Aims. Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD. Methods. A retrospective review at three paediatric tertiary centres identified 101 cases of tibial OFD in 99 patients. The clinical records, radiological images, and histology were analyzed. Results. Mean age at presentation was 13.5 years (SD 12.4), and mean follow-up was 5.65 years (SD 5.51). At latest review, 62 lesions (61.4%) were in skeletally mature patients. The most common site of the tibial lesion was the anterior (76 lesions, 75.2%) cortex (63 lesions, 62.4%) of the middle third (52 lesions, 51.5%). Pain, swelling, and fracture were common presentations. Overall, 41 lesions (40.6%) presented with radiological deformity (> 10°): apex anterior in 97.6%. A total of 41 lesions (40.6%) were treated conservatively. Anterior bowing < 10° at presentation was found to be related to successful conservative management of OFD (p = 0.013, multivariable logistic regression). Intralesional excision was performed in 43 lesions (42.6%) and a wide excision of the lesion in 19 (18.8%). A high complication rate and surgical burden was found in those that underwent a wide excision regardless of technique employed. There was progression/recurrence in nine lesions (8.9%) but statistical analysis found no predictive factors. No OFD lesion transformed to adamantinoma. Conclusion. This study confirms OFD to be a benign bone condition with low rates of local progression and without malignant transformation. It is important to distinguish OFD from adamantinoma by a histological diagnosis. Focus should be on angular deformity, monitored with full-length tibial radiographs. Surgery is indicated in symptomatic patients and predicted by the severity of the initial angular deformity. Surgery should focus more on the deformity rather than the lesion. Cite this article: Bone Joint J 2022;104-B(2):302–308

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

Aims. Congenital pseudarthrosis of the tibia (CPT) has traditionally been a difficult condition to treat, with high complication rates, including nonunion, refractures, malalignment, and leg length discrepancy. Surgical approaches to treatment of CPT include intramedullary rodding, external fixation, combined intramedullary rodding and external fixation, vascularized fibular graft, and most recently cross-union. The current study aims to compare the outcomes and complication rates of cross-union versus other surgical approaches as an index surgery for the management of CPT. Our hypothesis was that a good index surgery for CPT achieves union and minimizes complications such as refractures and limb length discrepancy. Methods. A multicentre study was conducted involving two institutions in Singapore and China. All patients with CPT who were surgically managed between January 2009 and December 2021 were included. The patients were divided based on their index surgery. Group 1 included patients who underwent excision of hamartoma, cross-union of the tibia and fibula, autogenic iliac bone grafting, and internal fixation for their index surgery. Group 2 included patients who underwent all other surgical procedures for their index surgery, including excision of hamartoma, intramedullary rodding, and/or external fixation, without cross-union of the tibia and fibula. Comparisons of the rates of union, refracture, limb length discrepancy, reoperations, and other complications were performed between the two groups. Results. A total of 36 patients were included in the study. Group 1 comprised 13 patients, while Group 2 comprised 23 patients. The mean age at index surgery was four years (1 to 13). The mean duration of follow-up was 4.85 years (1.75 to 14). All patients in Group 1 achieved bony union at a mean of three months (1.5 to 4), but ten of 23 patients in Group 2 had nonunion of the pseudarthrosis (p = 0.006). None of the patients in Group 1 had a refracture, while seven of 13 patients who achieved bony union in Group 2 suffered a refracture (p = 0.005). None of the patients in Group 1 had a limb length discrepancy of more than 2 cm, while ten of 23 patients in Group 2 have a limb length discrepancy of more than 2 cm (p = 0.006). In Group 1, four of 13 patients had a complication, while 16 of 23 patients in Group 2 had a complication (p = 0.004). Excluding removal of implants, four of 13 patients in Group 1 had to undergo additional surgery, while 18 of 23 patients in Group 2 had to undergo additional surgery following the index surgery (p = 0.011). Conclusion. A good index surgery of excision of hamartoma, cross-union of the tibia and fibula, autogenic iliac bone grafting, and internal fixation for CPT achieves union and minimizes complications such as refractures, limb length discrepancy, and need for additional surgeries

