We reviewed 31 consecutive patients with Friedreich’s ataxia and scoliosis. There were 24 males and seven females with a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean curve of 51° (13° to 140°). A total of 12 patients had thoracic curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar. Two patients had long thoracolumbar collapsing scoliosis with pelvic obliquity and four had hyperkyphosis. Left-sided thoracic curves in nine patients (45%) and increased thoracic kyphosis differentiated these deformities from adolescent idiopathic scoliosis. There were 17 patients who underwent a posterior instrumented spinal fusion at mean age of 13.35 years, which achieved and maintained good correction of the deformity. Post-operative complications included one death due to cardiorespiratory failure, one revision to address nonunion and four patients with proximal junctional kyphosis who did not need extension of the fusion. There were no neurological complications and no wound infections. The rate of progression of the scoliosis in children kept under simple observation and those treated with bracing was less for lumbar curves during bracing and similar for thoracic curves. The scoliosis progressed in seven of nine children initially treated with a brace who later required surgery. Two patients presented after skeletal maturity with balanced curves not requiring correction. Three patients with severe deformities who would benefit from corrective surgery had significant cardiac co-morbidities.

This review of the literature presents the current understanding of Scheuermann’s kyphosis and investigates the controversies concerning conservative and surgical treatment. There is considerable debate regarding the pathogenesis, natural history and treatment of this condition. A benign prognosis with settling of symptoms and stabilisation of the deformity at skeletal maturity is expected in most patients. Observation and programmes of exercise are appropriate for mild, flexible, non-progressive deformities. Bracing is indicated for a moderate deformity which spans several levels and retains flexibility in motivated patients who have significant remaining spinal growth.

The loss of some correction after the completion of bracing with recurrent anterior vertebral wedging has been reported in approximately one-third of patients. Surgical correction with instrumented spinal fusion is indicated for a severe kyphosis which carries a risk of progression beyond the end of growth causing cosmetic deformity, back pain and neurological complications. There is no consensus on the effectiveness of different techniques and types of instrumentation. Techniques include posterior-only and combined anteroposterior spinal fusion with or without posterior osteotomies across the apex of the deformity. Current instrumented techniques include hybrid and all-pedicle screw constructs.

Between June 1988 and December 1997, we treated 332 babies with 546 dysplastic hips in a Pavlik harness for primary developmental dysplasia of the hip as detected by the selective screening programme in Southampton. Each was managed by a strict protocol including ultrasonic monitoring of treatment in the harness. The group was prospectively studied during a mean period of 6.5 ± 2.7 years with follow-up of 89.9%. The acetabular index (AI) and centre-edge angle of Wiberg (CEA) were measured on annual radiographs to determine the development of the hip after treatment and were compared with published normal values. The harness failed to reduce 18 hips in 16 patients (15.2% of dislocations, 3.3% of DDH). These required surgical treatment. The development of those hips which were successfully treated in the harness showed no significant difference from the normal values of the AI for the left hips of girls after 18 months of age. Of those dysplastic hips which were successfully reduced in the harness, 2.4% showed persistent significant late dysplasia (CEA < 20°) and 0.2% persistent severe late dysplasia (CEA < 15°). All could be identified by an abnormal CEA (< 20°) at five years of age, and many from the progression of the AI by 18 months. Dysplasia was considered to be sufficient to require innominate osteotomy in five (0.9%). Avascular necrosis was noted in 1% of hips treated in the harness. We conclude that, using our protocol, successful initial treatment of DDH with the Pavlik harness appears to restore the natural development of the hip to normal. We suggest that regular radiological surveillance up to five years of age is a safe and effective practice

The December 2023 Research Roundup³⁶⁰ looks at: Tissue integration and chondroprotective potential of acetabular labral augmentation with autograft tendon: study of a porcine model; The Irish National Orthopaedic Register under cyberattack: what happened, and what were the consequences?; An overview of machine learning in orthopaedic surgery: an educational paper; Beware of the fungus…; New evidence for COVID-19 in patients undergoing joint replacement surgery.

