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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Bone-marrow transplantation has increased the survival of patients with mucopolysaccharidosis-I. We describe the spinal problems and their management in 12 patients with this disorder who have been followed up for a mean of 4.5 years since transplantation. High lumbar kyphosis was seen in ten patients which was associated with thoracic scoliosis in one. Isolated thoracic scoliosis was seen in another. One patient did not have any significant problems in the thoracic or lumbar spine but had odontoid hypoplasia, which was also seen in three other children. Four of the eight patients in whom MRI of the cervical spine had been performed had abnormal soft tissue around the tip of the odontoid. Neurological problems were seen in two patients. In one it was caused by cord compression in the lower dorsal spine 9.5 years after posterior spinal fusion for progressive kyphosis, and in the other by angular kyphosis with thecal indentation in the high thoracic spine associated with symptoms of spinal claudication.
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PMID:Spinal problems in mucopolysaccharidosis I (Hurler syndrome). 895 Oct 11

In case of painful scoliosis, a search for etiologic factors in the vertebral spinal canal and adjacent structures should be made. MRI with Gadolinium is useful to look for various pathology in and around the spine in particular of spinal cord. CT scanning is indispensible for bony details. Thus CT and MRI are complementary in evaluation of pathology of spine in any modern imaging centers.
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PMID:Painful scoliosis. 911 20

The authors analysed the clinical and radiological findings and the surgical management of 25 patients admitted for scoliosis classified as idiopathic at first presentation, but in fact associated with spinal cord and/or brain stem anomalies. Twenty patients had syringomyelia, 19 had Chiari malformation. Scoliosis was the only presenting symptom when all these patients were referred to the orthopaedic surgeon. On examination, five patients had normal neurological findings, while the others showed very mild neurological deficits. The diagnosis of syringomyelia and Chiari malformation was established by MRI, which is the best form of neuroradiological examination for discovering spinal abnormalities. Neurosurgical treatment is strongly recommended as the first step in the management of "pseudo" idiopathic scoliosis.
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PMID:Syringomyelia and Arnold Chiari in scoliosis initially classified as idiopathic: experience with 25 patients. 1055 28

The clinical presentation and treatment of a case of osteoblastoma of the lumbar spine (L4) is described, with a notable delay between the onset of symptoms and the definitive diagnosis (2 years). Besides scoliosis, the usual roentgenograms, bone scans, CAT and MRI, did not show any relevant abnormality, which is a possibility that does not allow us to exclude the diagnosis of this tumor in a young patient with lower back pain and a rigid scoliosis.
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PMID:[Scoliosis of sudden evolution due to lumbar osteoblastoma]. 928 42

A 13 year-old girl with scoliosis and central core disease is reported. She was noted to have mild psychomotor developmental delay since early infancy. Scoliosis with minimal muscle weakness was noted at about five years old. The neurological examination disclosed absent knee jerk. The spine MRI showed no significant finding. The serum CK revealed 518 U/L. The muscle biopsy obtained from the quadriceps femoris muscle showed Type 1 fiber atrophy and predominance, as is commonly seen in congenital myopathies. Under nicotinamide adenine dinucleotide dehydrogenase (NADH) and succinate dehydrogenase (SDH) stains, core structures were identified and the diagnosis of central core disease (CCD) was made. Since kyphoscoliosis usually becomes prominent as muscle weakness progresses to loss of ambulation in other myopathies, the disproportionate spinal involvement in central core disease appears to be a striking feature. We suggest that all patients with idiopathic scoliosis deserve a thorough neurological evaluation if congenital myopathies are suspected. Muscle biopsy should also be recommended for a confirmatory diagnosis even if only minimal muscle weakness present. Besides, early detection of CCD helps us to identify the population who are at a higher risk for malignant hyperthermia.
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PMID:Central core disease associated with scoliosis: report of one case. 929 32

The clinical presentation of tethered spinal cord and the results of tethered cord release were examined in a group of 30 low motor level (L3 and below) children with a history of myelomeningocele without concomitant CNS complications. Changes in orthopedic and/or neurologic status formed the basis of consideration for tethered cord release. Clinically, these patients presented with a new onset or recently progressing scoliosis, spasticity with or without contractures, decrease in motor function and low back pain at the site of closure. One or more of these findings was present in all cases and led to the suspicion of tethered spinal cord. The diagnosis of tethered cord was confirmed in all cases by MRI or CT myeolography. In order to isolate tethering as the etiology for the patients' clinical deterioration, patients with concomitant CNS complications, e.g. shunt dysfunction or hydromyelia were excluded from the study. Twenty-nine such patients, of an initial 59, who would have otherwise been considered, were excluded on the basis of this criteria of concomitant CNS complications. The results of release 1 year after the procedure were as follows: regarding scoliosis, in 75% of cases the curve either remained stable or decreased by more than 10 degrees, with 25% experiencing curve progression of > 10 degrees. The most recent follow-up in this group revealed that 11.8% experienced a decrease in curvature of >10 degrees; 47.1% remained stable, and 41.2% ultimately progressed 10 degrees. In the group with spasticity, 43.8% improved; 56.3% remained stable, and none worsened. Most (78.6%) of the children who had experienced a decline in motor function improved postoperatively, and all those with back pain experienced complete resolution. In conclusion, tethered cord release in symptomatic low lumbar and sacral level children with myelomeningocele appears to be of benefit, especially with respect to stabilization of scoliosis in selected patients, back pain at the site of closure, and prior decline in motor function. Results in the cases with spasticity were more equivocal.
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PMID:Tethered cord syndrome in low motor level children with myelomeningocele. 934 49

