The effect of posterior spinal fusion on respiratory function in Duchenne muscular dystrophy.

PURPOSE: Posterior instrumented spinal fusion is indicated for progressive scoliosis that develops in Duchenne muscular dystrophy (DMD) patients. Whilst spinal fusion is known to improve quality of life, there is inconsistency amongst the literature regarding its specific effect on respiratory function. Our objective was to determine the effect of scoliosis correction by posterior spinal fusion on respiratory function in a large cohort of patients with DMD. Patients with DMD undergoing posterior spinal fusion were compared to patients with DMD not undergoing surgical intervention. METHODS: An observational study of 65 patients with DMD associated scoliosis, born between 1961 and 2001: 28 of which underwent correction of scoliosis via posterior spinal fusion (Surgical Group) and 37 of which did not undergo surgical intervention (Non-Surgical Group). Pulmonary function was assessed using traditional spirometry. Comparisons were made between groups at set times, and by way of rates of change over time. RESULTS: There was no correlation between the level of respiratory dysfunction and the severity of scoliosis (as measured by Cobb angle) for the whole cohort. The Surgical Group had significantly worse respiratory function at a comparable age pre-operatively compared to the Non-Surgical Group, as measured by per cent predicted forced vital capacity (p = 0.02) on spirometry. The rate of decline of forced vital capacity and per cent predicted forced vital capacity was not slowed following surgery compared to the non-operated cases. There was no significant difference in survival between the two groups. CONCLUSIONS: Severity of scoliosis was not a key determinant of respiratory dysfunction. Posterior spinal fusion did not reduce the rate of respiratory function decline. These two points suggest that intrinsic respiratory muscle weakness is the main determinant of decline in respiratory function in DMD.

The mucopolysaccharidoses: advances in medical care lead to challenges in orthopaedic surgical care.

The mucopolysaccharidoses (MPS) are a group of inherited lysosomal storage disorders with clinical manifestations relevant to the orthopaedic surgeon. Our aim was to review the recent advances in their management and the implications for surgical practice. The current literature about MPSs is summarised, emphasising orthopaedic complications and their management. Recent advances in the diagnosis and management of MPSs include the recognition of slowly progressive, late presenting subtypes, developments in life-prolonging systemic treatment and potentially new indications for surgical treatment. The outcomes of surgery in these patients are not yet validated and some procedures have a high rate of complications which differ from those in patients who do not have a MPS. The diagnosis of a MPS should be considered in adolescents or young adults with a previously unrecognised dysplasia of the hip. Surgeons treating patients with a MPS should report their experience and studies should include the assessment of function and quality of life to guide treatment. Cite this article: Bone Joint J 2017;99-B:1132-9.

Obstacles to reduction in infantile developmental dysplasia of the hip.

PURPOSE: Identification of anatomical structures that block -reduction in developmental dysplasia of the hip (DDH) is -important for the management of this challenging condition. Obstacles to reduction seen on arthrogram are well-known. However, despite the increasing use of MRI in the assessment of adequacy of reduction in DDH, the interpretation of MRI patho-anatomy is ill-defined with a lack of relevant literature to guide clinicians. METHOD: This is a retrospective analysis of the MRI of patients with DDH treated by closed reduction over a five-year period (between 2009 and 2014). Neuromuscular and genetic disorders were excluded. Each MRI was analysed by two orthopaedic surgeons and a paediatric musculoskeletal radiologist to identify the ligamentum teres, pulvinar, transverse acetabular ligament (TAL), capsule, labrum and acetabular roof cartilage hypertrophy. Inter- and intraobserver reliability was calculated. The minimum follow-up was 12 months. RESULTS: A total of 29 patients (38 hips) underwent closed reduction for treatment of DDH. Eight hips showed persistent subluxation on post-operative MRI. Only three of these eight hips showed an abnormality on arthrogram. The pulvinar was frequently interpreted as 'abnormal' on MRI. The main obstacles identified on MRI were the ligamentum teres (15.8%), labrum (13.1%) and acetabular roof cartilage hypertrophy (13.2%). The inter-rater reliability was good for TAL, capsule and pulvinar; moderate for ligamentum teres and labrum; and poor for hypertrophied cartilage. CONCLUSION: The labrum, ligamentum teres and acetabular roof cartilage hypertrophy are the most important structures seen on MRI preventing complete reduction of DDH. Focused interpretation of these structures may assist in the management of DDH.

