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PubMed2006

Responses to lumbar magnetic stimulation in newborns with spina bifida.

Searching for a tool to quantify motor impairment in spina bifida, transcranial and lumbar magnetic stimulation were applied in affected newborn infants. Lumbar magnetic stimulation resulted in motor evoked potentials in both the quadriceps muscle and the tibialis anterior muscle in most (11/13) subjects. However, transcranial magnetic stimulation did not lead to any response at all. A strong left-to-right correlation existed for amplitude and for latency. Lumbar magnetic stimulation proved to be applicable in newborn infants with spina bifida. Although current concepts regarding spina bifida suppose lower motor neuron dysfunction, the results of this study suggest that lower motor neuron integrity is at least partly preserved after birth. Transcranial magnetic stimulation does not lead to responses in healthy newborn infants because of insufficient synaptogenesis, myelinogenesis, and axon thickness. Therefore, conclusions on upper motor neuron function in spina bifida cannot be drawn. To what extent the method used here can achieve the aim of quantifying motor impairment is a matter of further study.

Electromyography↗
PubMed2003

[Tethered cord syndrome: an unusual cause of adult bladder dysfunction].

Tethered cord syndrome is a complication of spinal dysraphism. The tethering of the cord does not permit the normal cranial migration of the conus within the vertebral canal. The result is a neural dysfunction due to a traction neuropathy. Although this condition commonly presents in childhood, less severe degrees of tethering may remain asymptomatic until adult life. The authors report a new case of tethered cord syndrome in a 25-years-old young girl, cause of bladder dysfunction. Clear improvement of urinary repercussions is obtained after surgery.

Adult↗
PubMed1976

Midline hamartomas masquerading as meningomyeloceles or teratomas in the newborn infant.

Congenital miline dorsal malformations in infants most commonly represent spina bifida cystica or teratoma. The five patients reported here are a distinct clinicopathologic entity. They presented at birth with a dorsal midline mass, with no neurologic impairment, and no hydrocephalus developed subsequently. The lesions were removed in all infants without significant mobidity. The masses were comprised prinicipally of mesodermal elements, i.e., bone, cartilage, fat, muscle, and fibrous tissue. The well-differentiated cellular components, forming mature structures and the lack of primitive organoid structures as well as the absence of neoplastic characteristics, favors a diagnosis of hamartoma rather than of teratoma.

Child, Preschool↗
PubMed1994

Pubovaginal slings for the management of urinary incontinence in female adolescents.

A pubovaginal sling is an effective treatment for type III incontinence secondary to poor proximal urethral sphincter function. We used a pubovaginal sling to treat incontinence in 15 female adolescents. The etiology of incontinence was spinal dysraphism in 10 patients and prior trauma in 3. Simultaneous bladder augmentation was performed in the remaining 2 patients for poor bladder compliance. Three patients required additional procedures including repeat slings in 2 and repeat augmentation in 1. Of 13 patients followed for more than 6 months 11 remain dry, 1 leaks small amounts and wears 1 pad per day, and 1 did not achieve acceptable continence and was subsequently managed with bladder augmentation and a Mitrofanoff procedure. The upper urinary tracts have remained normal in all 13 patients. The pubovaginal sling has proved to be safe and successful in these children. The overall continence rate of 92% compares favorably to other available modalities.

Adolescent↗
PubMed1992

Congenital spinal malformations.

When presented with an animal that has a congenital spinal malformation, the veterinarian needs to consider the clinical significance of the malformation, the possible presence of other anomalies--spinal and nonspinal, the heritability of the malformation, and potentially innovative treatment options. This article includes explanations of the conditions and information regarding diagnosis and treatment of hemivertebrae and block vertebrae, malformations at the cranial-vertebral junction, osteocartilaginous exostoses, spinal bifida, and spinal stenosis.

Animals↗
PubMed2006

Visual side-effects from transdermal scopolamine (hyoscine).

