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. Methods. This multicentre retrospective study was conducted in 20 tertiary paediatric hospitals in France, Belgium, and Switzerland, and included patients aged < 16 years admitted for UBC treatment in the proximal femur from January 1995 to December 2017. UBCs were divided into seven groups based on the index treatment, which included elastic stable intramedullary nail (ESIN) insertion with or without percutaneous injection or grafting, percutaneous injection alone, curettage and grafting alone, and insertion of other orthopaedic hardware with or without curettage. Results. A total of 201 patients were included in the study. The mean age at diagnosis was 8.7 years (SD 3.9); 77% (n = 156) were male. The mean follow-up was 9.4 years (SD 3.9). ESIN insertion without complementary procedure had a 67% UBC healing rate after the first operation (vs 30% with percutaneous injection alone (p = 0.027), 43% with curettage and grafting (p = 0.064), and 21% with insertion of other hardware combined with curettage (p < 0.001) or 36% alone (p = 0.014)). ESIN insertion with percutaneous injection presented a 79% healing rate, higher than percutaneous injection alone (p = 0.017), curettage and grafting (p = 0.028), and insertion of other hardware combined with curettage (p < 0.001) or alone (p = 0.014). Patients who underwent ESIN insertion with curettage had a 53% healing rate, higher than insertion of other hardware combined with curettage (p = 0.009). The overall rate of postoperative complications was 25% and did not differ between groups (p = 0.228). A total of 32 limb length discrepancies were identified. Conclusion. ESIN insertion, either alone or combined with percutaneous injection or curettage and grafting, may offer higher healing rates than other operative procedures. Limb length discrepancy remains a major concern, and might be partly explained by the cyst’s location and the consequence of surgery. Therefore, providing information about this risk is crucial. Cite this article: Bone Joint J 2024;106-B(5):508–514

Background. Unicameral bone cysts (UBCs) are difficult to treat and have a high recurrence rate. Their pathogenesis is unknown making targeted therapies difficult. Attributed causes include venous and interstitial fluid obstruction, oxygen free radicals, lysosomal enzymes, prostaglandins and genetic factors. Skeletal stem cells (SSCs) are osteoblast precursors critical to bone formation and cyst fluid may influence their growth, however the association between SSCs and cyst fluid has never been investigated. Aim. To investigate the effect of UBC fluid on SSC growth. Methods. Fluid was aspirated from a UBC in the proximal femur of a nine year old boy and centrifuged to isolate the acellular supernatant. SSCs were harvested from bone marrow of a haematologically normal adult and cultured with graded concentrations of cyst fluid in culture media (0,10,25,50%). Cell growth was assessed by alkaline phosphatase staining, and cytokine levels in the fluid were measured. Results. High levels of cytokines known to be chemo-attractive for cells of the of macrophage-monocyte lineage were found, including Macrophage Chemotactic Protein-1 (1853pg/ml), Monokine Induced by γ-interferon (656pg/ml), Macrophage Inflammatory Protein (MIP)-1α (401pg/ml) and MIP-1β (34pg/ml) suggesting a role of osteoclasts in UBC pathogenesis. Furthermore, SSC growth in vitro was reduced in cyst fluid in a concentration dependent manner. Conclusion. This is the first time altered SSC and osteoprogenitor function has been associated with the fluid of a UBC. A negative effect on osteogenesis was demonstrated, the precise mechanisms of which are under investigation, and macrophage-monocyte chemokines suggest high osteoclast activity. This study has indicated a role of the cyst fluid in limiting osteogenesis and bone turnover, which may explain the high failure rate for current interventions. More patients are needed to validate these findings

In 79 consecutive patients with unicameral bone cysts we compared the results of aspiration and injection of bone marrow with those of aspiration and injection of steroid. All were treated by the same protocol. The only difference was the substance injected into the cysts. The mean radiological follow-up to detect activity in the cyst was 44 months (12 to 108). Of the 79 patients, 14 received a total of 27 injections of bone marrow and 65 a total of 99 injections of steroid. Repeated injections were required in 57% of patients after bone marrow had been used and in 49% after steroid. No complications were noted in either group. In this series no advantage could be shown for the use of autogenous injection of bone marrow compared with injection of steroid in the management of unicameral bone cysts

