Spinal dysraphism. Spinal cord lesions associated with spina bifida occulta.
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Magnetic resonance imaging of spinal dysraphism.
Spinal cord development occurs through three consecutive periods. Gastrulation (weeks 2-3) is characterized by conversion of the embryonic disk from a bilaminar to a trilaminar arrangement and establishment of a notochord. Primary neurulation (weeks 3-4) produces the uppermost nine tenths of the spinal cord. Secondary neurulation and retrogressive differentiation (weeks 5-6) result in formation of the conus tip and filum terminale. Defects in these early embryonic stages produce spinal dysraphisms, which are characterized by anomalous differentiation and fusion of dorsal midline structures. Spinal dysraphisms may be categorized clinically into two subsets. In open spinal dysraphisms, the placode (non-neurulated neural tissue) is exposed to the environment. These disorders include myelomeningocele, myeloschisis, hemimyelomeningocele, and hemimyelocele, and are always associated with a Chiari II malformation. Closed spinal dysraphisms are covered by intact skin, although cutaneous stigmata usually indicate their presence. Two subsets may be identified based on whether a subcutaneous mass is present in the low back. Closed spinal dysraphisms with mass comprise lipomyeloschisis, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise complex dysraphic states (ranging from complete dorsal enteric fistula to neurenteric cysts, split cord malformations, dermal sinuses, caudal regression, and spinal segmental dysgenesis), bony spina bifida, tight filum terminale, filar and intradural lipomas, and persistent terminal ventricle. Magnetic resonance imaging is the imaging method of choice for investigation of this complex group of disorders.
Adolescent↗
Examining the newborn with an open spinal dysraphism.
Spinal dysraphism includes a constellation of frequent and potentially complex malformations of the spine and spinal cord. Open spinal dysraphisms, the majority of which are myelomeningoceles, have a number of associated morbidities that require both immediate and lifelong medical care. The role of the neonatal nurse includes the immediate stabilization of the affected infant, systematic examination of the malformation, and implementation of evidence-based protocols to ameliorate the associated medical problems. Coordination of care and communication and support of the family are essential aspects of care. This article reviews the embryology of open spinal dysraphisms and the steps needed to stabilize and evaluate affected infants.
Humans↗
MR imaging of spinal dysraphism.
Spinal dysraphism includes anomalies of midline fusion involving bony, mesenchymal, and neural elements. MR imaging of the spine was performed as the initial imaging technique to determine its role in the evaluation of 31 children when spinal dysraphism was clinically suspected or when radiographs revealed errors of ossification of the posterior elements (spina bifida). Correlation of surgical findings in 17 of 18 abnormal examinations and metrizamide myelography with CT in six of these cases indicated that accurate diagnosis was provided by MR in all instances. Examples of spina bifida aperta (spina bifida cystica)--including myelomeningocele, myelocystocele, and lipomyelomeningocele--and those of occult spinal dysraphism--such as dorsal dermal sinus, spinal lipoma, and tight filum terminale (thickened filum)--are presented. These cases show MR to be a reliable technique in the initial evaluation of these disorders.
Adolescent↗
MR spectrum in spinal dysraphism.
Spinal dysraphism is a general term which encompasses a wide variety of anomalies of the spine, all of which result from imperfect midline fusion of the embryonic neural tube. This term refers to large defects that involve the spine and not to small vertical clefts commonly seen within the spinal process of L5 or S1. We present a spectrum of MR imaging findings selected from a retrospective review of 100 patients of spinal dysraphism evaluated at our institution.
Diagnosis, Differential↗
Leg lengthening in spinal dysraphism.
Spinal dysraphism may lead to limb inequality and foot deformities. Fears have been expressed regarding limb lengthening in such patients because of potential sequelae in relation to their peripheral neurologic abnormalities. We performed Ilizarov lengthening of the lower limb on seven community walkers with spinal dysraphism. The procedure was well tolerated with no increase in sepsis, soft-tissue, or bony complications compared with patients undergoing lengthening for reduction anomalies. All the patients mobilised rapidly after frame application and returned to their previous functional level within 3 months of plaster removal.
