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The Bone & Joint Journal
Vol. 97-B, Issue 11 | Pages 1577 - 1581
1 Nov 2015
Balci HI Kocaoglu M Sen C Eralp L Batibay SG Bilsel K

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


The Bone & Joint Journal
Vol. 102-B, Issue 6 | Pages 755 - 765
1 Jun 2020
Liebs TR Burgard M Kaiser N Slongo T Berger S Ryser B Ziebarth K

Aims. We aimed to evaluate the health-related quality of life (HRQoL) in children with supracondylar humeral fractures (SCHFs), who were treated following the recommendations of the Paediatric Comprehensive AO Classification, and to assess if HRQoL was associated with AO fracture classification, or fixation with a lateral external fixator compared with closed reduction and percutaneous pinning (CRPP). Methods. We were able to follow-up on 775 patients (395 girls, 380 boys) who sustained a SCHF from 2004 to 2017. Patients completed questionnaires including the Quick Disabilities of the Arm, Shoulder and Hand questionnaire (QuickDASH; primary outcome), and the Pediatric Quality of Life Inventory (PedsQL). Results. An AO type I SCHF was most frequent (327 children; type II: 143; type III: 150; type IV: 155 children). All children with type I fractures were treated nonoperatively. Two children with a type II fracture, 136 with a type III fracture, and 141 children with a type IV fracture underwent CRPP. In the remaining 27 children with type III or IV fractures, a lateral external fixator was necessary for closed reduction. There were no open reductions. After a mean follow-up of 6.3 years (SD 3.7), patients with a type I fracture had a mean QuickDASH of 2.0 (SD 5.2), at a scale of 0 to 100, with lower values representing better HRQoL (type II: 2.8 (SD 10.7); type III: 3.3 (SD 8.0); type IV: 1.8 (SD 4.6)). The mean function score of the PedsQL ranged from 97.4 (SD 8.0) for type I to 96.1 (SD 9.1) for type III fractures, at a scale of 0 to 100, with higher values representing better HRQoL. Conclusion. In this cohort of 775 patients in whom nonoperative treatment was chosen for AO type I and II fractures and CRPP or a lateral external fixator was used in AO type III and IV fractures, there was equally excellent mid- and long-term HRQoL when assessed by the QuickDASH and PedsQL. These results indicate that the treatment protocol followed in this study is unambiguous, avoids open reductions, and is associated with excellent treatment outcomes. Cite this article: Bone Joint J 2020;102-B(6):755–765


The Bone & Joint Journal
Vol. 102-B, Issue 9 | Pages 1248 - 1255
1 Sep 2020
Laufer A Frommer A Gosheger G Roedl R Broeking JN Toporowski G Rachbauer AM Vogt B

Aims. The treatment of tibial aplasia is controversial. Amputation represents the gold standard with good functional results, but is frequently refused by the families. In these patients, treatment with reconstructive limb salvage can be considered. Due to the complexity of the deformity, this remains challenging and should be staged. The present study evaluated the role of femoro-pedal distraction using a circular external fixator in reconstructive treatment of tibial aplasia. The purpose of femoro-pedal distraction is to realign the limb and achieve soft tissue lengthening to allow subsequent reconstructive surgery. Methods. This was a retrospective study involving ten patients (12 limbs) with tibial aplasia, who underwent staged reconstruction. During the first operation a circular hexapod external fixator was applied and femoro-pedal distraction was undertaken over several months. Subsequent surgery included reconstruction of the knee joint and alignment of the foot. Results. The mean follow-up was 7.1 years (2 to 10). The mean age of the patients at the time of the application of the fixator was 2.3 years (1.1 to 5.0). The mean time under distraction was 139.7 days (81.0 to 177.0). A mean fibular distalization of 38.7 mm (14.0 to 67.0) was achieved. Pin infections occurred in four limbs (33.3%) and osteitis in one. A femoral fracture occurred in one patient. Premature removal of the frame was not required in any patient. Sufficient realignment of the leg as well as soft tissue lengthening was achieved in all patients, allowing subsequent reconstruction. All patients were able to mobilize fully weight bearing after reconstruction. Functional outcome was limited in all limbs, and five patients (50.0%) required additional reconstructive operations. Conclusion. Regarding the functional results in the treatment of tibial aplasia, amputation remains superior to limb salvage. The latter procedure should only be performed in patients whose parents refuse amputation. Femoro-pedal distraction efficiently prepares the limb by realigning the leg and soft tissue lengthening. Minor complications are frequent, but usually do not hinder the continuation of distraction. Even though a fully weight-bearing limb is achieved, the functional outcome of reconstructive treatment remains limited. Recurrent deformities frequently occur and may require further operations. Cite this article: Bone Joint J 2020;102-B(9):1248–1255


