast to ultrasound, MRI is most useful to demonstrate the age-dependent myelinization pattern and clearly discriminates between the white and gray matter of the central nervous system. We therefore use MRI for the detection of complex malformations of the central nervous system in particular disorders of cell migration and for a final estimation of a perinatal brain injury. CONCLUSION: A precise ultrasound examination performed by an experienced physician who is familiar with the expected pathology and has access to a well-adjusted ultrasound machine should help to minimize the use of confirming CT and MRI investigations and to optimize their optional use in more complex cases.

Brain Diseases↗

PubMed2001

Long-term outcome for patients with split cord malformation.

OBJECT: Split cord malformations (SCMs) are relatively rare forms of occult spinal dysraphism (OSD) and tethered spinal cord syndrome. The majority of these cases present in early childhood, with neurocutaneous stigmata being an early presenting feature. Prophylactic detethering surgery is advocated by most neurosurgeons due to the risk of neurological deterioration over time caused by patient growth and activity. However, unlike other forms of OSD, the course of SCM progression after surgery is not well understood, and little has been published about long-term followup results. In this study the authors review the results obtained in 16 patients in whom the senior author performed surgery over a 13-year period (average length of follow up almost 8 years). METHODS: Presentation, surgical approach, and outcome are evaluated, and the long-term outcome of neurological status, pain, bowel/bladder disturbance, and spinal deformities are emphasized. CONCLUSIONS: The primary conclusion is that patients with SCM generally tolerate surgery well and experience few complications. Neurological deterioration is rare except in cases in which retethering occurs, (two patients in this series). Although impaired bowel and bladder function was stabilized or improved and pain was reliably relieved postoperatively, preexisting vertebral column deformities usually progressed after surgery and, in most cases, required spinal fusion.

Adolescent↗

PubMedDate not supplied

Sacral nevus flammeus simplex: the role of imaging.

Neonates with midline lumbar, thoracic, or occipital cutaneous lesions should be suspected of having spinal dysraphism and should undergo an imaging study. The aim of the present study was to evaluate whether sacral nevus flammeus simplex (SNFS) in neonates is associated with spinal dysraphism. During a period of 6 months, we prospectively examined all neonates in our center for the presence of SNFS. Those affected were evaluated for associated anomalies by physical and neurologic examination and ultrasound imaging of the lumbosacral area. Twenty-eight of 3623 neonates (0.77%) were diagnosed as having SNFS. In 64% of the SNFS patients, nevus flammeus simplex (NFS) was noted as well. Physical and neurologic examinations were unremarkable. Ultrasound imaging of the lumbosacral area was performed in 25 patients (89%) and revealed diastematomyelia in one. The results show that SNFS may constitute the only cutaneous sign of spinal dysraphism. In conclusion, we recommend that neonates with SNFS be investigated for occult spinal dysraphism.

Female↗

PubMed1989

Tethered cord syndrome: a pediatric case study.

Tethered cord syndrome, seen in patients with spinal dysraphism, is a progressive neurological deterioration due to stretching of the spinal cord. Spinal dysraphism results from defects during embryonic closure of the neural tube, usually in the lumbosacral region. In tethered cord syndrome an abnormally low conus medullaris is tethered by intradural abnormalities such as a short, thickened filum terminale, fibrous bands, a lipoma or diastematomyelia. This article distinguishes tethered cord syndrome from other forms of occult spinal dysraphism and describes and compares normal anatomy to pathological changes. Clinical symptoms and nursing management of the pediatric patient with tethered cord syndrome will be exemplified by a case study presentation.

Child↗

PubMed1999

Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes.

BACKGROUND: Most reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects the scoliosis associated with syringomyelia. METHODS: We reviewed the records of 16 pediatric patients with syringomyelia and Chiari Type I malformation. The patients' ages ranged from 3 to 15 years, with mean age of 9.8 years. None of the patients had spinal dysraphism. RESULTS: Nystagmus was observed in 2 of the 16 patients, motor weakness in 8 patients, sensory disturbance in 10 patients, and scoliosis in 13 patients. As the initial surgical procedure, foramen magnum decompression (FMD) was performed in seven patients and syringo-subarachnoid (S-S) shunting in nine patients. The motor function improved in 7 of the 8 affected patients, and the sensory disturbance improved in 9 of the 10 affected patients. The magnetic resonance images obtained after the surgery revealed marked decrease of the syrinx size in all patients. Of the 13 patients with scoliosis, 5 showed improvement, 5 stabilization, and 3 deterioration. CONCLUSIONS: Compared with adolescent and adult syringomyelia, pediatric syringomyelia shows a much lower incidence of sensory disturbance and pain, but quite a high incidence of scoliosis. Surgery is effective in improving or stabilizing scoliosis in these patients.

