Effects of surgical correction of neuromuscular scoliosis on gastric myoelectrical activity, emptying, and upper gastrointestinal symptoms.

OBJECTIVES: Gastrointestinal complications are frequent after surgical correction of neuromuscular scoliosis, but the effects of scoliosis surgery on gastric function and upper gastrointestinal symptoms over the long term are unknown. METHODS: Thirty-one children (16 spastic, 15 flaccid patients) who underwent surgical correction of neuromuscular scoliosis were included in a prospective follow-up study. Median (range) age at surgery was 14.9 (5-20) years and follow-up time 4.3 (2-8) years. Electrogastrography (n=28), gastric emptying scintigraphy (n=17), and structured upper gastrointestinal symptoms questionnaire (n=26) were evaluated before and after surgery. The results were related to patients' clinical state, type and extent of corrective spinal surgery, and gastrointestinal complications. RESULTS: The median main scoliosis curve of 81 degrees (51-129 degrees) was corrected to 25 degrees (1.0-85 degrees) after surgery. In electrogastrogram, power ratio increased from preoperative 1.4 (0.30-11) to postoperative 6.2 (1.2-26) in the spastic group (P=0.008), whereas in the flaccid group, power ratio remained unchanged at 2.2 (0.1-17). Patients with prolonged postoperative paralytic ileus had the most substantial increase in gastric power ratio (P=0.038). Correction of sagittal spinal balance correlated with increased postprandial normogastric activity after surgery (R=0.459; P=0.004). The gastric emptying results, upper gastrointestinal symptoms, and body mass index were not significantly altered after scoliosis surgery. CONCLUSIONS: Gastric myoelectrical power increased after surgical correction of spastic neuromuscular scoliosis and was associated with prolonged postoperative paralytic ileus. Correction of poor, stooped spinal balance improved gastric myoelectrical activity. The net effect of scoliosis surgery on gastric emptying, upper gastrointestinal symptoms, and clinical nutritional state was minimal.

Tibial fractures in children. A retrospective 27-year follow-up study.

BACKGROUND: Tibial fracture is the third most common long-bone fracture in children. Traditionally, most tibial fractures in children have been treated non-operatively, but there are no long-term results. METHODS: 94 children (64 boys) were treated for a tibial fracture in Aurora City Hospital during the period 1980-89 but 20 could not be included in the study. 58 of the remaining 74 patients returned a written questionnaire and 45 attended a follow-up examination at mean 27 (23-32) years after the fracture. RESULTS: 89 children had been treated by manipulation under anesthesia and cast-immobilization, 4 by skeletal traction, and 1 with pin fixation. 41 fractures had been re-manipulated. The mean length of hospital stay was 5 (1-26) days. Primary complications were recorded in 5 children. The childrens' memories of treatment were positive in two-thirds of cases. The mean subjective VAS score (range 0-10) for function appearance was 9. Leg-length discrepancy (5-10 mm) was found clinically in 10 of 45 subjects and rotational deformities exceeding 20° in 4. None of the subjects walked with a limp. None had axial malalignment exceeding 10°. Osteoarthritis of the hip and/or knee was seen in radiographs from 2 subjects. INTERPRETATION: The long-term outcome of tibial fractures in children treated non-operatively is generally good.

[Multi-level surgery of a limb improves walking ability of a CP child]. / Alaraajojen monitasokirurgia parantaa CP-vammaisen lapsen kävelykykyä.

UNLABELLED: BACKGROUND. Correction of the deformities of the lower limbs in CP children is currently attempted in one go by using multi-level surgery in one operation involving multiple procedures. MATERIAL AND METHODS. We examined from patient records the surgical outcomes and operative harms of 40 CP children operated in the HUS Department of Gynecology and Pediatrics in 2000 to 2010. Clinical examination and analysis of gait were the most important methods of assessment. RESULTS AND CONCLUSION: Most CP children benefited from the operation, with a considerable improvement in their walking posture.

Childhood femoral fracture can lead to premature knee-joint arthritis. 21-year follow-up results: a retrospective study.