The Journal of Bone & Joint Surgery British Volume
Vol. 89-B, Issue 11 | Pages 1509 - 1514
1 Nov 2007
Zhang X Liu T Li Z Peng W

We present a retrospective study of 27 patients treated by callus distraction using a unilateral external fixator of our own design for nonunion with bone loss and shortening of the femur caused by suppurative osteomyelitis. The unilateral external fixator was used either alone or in combination with an intramedullary nail. The mean age of the patients was 13.6 years (8 to 18). The fixator was used alone in 13 patients and with an intramedullary nail in 14. The bone results at a mean follow-up of 88 months (37 to 144) were excellent in 16 patients and good in 11. The functional results were excellent in 18 patients and good in nine. However, four patients still had draining sinuses at the latest follow-up. A residual deformity greater than 7° was present in seven femora, but this did not adversely affect function or require further treatment


The Journal of Bone & Joint Surgery British Volume
Vol. 90-B, Issue 6 | Pages 795 - 800
1 Jun 2008
Liu T Zhang X Li Z Zeng W Peng D Sun C

Nonunion of the humerus with bone loss and shortening due to osteomyelitis is rare but difficult to treat. We describe our experience with a callus distraction technique using a monolateral external fixator for the treatment of this condition. Between October 1994 and January 2004, 11 patients were treated. There were seven males and four females, with a mean age of 14 years (10 to 17). The mean bone loss was 1.9 cm (1 to 2.7) and the mean length discrepancy in the upper limb was 5.6 cm (3.5 to 8.0). The mean follow-up was for 106 months (54 to 166). The mean external fixation index was 34.8 days/cm (29.8 to 40.5). The mean lengthening was 9.5 cm (5.5 to 13.4). There were seven excellent results, three good and one poor. There were nine excellent functional results and two good. The treatment of humeral nonunion with bone loss and shortening due to osteomyelitis by callus distraction is a safe and effective means of improving function and cosmesis


The Journal of Bone & Joint Surgery British Volume
Vol. 87-B, Issue 4 | Pages 540 - 543
1 Apr 2005
Kazuki K Hiroshima K Kawahara K

Deformity of the forearm due to growth disturbance of the ulna occurs in a number of conditions such as ulnar deficiency, multiple exostoses, and neurofibromatosis. We report a previously unrecognised form, caused by focal cortical indentation. We have treated five children with this condition, three girls and two boys; the mean age at presentation was 5 years (2 to 8). The deformity was first recognised about the age of two years, and progressed gradually. The radiological findings were the same in all cases. The focal cortical indentation was seen at the distal end of the ulna with anteromedial bowing and dysplasia. The radial head was dislocated posterolaterally. In one patient the histological findings at the site of indentation were of a fold of tissue resembling periosteum, which interfered with enchondral ossification. Treatment by ulnar lengthening using an external fixator and osteotomy which corrected both the ulnar deformity and reduced the dislocated radial head in two cases gave the best results


