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Query: UMLS:C0700208 (scoliosis)
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Sixty-seven patients who had a diagnosis of Scheuermann kyphosis and a mean angle of kyphosis of 71 degrees were evaluated after an average follow-up of thirty-two years (range, ten to forty-eight years) after the diagnosis. All sixty-seven patients completed a questionnaire; fifty-four had a physical examination and radiographs; fifty-two, pulmonary function testing; and forty-five, strength-testing of the trunk muscles. The results were compared with those in a control group of thirty-four subjects who were matched for age and sex. The patients who had Scheuermann kyphosis had more intense back pain, jobs that tended to have lower requirements for activity, less range of motion of extension of the trunk and less-strong extension of the trunk, and different localization of the pain. No significant differences between the patients and the control subjects were demonstrated for level of education, number of days absent from work because of low-back pain, extent that the pain interfered with activities of daily living, presence of numbness in the lower extremities, self-consciousness, self-esteem, social limitations, use of medication for back pain, or level of recreational activities. Also, the patients reported little preoccupation with their physical appearance. Normal or above-normal averages for pulmonary function were found in patients in whom the kyphosis was less than 100 degrees. Patients in whom the kyphosis was more than 100 degrees and the apex of the curve was in the first to eighth thoracic segments had restrictive lung disease. Five patients had an unexplained, mildly abnormal neurological examination. Mild scoliosis was common; spondylolisthesis was not observed.
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PMID:The natural history and long-term follow-up of Scheuermann kyphosis. 842 84

We investigated the long-term pulmonary sequelae of 38 children surviving 3 to 11.5 years (median 7 years) after high-dose chemotherapy (HDC) and autologous bone marrow transplantation (ABMT) without TBI. This cross-sectional study included patients with neuroblastoma (21), non-Hodgkin's lymphoma (7), Ewing's sarcoma (5), rhabdomyosarcoma (3), medulloblastoma (1) and ALL (1). They were asked and examined for clinical signs and underwent a physical examination with chest X-ray; 33/38 had pulmonary function tests (PFT) performed. No obstructive disease was found. Fifteen out of 32 evaluable PFT (47%) were abnormal with a pulmonary restrictive syndrome in 10, and borderline values in five patients. Four of these 15 patients were symptomatic with exertional dyspnea and two of four had abnormal chest X-rays. The etiology was mainly multifactorial, associating HDC with thoracic radiotherapy +/- scoliosis/kyphosis +/- previous thoracotomy +/- post-ABMT interstitial pneumonitis. Only 3/10 patients with a restrictive syndrome had HDC containing BCNU or busulfan as the only risk factor for lung disease. We conclude that the prevalence of late pulmonary sequelae after ABMT without TBI is moderate and rarely due to HDC alone, since most abnormal PFT can be explained by heavy pretreatment prior to ABMT. As symptoms are scarce even in advanced disease, repeated testing and very long-term follow-up are needed.
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PMID:Long-term pulmonary sequelae after autologous bone marrow transplantation in children without total body irradiation. 875 Feb 68

Mitral valve prolapse (MVP) is known to be associated with thoracic skeletal anomalies. To determine the incidence and risk factors for mitral valve prolapse in the adolescent population with severe idiopathic scoliosis (IS), a prospective follow-up study on 139 adolescent patients with IS from the Pediatric Orthopedic Service was undertaken. Data collected included age, sex, medical and family history, physical exam, electrocardiogram and echocardiogram, spinal x-rays, and pulmonary function tests. MVP was detected by echocardiogram in 13.6% (19/139) of patients with IS as compared with 3.2% in 154 age- and weight-matched controls (p < 0.006). All patients with MVP were asymptomatic and a systolic click or murmur was detected on the single preoperative exam only in 37% (7/19) of them. Patients with MVP and IS weighed less (45.1 +/- 2.0 vs 51.8 +/- 0.1 kg, p < 0.002) as compared with those IS patients without MVP. The electrocardiogram was abnormal in 21% (4/19) of patients with MVP as compared with only 1.6% (2/120) of patients with IS but no MVP. The two groups did not differ with respect to age at diagnosis, severity of scoliosis, positive family history of scoliosis, or the presence of restrictive lung disease. Though IS was more prevalent in females (79%), the presence of MVP was not related to gender. MVP was persistent in 10 of the 19 patients reevaluated by echocardiogram 2-4 years after spinal surgery. We conclude that MVP is four times more common in patients with severe IS than in the normal adolescent population, and is associated with a lower body weight in IS patients with MVP than in IS patients without MVP. The persistent nature of MVP, even after corrective spinal surgery, may be related to factors other than geometric changes of the heart caused by abnormal thoracic curvature.
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PMID:Incidence and risk factors for mitral valve prolapse in severe adolescent idiopathic scoliosis. 932 88