Adolescent↗

PubMed1993

Evaluation of tethered cord syndrome associated with anorectal malformations.

The clinical courses of four patients with occult spinal dysraphism who were also found to have anorectal malformations and tethered cord syndrome are evaluated in this report. These patients were among 27 patients with occult spinal dysraphism treated in the Division of Neurosurgery of Tohoku University School of Medicine during the last 3 years. All four patients experienced urinary disturbances early in life, and initially, these disturbances were considered to be caused by urogenital malformations. As a result, treatments were delayed. Only one patient with male Turner's syndrome exhibited mental retardation. Other symptoms, such as sensory disturbance and pain in the lower extremities, became apparent from complaints by the patients without atresia ani. When surgery was performed earlier in our patients with occult spinal dysraphism, the outcome was better. We believe in early surgery for tethered cord syndrome, although there is no consensus for the timing of this surgery. Unfortunately, the diagnosis of tethered cord syndrome was delayed in our four patients. However, because occult spinal dysraphism and anorectal malformations seem to be closely related, the presence of anorectal malformations may result in an earlier diagnosis of tethered cord syndrome.

Anus, Imperforate↗

PubMed2004

Cloacal exstrophy: morbidity associated with abnormalities of the gastrointestinal tract and spine.

BACKGROUND/PURPOSE: The aim of this study was to review the management of the gastrointestinal tract in cloacal exstrophy and to assess the effect of spinal dysraphism on nutritional outcome. METHODS: Twenty-two patients with cloacal exstrophy were reviewed retrospectively. Gastrointestinal and spinal anomalies were documented. The need for nutritional supplementation was recorded. Weights at 1 and 5 years of age were used to assess growth in childhood. RESULTS: Ten patients were treated initially with ileostomy, 7 with colostomy, and 3 without stoma. Two patients died before surgical reconstruction. Total parenteral nutrition (TPN) was utilized in 8 infants for a median of 10 days (range, 5 to 200). Three patients had complications with colostomies requiring conversion to ileostomy. Patients with an ileostomy required more nutritional supplementation compared with patients with a colostomy (50% v 28%). High-output stoma losses were more common in patients with an ileostomy (40% v 14%). Fourteen patients (67%) with spinal dysraphism had a higher incidence of failure to thrive in the first year of life (69% v 26%) and multiple episodes of enteritis (40% v 25%). Two neonates with duodenal atresia and small bowel deletion died within the first month of life. One patient with short bowel syndrome died of TPN-associated liver disease at 6 months of age. There were no other deaths. CONCLUSIONS: The gastrointestinal tract contributes significantly to the morbidity and mortality in cloacal exstrophy. Nutritional supplementation is more frequently required in patients with an ileostomy. Stoma complications were higher in those with a colostomy. Morbidity is high in patients with spinal dysraphism.

Abnormalities, Multiple↗

PubMed1967

Spinal cord dysraphism in a dog.

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Animals↗

PubMed1997

Urodynamic findings in the tethered spinal cord syndrome: does surgical release improve bladder function?

PURPOSE: We retrospectively reviewed 39 patients with a tethered spinal cord to evaluate whether surgical release positively influenced urological symptoms or urodynamic findings. MATERIALS AND METHODS: The patients were divided into 2 groups: group 1-11 with occult spinal dysraphism and group 2-28 with secondary spinal cord tethering after previous closure of a myelomeningocele or resection of a lipomyelomeningocele. Diagnosis was confirmed in all cases by magnetic resonance imaging or spinal ultrasound. A comprehensive urodynamic evaluation was done immediately preoperatively and 2 to 21 months (mean 7) postoperatively. RESULTS: In group 1 the most common preoperative urodynamic finding was hyperreflexia, which improved or resolved after untethering in 62.5% of the patients. Four adults also reported improved bladder sensation or decreased urgency. In group 2 the most common urodynamic finding was impaired compliance, followed closely by detrusor hyperreflexia. Urodynamic patterns of detrusor hyperreflexia or compliance improved in only 30% of the patients, while 48% had worsened patterns. Only 14% of group 2 had improved symptoms of urinary control but 28% had improved lower extremity function. CONCLUSIONS: Urological symptoms and urodynamic patterns may be improved by early surgical intervention in patients with occult spinal dysraphism. However, untethering did not consistently benefit patients with secondary spinal cord tethering.