The Journal of Bone & Joint Surgery British Volume
Vol. 86-B, Issue 2 | Pages 259 - 265
1 Mar 2004
Saldanha KAN Saleh M Bell MJ Fernandes JA

We performed limb lengthening and correction of deformity of nine long bones of the lower limb in six children (mean age, 14.7 years) with osteogenesis imperfecta (OI). All had femoral lengthening and three also had ipsilateral tibial lengthening. Angular deformities were corrected simultaneously. Five limb segments were treated using a monolateral external fixator and four with the Ilizarov frame. In three children, lengthening was done over previously inserted femoral intramedullary rods. The mean lengthening achieved was 6.26 cm (mean healing index, 33.25 days/cm). Significant complications included one deep infection, one fracture of the femur and one anterior angulation deformity of the tibia. The abnormal bone of OI tolerated the external fixators throughout the period of lengthening without any episodes of migration of wires or pins through the soft bone. The regenerate bone formed within the time which is normally expected in limb-lengthening procedures performed for other conditions. We conclude that despite the abnormal bone characteristics, distraction osteogenesis to correct limb-length discrepancy and angular deformity can be performed safely in children with OI


The Journal of Bone & Joint Surgery British Volume
Vol. 94-B, Issue 7 | Pages 989 - 993
1 Jul 2012
Monsell FP Howells NR Lawniczak D Jeffcote B Mitchell SR

Between 2005 and 2010 ten consecutive children with high-energy open diaphyseal tibial fractures were treated by early reduction and application of a programmable circular external fixator. They were all male with a mean age of 11.5 years (5.2 to 15.4), and they were followed for a mean of 34.5 months (6 to 77). Full weight-bearing was allowed immediately post-operatively. The mean time from application to removal of the frame was 16 weeks (12 to 21). The mean deformity following removal of the frame was 0.15° (0° to 1.5°) of coronal angulation, 0.2° (0° to 2°) sagittal angulation, 1.1 mm (0 to 10) coronal translation, and 0.5 mm (0 to 2) sagittal translation. All patients achieved consolidated bony union and satisfactory wound healing. There were no cases of delayed or nonunion, compartment syndrome or neurovascular injury. Four patients had a mild superficial pin site infection; all settled with a single course of oral antibiotics. No patient had a deep infection or re-fracture following removal of the frame. The time to union was comparable with, or better than, other published methods of stabilisation for these injuries. The stable fixator configuration not only facilitates management of the accompanying soft-tissue injury but enables anatomical post-injury alignment, which is important in view of the limited remodelling potential of the tibia in children aged > ten years. Where appropriate expertise exists, we recommend this technique for the management of high-energy open tibial fractures in children


Bone & Joint Open
Vol. 5, Issue 7 | Pages 581 - 591
12 Jul 2024
Wang W Xiong Z Huang D Li Y Huang Y Guo Y Andreacchio A Canavese F Chen S

Aims

To investigate the risk factors for unsuccessful radial head reduction (RHR) in children with chronic Monteggia fractures (CMFs) treated surgically.

Methods

A total of 209 children (mean age 6.84 years (SD 2.87)), who underwent surgical treatment for CMFs between March 2015 and March 2023 at six institutions, were retrospectively reviewed. Assessed risk factors included age, sex, laterality, dislocation direction and distance, preoperative proximal radial metaphysis width, time from injury to surgery, reduction method, annular ligament reconstruction, radiocapitellar joint fixation, ulnar osteotomy, site of ulnar osteotomy, preoperative and postoperative ulnar angulation, ulnar fixation method, progressive ulnar distraction, and postoperative cast immobilization. Independent-samples t-test, chi-squared test, and logistic regression analysis were used to identify the risk factors associated with unsuccessful RHR.