Aims. Paediatric triplane fractures and adult trimalleolar ankle fractures both arise from a supination external rotation injury. By relating the experience of adult to paediatric fractures, clarification has been sought on the sequence of injury, ligament involvement, and fracture pattern of triplane fractures. This study explores the similarities between triplane and trimalleolar fractures for each stage of the Lauge-Hansen classification, with the aim of aiding reduction and fixation techniques. Methods. Imaging data of 83 paediatric patients with triplane fractures and 100 adult patients with trimalleolar fractures were collected, and their fracture morphology was compared using fracture maps. Visual fracture maps were assessed, classified, and compared with each other, to establish the progression of injury according to the Lauge-Hansen classification. Results. Four stages of injury in triplane fractures, resembling the adult supination external rotation Lauge-Hansen stages, were observed. Stage I consists of rupture of the anterior syndesmosis or small avulsion of the anterolateral tibia in trimalleolar fractures, and the avulsion of a larger Tillaux fragment in triplanes. Stage II is defined as oblique fracturing of the fibula at the level of the syndesmosis, present in all trimalleolar fractures and in 30% (25/83) of triplane fractures. Stage III is the fracturing of the posterior malleolus. In trimalleolar fractures, the different Haraguchi types can be discerned. In triplane fractures, the delineation of the posterior fragment has a wave-like shape, which is part of the characteristic Y-pattern of triplane fractures, originating from the Tillaux fragment. Stage IV represents a fracture of the medial malleolus, which is highly variable in both the trimalleolar and triplane fractures. Conclusion. The paediatric triplane and adult trimalleolar fractures share common features according to the Lauge-Hansen classification. This highlights that the adolescent injury arises from a combination of ligament traction and a growth plate in the process of closing. With this knowledge, a specific sequence of reduction and optimal screw positions are recommended. Cite this article: Bone Joint J 2024;106-B(9):1008–1014

Aims. Eight-plates are used to correct varus-valgus deformity (VVD) or limb-length discrepancy (LLD) in children and adolescents. It was reported that these implants might create a bony deformity within the knee joint by change of the roof angle (RA) after epiphysiodesis of the proximal tibia following a radiological assessment limited to anteroposterior (AP) radiographs. The aim of this study was to analyze the RA, complemented with lateral knee radiographs, with focus on the tibial slope (TS) and the degree of deformity correction. Methods. A retrospective, single-centre study was conducted. The treatment group (n = 64 knees in 44 patients) was subclassified according to the implant location in two groups: 1) medial hemiepiphysiodesis; and 2) lateral hemiepiphysiodesis. A third control group consisted of 25 untreated knees. The limb axes and RA were measured on long standing AP leg radiographs. Lateral radiographs of 40 knees were available for TS analysis. The mean age of the patients was 10.6 years (4 to 15) in the treatment group and 8.4 years (4 to 14) in the control group. Implants were removed after a mean 1.2 years (0.5 to 3). Results. No significant differences in RA (p = 0.174) and TS (p = 0.787) were observed. The limb axes were significantly corrected in patients with VVD (p < 0.001). The change in tibial slope (∆TS) did not correlate (r = -0.026; p = 0.885) to the plate’s position on the physis when assessed by lateral radiographs. Conclusion. We were not able to confirm the reported change in the bony morphology of the proximal tibia on AP radiographs in our patient population. In addition, no significant change in TS was detected on the lateral radiographs. A significant correction of the VVD in the lower limb axes was evident. Position of the implant did not correlate with TS change. Therefore, eight-plate epiphysiodesis is a safe and effective procedure for correcting VVD in children without disturbing the knee joint morphology. Cite this article: Bone Joint J 2020;102-B(10):1412–1418