Differences in risk factors between early and late diagnosed developmental dysplasia of the hip.

BACKGROUND: Developmental dysplasia of the hip (DDH) is common, affecting 7.3 per 1000 births in South Australia. Clinical screening programmes exist to identify the condition early to gain the maximum benefit from early treatment. Although these screening programmes are effective, there are still cases that are missed. Previous research has highlighted key risk factors in the development of DDH. OBJECTIVE: To compare the risk factors of cases of DDH identified late with those that were diagnosed early. METHODS: A total of 1281 children with DDH born in 1988-1996 were identified from the South Australian Birth Defects Register. Hospital records of those who had surgery for DDH within 5 years of life were examined for diagnosis details. Twenty seven (2.1%) had been diagnosed at or after 3 months of age and were considered the late DDH cases (a prevalence of 0.15 per 1000 live births). Various factors were compared with early diagnosed DDH cases. RESULTS: Female sex, vertex presentation, normal delivery, rural birth, and discharge from hospital less than 4 days after birth all significantly increased the risk of late diagnosis of DDH. CONCLUSIONS: The results show differences in the risk factors for early and late diagnosed DDH. Some known risk factors for DDH are in fact protective for late diagnosis. These results highlight the need for broad newborn population screening and continued vigilance and training in screening programmes.

Physeal reconstruction using tissue donated from early postnatal limbs in a murine model.

Physeal reconstruction was performed in a murine model by transplanting corresponding postnatal tissue from 4-day-old C57B mice to resection defects. The site of the reconstruction, the murine distal femoral epiphysis, is completely cartilaginous and avascular at this stage of development. The tissue transplanted into the defect was demonstrated to have high kinetic activity by its incorporation of tritiated thymidine. The physeal reconstruction as performed restored only 25% of normal growth. While transplanting cell populations is feasible, the method will require a great deal of work before clinical application.

Evaluation of treatment modalities for septic arthritis with histological grading and analysis of levels of uronic acid, neutral protease, and interleukin-1.

We compared the effectiveness of antibiotics alone and in combination with arthroscopy, arthroscopy with debridement, arthrotomy, or needle aspiration for the treatment of septic arthritis. Each modality has its proponents, but, to our knowledge, no comparative studies have been conducted in animals. We used biochemical and histological analysis to compare these methods of treatment in an experimental model. The right hind knee of thirty goats was injected with 1 x 10(5) Staphylococcus aureus bacilli. The left hind knee was not inoculated and served as the normal control. Seventy-two hours after inoculation, a two-week course of treatment with intramuscular administration of cefuroxime sodium, either alone or in combination with another mode of treatment, was initiated in each of five groups. The cartilage was evaluated histologically with biochemical, enzymatic, and interleukin-1 analyses. Despite the early therapeutic intervention, on the average, there was a 25 per cent loss of uronic acid (t test, p < 0.001) and a 43 per cent increase in neutral protease activity (signed-rank test, p = 0.003) in the treatment groups. There were no significant intergroup differences with regard to the histochemical-histological rating or the levels of uronic acid, neutral protease, or interleukin-1.

Perinatal risk factors for developmental dysplasia of the hip.

AIMS: To identify perinatal risk factors for developmental dysplasia of the hip (DDH) and define the risk for each factor. METHODS: In this case control study, using logistic regression analysis, all 1127 cases of isolated DDH live born in South Australia in 1986-93 and notified to the South Australian Birth Defects Register were included; controls comprised 150130 live births in South Australia during the same period without any notified congenital abnormalities. RESULTS: Breech presentation, oligohydramnios, female sex and primiparity were confirmed as risk factors for DDH. Significant findings were an increased risk for vaginal delivery over caesarean section for breech presentation (as well as an increased risk for emergency section over elective section), high birthweight (> or = 4000 g), postmaturity and older maternal age; multiple births and preterm births had a reduced risk. There was no increased risk for caesarean section in the absence of breech presentation. For breech presentation, the risk of DDH was estimated to be at least 2.7% for girls and 0.8% for boys; a combination of factors increased the risk. CONCLUSIONS: It is suggested that the risk factors identified be used as indications for repeat screening at 6 weeks of age and whenever possible in infancy. Other indications are family history and associated abnormalities.

Cotrel-Dubousset instrumentation and vertebral rotation in adolescent idiopathic scoliosis.

