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Use of anal sphincter electromyography during operations on the conus medullaris and sacral nerve roots.

The mechanical activity of the anal sphincter can be translated into electrical activity and recorded on graph paper or an oscilloscope. The activity of the anal sphincter may be extrapolated to activity of the external urethral sphincter because both are striated muscles innervated by the pudendal nerve that arises from S-2, S-3, and S-4. Stimulation of these nerves causes contraction of the sphincter muscles, and a deflection of the recording device occurs. This technique was employed intraoperatively in monitoring operations on the conus medullaris and sacral nerve roots in 10 patients with spinal dysraphism (age range, 3 weeks to 15 years). Their diagnoses were tethered conus, 4; lipomeningocele, 3; spinal hamartoma, 1; syringocele, 1; and sacral arachnoiditis, 1. With general anesthesia, and the patient in the prone position, an electrode-containing anal plug was inserted or two needle electrodes were inserted into the anal sphincter muscle. The electrodes were connected to the electromyography recording stylus of the urodynamic bladder diagnostic unit. During the spinal operation, whenever a structure could not be identified clearly, it was stimulated with the disposable electrical stimulator and, if oscillations of the stylus occurred (indicating contraction of the anal sphincter), the structure was preserved. This technique permitted spinal operations in these 10 patients without changes in neurological or urological function.

Adolescent↗

Outcome of meningomyelocele/lipomeningomyelocele in children of northern India.

OBJECTIVE: To evaluate the clinical profile, associated anomalies and surgical outcome of children with meningomyelocele (MMC)/lipomeningomyelocele. METHODS: Out of a total of 181 cases of spinal dysraphism treated at our Institute between 1996 and 2004, 102 (56.35%) cases of MMC/lipomeningomyelocele were analyzed retrospectively and prospectively. The clinical profile and radiological findings of these children were recorded. Craniospinal MRI was the essential investigation and was done as a management protocol at our Institute for these children. Eighty-two out of 102 (80.3%) cases had pure MMC/lipomeningomyelocele and 20/102 (19.6%) had associated split cord malformation (SCM; complex spina bifida). All these children underwent surgery for their primary and associated malformations as indicated. They were clinically assessed over a mean follow-up period of 3.6 years ranging from 1.5 months to 8 years. No urodynamic or evoked potential studies were done to assess the sphincteric outcome following surgery. RESULTS: Forty-six (45.1%) of children with MMC had other associated tethering lesions, including the presence of SCM. Craniospinal axis screening remained an important tool to understand the associated tethering lesions and malformations. Only 58.8% of children had hydrocephalus; thus the incidence was much lower compared with reports from the western literature (80-96%). 63.3% of children with overt hydrocephalus required shunt surgery prior to the definitive surgery; however, 23.3% of cases required a shunt after the MMC has been closed. Improvement in clinical profile following microneurosurgery was observed in 42.8% of cases with motor involvement, 46.8% of cases with sensory dysfunction and 39.5% of cases with sphincteric involvement. Motor improvement was seen in 43.6% of cases of pure MMC/lipomeningomyelocele as compared to only 40.0% of cases of complex spina bifida. Sensory improvement was also better in pure MMC/lipomeningomyelocele group being seen in 48.0% of cases as compared to only 42.9% of cases of complex spina bifida. CONCLUSION: Presence of SCM with MMC is referred to as complex spina bifida and is seen in a significant proportion (19.6%) of all cases of MMC. Thorough assessment of the craniospinal imaging needs to be done to look for the presence of multiple tethering lesions which could be present in the same case. Not all children with spinal dysraphism with hydrocephalus required shunt surgery or CSF diversion but a constant and vigilant follow up could avoid it in 13.3% of cases. Improvement in motor and sensory dysfunction was better in the pure MMC/lipomeningomyelocele group than in the complex spina bifida group. Improvement in sphincteric dysfunction, although seen in significant cases, was less compared with improvement in motor and sensory dysfunction. This probably represents a lack of definitive objective criteria for urodynamic improvement and a lack of proper rehabilitation. Electromyographic studies and uroflowmetry are required to asses the true sphincteric outcome following surgery.