Transdermal scopolamine may be used to reduce drooling in children with disabilities. Side-effects include dilated pupils and a reduction in the near point of accommodation (the closest point at which clear vision is possible). Two male children with epilepsy, one with spinal dysraphism (aged 7y 6mo) and one with cerebral palsy (aged 5y 8mo), who have undergone treatment for drooling with transdermal scopolamine are described. Near visual acuity was reduced, and both children showed dilated pupils with reduced or no response to light. These responses became normal on cessation of the scopolamine patch. As the effect of this drug may be cumulative, and many patients are unable to communicate difficulties, clinicians need to be aware of these possible side-effects.

Administration, Cutaneous↗
PubMed2002

A comparison of visual function scores in hydrocephalic infants with and without lumbosacral myelomeningocoele.

PURPOSE: The cerebrum is frequently malformed in children with myelomeningocoele. This anomaly renders them potentially susceptible to cerebral visual impairment. In these patients, hydrocephalus is an important and frequent complicating lesion which compromises intellectual function and may also cause cerebral visual impairment. In this study, we determined whether hydrocephalic patients with lumbar myelomeningocoele (HLM) are at a greater risk of visual impairment than hydrocephalic patients without this lesion (H). METHODS: In this prospective study, we assessed five parameters of visual function in 20 hydrocephalic children with lumbar myelomeningocoele and compared the total visual function scores (TVFS) obtained with those from hydrocephalic children without overt spinal dysraphism, but similar in age, sex and ventricular size. The parameters, which were assessed with the aid of a quantitative grading scale, were pupillary size and reaction, optic atrophy, visual fixation and tracking. RESULTS: The age and sex distributions of the patients in the two groups were similar. The anterior and posterior dimensions of the lateral ventricles were also similar. The mean (SD) of the TVFS were 24.25 (3.63) and 24.20 (3.47) respectively for the two groups (P = 0.90). CONCLUSIONS: The results suggest that, in hydrocephalic infants with lumbar myelomeningocoele, visual function is not further diminished by the associated dysraphism and that ventricular dilatation is the major determinant of visual impairment.

Age Distribution↗
PubMed2001

Benign symmetric lipomatosis with myelomeningocele in an adolescent: An uncommon association-case report.

The authors report an unusual localization of symmetric adipose tumors associated with spinal dysraphism. Initially, the patient underwent a closure of the lumbosacral myelomeningocele. At that time, the tumors were not evident and remained undiscovered up until puberty. However, during puberty, the symmetric lipomatous masses grew at the perineal region. Except the patient's age, all findings and the clinical picture supported the diagnosis of a benign symmetric lipomatosis (BSL). To the authors' knowledge, the following case has not been described previously.

Adolescent↗
PubMed2001

Idiopathic megarectum in children.

There is scant information about the management of idiopathic megarectum in childhood. Children with idiopathic megarectum referred to a single institution between 1994 and 1998 were identified prospectively. Those with Hirschsprung's disease or an anorectal malformation were excluded. The remaining patient group, 22 boys and 7 girls, had a median age of 8.0 years (range 3.5-14.0 y). Median duration of symptoms prior to referral was 2.0 years (range 0.4-11 y). Chronic soiling was the dominant complaint in 28/29 (97%) cases. 23 children had received regular stimulant laxatives for periods ranging from 1 month to 11 years, and 9 children had been treated with regular enemas. The degree of megarectum assessed by both abdominal palpation and plain radiography was: grade 1 (below umbilical level) n=6; grade 2 (at umbilical level) n=15; and grade 3 (above umbilical level) n=8. Hirschsprung's disease was specifically excluded by rectal biopsy in all cases and no patient had evidence of spinal dysraphism. Three boys with massive megarectums and intractable symptoms were treated by a staged Duhamel sigmoid pull-through with excellent functional results. Fifteen patients (52%) were treated by a single manual evacuation under general anaesthesia followed by a daily Bisacodyl 5-10 mg suppository. After a median follow-up of 16 months, 13 continue to respond well with a daily bowel action and no soiling (4 of the 13 have discontinued treatment and remain well). The remaining 11 patients (38%) have continued conventional treatment with oral laxatives but with limited success. Idiopathic megarectum is poorly described in children. It is more common in boys and is often resistant to laxative therapy alone. After appropriate preparation, treatment with stimulant suppositories can be effective. Surgery has a valuable role in selected patients with a massive megarectum.