BACKGROUND AND PURPOSE: During the past decades, treatment of pediatric femoral fractures in Finland has changed from mostly non-operative to more operative. In this retrospective study, we analyzed the long-term results of treatment. PATIENTS AND METHODS: 74 patients (mean age 7 (0-14) years) with a femoral fracture were treated in Aurora City Hospital in Helsinki during the period 1980-89. 52 of 74 patients participated in this clinical study with a mean follow-up of 21 (16-28) years. Fracture location, treatment mode, time of hospitalization, and fracture alignment at union were assessed. Subjective assessment and range of motion of the hip and knee were evaluated. Leg-length discrepancy and alignment of the lower extremities were measured both clinically and radiographically. RESULTS: Of the 52 children, 28 had sustained a shaft fracture, 13 a proximal fracture, and 11 a distal fracture. 44 children were treated with traction, 5 by internal fixation, and 3 with cast-immobilization. Length of the hospital treatment averaged 58 (3-156) days and the median traction time was 39 (3-77) days. 21 of the 52 patients had angular malalignment of more than 10 degrees at union. 20 patients experienced back pain. Limping was seen in 10 patients and leg-length discrepancy of more than 15 mm was in 8 of the 52 patients. There was a positive correlation between angular deformity and knee-joint arthritis in radiographs at follow-up in 6 of 15 patients who were over 10 years of age at the time of injury. INTERPRETATION: Angular malalignment after treatment of femoral fracture may lead to premature knee-joint arthritis. Tibial traction is not an acceptable treatment method for femoral fractures in children over 10 years of age.

Congenital upper limb deficiencies and associated malformations in Finland: a population-based study.

PURPOSE: To calculate the national incidence of upper limb deficiencies and associated infant mortality in children in Finland using the International Federation of Societies for Surgery of the Hand (IFSSH) classification. Radial ray deficiency, ulnar ray deficiency, central ray deficiency, transverse arrest, phocomelia, undergrowth, and constriction band syndrome with skeletal defects were evaluated. METHODS: We reviewed upper limb deficiencies among all 753,342 births in Finland during 1993 to 2005 reported to the Finnish Register of Congenital Malformations. Classification of these upper limb deficiencies was done according to a modified IFSSH system. We calculated incidence, gender and side distributions, frequency of associated anomalies, and infant mortality rates in different subtypes of the deficiencies. Familial occurrence of congenital upper limb defects was recorded. RESULTS: A total of 419 cases (234 male, 185 female) of upper limb deficiencies were identified. The national incidence of upper limb deficiencies was 5.56 per 10,000 births and 5.25 per 10,000 live births. The most common upper limb abnormality was radial ray deficiency (138), followed by subgroups of undergrowth (91), upper limb defects due to constriction band syndrome (51), central ray deficiency (41), and ulnar ray deficiency (33). Perinatal mortality was 14%. Infant mortality among children with upper limb deficiencies was 137 per 1,000 live births, compared with an overall infant mortality of 3.7 per 1,000 live births in Finland. Additional birth defects were found in 60% of these children. Prevalence of upper limb defects in relatives of the census population was 2% (11 of 419). CONCLUSIONS: The national incidence of upper limb deficiencies is 5.25 per 10,000 live births. Congenital upper limb deficiencies are associated with additional birth defects in two thirds of cases. These children, especially children with radial ray deficiency, have a high perinatal mortality rate. When divided into subgroups using IFSSH classification, differences emerge in both associated anomalies and mortality.

Treatment of shoulder sequelae in brachial plexus birth injury.