We prospectively evaluated the percutaneous injection of autogenous bone marrow for the treatment of active simple bone cysts in ten consecutive children with cysts in the proximal humerus, proximal femur or tibia. The treatment included percutaneous biopsy, aspiration of fluid and the injection of autogenous bone marrow aspirated from the iliac crest. All the patients became painfree after a mean of two weeks and resumed full activities within six weeks. All ten cysts consolidated radiologically and showed remarkable remodelling within four months. Review at 12 to 48 months showed satisfactory healing without complications. Percutaneous injection of autologous bone marrow appears to be an effective treatment for active simple bone cysts

Aim. The purpose of this study was to quantify the number of children treated with ESIN (elastic stable intra-medullary nails) at our institution and to determine what proportion of these nails have been removed and the time and difficulty taken to do so. Methods. Patients treated with ESIN between 2000 and 2005 were identified by database search. Their notes were reviewed to record patient and initial nailing details, and timing, difficulty and length of metalwork removal procedures. Complications leading to premature removal or following nail extraction were noted. Results. Eighty-seven patients were identified who fitted our study criteria (68% male, 32% female). Mean age was 10.9 years (4.7–15.9 years). Twelve of the procedures were for open fractures. Seventy-nine patients underwent nail removal at our institution. Four were followed up elsewhere and a decision to leave the nails in was made in another four patients (usually underlying bone pathology). Four patients had surgery earlier than planned to deal with implant problems: 3 to address prominent nails and 1 for re-fracture. The nails were removed at a mean of 8 months after insertion. Mean time taken to remove the metalwork was 25 minutes (10 to 75 minutes). Eight of the 79 removal procedures were graded as difficult, with one being extremely difficult: this was not predictable from pre-operative radiographs. There were 2 complications following metalwork removal. One was a re-fracture through a unicortical bone cyst 2 years after removal, which was successfully treated with ESIN again. The other was a superficial wound infection, which settled with antibiotics. Conclusion. It is routine policy at our institution to remove ESIN after fracture healing, but it should be noted that removal procedures can be difficult and lengthy. There is, however, a low complication rate following removal, and routine follow-up may not be necessary

Aims

Our aim was to describe the mid-term appearances of the repair process of the Achilles tendon after tenotomy in children with a clubfoot treated using the Ponseti method.

Congenital pseudarthrosis of the tibia (CPT) is a rare but well recognised condition. Obtaining union of the pseudarthrosis in these children is often difficult and may require several surgical procedures. The treatment has changed significantly since the review by Hardinge in 1972, but controversies continue as to the best form of surgical treatment. This paper reviews these controversies.

Cite this article: Bone Joint J 2013;95-B:1027–34.

Congenital unilateral anterolateral tibial bowing in combination with a bifid ipsilateral great toe is a very rare deformity which resembles the anterolateral tibial bowing that occurs in association with congenital pseudarthrosis of the tibia. However, spontaneous resolution of the deformity without operative treatment and with a continuously straight fibula has been described in all previously reported cases. We report three additional cases and discuss the options for treatment. We suggest that this is a specific entity within the field of anterolateral bowing of the tibia and conclude that it has a much better prognosis than congenital pseudarthrosis of the tibia, although conservative treatment alone may not be sufficient.

Osteofibrous dysplasia is an unusual developmental condition of childhood, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial and some authors believe that they are part of one histological process.

We retrospectively reviewed 16 patients who were diagnosed as having osteofibrous dysplasia initially or on the final histological examination. Their management was diverse, depending on the severity of symptoms and the extent of the lesion. Definitive (extraperiosteal) surgery was localised ‘shark-bite’ excision for small lesions in five patients. Extensive lesions were treated by segmental excision and fibular autograft in six patients, external fixation and bone transport in four and proximal tibial replacement in one. One patient who had a fibular autograft required further excision and bone transport for recurrence. Six initially underwent curettage and all had recurrence. There were no recurrences after localised extraperiosteal excision or bone transport. There were three confirmed cases of adamantinoma.