Aims. Following the introduction of national standards in 2009, most major paediatric trauma is now triaged to specialist units offering combined orthopaedic and plastic surgical expertise. We investigated the management of open tibia fractures at a paediatric trauma centre, primarily reporting the risk of infection and rate of union. Patients and Methods. A retrospective review was performed on 61 children who between 2007 and 2015 presented with an open tibia fracture. Their mean age was nine years (2 to 16) and the median follow-up was ten months (interquartile range 5 to 18). Management involved IV antibiotics, early debridement and combined treatment of the skeletal and soft-tissue injuries in line with standards proposed by the British Orthopaedic Association. Results. There were 36 diaphyseal fractures and 25 distal tibial fractures. Of the distal fractures, eight involved the physis. Motor vehicle collisions accounted for two thirds of the injuries and 38 patients (62%) arrived outside of normal working hours. The initial method of stabilisation comprised: casting in nine cases (15%); elastic nailing in 19 (31%); Kirschner (K)-wiring in 13 (21%); intramedullary nailing in one (2%); open reduction and plate fixation in four (7%); and external fixation in 15 (25%). Wound management comprised: primary wound closure in 24 (39%), delayed primary closure in 11 (18%), split skin graft (SSG) in eight (13%), local flap with SSG in 17 (28%) and a free flap in one. A total of 43 fractures (70%) were Gustilo-Anderson grade III. There were four superficial (6.6%) and three (4.9%) deep infections. Two deep infections occurred following open reduction and plate fixation and the third after K-wire fixation of a distal fracture. No patient who underwent primary wound closure developed an infection. All the fractures united, although nine patients required revision of a mono-lateral to circular frame for delayed union (two) or for altered alignment or length (seven). The mean time to union was two weeks longer in diaphyseal fractures than in distal fractures (13 weeks versus 10.8 weeks, p = 0.016). Children aged > 12 years had a significantly longer time to union than those aged < 12 years (16.3 weeks versus 11.4 weeks, p = 0.045). The length of stay in hospital for patients with a Gustilo-Anderson grade IIIB fracture was twice as long as for less severe injuries. . Conclusion. Fractures in children heal better than those in adults. Based on our experience of deep infection we discourage the use of internal fixation with a plate for open tibial fractures in children. We advocate aggressive initial wound debridement in theatre with early definitive combined orthopaedic and plastic surgery in order to obtain skeletal stabilisation and soft-tissue cover. Cite this article: Bone Joint J 2017;99-B:544–53

Flexible intramedullary nailing is gaining popularity as an effective method of treating long-bone fractures in children. We retrospectively reviewed the records and radiographs of 56 unstable fractures of the tibia in 54 children treated between March 1997 and May 2005. All were followed up for at least two months after the removal of the nails. Of the 56 tibial fractures, 13 were open. There were no nonunions. The mean time to clinical and radiological union was ten weeks. Complications included residual angulation of the tibia, leg-length discrepancy, deep infection and failures of fixation. All achieved an excellent functional outcome. We conclude that flexible intramedullary fixation is an easy and effective method of management of both open and closed unstable fractures of the tibia in children

Morbid obesity and its association with obstructive sleep apnoea syndrome have been increasingly recognised in children. Orthopaedic surgeons are often the primary medical contact for older children with tibia vara, which has long been associated with obesity, but are unfamiliar with the evaluation and treatment of sleep apnoea in children. We reviewed all children with tibia vara treated surgically at one of our institutions over a period of five years. Thirty-seven patients were identified; 18 were nine years of age or older and 13 of these (72%) had morbid obesity and a history of snoring. Eleven children were diagnosed as having sleep apnoea on polysomnography. The incidence of this syndrome in the 18 children aged nine years or older with tibia vara, was 61%. All these patients required pre-operative non-invasive positive-pressure ventilation; tonsillectomy and adenoidectomy were necessary in five (45%). No peri-operative complications related to the airway occurred. There is a high incidence of sleep apnoea in morbidly obese patients with tibia vara. These patients should be screened for snoring and, if present, should be further evaluated for sleep apnoea before corrective surgery is undertaken

The use of recombinant human bone morphogenetic protein-2 (rhBMP-2) for the treatment of congenital pseudarthrosis of the tibia has been investigated in only one previous study, with promising results. The aim of this study was to determine whether rhBMP-2 might improve the outcome of this disorder. We reviewed the medical records of five patients with a mean age of 7.4 years (2.3 to 21) with congenital pseudarthrosis of the tibia who had been treated with rhBMP-2 and intramedullary rodding. Ilizarov external fixation was also used in four of these patients. Radiological union of the pseudarthrosis was evident in all of them at a mean of 3.5 months (3.2 to 4) post-operatively. The Ilizarov device was removed after a mean of 4.2 months (3.0 to 5.3). These results indicate that treatment of congenital pseudarthrosis of the tibia using rhBMP-2 in combination with intramedullary stabilisation and Ilizarov external fixation may improve the initial rate of union and reduce the time to union. Further studies with more patients and longer follow-up are necessary to determine whether this surgial procedure may significantly enhance the outcome of congenital pseudarthrosis of the tibia, considering the refracture rate (two of five patients) in this small case series