BACKGROUND: Many children with permanent brachial plexus birth injury (BPBI) develop shoulder problems, with subsequent joint deformity without treatment. We assessed the indications and outcome of shoulder operations for BPBI. PATIENTS AND METHODS: 31 BPBI patients who had undergone a shoulder operation in our hospital between March 2002 and December 2005 were included in the study. Relocation of the humeral head had been performed in 13 patients, external rotation osteotomy of the humerus in 5 patients, subscapular tendon lengthening in 5 patients, and teres major transposition in 8 patients. Subjective results were registered. Shoulder range of motion was measured, and function assessed according to the Mallet scale. Magnetic resonance imaging (MRI) was performed pre- and postoperatively. Glenoscapular angle (GSA) and percentage of humeral head anterior to the middle of the glenoid fossa (PHHA) were measured. Congruency of the glenohumeral joint (GHJ) was estimated. The mean follow-up time was 3.8 (1.7-6.8) years. RESULTS: At follow-up, the subjective result was satisfactory in 30 of the 31 patients. There were 4 failures, which in retrospect were due to wrong choice of surgical method in 3 of these 4 patients. Mean increase in Mallet score was 5.5 after successful relocation, 1.4 after rotation osteotomy, 2.2 after subscapular tendon lengthening, and 3.1 after teres major transposition. Congruency of the shoulder joint improved in 10 of 13 patients who had undergone a relocation operation, with mean improvement in GSA of 33º and mean increase in PHHA of 25%. There were no substantial changes in congruency of the glenohumeral joint in patients treated with other operation types. INTERPRETATION: Restriction of the range of motion and malposition of the glenohumeral joint can be improved surgically in brachial plexus birth injury. Remodeling of the joint takes place after successful relocation of the humeral head in young patients.

Range of motion and strength after surgery for brachial plexus birth palsy.

BACKGROUND: There is little information about the range of motion (ROM) and strength of the affected upper limbs of patients with permanent brachial plexus birth palsy. PATIENTS AND METHODS: 107 patients who had brachial plexus surgery in Finland between 1971 and 1998 were investigated in this population-based, cross-sectional, 12-year follow-up study. During the follow-up, 59 patients underwent secondary procedures. ROM and isometric strength of the shoulders, elbows, wrists, and thumbs were measured. Ratios for ROM and strength between the affected and unaffected sides were calculated. RESULTS: 61 patients (57%) had no active shoulder external rotation (median 0° (-75-90)). Median active abduction was 90° (1-170). Shoulder external rotation strength of the affected side was diminished (median ratio 28% (0-83)). Active elbow extension deficiency was recorded in 82 patients (median 25° (5-80)). Elbow flexion strength of the affected side was uniformly impaired (median ratio 43% (0-79)). Median active extension of the wrist was 55° (-70-90). The median ratio of grip strength for the affected side vs. the unaffected side was 68% (0-121). Patients with total injury had poorer ROM and strength than those with C5-6 injury. Incongruity of the radiohumeral joint and avulsion were associated with poor strength values. INTERPRETATION: ROM and strength of affected upper limbs of patients with surgically treated brachial plexus birth palsy were reduced. Patients with avulsion injuries and/or consequent joint deformities fared worst.

Brachial plexus birth injury: US screening for glenohumeral joint instability.

PURPOSE: To prospectively evaluate the use and optimal timing of ultrasonographic (US) screening for posterior shoulder subluxation in infantswith brachial plexus birth injury (BPBI). MATERIALS AND METHODS: Approval of the ethics committee and informed consent of guardians was obtained. This population-based prospective study included neonates with BPBI who were born in Helsinki from January 1, 2003 through December 31, 2006, and in whom BPBI was verified with sequential clinical examinations. US was performed at 1, 3, 6, and 12 months. Size (width and height) of the humeral head and its ossification center and congruency of the shoulder (alpha angle) were measured. Frequency of BPBI and permanent changes were evaluated. This study also included patients who were referred from the tertiary catchment area. For statistical analysis, 95% confidence intervals were calculated, and analysis of variance was performed. RESULTS: BPBI was seen in 132 of 41980 neonates (3.1 per 1000). In 27 cases (0.64 per 1000), BPBI did not heal during the 1st year of life and was considered permanent. The humeral head and its ossification center were smaller on the affected side in permanent BPBI. Nine patients with permanent palsy had posterior subluxation of the humeral head depicted with US (alpha angle, >30 degrees ). In five patients, posterior subluxation [corrected] was detected at 3 months. Nineteen of 21 patients with BPBI from the tertiary catchment area had permanent palsy. Ten of 19 patients developed posterior subluxation of the shoulder, which was verified with US. Altogether, three of these cases were not detected by surgeons. Posterior subluxation of the humeral head developed during the 1st year of life in one-third of patients with permanent BPBI. In more than one-half (55% [five of nine]) of the patients, posterior subluxation [corrected] was detected with US at 3 months, and in 89% (eight of nine), it was detected at 6 months. CONCLUSION: US is a fast and useful tool for diagnosis of posterior subluxation of the humeral head, and examination of the glenohumeral joint should be performed at 3 and 6 months of age in infants with BPBI if symptoms persist.