In severe congenital scoliosis, traction (whether with a halo or instrumental) is known to expose patients to neurologic complications. However, patients with restrictive lung disease may benefit from halo traction during the course of the surgical treatment. The goal of treatment of such deformities is, therefore, twofold: improvement of the respiratory function and avoidance of any neurologic complications. We report our technique to treat a 17-year-old girl with a multi-operated congenital scoliosis of 145 degrees and cor pulmonale. Pre-operative halo gravity traction improved her vital capacity from 560 c.c. to 700 c.c., but led to mild neurologic symptoms (clonus in the legs). To avoid further neurologic compromise, her first surgery consisted of posterior osteotomies and the implantation of two sliding rods connected to loose dominoes without any attempt at correction. Correction was then achieved over a 3-week period with a halofemoral traction. This allowed the two rods to slide while the neurologic status of the patient was monitored. Her definitive surgery consisted of locking the dominoes and the application of a contralateral rod. Satisfactory outcome was achieved for both correction of the deformity (without neurologic sequels) and improvement of her pulmonary function (1200 c.c. at 2 years). This technique using sliding rods in combination with halofemoral traction can be useful in high-risk, very severe congenital scoliosis.
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PMID:Halo femoral traction and sliding rods in the treatment of a neurologically compromised congenital scoliosis: technique. 1048 37

The optimal management of paediatric empyema thoracis remains controversial. The objective of the study was to analyse evolving experience in clinical presentation, management, outcome and factors contributing to adverse morbidity in thoracic empyema. Forty-seven patients presenting to a paediatric surgical centre were studied in three consecutive 6-y periods during 1980-97 to compare any change in the pattern of disease influencing diagnosis and management. Patients were categorized into two treatment groups: (i) conservative management (antibiotics and/or tube thoracostomy), (ii) thoracotomy. The median duration of illness prior to hospital admission was 10 d (range 1-42 d). Ultrasound was increasingly utilized in the diagnosis and staging of empyema and played an important role in directing definitive management. The presence of loculated pleural fluid determined the need for thoracotomy. Sixteen of 20 patients (80%) who were initially treated with thoracocentesis or tube thoracostomy eventually needed thoracotomy. There was a positive shift in management towards early thoracotomy resulting in prompt symptomatic recovery. Significant complications were noted in seven children who had delayed thoracotomy. These included recurrent empyema with lung abscess (n = 2), scoliosis (n = 2), restrictive lung disease (n = 1), bronchopleural fistula (n = 1) and sympathetic pericardial effusion (n = 1). An unfavourable experience with delayed thoracotomy during the study period has led us to adopt a more aggressive early operative approach to empyema thoracis. The decision to undertake thoracotomy has been influenced by the ultrasound findings of organized loculated pleural fluid. Delayed surgery was associated with adverse outcome. Whilst fibrinolytics and thoracoscopy may provide attractive options for early empyema, thoracotomy can hasten patient recovery regardless of the stage of disease. Prospective randomized trials are required to assess the ideal therapy for childhood empyema.
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PMID:Evolving experience in the management of empyema thoracis. 1083 Apr 52

Patients with developmental disorders, including adolescents, comprise a large and heterogeneous group of individuals who vary in underlying diagnosis and degree of disability. The largest numbers of patients are those with cerebral palsy and with traumatic brain injury. While these conditions themselves do not directly cause airway or parenchymal lung dysfunction, consequences of neuromuscular dysfunction, especially aspiration and ineffective cough, may lead to lung damage. Poor nutritional status, impairment of airway clearance by muscular weakness or incoordination and poor pulmonary reserve (due to chest wall or spine deformity) increase the risk of significant morbidity and mortality from respiratory infections. Individuals who were premature infants or who had prolonged neonatal courses may also have residual chronic lung disease (bronchopulmonary dysplasia) contributing to their pulmonary problems. This review discusses conditions that have adverse effects on the airway and lung (drooling, feeding problems, gastroesophageal reflux, aspiration, spasticity, scoliosis) and some of the consequences of these insults (disordered airway clearance, pneumonia, sleep apnea). Also discussed are issues important to the prevention or amelioration of respiratory difficulties, including preventive care, the effects of exercise, dental hygiene, and surgical intervention.
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PMID:Respiratory problems in the adolescent with developmental delay. 1106 May 58

1. Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth. 2. The rare condition of fused ribs and congenital scoliosis may result in a three-dimensional thoracic deformity with adverse effects on thoracic growth and function with development of thoracic insufficiency syndrome. 3. The normal thorax is defined by two characteristics: normal, stable volume and the ability to change that volume. Volume depends on the width and depth of the rib cage, and the thoracic spine provides height. The ability to change volume, termed thoracic function, is provided by the diaphragm and the secondary muscles of respiration. 4. On radiographs, the loss of the vertical height of the lung of the concave, restricted hemithorax is defined by the percentage of space available for the lung. 5. Spine rotation causes a windswept thorax, with both restriction of the volume of the convex hemithorax and restriction of the motion of the involved ribs. 6. Constrictive three-dimensional deformity of the thorax may cause extrinsic, restrictive lung disease. 7. Progressive thoracic insufficiency syndrome is diagnosed on the basis of clinical signs of respiratory insufficiency, loss of chest wall mobility as demonstrated by the thumb excursion test, worsening indices of three-dimensional thoracic deformity on radiographs and computed tomography scans, or a relative decline in percent predicted vital capacity due to thoracic "failure to thrive," as demonstrated by pulmonary function tests. 8. Treatment of progressive thoracic insufficiency syndrome should provide an acute increase in the thoracic volume with stabilization of any flail chest-wall defects and maintain these improvements as the patient grows, without the need for spine fusion.
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PMID:The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. 1263 23