Adolescent↗
Spinal dysraphism.
Spinal dysraphism (SD) is characterized by maldevelopment of neural tube, notochord, mesoderm and cutaneous ectoderm. Incidence of SD is 2-4/1000 live births. One hundred and nineteen patients operated from January 1991-June 1996 at Department of Neurosurgery, All India Institute of Medical Sciences, were studied. Only 21 patients (17.6%) presented when they were less than one year old and 17 patients came in adult age group (> 16 years). Lumbar and lumbosacral region was the commonly involved site in 81 patients (74.7%). Weakness of lower limbs (74%), difficulty in walking (54%), muscle atrophy (41.2%) were the commonest indicators of motor system involvement. Loss of sensation, trophic ulcer, backache were seen in 45, 14, 10 patients respectively. Cutaneous lipoma (26%), hypertrichiosis (20%), dermal sinus (13.4%), midline dimples (7%) were the important cutaneous markers. Foot and limb deformity was seen in 25% cases. Tethering of cord, syringomyelia & split cord malformation were the most common radiological findings. Only 10% of our patients had hydrocephalus that required shunt. Out on 119 cases operated, 43 improved, Twenty had sensory improvement and 18 showed motor improvement. Fifteen patients regained continence. Twelve patients were lost to follow-up. Sixty-seven patients had no change in neurological status, post-operatively. Six cases deteriorated in terms of motor or sensory deficit and one patient lost continence. CSF leak (8%) and wound infection (6%) were the common complications. Six patients required second surgery as T.P. Shunt (4), rotation flap (1), reexploration and duraplasty (1).
Adolescent↗
Spinal dysraphism.
Spinal dysraphism is common in North America, affecting from one in 500 to one in 1,000 newborns. Although dysraphic conditions vary widely, most can be recognized before any significant neurologic harm occurs. Lesions include simple meningocele, myelomeningocele and tethered spinal cord. Modern imaging techniques delineate the anatomy of the defect, permitting prompt neurosurgical repair, correction of neurologic deficits and prevention of further deterioration.
Female↗
Spinal neurenteric cysts without associated malformations. Are they the same as those presenting in spinal dysraphism?
Spinal neurenteric cysts are rare intradural developmental lesions, usually composed of a thin-walled cyst whose lining mimicks gastro-intestinal or respiratory epithelium. A detailed review of the literature revealed 80 cases of solitary spinal neurenteric cysts (including a new case of ours), which were analysed and compared, regarding clinical and pathological aspects, with 56 such cases of those with concomitant evidence of dysraphism. Older age and cervical location were found to be statistically characteristic of the solitary cysts in contrast to younger age and lumbosarcal location for the 'dysraphic' cases. Magnetic resonance imaging is the diagnostic modality of choice. Good evidence of the developmental origin of incomplete separation of notochord and endoderm was given by the fact that histologically solitary cysts were mainly composed by endodermal derivatives, while 'dysraphic' had also mesenchymal and ectodermal elements. Thus, it seems that additional spinal dysraphic changes are more likely to be found when ectodermal and mesenchymal elements co-exist with endodermal ones, possibly indicating an earlier error in development than in other cases containing only endodermal elements.
Humans↗
[Occult spinal dysraphism].
BACKGROUND: Spinal dysraphism is a group of congenital anomalies of the spine characterized by a midline defect affecting the nervous tissue and its bony and soft tissue coverings. Closed spinal dysraphism, spina bifida occulta, refers to skin-covered lesions. However, there are a number of cutaneous stigmata or other physical signs that serve as indicators of an underlying dysraphic malformation of the lower spinal canal. MATERIAL AND METHODS: Based upon relevant literature, we present a review of occult spina bifida. RESULTS AND INTERPRETATION: The cutaneous stigmata that may indicate an underlying dysraphism are particularly hairy patches, subcutaneous lipomas, capillary hemangiomas, dorsal dermal sinuses and sacral cutaneous pits. The clinical examination of a child with lumbosacral cutaneous stigmata should focus on neurological signs and symptoms, urological disturbances and orthopaedic deformities in the lower limbs. Spinal MRI is the imaging modality of choice in children presenting with features consistent with occult spinal dysraphism. Children with occult spinal dysraphism should be referred to a neurosurgical unit.