Abnormalities, Multiple↗

Uterus-like mass of the conus medullaris with associated tethered cord.

A patient with a mass lesion of Müllerian origin associated with a spinal dysraphism is reported. The mass lesion was a genuine uterine formation, and a hemorrhage arose from a functional endometrium. A spinal dysraphism was associated, including a low-lying conus medullaris, a subcutaneous lipoma, and a sinus between the cul-de-sac and the lipoma. This ectopic and mature tissue may be differentiated from teratoma and endometriosis.

Adolescent↗

Giant terminal lipomyelocystocele.

Terminal myelocystocele is a rare form of occult spinal dysraphism in which the hydromyelic caudal spinal cord and the subarachnoid space are herniated through a posterior spina bifida. A 1-year-old female child presented with a large lumbosacral mass (30 x 20 x 10 cm), flaccid paraplegia and urinary incontinence since birth. Magnetic resonance imaging revealed a low-lying conus (with associated conus lipoma) and a dilated central canal surrounded by a meningocele suggestive of terminal lipomyelocystocele and was operated on successfully. In our experience, this was a giant terminal lipomyelocystocele and such a large lesion has not been reported in the literature before. Terminal myelocystocele should be included in the differential diagnosis of congenital lesions presenting as a lumbosacral mass and operated early.

Diagnosis, Differential↗

Terminal myelocystocele--a case report.

Terminal myelocystocele is a rare form of occult spinal dysraphism in which the hydromyelic caudal spinal cord and the subarachnoid space are hemiated through a posterior spina bifida. A 1.5 month old boy presented with a large lumbosacral mass and urinary incontinence. The magnetic resonance imaging, operative findings and pathological findings revealed a low lying conus with a dilated central canal dorsally attached to the subcutaneous tissue. Ventral subarachnoid space was enlarged and herniated through the laminar defect of the sacrum. The lesion was typical of a terminal myelocystocele. The clinical features are different from those of myelomeningocele in many aspects. Though the incidence is low, terminal myelocystocele should be included in the differential diagnosis of congenital lesions presenting as a lumbosacral mass.

Arachnoid↗

Postoperative spinal ultrasonography findings in spinal dysraphia.

Diastematomyelia is a form of spinal dysraphism involving sagittal clefting of the spinal cord, conus medullaris, and/or filum terminale into two hemicords. It can be an isolated finding or can be associated with meningomyelocele or meningocele. In this report, we present postoperative spinal ultrasonography findings in a patient with diastematomyelia and a tethered cord.

Adolescent↗

Sonography for detection of spinal dermal sinus tracts.

OBJECTIVE: It is well known that spinal dysraphism may be complicated by meningitis as a result of infection traveling from the skin along a patent dermal sinus tract. The only clue is the lower back cutaneous lesions. Our aim was to investigate the correlation between cutaneous lesions, patent dermal sinus tracts, and spinal dysraphism and their complications. METHODS: Five patients (3 female and 2 male) with spinal patent dermal sinus tracts were studied. We used a 7-MHz linear transducer with a two-dimensional real-time sonographic system to insonate and obtain transverse and longitudinal views of the spinal cord and subcutaneous area, extending from the cervical cord to the sacral areas. Subsequently, we performed spinal magnetic resonance imaging in every patient to confirm the diagnosis and to evaluate the intraspinal conditions. RESULTS: The associated central nervous system anomalies and complications were tethered cords (n = 5), dermoid cysts (n = 3), lipoma (n = 2), central nervous system infections (n = 2), and syringomyelia (n = 1). The outcomes were better in those who received surgical intervention before they were infected. CONCLUSIONS: Early detection of spinal patent dermal sinus tracts and related anomalies was accomplished with spinal sonography and allowed for prophylactic treatment (e.g., early surgical intervention) before the onset of neurologic deficits.