We have studied 34 consecutive patients receiving Cotrel- Dubousset instrumentation for a single and flexible thoracic scoliotic curve, evaluating the rib hump deformity from a single CT scan through the apical vertebra of the curve. Using two measures of rotation we found a mean improvement of 25% in the rotation of the vertebra after operation. Any, usually minor, deterioration occurred in the first six months postoperatively, and there was no significant further deterioration in 19 patients assessed over two years after surgery. Cotrel-Dubousset instrumentation can produce a significant correction of vertebral rotation and of the associated rib hump deformity.

Synthetic versus plaster of Paris casts in the treatment of fractures of the forearm in children: a randomised trial of clinical outcomes and patient satisfaction.

Fractures of the forearm (radius or ulna or both) in children have traditionally been immobilised in plaster of Paris (POP) but synthetic cast materials are becoming more popular. There have been no randomised studies comparing the efficacy of these two materials. The aim of this study was to investigate which cast material is superior for the management of these fractures. We undertook a single-centre prospective randomised trial involving 199 patients with acute fractures of the forearm requiring general anaesthesia for reduction. Patients were randomised by sealed envelope into either a POP or synthetic group and then underwent routine closed reduction and immobilisation in a cast. The patients were reviewed at one and six weeks. A satisfaction questionnaire was completed following the removal of the cast. All clinical complications were recorded and the cast indices were calculated. There was an increase in complications in the POP group. These complications included soft areas of POP requiring revision and loss of reduction with some requiring re-manipulation. There was an increased mean padding index in the fractures that lost reduction. Synthetic casts were preferred by the patients. This study indicates that the clinical outcomes and patient satisfaction are superior using synthetic casts with no reduction in safety.

Slipped capital femoral epiphysis: rising rates with obesity and aboriginality in South Australia.

We analysed the incidence of slipped capital femoral epiphysis (SCFE) in South Australia, investigating possible associations between an increased incidence of SCFE, the local indigenous population and the Australian obesity epidemic during the last 20 years. Data including race, age and gender were collected to obtain a profile of the South Australian SCFE patient, and were then compared with epidemiological data for South Australian adolescents. We concluded that the incidence of both obesity and SCFE is increasing. We also noted that the median weight of SCFE patients has increased and the mean age at diagnosis has decreased. Despite weight profiles comparable with those of the general population, we noted that an indigenous child was three times more likely to develop SCFE than a non-indigenous child. As far as we know there is no published literature on the predisposition of Aboriginal Australians to SCFE.

A ten-year review of treatment of delayed union and nonunion with an implanted bone growth stimulator.

A ten-year clinical and roentgenographic review was conducted on the patients in the original Australian multicenter trial that evaluated use of an implantable bone growth stimulator for delayed union and nonunion. Of the original 81 patients, 38 patients were located, seven patients had died from unrelated causes, and 36 were unlocatable. Of the 38 patients located, 37 patients (representing 38 fractures) participated in a detailed clinical review and had a roentgenographic assessment. All fractures had remained united, and normal bone remodeling had occurred. There were no adverse effects of the generator or cathode wire. Six patients initially reported as failures had healed after further surgical intervention. Thus, normal osteogenesis occurs in association with electrical stimulation using an implantable bone growth stimulator. This ten-year review supports the long-term safety and effectiveness of this technique in treating nonuniting fractures.

Synovial hemangioma of the knee.

Hemangioma of the knee is a cause of pain and recurrent hemarthroses in the pediatric age group, often seen as an internal derangement of the knee. Historically, long delays in diagnosis have occurred. Conventional radiographic techniques can miss the presence of the lesion. However, magnetic resonance imaging (MRI) is a noninvasive and frequently diagnostic investigative modality. Five cases of diffuse synovial hemangioma of the knee are presented. Diffuse lesions are difficult to excise arthroscopically, and open wide excision is recommended after MRI and arthroscopic assessment.

Proximal humeral fractures. Management techniques and expected results.

