Increased incidence of spina bifida occulta in fluorosis prone areas

Spina bifida, a congenital deformity of the posterior wall of vertebrae of the spine, is a midline defect of skin, vertebral arches and neural tube, usually in the lumbosacral region. Its incidence is reported to be 0.2 to 0.4 per 1000 live births. Various hypotheses have been put forward as etiological factors for spina bifida including consumption of potato affected by blight and hardness of drinking water but these have not been proven. Two groups of 50 randomly chosen children were established. The study group consisted of children aged 5 to 12 years, weighing 15 to 30 kg, consuming fluoride rich drinking water (4.5 and 8.5 ppm fluoride; WHO permissible limit is 1.5 ppm fluoride), and manifesting either clinical, dental and/or skeletal fluorosis. The control group consisted of age and weight-matched children, consuming less than or equal to 1.5 ppm fluoride in drinking water and not showing any evidence of fluoride toxicity. These children were evaluated for antenatal history, general clinical examination (especially for dimples, tufts of hair, haemangioma on skin throughout the length of spine), other congenital abnormalities, evidence of fluoride toxicity, biochemical estimation for fluoride levels in blood and serum and by skiagrams of the spine to examine for the presence of spina bifida occulta. A total of 22 (44%) of the 50 children in group A, the study group, and 6 (12%) of the 50 children in group B, the control group, revealed spina bifida occulta in the lumbosacral region.(ABSTRACT TRUNCATED AT 250 WORDS)     

Clinical consequences of spina bifida occulta

OBJECTIVE: To study data concerning the pathogenesis, frequency of occurrence, clinical manifestations and associated abnormalities of spina bifida occulta (SBO) and re-evaluate the clinical importance of the lesion. DATA SOURCES: International journal articles indexed through Medline, and specific related texts, the majority of which were published after 1989. Key indexing terms used were spina bifida occulta, tethered cord syndrome and spondylolysis. RESULTS: The reported frequency of occurrence of SBO varies widely, depending largely on the age groups included in a particular study. The most accurate estimate of occurrence rate is 17% of examined spines. There is a significant association of some cutaneous stigmata, most notably hypertrichosis, with midline posterior arch defects. An increasing amount of evidence links SBO with a number of specific anomalies and clinical syndromes, including intraspinal lipoma, tethered cord syndrome, genitourinary dysfunction, increased incidence of disc pathology, lumbar spondylolysis, foot deformities and syringomyelia. A questionable association exists with epilepsy. A supposed link between constipation and SBO is lacking sufficient data to support it. CONCLUSIONS: SBO may be associated with pathology and significant sequelae, although the majority of lesions pose no clinical threat. The predictive value for adverse sequelae in a particular lesion is difficult to assess; however, multilevel occurrence and more expansive involvement in a given segment seem to be associated with higher risk of sequela. The treatment for SBO with progressive neurologic deficit is surgical intervention; however, reversal of the deficit is unusual and a halting of neurologic deterioration is a more realistic goal. Early diagnosis of this lesion, before the age of 3 yr, is associated with better surgical outcomes.     

Peripheral neurological involvement as the first manifestation of spina bifida occulta

A 42-year-old male was admitted for right-sided sciatica with asymptomatic septic arthritis of the fifth toe of the right foot. He had a history of active chronic hepatitis C and septic arthritis of the fifth toe of the left foot. His symptoms included low back pain, poorly systematized right-sided sciatica, impairment of all forms of sensation in both lower limbs, absent ankle jerks, episodes of urinary retention, urgency, and painless septic arthritis of the fifth toe of the right foot. Roentgenograms showed a spina bifida occulta of L5 and a bony erosin in the distal interphalangeal joint of the right fifth toe. Distal denervation in the territory of L5 was demonstrated by the electromyographic study. Magnetic resonance imaging disclosed an area of high signal on T1 and T2 images, located within the spinal canal opposite L4 and suggestive of an intraspinal lipoma, as well as tethering of the spinal cord in an abnormally distal position. Antimicrobial therapy was effective in ensuring resolution of the infectious arthritis. The low back pain and sciatica responded to nonsteroidal antiinflammatory drug therapy and did not recur subsequently. Many patients who have roentgenograms taken to evaluate low back pain and sciatica are found to have a spina bifida occulta. This complex birth defect involving the spinal canal, meninges and spinal cord or cauda equina can cause neurologic and/or urinary symptoms in adulthood. Magnetic resonance imaging is essential in this situation to evaluate the spinal cord and to look for an intraspinal lipoma.     

