Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0700208 (scoliosis)
 8,574 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From 1958 to March 1987 we corrected 704 patients with pectus excavatum. The condition occurred more frequently in boys (544 patients) than girls (160 patients). In the majority of patients (86%), the defect was evident at birth or within the first year of life. Musculoskeletal abnormalities were identified in 133 patients (scoliosis, 107; kyphosis, 4; myopathy, 3; Poland's syndrome, 3; Marfan's syndrome, 2; Pierre Robin syndrome, 2; prune belly syndrome, 2; neurofibromatosis, 3; cerebral palsy, 4; tuberous sclerosis, 1; and congenital diaphragmatic hernia, 2). Sixteen patients had associated congenital heart disease. A family history of chest wall deformity was present in 37% of the cases and a history of scoliosis in 11%. Surgical correction was performed using a uniform technique for bilateral subperichondrial resection of the deformed costal cartilages and sternal osteotomy resecting a wedge of the anterior cortex and fracturing the posterior cortex. Anterior displacement was maintained with silk sutures closing the osteotomy defect. In 28 early cases, the sternum was secured by intramedullary fixation with a Steinman pin. All repairs were completed with a low complication rate (4.4%; pneumothorax, 11; wound infection, 5; wound hematoma, 3; wound dehiscence, 5; pneumonia, 3; seroma, 1; hemoptysis, 1; hemopericardium, 1). Six complications were associated with Steinman pin fixation (hemoptysis, seroma, hemopericardium, pneumothorax, 3). Major recurrence occurred in 17 patients (2.7%) and led to revision in 12. Satisfactory long-term results were achieved in the remaining 687 patients, with follow-up ranging from 2 weeks to 27 years. Mean follow-up was 4.3 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Surgical repair of pectus excavatum. 320 60

Forty children were managed for prune-belly syndrome between 1979 and 1989, and twenty-five of them had musculoskeletal abnormalities. The musculoskeletal abnormalities were primary in twenty-two children, secondary to renal osteodystrophy in one, and both primary and secondary to renal osteodystrophy in two. Thirteen children had marked abnormality of the hip, and congenital dislocation was typically resistant to conventional treatment. Scoliosis was seen in seven patients. Pectus excavatum (a chest-wall deformity) was seen in eleven patients, including five of the six who had an idiopathic-like curve. Although prune-belly syndrome is uncommon, the diagnosis necessitates a thorough orthopaedic evaluation because of the high prevalence of associated musculoskeletal abnormalities.
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PMID:The orthopaedic manifestations of prune-belly (Eagle-Barrett) syndrome. 784 32

In prune belly syndrome (PBS), spine deformities, particularly scoliosis, are the most frequent accompanying orthopedic problem (18%). We give a detailed description of two cases with prune belly-associated spine deformities. A combined anterior-posterior fusion procedure with posterior correction and stabilization according to the Galveston-Luque technique was carried out in both patients. In one patient insufficiency of the pelvic fixation developed with loss of balance. Reoperation was performed. In the second patient correct alignment and balance was achieved, breakage of both metal rods occurred over time. The PBS represents a model of how scoliosis may emerge. The lack of the abdominal musculature subsequently leads to congenial imbalance, which may cause lordo-scoliotic deformity. The operative treatment of spine deformities in the PBS in the presented technique is efficient. In great deformities balance is difficult to achieve and maintain especially in the lumbo-sacral junction.
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PMID:Surgical treatment with the technique of Galveston-Luque of spine deformities associated with the prune belly syndrome. 1519 77