The Journal of Bone & Joint Surgery British Volume
Vol. 88-B, Issue 6 | Pages 799 - 806
1 Jun 2006
Jones D Parkinson S Hosalkar HS

We reviewed retrospectively 45 patients (46 procedures) with bladder exstrophy treated by bilateral oblique pelvic osteotomy in conjunction with genitourinary repair. The operative technique and post-operative management with or without external fixation are described. A total of 21 patients attended a special follow-up clinic and 24 were interviewed by telephone. The mean follow-up time was 57 months (24 to 108). Of the 45 patients, 42 reported no pain or functional disability, although six had a waddling gait and two had marked external rotation of the hip. Complications included three cases of infection and loosening of the external fixator requiring early removal with no deleterious effect. Mid-line closure failed in one neonate managed in plaster. This patient underwent a successful revision procedure several months later using repeat osteotomies and external fixation. The percentage pubic approximation was measured on anteroposterior radiographs pre-operatively, post-operatively and at final follow-up. The mean approximation was 37% (12% to 76%). It varied markedly with age and was better when external fixation was used. The wide range reflects the inability of the anterior segment to develop naturally in spite of close approximation at operation. We conclude that bilateral oblique pelvic osteotomy with or without external fixation is useful in the management of difficult primary closure in bladder exstrophy, failed primary closure and secondary reconstruction


The Bone & Joint Journal
Vol. 106-B, Issue 7 | Pages 751 - 758
1 Jul 2024
Yaxier N Zhang Y Song J Ning B

Aims

Given the possible radiation damage and inaccuracy of radiological investigations, particularly in children, ultrasound and superb microvascular imaging (SMI) may offer alternative methods of evaluating new bone formation when limb lengthening is undertaken in paediatric patients. The aim of this study was to assess the use of ultrasound combined with SMI in monitoring new bone formation during limb lengthening in children.

Methods

In this retrospective cohort study, ultrasound and radiograph examinations were performed every two weeks in 30 paediatric patients undergoing limb lengthening. Ultrasound was used to monitor new bone formation. The number of vertical vessels and the blood flow resistance index were compared with those from plain radiographs.


The Bone & Joint Journal
Vol. 104-B, Issue 2 | Pages 302 - 308
1 Feb 2022
Dala-Ali B Donnan L Masterton G Briggs L Kauiers C O’Sullivan M Calder P Eastwood DM

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.


The Bone & Joint Journal
Vol. 106-B, Issue 3 | Pages 277 - 285
1 Mar 2024
Pinto D Hussain S Leo DG Bridgens A Eastwood D Gelfer Y

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.


The Bone & Joint Journal
Vol. 103-B, Issue 6 | Pages 1160 - 1167
1 Jun 2021
Smith JRA Fox CE Wright TC Khan U Clarke AM Monsell FP

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.

Methods

Data were extracted from medical records and databases for patients with an open tibial fracture aged < 16 years who presented between 1 May 2014 and 30 April 2019. Patients who were transferred from elsewhere were excluded, yielding 44 open fractures in 43 patients, with a minimum follow-up of one year. Management was reviewed from the time of injury to discharge. Primary outcome measures were the rate of deep infection, time to union, and the Modified Enneking score.


The Journal of Bone & Joint Surgery British Volume
Vol. 83-B, Issue 4 | Pages 547 - 550
1 May 2001
Exner GU

Two consecutive cases of chronic dislocation of the head of the radius after missed Bado type-I Monteggia lesions are presented. Reduction was successfully achieved in both patients after ulnar corticotomy, gradual lengthening and angulation of the ulna using an external fixator. Open reduction or reconstruction of the radio-ulnar capitellar joint was not undertaken. The age at injury was seven years in the older and two years in the younger patient. The time from injury to treatment was five years in the older and three months in the younger child. At follow-up, nine years after completion of treatment in the older and eight months in the younger patient, both show satisfactory movement, function of the forearm and reduction of the head of the radius. This technique may be considered in missed Monteggia lesions before open procedures on the radio-ulnar capitellar joint are undertaken