Treatment injuries are rare in children's femoral fractures.

BACKGROUND AND PURPOSE: The current treatment for femoral fractures in children is mostly operative, which contrasts with treatment of other long bone fractures in children. We analyzed treatment injuries in such patients in Finland in order to identify avoidable injuries. Our other aims were to calculate the incidence of these fractures and to describe the treatment method used. METHODS: The Patient Insurance Centre (PIC) provides financial compensation of patients who have sustained an injury in connection with medical care. We retrospectively analyzed incidence, treatment methods, and all compensation claims concerning treatment of femoral fractures in children who were 0-16 years of age during the 8-year period 1997-2004. RESULTS: The incidence of childhood femoral fractures in Finland was 0.27 per 1,000 children aged < 17 years, and two-thirds of the patients were treated operatively during the study period. 30 compensation claims were submitted to PIC during the 8-year study period. The compensation claims mainly concerned pain, insufficient diagnosis or treatment, extra expenses, permanent disability, or inappropriate behavior of medical personnel. Of the claims, 16 of 30 were granted compensation. Compensation was granted for delay in treatment, unnecessary surgery, and for inappropriate surgical technique. The mean amount of compensation was 2,300 euros. Of the injuries that led to compensation, 11 of 16 were regarded as being avoidable in retrospect. INTERPRETATION: The calculated risk of a treatment injury in childhood femoral fracture treatment in Finland is approximately 2%, and most of these injuries can be avoided with proper treatment.

[Lengthening of lower limbs--implantable therapeutic devices in distraction osteogenesis]. / Alaraajojen pidennys--implantoitavat hoitolaitteet venytysluudutuksessa.

Severe anisomelia and stunting of the lower limbs are treated with distraction osteogenesis, which involves osteotomy of the femur and tibia and distraction of bone ends by using a device designed for this purpose. Sufficiently slow distraction induces bone growth into the generated gap, whereby the limb can be lengthened by up to 15 cm at a time. Attempts have been made to diminish the disadvantages associated with commonly utilized external distraction devices by using completely implantable lengthening devices, which have been shown to result in an at least equal treatment outcome.

Clinically distinct epigenetic subgroups in Silver-Russell syndrome: the degree of H19 hypomethylation associates with phenotype severity and genital and skeletal anomalies.

CONTEXT: The H19 imprinting control region (ICR), located on chromosome 11p15.5, has been reported hypomethylated in 20-65% of Silver-Russell syndrome (SRS) patients. OBJECTIVE: We investigated the methylation status of 11p15.5 ICRs in SRS patients and children born small for gestational age (SGA) to clarify the relationship between phenotype and H19 methylation status. METHODS: We performed methylation screens of the H19 and KCNQ1OT1 ICRs in 42 SRS patients, including seven maternal uniparental disomy of chromosome 7 patients, and 90 SGA children without SRS. Clinical data were evaluated from patient records, and seven hypomethylated patients were clinically and radiologically reexamined. RESULTS: H19 ICR hypomethylation was found in 62% of SRS patients but in no SGA children. A clinical severity score demonstrated strong correlation between hypomethylation level and phenotype severity. Hypomethylation related to a more severe SRS phenotype, in which especially asymmetry and micrognathia were significantly more common. Extremely hypomethylated patients had abnormally high lumbar vertebrae, lumbar hypomobility, elbow subluxations, and distinct hand and foot anomalies. They also presented with congenital aplasia of the uterus and upper vagina, equivalent to the Mayer-Rokitansky-Küster-Hauser syndrome in females, and cryptorchidism and testicular agenesis in males. CONCLUSIONS: We found a dose-response relationship between the degree of H19 hypomethylation and phenotype severity in SRS. We report for the first time the association of specific anomalies of the spine, elbows, hands and feet, and genital defects in SRS with severe H19 hypomethylation. Classical SRS features were found in H19 hypomethylation and milder symptoms in maternal uniparental disomy of chromosome 7, thus distinguishing two separate clinical and etiological subgroups.

