Introduction

The effect of lower extremity lengthening on physeal growth is still controversial. We aim to compare data of the patients who had bilateral simultaneous femur and tibia lengthening with the patients who underwent the lengthening surgery separately for the femur and tibia at the end of completed skeletal maturation in terms of the effect of physeal growth.

Introduction

This study aims to evaluate the effect of using different types of fixator on the quality of callus and complications during distraction osteogenesis in patients with achondroplasia.

A retrospective study was performed in 18 patients with achondroplasia, who underwent bilateral humeral lengthening between 2001 and 2013, using monorail external fixators. The mean age was ten years (six to 15) and the mean follow-up was 40 months (12 to 104).

The mean disabilities of the arm, shoulder and hand (DASH) score fell from 32.3 (20 to 40) pre-operatively to 9.4 (6 to 14) post-operatively (p = 0.037). A mean lengthening of 60% (40% to 95%) was required to reach the goal of independent perineal hygiene. One patient developed early consolidation, and fractures occurred in the regenerate bone of four humeri in three patients. There were three transient radial nerve palsies.

Humeral lengthening increases the independence of people with achondroplasia and is not just a cosmetic procedure.

Cite this article: Bone Joint J 2015;97-B:1577–81.

We present the results of the surgical correction of lower-limb deformities caused by metabolic bone disease. Our series consisted of 17 patients with a diagnosis of hypophosphataemic rickets and two with renal osteodystrophy; their mean age was 25.6 years (14 to 57). In all, 43 lower-limb segments (27 femora and 16 tibiae) were osteotomised and the deformity corrected using a monolateral external fixator. The segment was then stabilised with locked intramedullary nailing. In addition, six femora in three patients were subsequently lengthened by distraction osteogenesis. The mean follow-up was 60 months (18 to 120). The frontal alignment parameters (the mechanical axis deviation, the lateral distal femoral angle and the medial proximal tibial angle) and the sagittal alignment parameters (the posterior distal femoral angle and the posterior proximal tibial angle) improved post-operatively. The external fixator was removed either at the end of surgery or at the end of the lengthening period, allowing for early mobilisation and weight-bearing. We encountered five problems and four obstacles in the programme of treatment.

The use of intramedullary nails prevented recurrence of deformity and refracture.

The nonrhabdomyosarcoma soft tissue sarcomas constitute a heterogenous group of rare mesenchymal tumors that account for less than %5 of pediatric cancers. Their biology and optimal treatment is not well understood. This study retrospectively analyses a small subset of surgically treated patients.

Fifteen patients with a mean age of 11.4 years (4 months – 16 years) were followed-up for a mean of 48 (2–124) months. The histologic diagnosis was synovial sarcoma in 5 patients; soft tissue Ewing Sarcoma in 3; fusiform cell sarcoma in 3; malignant peripheral nerve sheath tumor in 2; fibromyxoid sarcoma in 1 and myxoid liposarcoma in 1. The tumor was located in the upper extremity in 6 patients; thigh in 4; inguinal region in 2, foot in 2 and gluteal region in 1. Eight patients received preoperative chemotherapy and 5 received preoperative radiotherapy. Two patients had pulmonary metastasis at the time of admission. Thirteen patients were operated by limb salvage procedures and 2 underwent amputation.

Tumor resection was radical in 2 patients, wide in 12 and marginal in 1. Five patients received adjuvant chemotherapy and 5 received adjuvant radiotherapy. There were 5 local recurrences after 23.8 (14–40) months; three patients underwent wide resection for recurrence and two received chemotherapy and radiotherapy. Three patients had systemic metastases after a mean of 32.3 (27–40) months. There were no major complications but local wound problems were encountered. Three patients died of disease after a mean of 65.3 months (32–124 months). Two patients had metastatic disease and 10 had no evidence of disease in the last follow-up visit.

Pediatric soft tissue sarcomas can be treated following the principles of adult soft tissue sarcomas, except for wide utilization of radiotherapy in neoadjuvant and adjuvant setting. Treatment results are similar to adult patients.

Aneurysmal bone cysts are benign lesions of bone that tend to recur if they display an aggressive behaviour. In this study, an aggressive lesion is described as one with almost completely lost cortices on standard x-rays, mimicking ‘vanishing bone disease’. Purpose of this study is to retrospectively analyse the treatment results in this special patient subgroup.