Child↗
Spinal dysraphism: MR imaging rationale.
Spinal cord development occurs through the three consecutive periods of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal cord malformations derive from defects in these early embryonic stages, and are collectively called spinal dysraphisms. Spinal dysraphisms may be categorized clinically into open and closed, based on whether the abnormal nervous tissue is exposed to the environment or covered by skin. Open spinal dysraphisms include myelomeningocele and other rare abnormalities such as myelocele, hemimyelomeningocele, and hemimyelocele, and are always associated with a Chiari II malformation. Closed spinal dysraphisms are further divided into two subsets based on whether a subcutaneous mass is present in the low back. Closed spinal dysraphisms with mass comprise 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 involves abnormal notochordal development, either in the form of failed midline integration (ranging from complete dorsal enteric fistula to neurenteric cysts and diastematomyelia) or of segmental agenesis (caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging is the imaging modality of choice for evaluation of this complex group of disorders.
Adolescent↗
An unusual presentation of occult spinal dysraphism.
Occult spinal dysraphism can lead to irreversible neurological complications, early diagnosis and treatment are necessary. It can be suspected from the presence of any cutaneous abnormality. We report a case with bony spur formation on the top of the 5th lumbar vertebra spinose process covered with skin mimicking a meningocel sac. By the help of this bony spur tethered cord could be diagnosed before any neurological deficit.
Bone Diseases↗
Imaging in hydrocephalus and spinal dysraphism.
Hydrocephalus and spinal dysraphism are two commonly encountered clinical entities where imaging plays a vital role. Sonography, Computed Tomography and Magnetic Resonance Imaging are the modalities used for the evaluation of hydrocephalus. Ultrasound is useful in small infants with open fontanella, is non-invasive and can be performed at cribside. However, it has limitation in identifying the etiology. CT is the most cost effective modality in the evaluation of hydrocephalus and gives detailed anatomical information. MRI offers the added advantage of multiplanar display and can also assess stenosis/patency of CSF pathways, using phase contrast cine techniques. MRI is the modality of choice for evaluating spinal dysraphism and gives excellent information regarding contents of back mass, spinal cord status and associated anomalies like Chiari malformation. Myelography coupled with CT should be reserved for equivocal cases or in centres where MRI is not available.
Cerebrospinal Fluid Shunts↗
[Ultrasonography in spinal dysraphism and tethered cord].
Spinal dysraphism is relatively common in children and includes a wide spectrum of congenital anomalies in the normal closure of the posterior elements of the spine. The prognosis will depend mostly on early diagnosis and treatment. Occult spinal dysraphism may present without external anomalies and the diagnosis could be suspected lately, when neurological symptoms are present and often irreversible. Occult spinal dysraphism is frequently associated to a tethered cord, most commonly secondary to the presence of a lipoma. Ultrasonography has been proven highly sensitive in the detection of intraspinal anomalies, especially in the diagnosis of tethered cord, in children under two years of age due to lack of ossification of the posterior elements of the spine. Today ultrasonography should be the examination of choice in all those patients in whom some kind of spinal dysraphism is suspected. We report our experience with three infants with occult spinal dysraphism in whom diagnosis was initially made by US and later on proved by either computed tomography, magnetic resonance or myelography. All of them had corrective surgery and neurological abnormalities were not detected afterwards.
Humans↗
[Neurogenic disorders of bladder emptying in closed spinal dysraphism].