Injuries as a result of treatment of tibial fractures in children: Claims for compensation submitted to the Patient Insurance Center in Finland.

BACKGROUND AND PURPOSE: Tibial fractures comprise 10% of all fractures in children. To our knowledge there have been no previous reports of treatment injuries in these fractures. We analyzed compensation claims concerning treatment of these fractures in Finland. We used this information to determine preventable causes of treatment injuries. MATERIAL AND METHODS: In Finland, the Patient Insurance Center (PIC) provides financial compensation for patients who have sustained an injury in connection with medical treatment or operation. We retrospectively analyzed all claims for compensation arising from treatment of tibial fractures in children that had been received by the PIC between 1997 and 2004. The mode of treatment, complications, and permanent sequelae were assessed. We also estimated the number of avoidable treatment injuries. RESULTS AND INTERPRETATION: The PIC received 50 claims for compensation during the 8-year study period. The claims were based on the following issues: pain, incorrect diagnosis and treatment, permanent disability, extra treatment expenses, inappropriate behavior of the medical personnel, and loss of income of the parents. 35/50 claims had received compensation, of which 32 were related to the treatment and 3 to infections. The treatment injuries that had led to compensation comprised a delay in diagnosis and treatment in 15 patients, inappropriate casting in 9, inappropriate operative treatment in 5, and other causes in 3 patients. An unsatisfactory standard of treatment and missed diagnosis were the most common reasons for compensation. In restrospect, all but 1 of the 35 injuries that had led to compensation were considered to be avoidable.

Long-term results of surgery for brachial plexus birth palsy.

BACKGROUND: The long-term results of surgical treatment of brachial plexus birth palsy have not been reported. We present the findings of a nationwide study, with a minimum five-year follow-up, of the outcomes of surgery for brachial plexus birth palsy in Finland. METHODS: Of 1,717,057 newborns, 1706 with brachial plexus birth palsy requiring hospital treatment were registered in Finland between 1971 and 1997. Of these patients, 124 (7.3%) underwent surgery on the brachial plexus at a mean age of 2.8 months (range, 0.4 to 13.2 months). The most commonly performed surgical procedure was direct neurorrhaphy after neuroma resection. One hundred and twelve patients (90%) returned for a clinical and radiographic follow-up examination after a mean of 13.3 years. Activities of daily living were recorded on a questionnaire, and the affected limb was assessed with use of joint-specific functional measures. RESULTS: Two-thirds (63%) of the patients were satisfied with the functional outcome, although one-third of all patients needed help in activities of daily living. One-third of the patients, including all nine with a clavicular nonunion from the surgical approach, experienced pain in the affected limb. All except four patients used the hand of the unaffected limb as the dominant hand. Shoulder function was moderate, with a mean Mallet score of 3.0. Both elbow and hand function were good, with a mean score on the Gilbert elbow scale of 3 and a mean Raimondi hand score of 4. Incongruence of the glenohumeral joint was noted in sixteen (16%) of the ninety-nine patients in whom it was assessed, and incongruence of the radiohumeral joint was noted in twenty-one (21%). The extent of the brachial plexus injury was found to be strongly associated with the final shoulder, elbow, and hand function in a multivariate analysis. CONCLUSIONS: Following surgical treatment of brachial plexus birth palsy, substantial numbers of the patients continued to need help performing activities of daily living and had pain in the affected limb, with the pain due to a clavicular nonunion in one-fourth of the patients. The strongest prognostic factor predicting outcome appears to be the extent of the primary plexus injury.

Therapy insight: orthopedic complications after solid organ transplantation in childhood.