Sixteen patients with a mean age of 23.1 (6–44) were included in this study. Femur (%25) was the most commonly affected bone. Spinal lesions were excluded. All lesions were diagnosed by preoperative tru-cut biopsy, however open biopsy was also done if requested by the pathologist. All lesions were preoperatively examined by contrast enhanced MRI. They were evaluated as having almost no cortical bone rim but a periosteum like soft tissue envelope. Intraoperatively, following an extended curettage, this tissue was observed as an alive periosteal layer. Phenolisation was added and cavities were filled with allograft bone chips. The periosteum was sutured around the grafts. Additional stabilisation was performed by external fixator in 3 patients, dynamic compression hip screw in 1.

Patients were followed up for a mean period of 47.8 months (9–84). Lesions healed after a mean period of 28.2 weeks (6–126). There was recurrence in 5 patients. The number of additional procedures necessary for relapsed lesions was 3 for one patient, 2 for another patient and 1 for the remaining 2 patients. Time to healing was not included for 1 patient who refused surgery for relapse and another patient who recently underwent surgery for relapse. One patient healed with a deformity.

Aneurysmal bone cysts may present as highly aggressive local bone lesions. Even in such a subgroup, resection to prevent relapses seems an exaggerated procedure. Extended curretage and packing may yield satisfactory results, with acceptable recurrence rates.

In an era where the survival rates of oncologic patients are improving, biologic reconstruction is the treatment of choice, however, it has its complications and fortunately we have the solutions.

Biological reconstruction was performed on 52 patients with a mean age of 11.3 (1.5–16) after malignant bone tumor resection in our institution between 1991 and 2008. Patients were followed up for a mean period of 49 months (3–216). Twenty-nine patients were diagnosed with osteosarcoma, 22 with Ewing sarcoma and 1 with adamantinoma. A wide range of vascular and nonvascular autografts, allografts, fibular transposition, bone regeneration and bone recycling techniques were utilised alone or in combination for reconstruction. Crucial anatomical parts (epiphyses, apophyses, triradiate cartilage, glenoid) were preserved in 41 patients while maintaining safe surgical margins.

Wound problem was the most common early complication. The most common late complications were nonunion, limb length discrepancy, limitation of range of motion (ROM), deformity, implant or external fixator failure and fibular graft fracture. Local recurrence was seen in only 2 patients. Patients underwent a mean of 0.8 (1–10) additional surgical interventions for treatment of complications. Thirty-one out of 43 lower extremity patients became ambulatory with full weight bearing and near full ROM while 4 died of disease and 2 were disarticulated prior to healing or treating of complications. Six patients with reconstructions around the glenohumeral joint had functional outcomes varying from excellent to poor with defect pseudoarthrosis. Two patients with successful pelvic reconstructions suffered from co-morbidities and disease itself. Implants and fixators, orthoses, physiotherapy and antibiotherapy were used as necessary for treatment of complications.

Biologic reconstruction yields good functional outcomes and allows more flexibility while treating complications since it preserves bone stock and epiphyses.

Autografts produced by recycling of tumor-bearing bone have been used for bridging intercalary bone defects but they are known to act as massive allografts after recycling procedures due to devitalisation. Recycled bone is superior to massive allografts since it allows anatomical reconstruction. Vascularised fibular grafts are inserted into recycled bone segments to provide biological support and to promote healing.

Twelve patients with a mean age of 13.3 years (6–31), who had undergone curative resection of malignant bone tumor followed by biological reconstruction comprised of recycled bone combined with vascularised fibula, were followed up for a mean period of 16.8 (6–46). The tumor was located in distal femur in 7 patients, proximal femur in 2, proximal tibia in 2 and mid-diaphyseal tibia in 1. Cryopreservation with liquid nitrogen was employed for all patients. Contralateral single strut vascularised fibular grafts were used in all except one patient for whom bilateral fibula grafts were harvested to span a longer defect. Plates were used for fixation in 11 patients, and intramedullary nailing in one case. Mean length of bony defect was 16.1 cm (9.0–25.0). Mean fibular graft length was 17.5 cm (10.0–23.0 cm).