Scoliosis is associated with progressive restrictive lung disease and an increased risk of pulmonary complications following surgical correction. Identification of higher risks for prolonged postoperative mechanical ventilation (MV) improves postoperative care. Our objective was to determine if preoperative pulmonary function tests (PFT) predict prolonged postoperative MV (defined as MV >or=3 days). We correlated preoperative PFT (forced expired volume in 1 sec, FEV1; vital capacity, VC; inspiratory capacity, IC; maximal inspiratory pressure, MIP; total lung capacity, TLC; and residual volume, RV) and postoperative MV days in 125 patients who had scoliosis surgery (aged 13.7 +/- 3.0 (SD) years) from January 1990-July 2001. We had 71 male and 54 female patients. Scoliosis types were 13 congenital, 27 idiopathic, 57 neuromuscular, 23 syndrome/tumor, and 5 kyphoscoliosis. Forty patients (32%) had postoperative MV >or=3 days. Independent factors likely requiring postoperative MV >or=3 days were neuromuscular scoliosis (P < 0.001) and FEV1 <40% predicted. Independent factors most likely were: neuromuscular scoliosis with preoperative FEV1 <40% predicted (P < 0.01). Independent factors most unlikely were: idiopathic scoliosis (P < 0.002). VC <60% predicted, IC <30 ml/kg, TLC <60% predicted, and MIP <60 cm H2O correlated with postoperative MV >or=3 days (P < 0.05). We found no association between RV and postoperative MV. FEV1 <40% predicted, VC <60% predicted, IC <30 ml/kg, TLC <60% predicted, MIP <60 cm H2O, and neuromuscular disease each correlated with prolonged postoperative MV. Neuromuscular disease or a preoperative FEV(1) <40% predicted were more likely, and older children with neuromuscular disease and FEV1 <40% predicted were most likely to require prolonged postoperative MV (P < 0.01). Clearly FEV1, and possibly VC, IC, TLC, and MIP, may increase accuracy in predicting the need for prolonged postoperative MV.
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PMID:Preoperative predictors of prolonged postoperative mechanical ventilation in children following scoliosis repair. 1614 95

Patients with neuromuscular scoliosis (NMS) are frequently considered at high risk for postoperative complications based on their underlying disease and comorbidities. Postoperative complications include prolonged mechanical ventilation (MV), defined longer than 72 h, at the paediatric intensive care unit. The objectives of this retrospective study were to assess the incidence of prolonged MV in patients with NMS following scoliosis surgery and to identify predictive risk factors. A total of 46 consecutive patients underwent surgical spinal fusion and instrumentation for progressive NMS. Prolonged MV was required in seven of 46 patients (15%). The only risk factor for prolonged MV was a decreased preoperative pulmonary function. Forced expired volume in 1 s and vital capacity were significantly decreased in patients with MV >72 h compared with patients with MV <72 h. Routine preoperative pulmonary function testing may reveal important information with regard to restrictive lung disease in the preoperative assessment of patients with NMS and predict the early postoperative clinical course.
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PMID:Incidence and risk factors of prolonged mechanical ventilation in neuromuscular scoliosis surgery. 1852 79

Quadriceps muscle weakness is an important component of chronic obstructive pulmonary disease (COPD). We hypothesised that quadriceps weakness would also be a feature of restrictive lung disease due to scoliosis. We studied 10 patients with severe scoliosis (median (interquartile range (IQR)) forced expiratory volume in 1 s (FEV(1))() 35.3 (11)% predicted), 10 patients with severe COPD (FEV(1) 26.5 (9.0)% pred) and 10 healthy age-matched adults. We measured quadriceps strength, exercise capacity and analysed quadriceps muscle biopsies for myosin heavy-chain (MyHC) isoform expression and the presence of oxidative stress. Both groups exhibited quadriceps weakness with median (IQR) maximal voluntary contraction force being 46.0 (17.0) kg, 21.5 (21.0) kg and 31.5 (11.0) kg, respectively (p = 0.02 and 0.04, respectively, for each patient group against controls). Oxidative stress was significantly greater in the quadriceps of both restrictive and COPD patients. The scoliosis patients exhibited a decrease in the proportion of MyHC type I compared with controls; median (IQR) 35.3 (18.5)% compared with 47.7 (9.3)%, p = 0.028. The scoliosis patients also showed an increase in MyHC IIx (26.3 (15.5)% compared with 11.3 (13.0)%, p = 0.01. Quadriceps weakness is a feature of severe scoliosis; the similarities between patients with scoliosis and patients with COPD suggest a common aetiology to quadriceps weakness in both conditions.
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PMID:Quadriceps muscle strength in scoliosis. 2043 81


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