Several factors, such as immobilization, metabolic bone disease and immunosuppressive drugs, can compromise the quality of bone in children who have undergone solid organ transplantation. In contrast to adults, decreased bone mineral density has been reported in only a small proportion of pediatric transplant patients, and the relationship between low bone mineral density and fracture risk has not been established in children. Nevertheless, fractures, scoliosis, and joint and spinal degeneration are common in patients who received solid organ grafts as children. Avascular bone necrosis occurs infrequently in this patient population. Future studies should evaluate the effects of the underlying disease, transplantation and immunosuppression on the metabolism of bone and cartilage. On the basis of our own clinical experience and literature review, the growing spine of children who have received transplants should be continuously evaluated, and follow-up of bone mineral density is indicated. By contrast, routine MRI of the joints seems unnecessary.

Incidence and predictors of fractures in children after solid organ transplantation: a 5-year prospective, population-based study.

UNLABELLED: In this population-based prospective follow-up study, children undergoing solid organ transplantation had a highly elevated risk for fractures: The incidence of all fractures was 6-fold higher (92 versus 14 fractures/1000 persons/year; p < 0.001) and vertebral fractures was 160-fold higher (57 versus 0.35 fractures/1000 persons/year; p < 0.001) in the study group compared with the control population. Thus, screening of vertebral fractures at regular intervals is recommended, and preventive strategies should be studied. INTRODUCTION: The incidence and predictors of fractures after solid organ transplantation are not well documented in the pediatric age group. MATERIALS AND METHODS: A total of 196 children, which is 93% of patients surviving kidney, liver, and heart transplantation in our country, participated in a retrospective chart review at enrollment followed by a 5-year prospective follow-up study between January 1999 and December 2004. Hospital and medical records were reviewed. All children underwent clinical examinations and answered questionnaires concerning fracture history at the beginning and at the end of the prospective follow-up. Radiographs of the thoracic and lumbar spine were obtained. The fracture incidence was compared with data obtained from public health registries. RESULTS: Seventy-five (38%) of the transplant patients suffered from a total of 166 fractures after organ transplantation. The incidence of all fractures was 6-fold higher (92 versus 14 fractures/1000 persons/year; p < 0.001) and vertebral fractures was 160-fold higher (57 versus 0.35 fractures/1000 persons/year; p < 0.001) in the study group compared with the control population. The age- and sex-adjusted hazard ratios (95% CI) were 61.3 (40.7-92.4) for vertebral, 17.9 (8.96-35.8) for symptomatic vertebral, 0.99 (0.65-1.50) for nonvertebral, and 2.90 (2.25-3.73) for all fractures in the patients compared with the control population. In a multivariate analysis, older age (hazard ratio [95% CI]; 2.02 [1.07-3.83]), male sex (2.15 [1.22-3.81]), liver transplantation (1.78 [1.01-3.14]), and fractures before transplantation (2.02 [0.92-4.47]) were the most significant independent risk factors. CONCLUSIONS: Children undergoing solid organ transplantation have a highly elevated risk for fractures. Screening of vertebral fractures at regular intervals is recommended, and preventive strategies should be studied.

Avascular bone necrosis of the hip joint after solid organ transplantation in childhood: a clinical and MRI analysis.

BACKGROUND: Aseptic osteonecrosis is a well-known complication after solid organ transplantation in adults. The occurrence of osteonecrosis in growing age has been studied after kidney transplantation, but no systematic evaluation of the joints has been reported after heart or liver transplantation in childhood. METHODS: A total of 196 children--93% of patients surviving kidney, liver and heart transplantation in Finland--participated in a cross-sectional survey. All children underwent a detailed clinical examination and filled out a questionnaire on musculoskeletal symptoms. Radiographs were taken in case of joint pain or abnormal clinical findings. In addition, magnetic resonance imaging (MRI) from the hips was taken on a random basis from 34 adult patients transplanted as a child. The mean follow-up time of all patients after transplantation was 9.2 years (range, 2.4 to 20.5 years). RESULTS: Twenty-eight (14%) patients reported prolonged joint or limb pain without previous trauma. Specific etiology for the limb pain was not found in 10 (5.1%) patients. Osteonecrosis seen in radiographs or MRI was noted in seven (3.6%) patients, of which three had received kidney, three liver, and one heart graft. Femoral head was affected in five patients, as well as talus bilaterally in one patient and lateral femoral condyle in one patient. All patients were older than 12 years at the time of diagnosis of the osteonecrosis. MRI of the hips of 34 randomly selected patients showed only one asymptomatic necrosis of the femoral head. CONCLUSIONS: Symptomatic osteonecrosis of the hip is uncommon after solid organ transplantation in childhood using the current immunosuppressive medications.