Complete union and full weight bearing was achieved in 6 patients, and mean time to detect the commencement of union was 6 months (4–8). Incomplete union was detected in 4 patients and no union in 2. Five patients were complicated by implant failure, 1 with deep infection and 1 with drop foot.

In order to fill large defects after resection of bone tumors, recyled bone combined with vascularised fibular graft is an effective reconstructive tool. Union rate of this technique is quite satisfactory with good functional results.

Background: The occurrence of congenitally short metatarsals is associated with an abnormal gait and an aesthetically displeasing appearance. Similarly, short metacarpals result in severe cosmetic disfigurement, particularly in young female patients.

Methods: We examined and performed bone lengthening surgery in 13 female and 2 male patients. Of these, procedures were conducted on 12 metatarsals of 8 patients, 4 metacarpals and 1 metatarsal of a single patient, 1 metacarpal and 1 metatarsal of a single patient and 7 metacarpals of 5 patients. The mean age of the patients who underwent metacarpal procedures was 14.5 (10–21) years while the mean age of those who underwent metatarsal procedures was 17.5 (10–25) years. The callotasis method was employed for these procedures and we used either a unilateral external fixator and/or a circular external fixator.

Results: The mean healing index and increase in metacarpal length was 1.6 (1.1–2.3) months/cm and 17.6 (13–26) mm, respectively. The mean follow-up period for patients who underwent metacarpal lengthening was 57.5 (12–96) months. The mean healing index and increase in metatarsal length was 1.6 (1.0–2.0) months/cm and 24.3 (20–30) mm respectively. The mean follow-up period for patients who underwent metatarsal lengthening was 48.3 (12–72) months. The preoperative AOFAS (American Orthopaedic Foot and Ankle Society) scores were good in 5 and excellent in 9 cases. The functional scores of metatarso-phalangial (MTP) joint of lengthened metatarsals for the lesser toe were excellent in 12 and good in 2 cases based on the AOFAS scoring system. All patients who underwent metacarpal lengthening reported that they were satisfied and could conduct their daily activities with good functional and aesthetic results. Complications included 4 angulations, 1 subluxation and 1 non-union and were seen in 6 of the metatarsal lengthening cases that exceeded 40% (or > 20 mm) of the total length of the original bone.

Interpretation: There are recommendations in the literature that allow for the avoidance of severe complications and for the shortening of the consolidation period. We conclude that the periosteum must be protected with percutaneus osteotomy and lengthening should be performed at a rate of 0.25 mm twice a day, should not exceeding 40% of the original bone length (or > 20 mm). If the anticipated lengthening exceeds these predefined values then we suggest that the procedure should be performed using a circular external fixator with temporary fixation of the MCP or the MTP joint and the inclusion of the proximal phalanx in the frame.

We report the results of using a combination of fixator-assisted nailing with lengthening over an intramedullary nail in patients with tibial deformity and shortening. Between 1997 and 2007, 13 tibiae in nine patients with a mean age of 25.4 years (17 to 34) were treated with a unilateral external fixator for acute correction of deformity, followed by lengthening over an intramedullary nail with a circular external fixator applied at the same operating session. At the end of the distraction period locking screws were inserted through the intramedullary nail and the external fixator was removed.

The mean amount of lengthening was 5.9 cm (2 to 8). The mean time of external fixation was 90 days (38 to 265). The mean external fixation index was 15.8 days/cm (8.9 to 33.1) and the mean bone healing index was 38 days/cm (30 to 60).

One patient developed an equinus deformity which responded to stretching and bracing. Another developed a drop foot due to a compartment syndrome, which was treated by fasciotomy. It recovered in three months. Two patients required bone grafting for poor callus formation.

We conclude that the combination of fixator-assisted nailing with lengthening over an intramedullary nail can reduce the overall external fixation time and prevent fractures and deformity of the regenerated bone.

Callus distraction over an intramedullary nail is a rarely used technique for the reconstruction of intercalary defects of the femur and tibia after radical debridement of chronic osteomyelitic foci.

The aim of this study was to summarize our experience of distraction osteogenesis with an external fixator combined with an intramedullary nail for the treatment of bone defects and limb shortening produced as a result of radical debridement of chronic osteomyelitis.