Closed (occult) spinal dysraphism, e.g. lipomyelomeningocele, intraspinal lipoma, diastematomyelia, the tethered spinal cord in its various forms and dysgenesis of the sacrum, is often diagnosed late and only symptoms of neurogenic bladder dysfunction are present. A lipomyelomeningocele mostly causes detrusor and sphincter dysfunction, as was the case in five of six children among our patients. However, improvement of neurological and urological symptoms after the operation can only be achieved in about 40%. Four of eight children with diastematomyelia suffered from neurogenic bladder dysfunction; three have meanwhile undergone surgery with complete recovery in one, no relevant change in the second, and worsening in the third. Originally a specific term, the "tethered spinal cord" when associated with spinal dysraphism has taken on a more general meaning. Nowadays this term is not only used for a short, thickened and tight filum terminale, but comprises any pathology, which prevents the spinal cord from ascending. MRI examination of the craniovertebral junction and spinal cord of patients with treated myelomeningocele often reveals secondary pathologic changes: these may be areas of cord atrophy, hydromyelic cavitation or ventral compression from arachnoid cysts with clinical symptoms mostly after the age of 5 years. In these children a changing urodynamic pattern may therefore be caused by such a pathology and is an indication for a thorough neurological examination including MRI. Of all the dysrhaphic states mentioned above, sacral dysgenesis is the most frequent. The sacral osteological anomaly, as a numerical and as a structural anomaly, also determines the neuro-urological deficit.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Screening of occult spinal dysraphism by ultrasonography].
Ultrasonography for diagnosis of spinal cord diseases has been used since the eighties. There are different fields for its use: traumatic spine lesions, intra-operative use in trauma and spine tumors surgery, pre-operative and follow up study for spinal dysraphism, occult spinal dysraphism of the newborn. Patients with suspected occult spinal dysraphism are the most important indication for the method. Ultrasonography was performed in 292 patients of Hospital das Clínicas, Universidade Federal de Minas Gerais. Lesions such as conus medullaris lipomas, low level of conus medullaris, filum terminale tethering, cauda equina cysts and dermal sinuses can be well demonstrated. There is no difficulty in interpreting the image. The exam does not need sedation; it is safe, fast and cheap. Ideally it should be performed by the neurosurgeon as it may provide many information during the examination. The ultrasonography does not replace magnetic resonance imaging but it is an excellent method for screening.
Humans↗
Magnetic resonance imaging of pediatric spinal dysraphism.
Thirty pediatric patients with spinal dysraphism were studied with magnetic resonance imaging. A variety of dysraphic anomalies were visualized, including syringohydromyelia, tethered cord, widened spinal canal, meningomyelocele, lipomyelomeningocele, lipomeningocele, intraspinal lipoma, congenital dermal sinus tract, and scoliosis. In 29 of 30 cases, MRI diagnostically visualized spinal cord and dysraphic anatomy. A combination of T1-weighted sagittal and axial imaging planes provided the most complete visualization. In specific cases, T2-weighted examinations were useful in demonstrating likely regions of inflammation bordering infected sinus tracts. MRI demonstrates a wide variety of dysraphic spinal anomalies and effectively screens children for occult spinal dysraphism.
Adult↗
Neurovesical dysfunction due to spinal dysraphism in anorectal anomalies.
Spinal dysraphism (SD) has been found associated with functional abnormalities of anorectal anomalies (ARA). The incidence of SD in these children is probably underestimated and a complete neuroradiological investigation of the lower urinary tract function has not been carried out routinely. In a 2 years time frame we performed urodynamic (UDS) evaluations on 14 patients (8 males and 6 females) with ARA who showed SD at Magnetic Resonance Imaging (MRI) studies. We divided them into 2 groups by age: group A (5 to 18 months) and group B (3 to 12 years). The UDS findings were classified as upper (UMN) and lower motor neuron (LMN) lesion. Out of the 9 children included in group A, 5 showed normal urodynamic pattern, while 4 had pathological UDS findings suggesting UMN lesion. Among the older (group B) children only one had normal and 4 had pathological findings: 2 hyperreflexia suggesting UMN lesion and 2 external sphincter denervation suggesting LMN lesion. These data support the hypothesis that the neurovesical dysfunction found in children with anorectal anomalies results from a possible association with spinal dysraphism. An early morphological evaluation of the spinal cord is mandatory in all children with ARA prior to definitive surgical correction of the malformation. The low incidence of pathological UDS findings in small children if compared to older ones suggest that SD, although present, may be asymptomatic.
Age Factors↗