Lung function in diastrophic dysplasia.

Diastrophic dysplasia (DD) is a rare skeletal dysplasia characterized by short-limbed short stature, contractures and early degeneration of joints, and spinal deformities such as scoliosis. Mortality is increased in the neonatal period, in part due to tracheo- and bronchomalacia. Additionally, spinal deformities are very rigid, decreasing mobility of the chest cage. The aim of our study was to evaluate lung volumes and airway flow dynamics in patients with DD. A total of 31 patients (12 males, 19 females) underwent a detailed clinical examination measurements of standing height PA-radiography of the spine, flow-volume spirometry, and body plethysmography. The patients were assigned to two groups: children and adolescents (0-18 years, n = 18) and adults (over 18 years, n = 13). The mean spirometric parameters were mostly within the predicted value range, although the variation was wide. At least one abnormally low spirometry parameter was found in 6 (33%) of the children and adolescents and in 7 (54%) of the adults. Mean forced vital capacity (FVC) was 104% (range 48-163%) of predicted values in the children and adolescents and 95% (58-140%) of the adults. Peak expiratory flow (PEF) values were abnormal in 4 (22%) of the children and adolescents and in 5 (39%) adults. The mean plethysmographic parameters were all within the predicted value range. At least one abnormal plethysmographic value was found in 6 (33%) of the children and adolescents and in 4 (31%) of the adults. Airway resistance (Raw) was significantly higher in the adults than in the children and adolescents (P = 0.016), and was abnormally high in 3 (23%) of the adults. The angle of thoracic or thoracolumbar scoliosis correlated with the percentages of the predicted values of FVC (r(s) = - 0.66), forced expired volume in 1 sec (FEV(1)) (r(s) = - 0.56), and total lung capacity (TLC) (r(s) = - 0.67). Age correlated with the FEV(1)/FVC ratio (r(s) = - 0.41), with the maximal expired flow at 50% FEV (MEF(50)) values (r(s) = - 0.55), with the residual volume (RV) values (r(s) = - 0.47), and with the RV/TLC ratio (r(s) = - 0.43). Variable bronchial obstruction was found in 1 (6%) child and in 2 (17%) adults. Although the patients with DD had, on average, normal lung volumes, large individual variation occurred. Airway resistance was increased in adults. The angle of scoliosis correlated inversely with lung volume parameters.

Total hip arthroplasty in diastrophic dysplasia.

BACKGROUND: Diastrophic dysplasia results in severe disproportionate growth failure, multiple joint deformities, and early osteoarthritis of the hips. Mortality is increased in early childhood, but thereafter life expectancy is normal. Because of severe flexion deformities, resting pain, and diminished movements of the hip joints, total hip arthroplasty is indicated at an early age. The purpose of our study was to evaluate prospectively the midterm results of total hip arthroplasty in a consecutive series of patients with diastrophic dysplasia. METHODS: Between 1982 and 1996, forty-one total hip replacements were performed in twenty-four consecutive patients with diastrophic dysplasia (mean age, forty-one years) at our hospital. The patients were followed prospectively for a minimum of five years with clinical examination, determination of Harris hip scores, and radiographs. Twenty-two patients (thirty-eight hips) were examined clinically and radiographically at the time of follow-up, and the remaining two patients (three hips) were contacted only by telephone. The mean duration of follow-up was 7.8 years. RESULTS: The mean Harris hip score increased from 44 points (range, 25 to 66 points) before the operation to 70 points (range, 37 to 89 points) at the final follow-up examination (p < 0.001). Ten complications (24%) were recorded. Five (12%) of the forty-one hips required revision because of aseptic loosening of the acetabular component at a mean of 9.4 years after the primary operation. No revisions were due to aseptic failure of the femoral component. CONCLUSIONS: Implant survival was good and the Harris hip scores increased significantly after total hip arthroplasty in patients with diastrophic dysplasia. However, shortening femoral osteotomy and transposition of the greater trochanter, adductor and flexor tenotomies, and modification of the femoral stem were frequently needed. Total hip arthroplasty is recommended for patients with diastrophic dysplasia and severe degeneration of the hip joints, even for those who are relatively young. LEVEL OF EVIDENCE: Therapeutic study, Level IV (case series [no, or historical, control group]). See Instructions to Authors for a complete description of levels of evidence.