The Journal of Bone & Joint Surgery British Volume
Vol. 85-B, Issue 2 | Pages 261 - 264
1 Mar 2003
Hosalkar HS Jones S Chowdhury M Hartley J Hill RA

We review the results of a modified quadricepsplasty in five children who developed stiffness of the knee after femoral lengthening for congenital short femur using an Ilizarov external fixator which spanned the knee. All had a full range of movement of the knee before lengthening was undertaken. Unifocal lengthening was carried out in the distal metaphysiodiaphyseal region of the distal femur with a mean gain of 6.5 cm. The mean percentage lengthening was 24%. At the end of one year after removal of the Ilizarov frame and despite intensive physiotherapy all patients had stiffness. Physiotherapy was continued after the quadricepsplasty and, at the latest follow-up (mean 27 months), the mean active flexion was 102° (80 to 130). The gain in movement ranged from 50° to 100°. One patient had a superficial wound infection which settled after a course of oral antibiotics. None developed an increased extension lag after surgery and all were very satisfied with the results. Quadricepsplasty is a useful procedure for stiffness of the knee after femoral lengthening which has not responded to physiotherapy


The Bone & Joint Journal
Vol. 104-B, Issue 4 | Pages 510 - 518
1 Apr 2022
Perry DC Arch B Appelbe D Francis P Craven J Monsell FP Williamson P Knight M

Aims

The aim of this study was to evaluate the epidemiology and treatment of Perthes’ disease of the hip.

Methods

This was an anonymized comprehensive cohort study of Perthes’ disease, with a nested consented cohort. A total of 143 of 144 hospitals treating children’s hip disease in the UK participated over an 18-month period. Cases were cross-checked using a secondary independent reporting network of trainee surgeons to minimize those missing. Clinician-reported outcomes were collected until two years. Patient-reported outcome measures (PROMs) were collected for a subset of participants.


The Bone & Joint Journal
Vol. 100-B, Issue 1 | Pages 109 - 118
1 Jan 2018
Talbot C Davis N Majid I Young M Bouamra O Lecky FE Jones S

Aims

The aim of this study was to describe the epidemiology of closed isolated fractures of the femoral shaft in children, and to compare the treatment and length of stay (LOS) between major trauma centres (MTCs) and trauma units (TUs) in England.

Patients and Methods

National data were obtained from the Trauma and Audit Research Network for all isolated, closed fractures of the femoral shaft in children from birth to 15 years of age, between 2012 and 2015. Age, gender, the season in which the fracture occurred, non-accidental injury, the mechanism of injury, hospital trauma status, LOS and type of treatment were recorded.


The Bone & Joint Journal
Vol. 100-B, Issue 3 | Pages 396 - 403
1 Mar 2018
Messner J Johnson L Taylor DM Harwood P Britten S Foster P

Aims

The aim of this study was to report the clinical, functional and radiological outcomes of children and adolescents with tibial fractures treated using the Ilizarov method.

Patients and Methods

Between 2013 and 2016 a total of 74 children with 75 tibial fractures underwent treatment at our major trauma centre using an Ilizarov frame. Demographic and clinical information from a prospective database was supplemented by routine functional and psychological assessment and a retrospective review of the notes and radiographs.


The Journal of Bone & Joint Surgery British Volume
Vol. 91-B, Issue 12 | Pages 1612 - 1617
1 Dec 2009
Venkatesh KP Modi HN Devmurari K Yoon JY Anupama BR Song HR

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.


The Bone & Joint Journal
Vol. 99-B, Issue 4 | Pages 544 - 553
1 Apr 2017
Nandra RS Wu F Gaffey A Bache CE

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.