Sixteen patients aged 16 to 63 years underwent radical debridement to treat nonunion associated with chronic osteomyelitis of 8 tibias and 8 femurs. The lesions were staged as Cierny and Mader type IVA (10) and IVB (6). The resulting segmental defects and any limb length discrepancy were then reconstructed by distraction osteogenesis over an intramedullary nail. A monolateral frame was used for the femur, and a ring type external fixator for the tibia. Two patients required local gastrocnemius flaps. Free non-vascularised fibula grafts were added to the regenerate for augmentation of a femoral defect at the time of external fixator removal and locking of the nail. At the latest follow-up, functional and radiographic results were evaluated using the Paley’s criteria.

In the femur, the mean defect was 10 centimeters (range 6 to 13 centimeters), while in the tibia it was 8.4 centimeters (range 5 to 11 centimeters). The mean external fixator index was 13.5 days per centimeter, the consolidation index was 36 days per centimeter and the mean time to union at the docking site was 9 (range 5–16) months. The average follow-up was 31.3 months. We obtained 81.25% (13 of 16) excellent results in terms of both bone and functional assessment. There were two recurrences of infection necessitating nail removal. These patients underwent revision with an Ilizarov fixator. Subsequently, infection was controlled and the non-unions healed.

This combined method may prove to be an improvement on the classic techniques for the treatment of long bone nonunions associated with chronic osteomyelitis, in terms of external fixation period and consolidation index. These appears to be no increase in the risk of complications, and the earlier removal of the external fixator is associated with patient comfort, decreases the complication rate and facilitates convenient and quick rehabilitation.

Infection of a megaprosthesis implanted following tumor resection leads to major morbidity and sometimes amputation. Their treatment is like treatment of infected total knee or hip prosthesis, except there is a dramatically larger dead or infected tissue, and immune deficiency due to oncologic treatments. Two-stage revision of infected megaprosthesis seems to be the safest solution except amputation.

Between 1990–2005, we have implanted 282 megaprosthesis in the upper and lower extremity following tumor resection. Sixteen prostheses became infected after a median of 48 months (2–96) following the index intervention. All of them underwent two-stage revision. The infections were staged after Mc Pherson classification, revealing that 75% of them were ‘stage IIIB’. The first stage included debridement, insertion of culture specific antibiotic-loaded bone cement in the form of beads and/or rods, temporary fixation with a custom made IM nail or self-designed, mobile hinged-joint prosthesis covered with antibiotic- loaded PMMA. The most common grown microorganism was MRSA. Following parenteral antibiotherapy, the second staging was performed after a median of 6 weeks (5–11). The reconstruction stage included reimplantation of a cemented prosthesis in 5 patients, a cemented prosthesis in 6 patients, arthrodesis through segment transfer with an external fixator in 4 patients. Eight patients necessitated a local or a distant flap for soft tissue coverage. A patient with recurrent deep infection was amputated.

Patients were followed up for a median of 86 months (24–146). Infection was controlled in 15 patients, with an overall success rate of 94%. The mean functional outcome for the retained limb using the MSTS score was 70%.

Two-stage revision in infected mega prosthesis yields results close to conventional joint replacements, if general guidelines are followed and good soft tissue coverage is provided.

In this retrospective study we evaluated the method of acute shortening and distraction osteogenesis for the treatment of tibial nonunion with bone loss in 17 patients with a mean age of 36 years (10 to 58). The mean bone loss was 5.6 cm (3 to 10). In infected cases, we performed the treatment in two stages. The mean follow-up time was 43.5 months (24 to 96). The mean time in external fixation was 8.0 months (4 to 13) and the mean external fixator index was 1.4 months/cm (1.1 to 1.8). There was no recurrence of infection. The bone evaluation results were excellent in 16 patients and good in one, while functional results were excellent in 15 and good in two. The complication rate was 1.2 per patient.

We conclude that acute shortening and distraction osteogenesis is a safe, reliable and successful method for the treatment of tibial nonunion with bone loss, with a shorter period of treatment and lower rate of complication.

We report two cases with windswept deformities of the lower extremities. All deformities were corrected by fixator-assisted intramedullary nailing. At the latest follow-up, the patients had normal alignment, without symptoms and no loss of correction.