Three-dimensional gait analysis in spina bifida

This study was designed to determine gait patterns in children with lumbar and sacral neurologic level spina bifida. We studied a group of 28 children: 10 had L4-level lesions and a mean age of 11 years; eight had L5-level lesions and a mean age of 8 years; and 10 had S1-level lesions with a mean age of 12 years. A group of 15 normal children, mean age 10 years, was used for comparison. Each child underwent three-dimensional gait analysis using the Vicon system. We found that there were recognisable gait patterns for each level of spina bifida and that the abnormalities accurately reflected the muscle deficiencies present. The gait patterns approximated more closely to those of the normal group as the neurological level descended. The most important findings were of increased pelvic obliquity and rotation with hip abduction in stance (reflecting the gross Trendelenburg-type gait seen in these children) and persistent knee flexion throughout stance as a result of the absence of the plantar flexion-knee extension couple. We found that gait was not improved by tendon transfers performed either at the hip (posterolateral psoas transfer) or at the ankle (tibialis anterior transfer).     

The management of deformities of the foot in children with spina bifida

A simple photographic technique is described which measures the weight-bearing and non-weight-bearing areas of the feet in the standing child. The child stands on a rectangular sheet of thick glass which is set into the floor. Beneath the floor is a cellar, from which the soles of the feet may be observed and photographed. A transparent grid placed over the photographic print enables the weight-bearing and non-weight-bearing areas of the foot to be measured, and thus to express the weight-bearing areas as a percentage of the total area of the sole of the foot. The procedure was used to assess 55 spina-bifida children who had had surgical correction of deformities of the feet. The findings are discussed.     

Depressive symptoms and self-concept in young people with spina bifida

Examined self-reported depressive symptoms in 72 young people with spina bifida, ages 9 to 18:11, and matched able-bodied comparison subjects, using the Dimensions of Depression Profile for Children and Adolescents (Harter & Nowakowski, 1987). Independent variables included gender, self-perceptions (including physical appearance), and perceived social support. Young people with spina bifida were at greater risk of depressive mood, low self-worth, and suicidal ideation. Girls, independent of disability, were at greater risk of depressive mood, low self-worth, and self-blame. Multiple regression analyses suggest that global self-worth serves as a mediating variable for the effect of physical appearance self-concept on depressed mood (particularly in young people with spina bifida), and that perceived parental social support has a direct effect on depressed mood (particularly in girls).     

Genetics of idiopathic scoliosis

To define the luminal agent(s) responsible for the reduction of nephron filtration rate following increases of loop of Henle flow rate early proximal flow rate (EPFR) during loop perfusion with 17 different salt solutions were compared to the non-perfused tubules. During orthograde microperfusions a reduction of EPFR as indication of a feedback response was noted with a number of monovalent Cl- and Br- salts (LiCl, KCl, NaCl, RbCl, CsCl, NH4Cl, choline Cl, NaBr, KBr), with Na+ salts except Na acetate (NaHCO3, NaNO3, NaF, NaI, NaSCN), and with CaCl2 and MgCl2. These latter 2 solutions where used in a concentration of 70 mM while all other solutions had a concentration of 140 mM. During retrograde perfusion from the distal to the proximal end of the loop of Henle EPFR fell significantly with Cl- and Br- salts with percentage changes of EPFR ranging from -8.0 to -44.3%. In contrast, Cl- free salts and Cl- salts of divalent cations were associated with percentage changes of EPFR ranging from +7.1 to -6.2%, significance being reached only during perfusion with NaSCN. When furosemide (5 x 10(-4) M) was added to NaBr or KBr a feedback response was not observed. During orthograde perfusion with NaNO3 distal Cl- concentrations were 44.2 +/- 5.08, mM (mean +/- S.E.) at a perfusion rate of 10 nl/min and 59.1 +/- 3.93 mM at a rate of 40 nl/min. CaCl2 perfusion induced a marked elevation of distal Cl- concentrations to levels higher than 140 mM. Loop chloride handling was normal during RbCl perfusion. The magnitude of the feedback response during retrograde perfusion was not changed by lowering NaCl concentration from 140 to 60 mM, but fell when NaCl concentration was further reduced. In contrast to orthograde perfusions it was insensitive to changes in flow rate. Our results are compatible with the thesis that feedback responses depend critically upon the rate of Cl- transport probably across the macula densa cells. Br- ions can replace Cl- because they appear to share a common transport pathway which can be inhibited with furosemide. Unspecificity of feedback responses during orthograde microperfusions is due to presence of Cl- ions in the macula densa region even when solutions are initially Cl- free. Cl- salts of divalent cations do not elicit a feedback response because Cl- transport is severely curtailed.     