The Bone & Joint Journal
Vol. 96-B, Issue 1 | Pages 137 - 142
1 Jan 2014
Nayagam S Davis B Thevendran G Roche AJ

We describe the technique and results of medial submuscular plating of the femur in paediatric patients and discuss its indications and limitations. Specifically, the technique is used as part of a plate-after-lengthening strategy, where the period of external fixation is reduced and the plate introduced by avoiding direct contact with the lateral entry wounds of the external fixator pins. The technique emphasises that vastus medialis is interposed between the plate and the vascular structures.

A total of 16 patients (11 male and five female, mean age 9.6 years (5 to 17)), had medial submuscular plating of the femur. All underwent distraction osteogenesis of the femur with a mean lengthening of 4.99 cm (3.2 to 12) prior to plating. All patients achieved consolidation of the regenerate without deformity. The mean follow-up was 10.5 months (7 to 15) after plating for those with plates still in situ, and 16.3 months (1 to 39) for those who subsequently had their plates removed. None developed a deep infection. In two patients a proximal screw fractured without loss of alignment; one patient sustained a traumatic fracture six months after removal of the plate.

Placing the plate on the medial side is advantageous when the external fixator is present on the lateral side, and is biomechanically optimal in the presence of a femoral defect. We conclude that medial femoral submuscular plating is a useful technique for specific indications and can be performed safely with a prior understanding of the regional anatomy.

Cite this article: Bone Joint J 2014;96-B:137–42.


The Journal of Bone & Joint Surgery British Volume
Vol. 89-B, Issue 11 | Pages 1515 - 1518
1 Nov 2007
Zhang X Duan L Li Z Chen X

We report our experience of the use of callus distraction with a monolateral fixator for the treatment of acquired radial club-hand deformity after osteomyelitis. Between 1994 and 2004, 13 patients with a mean age of eight years (4 to 15) were treated by callus distraction with a monolateral fixator after a preliminary period of at least four weeks in a corrective short-arm cast.

All patients achieved bony union and were satisfied with the functional and cosmetic outcome. There were no major complications, but three patients required cancellous bone grafting at the docking site for delayed union. Local treatment and oral antibiotics were required for pin-site infection in six patients. There were no deep infection or recurrence of osteomyelitis.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 7 | Pages 984 - 989
1 Jul 2011
Park DH Bradish CF

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.


The Bone & Joint Journal
Vol. 97-B, Issue 3 | Pages 412 - 419
1 Mar 2015
Walton RDM Martin E Wright D Garg NK Perry D Bass A Bruce C

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.


The Bone & Joint Journal
Vol. 96-B, Issue 9 | Pages 1269 - 1273
1 Sep 2014
Kitoh H Mishima K Matsushita M Nishida Y Ishiguro N

Two types of fracture, early and late, have been reported following limb lengthening in patients with achondroplasia (ACH) and hypochondroplasia (HCH).

We reviewed 25 patients with these conditions who underwent 72 segmental limb lengthening procedures involving the femur and/or tibia, between 2003 and 2011. Gender, age at surgery, lengthened segment, body mass index, the shape of the callus, the amount and percentage of lengthening and the healing index were evaluated to determine predictive factors for the occurrence of early (within three weeks after removal of the fixation pins) and late fracture (> three weeks after removal of the pins). The Mann‑Whitney U test and Pearson’s chi-squared test for univariate analysis and stepwise regression model for multivariate analysis were used to identify the predictive factor for each fracture. Only one patient (two tibiae) was excluded from the analysis due to excessively slow formation of the regenerate, which required supplementary measures. A total of 24 patients with 70 limbs were included in the study.

There were 11 early fractures in eight patients. The shape of the callus (lateral or central callus) was the only statistical variable related to the occurrence of early fracture in univariate and multivariate analyses. Late fracture was observed in six limbs and the mean time between removal of the fixation pins and fracture was 18.3 weeks (3.3 to 38.4). Lengthening of the tibia, larger healing index, and lateral or central callus were related to the occurrence of a late fracture in univariate analysis. A multivariate analysis demonstrated that the shape of the callus was the strongest predictor for late fracture (odds ratio: 19.3, 95% confidence interval: 2.91 to 128). Lateral or central callus had a significantly larger risk of fracture than fusiform, cylindrical, or concave callus.