During a ten-year period (1978-1988), 143 of 1386 patients with proximal humeral fractures were treated with internal osteosynthesis. Ninety-seven proximal humeral osteosynthesis cases had adequate documentation, and 77 (80%) were available for clinical review. The 97 fractures were graded by the AO/ASIF classification and included 44 Group A, 32 Group B, and 21 Group C fractures. Exercise-stable osteosynthesis using T-plate, cloverleaf plate, or small condylar plate was performed in 70% of patients. In the remaining patients, a less rigid fixation, with Kirschner wires or screws and cerclage wires, was used. Fifty-two percent of the patients had excellent and good results, 15% had fair results, and 33% had poor results. Most poor results occurred in patients with four-part fractures (61% of poor results). Of the cases involving four-part fractures, however, 22% had an excellent result after internal fixation. Displaced four-part fractures or fracture-dislocations should be treated by reconstruction of the proximal humerus, especially in young patients. The use of minimal fixation rather than rigid fixation is considered after careful assessment of the condition of the soft tissue and blood supply of the humeral head fragments. Primary treatment with endoprostheses is required when internal fixation is impractical in AO/ASIF fracture Types C 2/3 and C3 fractures. Improved clinical results may be achieved, particularly in the more severe fracture types, with increased experience in techniques of internal fixation of proximal humeral fractures.

Three-dimensional imaging of a juvenile Tillaux fracture.

The juvenile Tillaux fracture is an injury involving the articular surface of the ankle joint. The fracture sometimes requires open reduction and internal fixation. The decision to adopt an operative or nonoperative policy in each case can only be made with precise imaging of the anatomy which can be provided by three-dimensional reformation of computed tomography (CT) scanning.

Late diagnosis of congenital dislocation of the hip and presence of a screening programme: South Australian population-based study.

BACKGROUND: The Medical Research Council Working Party on Congenital Dislocation of the Hip have reported an ascertainment-adjusted incidence of a first operative procedure for congenital dislocation of the hip (CDH) of 0.78 per 1000 livebirths, which is similar to the incidence of CDH before the start of the UK screening programme. The report showed that CDH had not been detected by routine screening before age 3 months in 70% of children reported to the national orthopaedic surveillance scheme. This report raised concerns about the merit of screening at birth for CDH. We aimed to find out the incidence of an operative procedure for CDH in the first 5 years of life among children born in South Australia between 1988 and 1993, and the proportion of these patients that were detected at age 3 months or older. METHODS: The state's database for inpatient separations between January, 1988, and April, 1998 was searched. Case records were examined for the age and circumstances of diagnosis, and type of operative procedures. Prevalence rates of CDH were obtained from the South Australian Birth Defects Register, which receives notifications from a statutory perinatal data collection of birth defects detected at birth and subsequent voluntary notifications for children up to age 5 years. FINDINGS: Of the 55 children born in South Australia between 1988 and 1993 identified as having non-teratological CDH and operative procedures, only 22 (40%) had been diagnosed at age 3 months or older. 18 had an open reduction of the hip joint or osteotomy, or both, and the remainder had arthrograms, closed reductions, and/or tenotomy. The prevalence of non-teratological CDH in children was 7.74 per 1000 livebirths. The incidence of surgery for CDH in the first 5 years of life was 0.46 per 1000 livebirths (95% CI 0.34-0.59) and only 0.19 per 1000 livebirths (0.11-0.26) for those diagnosed late (age 3 months or older). These children diagnosed late represented 2.4% of all known cases of CDH. INTERPRETATION: Only 2.4% of known cases of CDH in children born in South Australia had been detected late and required surgery. These results show that a screening programme for CDH can be successful, contrary to the findings of the UK Medical Research Council Working Party.

Developmental dysplasia of the hip in South Australia in 1991: prevalence and risk factors.

OBJECTIVE: To determine the prevalence of developmental dysplasia of the hip (DDH) in South Australia (SA) in 1991, the proportion of cases detected in the neonatal period and the perinatal risk factors for DDH. METHODOLOGY: Cases of DDH born in SA in 1991 were identified from multiple sources and their clinical data linked to perinatal data provided by midwives; five controls per case were obtained randomly from SA livebirths without congenital abnormalities and adjusted odds ratios (OR) for potential risk factors obtained by logistic regression analysis. South Australia perinatal data were also used to estimate numbers of births with perinatal risk factors for targeted screening. RESULTS: Two hundred and six cases of isolated DDH were identified, giving a prevalence of 10.5 per 1000 births. Of these, 173 (84%) had been detected in the neonatal period. The perinatal risk factors for DDH were identified as breech presentation (OR 9.65), female babies (OR 4.04), first births (OR 1.91) and maternal age of 25 years or more (OR 1.53). Screening breech and firstborn female babies (23% of births) would yield approximately 51% of cases of DDH. CONCLUSIONS: Isolated DDH had a prevalence of 10.5 per 1000 births and 84% of cases had been detected in the neonatal period in SA. Repeated screening during infancy of "at risk' groups of babies is recommended.