Treatment of spinal deformities in patients with diastrophic dysplasia: a long-term, population based, retrospective outcome study.

STUDY DESIGN: A long-term, population based, retrospective follow-up study. OBJECTIVE: To evaluate long-term outcomes of brace and surgical treatment for spinal deformities in patients with diastrophic dysplasia (DD). SUMMARY OF BACKGROUND DATA: Literature on the brace treatment and surgery of spinal deformities in patients with DD is limited. METHODS: All patients with DD undergoing either brace treatment or surgery for spinal deformity with a minimum of 2 years follow-up were identified in our country. Eight patients had undergone brace treatment and 12 had been treated operatively. Two patients had early progressive and the rest idiopathic-like scoliosis. Five patients underwent posterior only, 1 anterior only, and 6 anteroposterior surgery. Patients' mean age at the beginning of brace treatment was 6.9 (range, 0.9-12.7) years and at the time of surgery 13.4 (range, 6.5-20.1) years. The follow-up time averaged 17 (range, 6.6-44.3) years for the brace and 14.0 (range, 2.1-37.2) years for the surgical treatment group. The radiographic follow-up rate was 100%. RESULTS: Both thoracic and lumbar curves progressed during brace treatment (mean major curve progression 12%, range, -43%-53%). Before surgery, the mean Cobb angle of the thoracic curve was 68 degrees (range, 42 degrees-100 degrees) and 46 degrees (25 degrees-68 degrees) in the lumbar spine. At final follow-up visit, the mean correction was 23% (-6%-76%) for the thoracic curve and 25% (-68%-82%) for the lumbar curve. The correction of the major curve was higher in patients undergoing anteroposterior versus posterior only (40% vs. 13%, P = 0.017). Five (42%) operated patients had significant complications. The SRS-24 yielded 92 (79-103) points for the brace treatment and 93 (73-114) points for the surgical group, respectively. CONCLUSION: Brace treatment does not prevent progression of the spinal deformity in patients with DD. Anteroposterior surgery is indicated in patients with severe spinal deformities. The risk for major complications is high especially in patients with marked kyphosis.

Natural history of spinal anomalies and scoliosis associated with esophageal atresia.

OBJECTIVE: Clinical characteristics of spinal deformities that commonly occur in patients with esophageal atresia (EA) are unclear. The objective of this study was to assess the incidence and natural history of spinal anomalies and scoliosis in patients with EA. METHODS: A population-based cohort of 100 adults who had an operation for EA in our hospital were examined clinically and radiographically for spinal deformities. The results were compared with data obtained from normal population-based controls. RESULTS: Vertebral anomalies were observed in 45 patients, predominating in the cervical spine in 38 patients. Any additional anomaly was the most significant risk factor (odds ratio [OR]: 27 [95% confidence interval (CI): 8-100]) for the occurrence of vertebral anomalies. Scoliosis of >10 degrees was observed in 56 patients, >20 degrees in 11 patients, and >45 degrees in 1 patient. The risk for scoliosis of >10 degrees was 13-fold (OR: 13 [95% CI: 8.3-21]), and the risk for scoliosis of >20 degrees was 38-fold (OR: 38 [95% CI: 14-106]) compared with those in the normal population. Thoracotomy-induced rib fusions (OR: 3.6 [95% CI: 0.7-19]) and other associated anomalies (OR: 2.1 [95% CI: 0.9-2.9]) were the strongest predictive factors for scoliosis. The general clinical course of spinal deformities was mild, and none of the patients had undergone spinal surgery. CONCLUSIONS: The risk of scoliosis is 13-fold after repair of EA in relation to general population. Nearly half of the patients have vertebral anomalies predominating in the cervical spine. Most of these deformities were not diagnosed primarily or during growth. Spinal surgery is rarely indicated.