Central Nervous System Cysts↗

Intramedullary spinal tumors of disordered embryogenesis.

Abnormal spinal embryogenesis is quite commonplace. While greater than 90 percent of these errors of embryogenesis leads to occult spinal dysraphism with minimal neurologic or orthopedic sequelae, there is a significant minority of these anomalies which leads to the formation of the so-called 'congenital tumors of disordered embryogenesis'. The purpose of this article is to discuss the embryology, presentation, diagnosis and management of the spinal dysraphic states with particular emphasis on those errors which lead to mass lesions in the spinal canal such as dermoids, epidermoids, lipoma/lipomyelomeningocoele and neurenteric cysts. We also include lesions such as dermal sinus tracts and thickened filum terminale in our discussion with particular emphasis on their relationship to the tethered cord syndrome. Proper surgical management of these various conditions necessitates a thorough understanding of their embryologic etiology and the anatomic/physiologic ramifications that such lesions have on the developing spinal cord.

Child↗

[Intraspinal lipoma in an infant: a case report of MRI study].

A 6-month-old boy having intraspinal lipoma with lumbosacral subcutaneous lipoma and occult spinal dysraphism is described. CT scan and magnetic resonance imaging (MRI) are demonstrated. On admission, the patient showed no neurological deficits. Lumbosacral hemangioma and subcutaneous lipoma were noticed on physical examination. A plain spinal roentogenogram revealed occult spinal dysraphism extending from L 4 level down to the sacral region. A plain CT scan disclosed a round-shaped low density area surrounded by a relatively high density area corresponding to the neural tissue in the spinal canal. MRI (1.5-T superconducting system) with sagittal views clearly showed an oval-shaped high signal intensity (SI) area-an intraspinal lipoma-with the neural tissue running longitudinally within and on its surface. The lipoma appeared to be attached to the spinal cord at L 1/2 level. On operation, we found an extramedullary lipoma which had a certain connection to the sacral epidural adipose tissue with an opening of the dural sac, as far as the subcutaneous lipoma. Tethered cord and thickened filum terminale were not identified. Generally, lipoma is demonstrated as a low density area of approximately -90 H.U. on a CT scan. In MRI, we can obtain a high contrast between the lipoma, the spinal cord, and the cerebrospinal fluid, since they are shown as a high, an intermediate, and a low SI area respectively, on T1-weighted spin echo images. Furthermore, sagittal section of MRI is regarded as a potent diagnostic modality to get the precise anatomy of the spinal cord lesion. These short spin echo images can be taken in a relatively brief time. It is a great advantage for patients with spinal lipoma, who are usually infants. MRI is considered to be a quite useful modality to diagnose spinal lipoma, and is able to lead to an early diagnosis non-invasively and accurately, facilitating appropriate surgical treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Humans↗

Exencephaly in Cantrell-Haller-Ravitsch Syndrome.

A case of exencephaly associated with spinal dysraphism and Cantrell-Haller-Ravitsch syndrome is described. Significant findings included exencephaly, multiple subcortical neuroepithelial islets with active proliferation, cerebellar aplasia, and abnormal flexion of the neuraxis with spinal dysraphism of the cervicothoracic region. The various features of exencephaly, anencephaly, and iniencephaly are discussed. The significance of subcortical growth zones is evaluated in the light of experimental observation.s

Abnormalities, Multiple↗

Surgical release of tethered spinal cord: survivorship analysis and orthopedic outcome.