Purpose of the Study. A cooled, side cutting burr designed for use in adult foot surgery has been used as a primary bone cutting device in children to facilitate a truly percutaneous method of performing osteotomies. Stabilisation of the femur was using a percutaneous locked nail and for the tibia percutaneous K-wires. The author describes the advantages and disadvantages of this method with results from the first cohort of patients treated. Method. Patients under going osteotomy of the femur, tibia and fibula using a 2 mm × 20 mm side cutting burr were followed prospectively and assessed for scar size, bone healing time and complications. Results. Thirty six osteotomies were performed in the femur and tibia in 25 patients. A fibula osteotomy was always performed with a tibial osteotomy. Scar size for the femoral osteotomy was <15 mm and for the nail insertion <25 mm. For the tibia & fibula the scar size was <10 mm. Healing time was by 6 weeks in the tibia and in the femur was within 16 weeks in non-lengthening cases in all but 1 case of non-union (associated with Vitamin D insufficiency). Three burr bits broke during the learning curve including 2 in tibial osteotomies and 1 in the femur. Cortical thickness and slow burr speeds were associated with burr breakage. All wounds healed without infection. The optimum speed for the burr at 50 Nm of torque was established as 200 rpm in children under the age of 13 yrs. The initial recommended speed of 300 rpm increases healing time when performing osteotomies in children. Conclusion. Truly percutaneous surgery can be performed to osteotomise the femur and tibia using a cooled, side cutting burr with a locked femoral nail and crossed tibial wires with excellent cosmetic results and minimal complications. Level of Evidence IV

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

A percutaneous supramalleolar osteotomy with multiple drill holes and closed osteoclasis was used to correct rotational deformities of the tibia in patients with cerebral palsy. The technique is described and the results in 247 limbs (160 patients) are reported. The mean age at the time of surgery was 10.7 years (4 to 20). The radiographs were analysed for time to union, loss of correction, and angulation at the site of the osteotomy. Bone healing was obtained in all patients except one in a mean period of seven weeks (5 to 12). Malunion after loss of reduction at the site of the osteotomy developed in one tibia. Percutaneous supramalleolar osteotomy of the tibia is a safe and simple surgical procedure

Aims

Open tibial fractures are limb-threatening injuries. While limb loss is rare in children, deep infection and nonunion rates of up to 15% and 8% are reported, respectively. We manage these injuries in a similar manner to those in adults, with a combined orthoplastic approach, often involving the use of vascularised free flaps. We report the orthopaedic and plastic surgical outcomes of a consecutive series of patients over a five-year period, which includes the largest cohort of free flaps for trauma in children to date.

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

Torsional deformities of the tibia are common in children, but in the majority both the torsion and the associated disturbance of gait resolve without intervention. There are, however, a significant number of children and adults with neuromuscular disease who present with pathological tibial torsion, which may require surgical correction. We conducted a prospective study in two centres, to investigate the outcome of supramalleolar derotation osteotomy of the tibia, using internal fixation with the AO-ASIF T plate. A range of outcome variables was collected, prospectively, for 57 patients (91 osteotomies), including thigh foot angle, foot progression angle, post-operative complications and serial radiographs. Correction of thigh foot angle and foot progression angle was satisfactory in all patients. Three major complications were recorded; one aseptic nonunion, one fracture through the osteotomy site after removal of the plate and one distal tibial growth arrest. We found that supramalleolar derotation osteotomy of the tibia, with AO-ASIF T plate fixation is an effective method for the correction of torsional deformities of the tibia and the associated disturbances of gait in children and adults with neuromuscular disease, with a 5.3% risk of major complications

We have observed the natural history without treatment of 46 limbs in 29 patients with infantile tibia vara and a metaphyseal-diaphyseal angle (MDA) of more than 11°. The femorotibial angle (FTA) and MDA were measured, and Langenskiöld’s classification of radiological changes in the proximal medial metaphysis of the tibia was used. In 22 limbs which were not in Langenskiöld stages II to III the condition resolved spontaneously without treatment. Of the remaining 24 which were in stages II to III, in 18 it resolved spontaneously by the age of six years, but six showed little or no improvement at the latest follow-up. It was impossible to differentiate by measuring the FTA or MDA whether spontaneous resolution could be expected before the age of four years. There was no difference in the rate of resolution of the deformity between those patients who had been treated by a brace and those who had received no treatment. We advise no initial treatment but review at six-monthly intervals until the age of four years, even in patients with Langenskiöld stage-II to stage-III deformity. When a deformity persisted or progressed we carried out a corrective osteotomy after the age of four years

