Aims. Children with spinal dysraphism can develop various musculoskeletal deformities, necessitating a range of orthopaedic interventions, causing significant morbidity, and making considerable demands on resources. This systematic review aimed to identify what outcome measures have been reported in the literature for children with spinal dysraphism who undergo orthopaedic interventions involving the lower limbs. Methods. A PROSPERO-registered systematic literature review was performed following PRISMA guidelines. All relevant studies published until January 2023 were identified. Individual outcomes and outcome measurement tools were extracted verbatim. The measurement tools were assessed for reliability and validity, and all outcomes were grouped according to the Outcome Measures Recommended for use in Randomized Clinical Trials (OMERACT) filters. Results. From 91 eligible studies, 27 individual outcomes were identified, including those related to clinical assessment (n = 12), mobility (n = 4), adverse events (n = 6), investigations (n = 4), and miscellaneous (n = 1). Ten outcome measurement tools were identified, of which Hoffer’s Functional Ambulation Scale was the most commonly used. Several studies used unvalidated measurement tools originally developed for other conditions, and 26 studies developed new measurement tools. On the OMERACT filter, most outcomes reported pathophysiology and/or the impact on life. There were only six patient- or parent-reported outcomes, and none assessed the quality of life. Conclusion. The outcomes that were reported were heterogenous, lack validation and failed to incorporate patient or family perceptions. Until outcomes can be reported unequivocally, research in this area will remain limited. Our findings should guide the development of a core outcome set, which will allow consistency in the reporting of outcomes for this condition. Cite this article: Bone Joint J 2024;106-B(3):277–285

Aims

The aim of this study is to evaluate the surgical treatment with the best healing rate for patients with proximal femoral unicameral bone cysts (UBCs) after initial surgery, and to determine which procedure has the lowest adverse event burden during follow-up.

Aims

Perthes’ disease is a condition which leads to necrosis of the femoral head. It is most commonly reported in children aged four to nine years, with recent statistics suggesting it affects around five per 100,000 children in the UK. Current treatment for the condition aims to maintain the best possible environment for the disease process to run its natural course. Management typically includes physiotherapy with or without surgical intervention. Physiotherapy intervention often will include strengthening/stretching programmes, exercise/activity advice, and, in some centres, will include intervention, such as hydrotherapy. There is significant variation in care with no consensus on which treatment option is best. The importance of work in this area has been demonstrated by the British Society for Children’s Orthopaedic Surgery through the James Lind Alliance’s prioritization of work to determine/identify surgical versus non-surgical management of Perthes’ disease. It was identified as the fourth-highest priority for paediatric lower limb surgery research in 2018.

We undertook a retrospective comparative study of all patients with an unstable slipped capital femoral epiphysis presenting to a single centre between 1998 and 2011. There were 45 patients (46 hips; mean age 12.6 years; 9 to 14); 16 hips underwent intracapsular cuneiform osteotomy and 30 underwent pinning in situ, with varying degrees of serendipitous reduction. No patient in the osteotomy group was lost to follow-up, which was undertaken at a mean of 28 months (11 to 48); four patients in the pinning in situ group were lost to follow-up, which occurred at a mean of 30 months (10 to 50). Avascular necrosis (AVN) occurred in four hips (25%) following osteotomy and in 11 (42%) following pinning in situ. AVN was not seen in five hips for which osteotomy was undertaken > 13 days after presentation. AVN occurred in four of ten (40%) hips undergoing emergency pinning in situ, compared with four of 15 (47%) undergoing non-emergency pinning. The rate of AVN was 67% (four of six) in those undergoing pinning on the second or third day after presentation.

Pinning in situ following complete reduction led to AVN in four out of five cases (80%). In comparison, pinning in situ following incomplete reduction led to AVN in 7 of 21 cases (33%). The rate of development of AVN was significantly higher following pinning in situ with complete reduction than following intracapsular osteotomy (p = 0.048). Complete reduction was more frequent in those treated by emergency pinning and was strongly associated with AVN (p = 0.005).