Between January 1988 and February 1995, 133 tethered spinal cord-release procedures in 88 consecutive patients were performed at our institution and were used to determine survivorship data for surgical release of tethered spinal cord. The diagnoses included spinal dysraphism (67), achondroplasia (nine), isolated tethered cord (nine), cerebral palsy (three), and others (seven). Survivorship data were calculated for the initial and first-revision tethered cord release. There was a 50% revision rate by 5 years after initial tethered-cord release and a 57% revision rate by 2 years after a second release. Thirty-six patients were excluded for having <2 years of clinical follow-up, leaving 97 spinal cord releases in 52 patients available for outcome analysis. At a mean follow-up of 4.4 years (range, 2-11.3), 58% of patients required one or more orthopedic procedures after tethered-cord release. The average number of orthopedic procedures per year before release (0.28/year) was found to increase after initial release (0.39/year; p < 0.05). These data demonstrate the frequent need for operative revision after index tethered-cord release, especially in children with spinal dysraphism. In addition, the need for orthopedic procedures after tethered spinal-cord release frequently persists.

Adolescent↗

Lumbar canal stenosis: a cause of late neurological deterioration in patients with spina bifida.

BACKGROUND: Patients diagnosed with spina bifida may show late deterioration. This worsening in their clinical symptoms has been attributed to a multiplicity of causes such as secondary tethering of the spinal cord, Chiari II anomaly, hydromyelia, diastematomyelia, arachnoid cysts, and dermoid tumors. METHODS: We searched the clinical records of patients diagnosed with spina bifida who were treated at our hospital for a period of 25 years for the purpose of ascertaining the number and etiology of cases of late neurological deterioration. RESULTS: Six of 144 patients with open spina bifida presented with late neurological deterioration. In one of these cases and in another patient with occult spina bifida the most relevant factor noted during surgery was the presence of marked lumbar canal stenosis. CONCLUSION: We suggest that certain cases of late clinical worsening in spina bifida patients are because of lumbar canal stenosis and that this condition should be added to the list of causes that may produce delayed neurological deterioration in patients with spinal dysraphism.

Adult↗

[Creation of a model for myelomeningocele in rabbit embryos].

UNLABELLED: Spinal dysraphism causes paraplegia, fecal incontinence, neurogenic bladder, sexual dysfunction, hydrocephalus and skeletal abnormalities in newborns. Its ethiology and pathogenesis are still not known, and probably multifactorial. AIM: To determine whether spinal cord exposition to the amniotic space causes a functional (impairment) and anatomic lesion similar to that of human myelomeningocele. MATERIAL AND METHODS: Forty-eight fetal rabbits underwent to create spina bifida on the 23rd gestational day (term is 31 days). The procedure consisted of lumbosacral skin excision and posterior laminectomy. The fetuses are allowed to continue their gestation. On the 30th gestational by the operated fetuses were harvested, together with a group of nonoperated littermates for control. A clinical and neurologic evaluation was done, as well a study of somato-sensorial evoked potentials in the upper and lower limb and histologic study of the affected vertebral and cordial segment. RESULTS: The 26 surviving animals had deformity and lack of movement of the lower limbs. Evoked potentials showed absent response to stimuli in the lower limbs of animals with spina bifida, whereas upper limbs and control animals did respond. Histologically the spinal cord of the operated rabbits was uncovered and flattened. CONCLUSION: This model of myelomeningocele in fetal rabbit reproduces a variety of features similar to human spinal dysraphism, and hence can be used to study the pathophysiology of spina bifida.

Animals↗

Urodynamic findings in the tethered spinal cord: the effect of tethered cord division on lower urinary tract functions.

PURPOSE: The aim of this study is to evaluate the effect of division of the tethered spinal cord urodynamically in spinal dysraphic cases. METHODS: Between 1995-1997, 20 cases (11 males, 9 females) aged from 5 months to 13 years with TSC were investigated. 13 cases (65%) were classed as belonging to the myelomeningocele group and 5 cases (35%) to the spina bifida occulta group. We used a computerized urodynamic system to evaluate the functions of the lower urinary tracts pre- and postoperatively. The definitive diagnosis of cord tethering was made using magnetic resonance imaging (MRI) in 19 cases (95%) and spinal ultrasound in 1 case (5%). Division of filum terminale and laminectomy were carried out in all cases by the Neurosurgery Department, and 2 cases with retethering were operated on twice. RESULTS: All of these cases were assessed urodynamically in the preoperative and postoperative period. Significant improvements were noted in detrusor functions (35%); electromyography recordings (45%); high leak point pressures (55%) and anal and urinary continence (70%). CONCLUSION: Lower urinary tract dysfunctions secondary to tethered cord syndrome are very common in spinal dysraphic cases and significant improvements can be achieved with a judiciously timed division of the spinal tethered cord.