We present two children with massive defects of the tibia and an associated active infection who were treated by medial transport of the fibula using the Ilizarov device. The first child had chronic discharging osteomyelitis which affected the whole tibial shaft. The second had sustained bilateral grade-IIIB open tibial fractures in a motor-car accident. The first child was followed up for three years and the second for two years. Both achieved solid union between the proximal and distal stumps of the tibia and the fibula, with hypertrophy of the fibula. The first child had a normal range of movement at the knee, ankle and foot but there was shortening of 1.5 cm. The second had persistent anterior angulation at the proximal tibiofibular junction and the ankle was stiff in equinus

We investigated patterns of refracture and their risk factors in patients with congenital pseudarthrosis of the tibia after Ilizarov osteosynthesis. We studied 43 cases in 23 patients. Temporal and spatial patterns of refracture and refracture-free survival were analysed in each case. The refracture-free rate of cumulative survival was 47% at five years and did not change thereafter. Refracture occurred at the previous pseudarthrosis in 16 of 19 cases of refracture. The risk of refracture was significantly higher when osteosynthesis was performed below the age of four years, when the tibial cross-sectional area was narrow, and when associated with persistent fibular pseudarthrosis. Refracture occurs frequently after successful osteosynthesis in these patients. Delaying osteosynthesis, maximising the tibial cross-sectional area and stabilising the fibula may reduce the risk of refracture

Introduction. We report 3 cases from different centres of infantile tibia vara in which the deformity was due to slippage of the proximal tibial epiphysis on the metaphysis; the aim of this study was to define the features of this previously unreported condition, and their implications for management. Method. Three cases of tibia vara secondary to atraumatic slippage of the upper tibial epiphysis on the metaphysis were identified from three different centres. The case notes and imaging studies were retrospectively reviewed to distinguish common clinical and radiographic features. Results. There were one male and two females, all of non-Caucasian origin, (age 3–7 years). All patients' weights were above the 97th centile for age. In all cases there was an infero-medial subluxation of the tibial epiphysis over a dome shaped proximal tibial metaphysis, with disruption of continuity between their lateral borders. The height of the medial tibial plateau was preserved in all cases. New bone formation suggests this is a chronic process. The evolution of one case indicates that pathogenesis is shared with infantile Blount's disease. A gradual deformity correction was performed in all cases using circular external fixation, with the proximal ring secured to both the proximal epiphysis and metaphysis. Conclusion. Slipped upper tibial epiphysis is an uncommon but distinct cause of tibia vara. The radiological features are completely different from those previously described for infantile tibia vara and not encompassed by the existing classification. The unusual morphology has consequences for treatment. Management is analogous to a slipped upper femoral epiphysis – the physis has to be stabilized to the metaphysis and an osteotomy performed to restore the mechanical axis. We believe this is best achieved with a circular external fixator because this permits multiaxial correction including translation and rotation

PURPOSE. To report the experience and evolution of treatment of Congenital Pseudarthrosis of the Tibia in a single surgeon, consecutive series of 19 patients. METHODS. A retrospective case series review, with radiological assessment and functional outcome scores including the Activity Scale for Kids (ASK) and the Oxford Ankle Foot Questionnaire (OAFQ) Child & Teenager version. Patients were classified as per Boyd & Andersen. Alteration in the Tibial mechanical axis was compared pre-treatment and at last review. RESULTS. The average follow up from presentation was 6.3 years, 7 patients had reached skeletal maturity. Union was obtained in 79% at the time of review with a mean time to union of an established pseudarthrosis of 15.2 months. 9 of 19 had an established diagnosis of Neurofibromatosis. 8 had a frank pseudarthrosis at presentation, 2 of which were present at birth. There were 3 re-fractures following treatment. One of the 19 required amputation after failed surgical treatment. Each patient had an average of 2.4 surgical procedures. 14 patients had either fixed or elongating rods, 10 had Ilizarov treatment. 2 have outstanding surgery planned. CONCLUSIONS. A philosophy of treatment has evolved which produces acceptable results. Splintage is used for antero-lateral bowing with corrective osteotomies and rodding, without extensive bone or periosteal resection, for significant or progressive deformity or fracture. Ilizarov treatment is used for recalcitrant cases over the age of 6 years. The technique has evolved over time to include bone graft wraps and the use of Bone Morphogenic protein in combination with the Ilizarov technique. Patients have below population and age matched average scores for both the ASK and OAFQ