A 9-year-old girl was discovered to have had a huge retroperitoneal mass causing moderate hydronephrosis and anterior displacement of the right kidney and which was found on a renal ultrasonographic examination. A series of examinations including (1)IVP, (2) abdominal computed tomography, (3) MRI and(4) incisional biopsy revealed a giant dumb-bell shaped retroperitoneal ganglioneuroma with intraspinal involvement. Secondary scoliosis was also noticed. We successfully resected the huge ganglioneuroma of the right retroperitoneum in a two stage procedure: her postoperative course was uneventful.
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PMID:A giant retroperitoneal ganglioneuroma with intraspinal involvement: report of one case. 940 Nov 85

Hydromyelia in patients with myelomeningocele and Chiari-II malformation is a relatively frequent finding on MRI studies. However, not all children develop symptoms from the hydromyelia that requires treatment. Furthermore, treatment of hydromyelia in spina bifida patients is rather complex due to the associated malformations. The authors retrospectively analyzed 231 MRI studies carried out on spina bifida patients who presented neurological deterioration. Hydromyelia was found in 48.5% of the patients. Forty-five children with severe hydromyelia required treatment. These patients were first divided into 2 groups: those with holocord hydromyelia, and those with a segmental lesion. Fifteen patients presented symptoms characteristic of symptomatic Chiari-II malformation: neck rigidity; swallowing difficulty; pain in the upper extremeties; weakness or spasticity in the upper extremeties. Eighteen patients presented symptoms typical of the tethered cord syndrome: scoliosis; worsening bladder and/or bowel function; pain in the lower extremeties; weakness or spasticity in the lower extremeties. Twelve patients presented a mixed-type symptomatology. These patients subsequently underwent posterior cervical decompression, tethered cord release or insertion of a hydromyelia-pleural shunt according to the type of presenting symptoms and to the extent of the hydromyelic lesion. A pattern of successful treatment was identified for each type of presenting clinical and radiological picture. This has allowed the authors to determine an algorithm for optimal treatment of hydromyelia associated with Chiari-II malformation and myelomeningocele, which is proposed here.
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PMID:Presentation and management of hydromyelia in children with Chiari type-II malformation. 941 35

The cases of six adolescent males with a mild thoracic scoliosis of unknown etiology and a marked limitation of neck flexion are reported. All six complained of mild thoracic spine pain and were 13-17 years old at the onset of their symptoms. In addition to the thoracic scoliosis, all were found to have a marked limitation of neck flexion. They were unable to touch their chest wall with their chin. None had a positive family history of scoliosis. Radiological examination revealed a thoracic scoliosis of 10 degrees-40 degrees, no abnormalities were detected on cervical spine plain radiographs. Extensive investigations, including MRI of the entire spine, failed to disclose the cause of the spinal deformity or the mechanism causing the limitation of neck motion. Apart from the limited cervical flexion and mild pain, this condition was found to be benign and the spine deformity to be mild or moderate in nature and with limited progression. Review of the literature failed to identify similar cases.
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PMID:Thoracic scoliosis and restricted neck motion: a new syndrome? A report of six cases. 962 40

The Chiari malformation, condition characterized by herniation of posterior fossa contents below the level of foramen magnum coexists very often with solitary or multiple syringomyelic cysts in spinal cord. The authors, on the basis of current opinions presented in literature and an own case present considerations concerning pathogenesis, operative procedures and prognostic factures which are of value in prediction of long-term outcome in patients which Chiari type I-Syringomyelia complex. The presence or absence of three preoperative signs as: muscular atrophy, ataxia and scoliosis facilitate, with confidence of 95%, prediction of long-term postoperative result. Preoperative incidence of all three above signs is much less favourable for outcome. In the presented case all unfavourable signs were observed preoperatively and, despite of good MRI-result, long-term outcome was only fair.
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PMID:[Type I Chiari malformation coexisting with syringomyelia: pathogenesis, treatment choice and prognostic factors for remote outcome in the light of own case]. 1035 45


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