1/12. Primary tethered cord syndrome: diagnosis and treatment of an insidious defect.Failure to recognize the signs and symptoms of tethered cord syndrome in patients with spina bifida occulta can result in tragic consequences. Of patients with tethered spinal cord, 35% have bowel and/or bladder dysfunction. scoliosis, foot or leg length discrepancies, pes cavus, and varus or valgus deformities also can occur. Early assessment and intervention by the neuroscience nurse who is familiar with these clinical signs can help prevent significant deformities and irreversible neurological deficits.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/12. spinal dysraphism presenting as acro-osteolysis: report of four cases.The acro-osteolyses are a heterogeneous group of disorders characterized by bone resorption. The disorder may occur as familial, idiopathic, or secondary to vascular, inflammatory, or neurologic conditions. acro-osteolysis is rare in association with spinal dysraphism. It is even rarer for it to be the presenting symptom in spinal dysraphism. We report here four patients in whom the diagnosis of spinal dysraphism was established while investigating for the various causes of acro-osteolysis. All four patients presented with trophic changes and acro-osteolysis. hyperhidrosis in the affected limb was seen in three patients. One patient had leg pain, the others had no sensory or motor deficits. magnetic resonance imaging showed spinal dysraphism in all four patients.- - - - - - - - - - ranking = 16684.193894874keywords = spinal dysraphism, dysraphism, spinal (Clic here for more details about this article) |
3/12. Occult spinal cord abnormalities in children referred for orthopedic complaints.This study reviews spinal cord abnormalities found in children initially referred for an orthopedic problem. Over a 5-year period in an academic pediatric orthopedic referral clinic, 167 children aged 3 months to 18 years (average, 9.4 years) underwent spine magnetic resonance imaging (MRI) scans and had records available for review. The patients were divided into 7 major groups based on the primary indication for the MRI. The frequency of spinal cord pathology was as follows: 3 of 35 patients with atypical idiopathic scoliosis, 1 of 19 with neuromuscular scoliosis, 6 of 18 with congenital scoliosis, 1 of 50 with unexplained back pain, 3 of 17 with gait abnormality, 5 of 14 with limb pain or weakness, and 4 of 8 with rigid or recurrent foot deformity. spine MRI was not very helpful in evaluating children who had some degree of back pain without neurological signs or symptoms. However, the spine MRI was helpful in evaluating children with atypical idiopathic scoliosis or congenital scoliosis, gait abnormality, limb pain or weakness, or rigid or recurrent foot deformities. Given the high frequency of occult spinal cord abnormality in children with severe foot deformity, the use of screening spine MRI may be especially useful in this group.- - - - - - - - - - ranking = 7keywords = spinal (Clic here for more details about this article) |
4/12. Spondylocarpotarsal synostosis syndrome (with a posterior midline unsegmented bar).Spondylocarpotarsal synostosis syndrome (SSS) is characterised by malsegmentation of the thoracic spine and carpal/tarsal fusions. A unilateral or bilateral unsegmented bar may be present in the thoracic spine. Presenting clinical signs are congenital scoliosis early in life, and shortening of the trunk with scoliosis and/or lordosis in older children. We report a 13-year-old girl with SSS and a midline unsegmented bar running along the spinal processes of T3 to L2 and extending into the posterior vertebral elements.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
5/12. Congenital lumbosacral limb duplication: a case report.We report a case of congenital lumbosacral limb duplication at the pelvis, lower limb, and foot of a 4-year-old boy. The patient presented with a supernumerary lower limb joined at the lumbosacral region by a bony connection. A pseudo-navel and a pseudo-penis were found on the supernumerary limb and foot duplication was noted at the end. radiography and a 3-dimensional computed-tomography reconstruction revealed duplication of an inversely oriented pelvis dorsal to the normal pelvis, semi-vertebrae in T7 to T9, thoracolumbar scoliosis, lumbosacral vertebral dysplasia, and dislocation of the left hip. magnetic resonance imaging demonstrated a tethered spinal cord and an adipoma in the sacral canal. Motor conduction velocity was reduced in the left peroneal nerve. Surgery to remove the supernumerary limb was successfully performed. The patient recovered well and further surgery is planned for functional reconstruction of the left lower limb.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
6/12. Acrodysostosis in two generations: an autosomal dominant syndrome.Acrodysostosis is a rare syndrome characterized by growth retardation, peripheral dysostosis and mental deficiency. x-rays reveal generalized shortening of metacarpals, metatarsals and phalanges, hyperplasia of the first ray of the feet and premature skeletal maturation. Occasionally abnormal interpedicular spinal spaces, increased mandibular angle and hearing loss have been observed. We report a 19-year-old woman and her daughter examined at birth and subsequently at 6 years of age. The clinical and radiological characteristics are those of acrodysostosis. The syndrome is easily recognized at birth. The generalized corporal shortening is progressive and could be due to premature closing of epiphyses. The finding of an affected mother and her daughter support the postulate that acrodysostosis is inherited as an autosomal dominant syndrome.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
7/12. The hand-foot-uterus syndrome: a case study.An autosomal dominant syndrome is described in 26 members of six generations in a single family. Distal extremity malformations are characteristic and superficially resemble those of arthrogryposis, chondroectodermal dysplasia, Cornelia de lange syndrome, Faconi's anemia or Holt-Oram syndrome. There is an absence of spinal deformity, and females of the disorder have duplication of the genital tract.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
8/12. Os calcis osteotomy in the management of deformities of the hindfoot in spinal dysraphism.The results of 52 primary os calcis osteotomies in 44 patients with spinal dysraphism are reviewed. There were 42 varus hindfoot deformities and ten valgus hindfoot deformities. The results indicate that the operation is simple and uncomplicated, but over a mean 5.8 year follow-up for the varus feet and 6.5 years for the valgus feet, over 50% of the feet developed either a moderate or severe recurrence of deformity. Os calcis osteotomy appeared from this series to be a moderately successful interim operation for the equinus varus foot, delaying the need for premature triple arthrodesis, but the long-term results in cavo-varus feet with claw toes were uniformly poor. Furthermore, it appeared to be unsatisfactory in the mobile flail valgus foot unless the subtalar joint had been stabilised by fusion. Correction of proximal joint or bone deformity and muscle imbalance is considered necessary if satisfactory results are to be achieved from the osteotomy.- - - - - - - - - - ranking = 17205.029980165keywords = spinal dysraphism, dysraphism, spinal (Clic here for more details about this article) |
9/12. hydrocephalus ex vacuo and clasp thumb deformity due to congenital cytomegalovirus infection.A case of congenital hydrocephalus in a male infant with flexion deformity of the thumbs and great toes is reported. A maternal uncle had undefined intellectual impairment and X-linked hydrocephalus was considered among the differential diagnoses. However, this diagnosis was considered unlikely as the pyramids were preserved at autopsy. In addition, postmortem histopathology and viral culture established cytomegalovirus (CMV) infection as the underlying cause of the hydrocephalus. Although CMV infection is a well recognized cause of congenital hydrocephalus, the associated flexion deformities of the thumbs and great toes have not been previously described and may reflect injury to the corticospinal tracts.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
10/12. anencephaly with spinal dysraphism, cleft lip and palate and limb reduction defects.We report three unrelated fetuses presenting with anencephaly, spinal dysraphism, cleft lip and palate and limb reduction defects. review of the literature suggests that this association may be more commonly found than previously recognized and may indicate severe disturbance in early embryogenesis.- - - - - - - - - - ranking = 17205.029980165keywords = spinal dysraphism, dysraphism, spinal (Clic here for more details about this article) |
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