Adolescent↗

PubMed2006

Neurenteric cyst: case report and a review of the potential dysembryology.

We report a 13-year-old female who presented to an outside emergency room following blunt trauma to the head. As part of her evaluation, an unenhanced CT of the face and radiographs of the cervical spine were performed. She was referred to our clinic with "abnormal" imaging. Radiographs revealed dysmorphic cervical spine and ventral clefting of the vertebral bodies from C7-T2. A subsequent MRI was obtained that demonstrated a large spinal cord mass. MRI demonstrated an unenhanced intramedullary mass of the cervicothoracic spinal cord measuring approximately 1.5 cm in greatest axial diameter. Surgical exploration of her intraspinal mass revealed a neurenteric cyst. Spinal neurenteric cysts are categorized in the spectra of occult spinal dysraphism and most likely arise from incomplete separation of the developing notochord and foregut in the embryo. We discuss potential etiologies for the formation of such cysts. The clinician should consider neurenteric cysts in their differential diagnosis of patients with spinal dysraphism and/or intraspinal masses.

Adolescent↗

PubMed1982

Functional improvement after spinal surgery for the dysraphic malformations.

Spinal surgery for the various dysraphic malformations is generally indicated to arrest disease progression. The results of the present study suggest that certain clinical conditions and presentations are associated with an exceptionally good surgical prognosis. Sixty-nine procedures were done in 55 patients. The diagnoses included hydromyelia and tethered cord related to diastematomyelia, lipoma, lipomeningocele, previous operation, and thickened filum terminale. The operative results were evaluated for significant functional neurological and urological improvement, as well as for the relief of pain. Pain relief was achieved in 92% of patients with preoperative pain. Significant functional return was seen in 67% of hydromyelia patients. On the other hand, surgical treatment of tethered cord caused improvement in 24% (lipoma) to 46% (thickened filum and diastematomyelia). These results suggest that patients with hydromyelia and patients with a dysraphic malformation and pain have an excellent prognosis after operation. A re-evaluation of the indications for surgery for these patients may be necessary.

Adolescent↗

PubMed1992

Occult dysraphism of the spine. A retrospective analysis of 88 operative cases, 1979-1989.

Eighty-eight children under the age of 12 years with occult spinal dysraphism were operated on at Red Cross War Memorial Children's Hospital from 1979 to 1989. There were 56 lumbosacral lipomas, 18 dermal sinuses and cysts, 7 isolated thickened filum terminales and 7 diastematomyelias. The incidence of neurological abnormality was significantly higher in children operated on after 12 months of age (15% v. 60%). At surgery the commonest variant of the lumbosacral lipoma was the dorsal type. The incidence of postoperative complications was low (CSF leak 10%; superficial infection 6%). Three children improved neurologically, but the vast majority remained unchanged. The desirability and safety of early prophylactic surgery for occult spinal dysraphism is supported by this study.

Age Factors↗

PubMed1985

Sonography of the spine and spinal cord.

Sonography has proved to be a valuable method for the examination of the spine in children with spinal dysraphism. It facilitates the diagnosis of meningocele, lipoma, and/or spinal cord tethering rapidly and noninvasively. It is suggested that sonography be utilized for screening infants with neurocutaneous signature of spinal dysraphism.

Astrocytoma↗

PubMed2006

The 'human tail' causing tethered cervical cord.

STUDY DESIGN: Human tails continue to elicit curiosity till the present times. A unique case of human cervical dysraphism is described. OBJECTIVES: In addition to the cosmetic stigma, these cutaneous markers provide a lead to reach the underlying spinal dysraphic states. SETTING: Plains of North India. METHODS: A case of human tail at the neck region is presented, whose cutaneous deformity lead the clinicians to his underlying spinal dysraphic state. RESULTS: The final outcome of the patient was favorable. CONCLUSION: Such markers should always be looked upon with a caution. The present report describes a unique case of a human neck tail, a causing cervical cord tethering.

Cervical Vertebrae↗