Treatment of idiopathic scoliosis in children

This is an update on the surgical and orthopedic management of scoliosis in children. After a review of the biomechanical factors underlying the scoliotic deformity in the three dimensions, methods and indications of conservative treatment combining physiotherapy and full-time or part-time bracing are discussed. Development of new segmental spinal instrumentation devices has modified the surgical treatment of scoliosis by allowing three-dimensional correction of deformities and obviating the need for postoperative immobilization in a cast or corset. Results of conservative treatment are analyzed in an original series of 56 children with progressive scoliosis treated when the angulation was still under 30 degrees. Mean follow-up since the end of treatment is 14 years. Results show that proper conservative treatment arrests progression and modifies the natural history of scoliosis. Outcome after surgical treatment is analyzed in an original and recent series composed of the 50 first children who had posterior surgery with the new Cotrel-Dubousset instrumentation. Results of anterior surgery (V.D.S. instrumentation), which is still indicated in some cases, are analyzed in an older series of 18 cases. When the deformities are too severe to allow conservative treatment, short segment fusion effectively corrects the scoliosis and reliably provides good cosmetic and functional results.     

Assessing the development of daily living skills in patients with spina bifida

The development of independence in daily living skills of 256 patients with spina bifida was assessed by means of a new Functional Activities scoring system. The score was derived from nine categories of activity; five are to do with self-care, three with locomotion and one with social interaction. The reasons for the variation in achievement between individuals with the same degree of paralysis are discussed, as are the differences in learning curves for single activities. The scoring system enables a quantitative assessment to be made of the current status of an individual patient, and enables more realistic therapeutic goals to be set.     

Faecal incontinence in children with spina bifida: the best conservative treatment

We report the case of a 75-year-old woman with an uneventful medical history who presented with sudden-onset diplopia immediately after neck trauma in a traffic accident. Clinical and ophthalmological examinations were consistent with bilateral abducens nerve palsy. The causal relationship, including the medicolegal implications and the pathophysiological mechanism are discussed. The case demonstrates that exceptionally benign trauma can cause severe diplopia. The delay to recovery may be very long, suggesting caution concerning the decision of surgical care.     

Arnold-Chiari-like malformation associated with a valproate model of spina bifida in the rat

The brains of rat fetuses exposed to saline and valproic acid (VA: 600 mg/kg or 1,200 mg/kg) at 10 days of gestation were examined for structural changes similar to that seen for the Arnold-Chiari malformation (ACM) in humans. Only fetuses exposed to the 1,200-mg/kg level VA demonstrated spina bifida (SB) type widening of the vertebral arch when compared to saline treated animals. When compared to controls, both 600-and 1,200-mg/kg VA animals demonstrated microencephaly. The brains of treated animals demonstrated alterations in their angular morphology such that the cerebellum was displaced toward the foramen magnum in a manner akin to human ACM. These findings indicate that valproate-induced SB in the rat more closely approximates SB in humans than was previously appreciated and provides a model for experimental study of both SB and ACM.     

Prevalence of adolescent idiopathic scoliosis in Hungary

An attempt of PCS was performed for the first time in Japan. Eighteen hundred twenty-six preschool and school children were examined and the result was compared with other studies (physician's auscultation and PCG). The time requirement for a single child is approximately 1.5 min. in primary school. It took longer time in kindergarten. The heart murmur detection rate is high and the sensitivity is fairly good, although it is not so good as well-trained physician's heart murmur detection rate. PCS is not perfect, but is fairly good tool for screening. It can be used only by technician. PCS may be able to save the physician's time.     

The correlation of idiopathic lumbar scoliosis and lumbar lordosis

A definite correlation exists between the presence of idiopathic lumbar scoliosis and hyperlordosis of the lumbar spine. Present data do not reveal whether hyperlordosis constitutes an element which makes a "spine at risk" for developing scoliosis. However, the presence of an extreme lordosis suggests that in any comprehensive plan of treatment of idiopathic lumbar scoliosis, provision should be made for correction of severe deformities of the lumbar spine.     

Trajectories of autonomy development across the adolescent transition in children with spina bifida.

Objective: The current study investigated individual growth in autonomy development across the adolescent transition, comparing the trajectories of children with and without spina bifida. Method: Individual growth curve modeling procedures were utilized to describe the developmental course of autonomy across four waves of data collection, from ages 9 to 15, and to test whether illness status [spina bifida vs. matched comparison group (N = 68 for both groups at Time 1)] would significantly predict individual variability in autonomy development. Potential moderators [child gender, SES, and Peabody Picture Vocabulary Test (PPVT) score] of the association between illness status and autonomy development were also examined. Results: Children with spina bifida demonstrated distinct developmental trajectories, though the nature of the group differences varied by type of autonomy development (emotional vs. behavioral), context (i.e. school vs. family), and reporter. Significant interactions with PPVT score and child gender were found. Conclusion: Overall, children with spina bifida show considerable developmental resiliency, but may lag behind their peers in specific areas of autonomy. Boys with spina bifida, and children with spina bifida who have lower than average levels of verbal intelligence, appear to be at greater risk for exhibiting delays in autonomy development. (PsycINFO Database Record (c) 2010 APA, all rights reserved)