The relevant literature is reviewed. We recommend extraperiosteal excision in all cases of osteofibrous dysplasia, with segmental excision and reconstruction in more extensive lesions.

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.

We present the results of treatment of developmental dysplasia of the hip in infancy with the Pavlik harness using a United Kingdom screening programme with ultrasound-guided supervision. Initially, 128 consecutive hips in 77 patients were reviewed over a 40-month period; 123 of these were finally included in the study. The mean age of the patients at the start of treatment was five weeks (1 to 12). All hips were examined clinically and monitored with ultrasound scanning. Failure of treatment was defined as an inability to maintain reduction with the harness. All hips diagnosed with dysplasia or subluxation but not dislocation were managed successfully in the harness. There were 43 dislocated hips, of which 39 were reducible, but six failed treatment in the harness. There were four dislocated but irreducible hips which all failed treatment in the harness. One hip appeared to be successfully treated in the harness but showed persistent radiological dysplasia at 12 and 24 months. Grade 1 avascular necrosis was identified radiologically in three patients at 12 months.

We prospectively studied the benefits and risks of prolonged treatment with the Pavlik harness in infants with idiopathic developmental dysplasia of the hip. Bracing was continued as long as abduction improved. It was started at a mean age of four months (1 to 6.9). Outcome measures were the number of successful reductions, the time to reduction, the acetabular index and evidence of avascular necrosis at follow-up at one year.

In 50 infants with 62 subluxated and dislocated hips (42 Graf type III and 20 type IV), 37 were reduced successfully with a mean time to reduction of 13.4 weeks (sd 6.8). Bracing was successful in 31 type-III (73.8%) and in only six type-IV hips (20%, p = 0.002). Avascular necrosis was seen in ten hips.

Prolonged treatment with the Pavlik harness for developmental dysplasia of the hip over the age of one month can be beneficial in type-III hips, but it is unclear as to whether this is the optimal treatment, since it may postpone the need for closed or open reduction to a more unfavourable age. The use of the Pavlik harness in type-IV hips in this age group is questionable, but if used, prolonged bracing is not advised.

Extensive limb lengthening may be indicated in achondroplastic patients who wish to achieve a height within the normal range for their population. However, increasing the magnitude of lengthening is associated with further complications particularly adjacent joint stiffness and fractures. We studied the relationship between the magnitude of femoral lengthening and callus pattern, adjacent joint stiffness and fracture of the regenerate bone in 40 femoral lengthenings in 20 achondroplastic patients. They were divided into two groups; group A had lengthening of less than 50% and group B of more than 50% of their initial femoral length. The patterns of radiological callus formation were classified according to shape, type and features. The incidence of callus features, knee stiffness and regenerate bone fracture were analysed in the two groups. Group B was associated with an increased incidence of concave, lateral and central callus shapes, adjacent joint and stiffness and fracture. Statistically, the incidence of stiffness in adjacent joints and regenerate bone fracture was significantly associated with the magnitude of lengthening.

We suggest that careful radiological assessment of the patterns of callus formation is a useful method for the evaluation and monitoring of regenerate bone.

Type BI rotationplasty is currently indicated for children with tumours of the proximal femur whereas type BIIIa rotationplasty is reserved for those in which the entire femur has to be removed. Our aim was to compare these two types of rotationplasty and determine whether the knee should be preserved in children with tumours of the proximal femur. We compared the post-operative complications, oncological outcome, range of movement, Enneking score and radiographs of six children, who had undergone type BI rotationplasty with those of 12 who had undergone type BIIIa rotationplasty.

Patients with type BI rotationplasty had a mean Enneking score of 21.6 compared with 24.4 in those with type BIIIa rotationplasty, and worse mean results in all of the parameters investigated. We conclude that type BI rotationplasty has a worse functional outcome and more complications than type BIIIa rotationplasty in children under the age of ten years.