Aims

Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed non-operatively or operatively in our institution.

Aims

We reviewed 34 consecutive patients (18 female-16 male) with isthmic spondylolysis and grade I to II lumbosacral spondylolisthesis who underwent in situ posterolateral arthodesis between the L5 transverse processes and the sacral ala with the use of iliac crest autograft. Ten patients had an associated scoliosis which required surgical correction at a later stage only in two patients with idiopathic curves unrelated to the spondylolisthesis.

A review of the current literature shows that there is a lack of consensus regarding the treatment of spondylolysis and spondylolisthesis in children and adolescents. Most of the views and recommendations provided in various reports are weakly supported by evidence. There is a limited amount of information about the natural history of the condition, making it difficult to compare the effectiveness of various conservative and operative treatments. This systematic review summarises the current knowledge on spondylolysis and spondylolisthesis and attempts to present a rational approach to the evaluation and management of this condition in children and adolescents.

We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity.

We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis.

Aims

Hip disease is common in children with cerebral palsy (CP) and can decrease quality of life and function. Surveillance programmes exist to improve outcomes by treating hip disease at an early stage using radiological surveillance. However, studies and surveillance programmes report different radiological outcomes, making it difficult to compare. We aimed to identify the most important radiological measurements and develop a core measurement set (CMS) for clinical practice, research, and surveillance programmes.

Aims

It is important to analyze objectively the hammering sound in cup press-fit technique in total hip arthroplasty (THA) in order to better understand the change of the sound during impaction. We hypothesized that a specific characteristic would present in a hammering sound with successful fixation. We designed the study to quantitatively investigate the acoustic characteristics during cementless cup impaction in THA.

Aims

It is important to analyze objectively the hammering sound in cup press-fit technique in total hip arthroplasty (THA) in order to better understand the change of the sound during impaction. We hypothesized that a specific characteristic would present in a hammering sound with successful fixation. We designed the study to quantitatively investigate the acoustic characteristics during cementless cup impaction in THA.

Aims

Periacetabular osteotomy (PAO) is widely recognized as a demanding surgical procedure for acetabular reorientation. Reports about the learning curve have primarily focused on complication rates during the initial learning phase. Therefore, our aim was to assess the PAO learning curve from an analytical perspective by determining the number of PAOs required for the duration of surgery to plateau and the accuracy to improve.

Aims

Acetabular retroversion is a recognized cause of hip impingement and can be influenced by pelvic tilt (PT), which changes in different functional positions. Positional changes in PT have not previously been studied in patients with acetabular retroversion.

An eight-week-old boy developed severe thoracic spondylodiscitis following pneumonia and septicaemia. A delay in diagnosis resulted in complete destruction of the T4 and T5 vertebral bodies and adjacent discs, with a paraspinal abscess extending into the mediastinum and epidural space. Antibiotic treatment controlled the infection and the abscess was aspirated. At the age of six months, he underwent posterior spinal fusion in situ to stabilise the spine and prevent progressive kyphosis. At the age of 13 months, repeat imaging showed lack of anterior vertebral body re-growth and he underwent anterior spinal fusion from T3 to T6 and augmentation of the posterior fusion. At the age of five years, he had no symptoms and radiographs showed bony fusion across the affected levels.

Spondylodiscitis should be included in the differential diagnosis of infants who present with severe illness and atypical symptoms. Delayed diagnosis can result in major spinal complications with a potentially fatal outcome.

Although about 200 000 cementless Zweymüller-Alloclassic total hip arthroplasties (THAs) were carried out worldwide in the last decade, the survival analysis of these prostheses was not available in the 2000 report of the Swedish national hip arthroplasty registry. We report a prospective survivorship analysis of 200 consecutive grit-blasted cementless Alloclassic primary THAs carried out since 1988.