Adolescent↗
PubMed1988

The management of dropfoot using the Lambrinudi arthrodesis.

The results of 12 Lambrinudi arthrodeses in 10 patients with spinal dysraphism were compared with 11 operations performed in patients with only motor nerve deficits performed over the same 10 year period. The early failure and complications were more common in these patients and the tendency to recurrence of dropfoot occurred earlier. Despite this, the operation was effective in the management of these patients, especially for the improvement of co-existent sores or the fitting of shoewear.

Adolescent↗
PubMed1984

Intrauterine treatment of spina bifida: primate model.

A spina-bifida-like condition was induced in 8 Macaca mulatta fetuses by intra-uterine lumbar laminectomy (L3-L5) followed by displacement of the spinal cord from the central canal. This condition was repaired in utero in 5 animals. A key feature of the repaired in utero in 5 animals. A key feature of the repair method was the use of allogeneic bone paste made from fetal bone particles suspended in an agar-thickened modified Dulbecco's medium. The bone paste was used to correct the bone deficit produced by the laminectomy. All of the monkey babies were delivered by cesarean section at near term (160-164 days of gestation). Neurological test on the neonates and subsequent morphological studies indicated that the in utero treated animals developed normally. In contrast, 3 control animals, which had the induced spinal dysraphism but were left untreated, showed severe spina-bifida-like abnormalities, including paraplegia, incontinence and somatosensory loss.

Animals↗
PubMed1992

Infantile hydrocephalus in the south-western region of Saudi Arabia.

During the period January 1988 to December 1990, the overall incidence of infantile hydrocephalus in the south-western region of Saudi Arabia was 0.81/1000. The series comprised 61 infants affected by infantile hydrocephalus. The total number of live births in this period was 74,923. Hydrocephalus associated with spinal dysraphism (spina bifida cystica and encephalocoele) constituted 24 cases (39.3%), and there was aqueduct stenosis in 10 cases (16.4%). Nine cases (11.9%) were post-meningitic, and seven (14.8%) post-haemorrhagic. There was Dandy-Walker malformation in five cases (8.2%), three (4.9%) had congenital idiopathic hydrocephalus, two (3.3%) congenital toxoplasmosis, and one (1.6%) isolated Arnold Chiari malformation. There were congenital causes in 45 cases, 73.7% of all the cases in this series (incidence: 0.6/1000 births). Of the remaining 16 cases (26.3%) which were due to postnatal factors, seven (11.5%) were caused by acquired cerebral haemorrhage, only two of them being premature, and the other nine (14.8%) were due to meningitis. The data indicate the predominance of prenatal causes of infantile hydrocephalus and the relatively low contribution of extreme prematurity. This is possibly due to the high mortality of this group of infants in this region.

Cerebral Hemorrhage↗
PubMed2000

A theory on the embryogenesis of oculo-auriculo-vertebral (Goldenhar) syndrome.

Oculo-auriculo-vertebral (OAV) syndrome is made up of anomalies, mainly of first and second branchial arch derivatives. Characteristic features include structural malformations of the external and middle ears, face, and jaw. It has been previously suggested that hemorrhage involving the first and second branchial arches causes hypoplasia and malformation of the face and auricle, but this theory cannot explain the multisystemic and protean manifestations of this disease. The theory set forth describes the cutaneous, facial, vertebral, and systemic anomalies in the OAV spectrum as a result of ectodermal nondisjunction early in development with subsequent mesodermal tethering. A subgroup of OAV may therefore be a disorder of ectodermal nondisjunction involving the otic placode similar to the spectrum of diseases such as occult spinal dysraphism that is associated with the same mechanism in the embryonic neuraxis. This would imply a molecular mechanism involving cell adhesion molecules that unify the two disease processes and explain the multisystem anomalies of the OAV syndrome.

Branchial Region↗