Non-emergency intracapsular osteotomy may have a protective effect on the epiphyseal vasculature and should be undertaken with a delay of at least two weeks. The place of emergency pinning in situ in these patients needs to be re-evaluated, possibly in favour of an emergency open procedure or delayed intracapsular osteotomy. Non-emergency pinning in situ should be undertaken after a delay of at least five days, with the greatest risk at two and three days after presentation. Intracapsular osteotomy should be undertaken after a delay of at least 14 days. In our experience, closed epiphyseal reduction is harmful.

Cite this article: Bone Joint J 2015;97-B:412–19.

We retrospectively reviewed the records of 16 children treated for spondylodiscitis at our hospital between 2000 and 2007. The mean follow-up was 24 months (12 to 38). There was a mean delay in diagnosis in hospital of 25 days in the ten children aged less than 24 months. At presentation only five of the 16 children presented with localising signs and symptoms. Common presenting symptoms were a refusal to walk or sit in nine children, unexplained fever in six, irritability in five, and limping in four. Plain radiography showed changes in only seven children. The ESR was the most useful investigation when following the clinical course of the disease. Positive blood cultures were obtained in seven children with Staphylococcus aureus being isolated in five. Antibiotics were used in 14 children and spinal bracing in six. Children with spondylodiscitis often present with a confusing clinical picture leading to late diagnosis.

The early use of MRI in the investigation of children with an atypical picture may avoid unnecessary delay in starting treatment and possibly prevent long-term problems. All except one of our children had made a complete clinical recovery at final follow-up. However, all six children in the > 24-month age group showed radiological evidence of degenerative changes which might cause problems in the future.

Congenital pseudarthrosis of the tibia is an uncommon manifestation of neurofibromatosis type 1 (NF1), but one that remains difficult to treat due to anabolic deficiency and catabolic excess. Bone grafting and more recently recombinant human bone morphogenetic proteins (rhBMPs) have been identified as pro-anabolic stimuli with the potential to improve the outcome after surgery. As an additional pharmaceutical intervention, we describe the combined use of rhBMP-2 and the bisphosphonate zoledronic acid in a mouse model of NF1-deficient fracture repair.

Fractures were generated in the distal tibiae of neurofibromatosis type 1-deficient (Nf1^+/−) mice and control mice. Fractures were open and featured periosteal stripping. All mice received 10 μg rhBMP-2 delivered in a carboxymethylcellulose carrier around the fracture as an anabolic stimulus. Bisphosphonate-treated mice also received five doses of 0.02 mg/kg zoledronic acid given by intraperitoneal injection.

When only rhBMP but no zoledronic acid was used to promote repair, 75% of fractures in Nf1^+/− mice remained ununited at three weeks compared with 7% of controls (p < 0.001). Systemic post-operative administration of zoledronic acid halved the rate of ununited fractures to 37.5% (p < 0.07).

These data support the concept that preventing bone loss in combination with anabolic stimulation may improve the outcome following surgical treatment for children with congenital pseudarthoris of the tibia and NF1.

Septicaemia resulting from meningococcal infection is a devastating illness affecting children. Those who survive can develop late orthopaedic sequelae from growth plate arrests, with resultant complex deformities. Our aim in this study was to review the case histories of a series of patients with late orthopaedic sequelae, all treated by the senior author (CFB). We also describe a treatment strategy to address the multiple deformities that may occur in these patients.

Between 1997 and 2009, ten patients (seven girls and three boys) were treated for late orthopaedic sequelae following meningococcal septicaemia. All had involvement of the lower limbs, and one also had involvement of the upper limbs. Each patient had a median of three operations (one to nine). Methods of treatment included a combination of angular deformity correction, limb lengthening and epiphysiodesis. All patients were skeletally mature at the final follow-up. One patient with bilateral below-knee amputations had satisfactory correction of her right amputation stump deformity, and has complete ablation of both her proximal tibial growth plates. In eight patients length discrepancy in the lower limb was corrected to within 1 cm, with normalisation of the mechanical axis of the lower limb.

Meningococcal septicaemia can lead to late orthopaedic sequelae due to growth plate arrests. Central growth plate arrests lead to limb-length discrepancy and the need for lengthening procedures, and peripheral growth plate arrests lead to angular deformities requiring corrective osteotomies and ablation of the damaged physis. In addition, limb amputations may be necessary and there may be altered growth of the stump requiring further surgery. Long-term follow-up of these patients is essential to recognise and treat any recurrence of deformity.