Adolescent↗

Cervical spina bifida cystica: MRI differentiation of the subtypes in children.

BACKGROUND: Cervical spinal dysraphism is a rare condition with only 37 cases reported in the literature, of which only seven patients had undergone preoperative magnetic resonance imaging (MRI). OBJECTIVE: By using illustrative cases, we demonstrate the value of MRI in distinguishing the two different subtypes of cervical spinal dysraphism, namely, myelocystocele and meningocele. METHOD: Retrospective review of the MRI scans of six patients with cervical (one high thoracic) posterior cystic swellings, which were diagnosed as cervical dysraphism, was done. RESULTS: Three patients were diagnosed with myelocystocele and three patients with meningocele. A comparison of MRI features between the two entities, as well as the associated anomalies, is discussed. CONCLUSION: MRI is the imaging modality of choice for suspected cervical dysraphism, prior to surgery. It helps distinguish the subtypes, as well as identify the associated anomalies.

Adolescent↗

[Fetal MRI: value and limitations--a review of 55 lesions].

OBJECTIVE: This study was undertaken to evaluate the usefulness and limitations of fetal MR imaging. MATERIALS AND METHODS: Fifty-five prenatal MRI examinations were performed, and the results were confirmed by postnatal follow-up or from pathological examination and clinical findings. All examinations were performed between 18 and 40 weeks of gestation without any premedication. A 1.5-Tesla superconductive MR imaging unit was used to obtain half-Fourier acquisition single-shot turbo spin-echo (HASTE) images. RESULTS: There were two false-positive cases on US. MRI allowed the diagnosis of serious unsuspected lesions such as hypoplastic lung and anorectal malformation. MRI was also useful for the precise diagnosis of ventriculomegaly. However, MRI missed two cases of spinal dysraphism and one case of laryngotracheoesophageal cleft. CONCLUSION: Fetal MRI is a valuable complementary tool when prenatal US is inadequate or doubtful. MRI is useful for the evaluation of pulmonary hypoplasia or hydrocephalus and may modify obstetric management. MRI may be able to diagnose spinal dysraphism by detecting mild widening of the CSF space. However, MRI currently is not useful for making a differential diagnosis between laryngotracheoesophageal cleft and esophageal atresia.

Congenital Abnormalities↗

Ultrasound evaluation of prenatal and neonatal spina bifida.

Interest in studying the role of sonography in the evaluation of newborns and infants with spinal dysraphism waned with the advent of MR imaging. Sonographic evaluation of the neonatal spine, however, can be performed portably, is relatively inexpensive when compared with MR imaging, and does not require sedation. Its recognized utility in evaluating the neonatal spine in both open and occult spinal dysraphisms makes sonography an ideal diagnostic modality for this patient population. Its most valuable contribution probably lies in screening for closed dysraphisms, but comparative studies with MR imaging are necessary to clarify sonography's role.

Cerebral Ventricles↗

Human tail associated with lipomeningocele--case report.

A 3-month-old boy presented with a tail associated with lipomeningocele. Computed tomography and magnetic resonance imaging clearly demonstrated the presence of spina bifida and lipoma continuous from the tail to the thickened conus medullaris. The human tail may be related to spinal dysraphism and requires detailed neuroimaging investigation, and possibly microsurgery to prevent the tethered cord syndrome.

Adipose Tissue↗