Using surgical, clinical and radiological endpoints for the stem and the threaded cup the ten-year survivorship was 91.5% for reoperation for any cause, 96.4% for hip pain (Merle d’Aubigné score < 5 points, clinical failure), 99.4% for definite aseptic loosening (radiological failure) and 99.3% for revision for aseptic loosening. Using the Swedish registry criteria of primary osteoarthritis and revision for aseptic loosening as the endpoint, the survival rate of 99.1% at ten years for the subgroup of 157 Alloclassic THAs in osteoarthritis compares favourably with that of the best modern cemented hip replacements reported in the Swedish arthroplasty registry.

Clinical, radiological, and Scoliosis Research Society-22 questionnaire data were reviewed pre-operatively and two years post-operatively for patients with thoracolumbar/lumbar adolescent idiopathic scoliosis treated by posterior spinal fusion using a unilateral convex segmental pedicle screw technique. A total of 72 patients were included (67 female, 5 male; mean age at surgery 16.7 years (13 to 23)) and divided into groups: group 1 included 53 patients who underwent fusion between the vertebrae at the limit of the curve (proximal and distal end vertebrae); group 2 included 19 patients who underwent extension of the fusion distally beyond the caudal end vertebra.

A mean scoliosis correction of 80% (45% to 100%) was achieved. The mean post-operative lowest instrumented vertebra angle, apical vertebra translation and trunk shift were less than in previous studies. A total of five pre-operative radiological parameters differed significantly between the groups and correlated with the extension of the fusion distally: the size of the thoracolumbar/lumbar curve, the lowest instrumented vertebra angle, apical vertebra translation, the Cobb angle on lumbar convex bending and the size of the compensatory thoracic curve. Regression analysis allowed an equation incorporating these parameters to be developed which had a positive predictive value of 81% in determining whether the lowest instrumented vertebra should be at the caudal end vertebra or one or two levels more distal. There were no differences in the Scoliosis Research Society-22 outcome scores between the two groups (p = 0.17).

In conclusion, thoracolumbar/lumbar curves in patients with adolescent idiopathic scoliosis may be effectively treated by posterior spinal fusion using a unilateral segmental pedicle screw technique. Five radiological parameters correlate with the need for distal extension of the fusion, and an equation incorporating these parameters reliably informs selection of the lowest instrumented vertebra.

Cite this article: Bone Joint J 2014;96-B:1082–9.

We report the incidence of and risk factors for complications after scoliosis surgery in patients with Duchenne muscular dystrophy (DMD) and compare them with those of other neuromuscular conditions.

We identified 110 (64 males, 46 females) consecutive patients with a neuromuscular disorder who underwent correction of the scoliosis at a mean age of 14 years (7 to 19) and had a minimum two-year follow-up. We recorded demographic and peri-operative data, including complications and re-operations.

There were 60 patients with cerebral palsy (54.5%) and 26 with DMD (23.6%). The overall complication rate was 22% (24 patients), the most common of which were deep wound infection (9, 8.1%), gastrointestinal complications (5, 4.5%) and hepatotoxicity (4, 3.6%). The complication rate was higher in patients with DMD (10/26, 38.5%) than in those with other neuromuscular conditions (14/84, 16.7% (p = 0.019). All hepatotoxicity occurred in patients with DMD (p = 0.003), who also had an increased rate of deep wound infection (19% vs 5%) (p = 0.033). In the DMD group, no peri-operative factors were significantly associated with the rate of overall complications or deep wound infection. Increased intra-operative blood loss was associated with hepatotoxicity (p = 0.036).

In our series, correction of a neuromuscular scoliosis had an acceptable rate of complications: patients with DMD had an increased overall rate compared with those with other neuromuscular conditions. These included deep wound infection and hepatotoxicity. Hepatotoxicity was unique to DMD patients, and we recommend peri-operative vigilance after correction of a scoliosis in this group.

Cite this article: Bone Joint J 2014; 96-B:943–9.