Radiological monitoring of the shape of the callus during distraction is important to prevent early and late fracture of lengthened limbs in patients with ACH or HCH. In patients with thin callus formation, some measures to stimulate bone formation should be considered as early as possible.

Cite this article: Bone Joint J 2014;96-B:1269–73.


The Journal of Bone & Joint Surgery British Volume
Vol. 94-B, Issue 4 | Pages 556 - 563
1 Apr 2012
Song SH Kim SE Agashe MV Lee H Refai MA Park YE Choi HJ Park JH Song HR

This study evaluated the effect of limb lengthening on longitudinal growth in patients with achondroplasia. Growth of the lower extremity was assessed retrospectively by serial radiographs in 35 skeletally immature patients with achondroplasia who underwent bilateral limb lengthening (Group 1), and in 12 skeletally immature patients with achondroplasia who did not (Group 2). In Group 1, 23 patients underwent only tibial lengthening (Group 1a) and 12 patients underwent tibial and femoral lengthening sequentially (Group 1b).

The mean lengthening in the tibia was 9.2 cm (59.5%) in Group 1a, and 9.0 cm (58.2%) in the tibia and 10.2 cm (54.3%) in the femur in Group 1b. The mean follow-up was 9.3 years (8.6 to 10.3). The final mean total length of lower extremity in Group 1a was 526.6 mm (501.3 to 552.9) at the time of skeletal maturity and 610.1 mm (577.6 to 638.6) in Group 1b, compared with 457.0 mm (411.7 to 502.3) in Group 2. However, the mean actual length, representing the length solely grown from the physis without the length of distraction, showed that there was a significant disturbance of growth after limb lengthening. In Group 1a, a mean decrease of 22.4 mm (21.3 to 23.1) (4.9%) was observed in the actual limb length when compared with Group 2, and a greater mean decrease of 38.9 mm (37.2 to 40.8) (8.5%) was observed in Group 1b when compared with Group 2 at skeletal maturity.

In Group 1, the mean actual limb length was 16.5 mm (15.8 to 17.2) (3.6%) shorter in Group 1b when compared with Group 1a at the time of skeletal maturity. Premature physeal closure was seen mostly in the proximal tibia and the distal femur with relative preservation of proximal femur and distal tibia.

We suggest that significant disturbance of growth can occur after extensive limb lengthening in patients with achondroplasia, and therefore, this should be included in pre-operative counselling of these patients and their parents.


The Bone & Joint Journal
Vol. 97-B, Issue 11 | Pages 1582 - 1587
1 Nov 2015
Suzuki T Seki A Nakamura T Ikegami H Takayama S Nakamura M Matsumoto M Sato K

This retrospective study was designed to evaluate the outcomes of re-dislocation of the radial head after corrective osteotomy for chronic dislocation. A total of 12 children with a mean age of 11 years (5 to 16), with further dislocation of the radial head after corrective osteotomy of the forearm, were followed for a mean of five years (2 to 10). Re-operations were performed for radial head re-dislocation in six children, while the other six did not undergo re-operation (‘non-re-operation group’). The active range of movement (ROM) of their elbows was evaluated before and after the first operation, and at the most recent follow-up.

In the re-operation group, there were significant decreases in extension, pronation, and supination when comparing the ROM following the corrective osteotomy and following re-operation (p < 0.05).

The children who had not undergone re-operation achieved a better ROM than those who had undergone re-operation.

There was a significant difference in mean pronation (76° vs 0°) between the non- re-operation and the re-operation group (p = 0.002), and a trend towards increases in mean flexion (133° vs 111°), extension (0° vs 23°), and supination (62° vs 29°). We did not find a clear benefit for re-operation in children with a re-dislocation following corrective osteotomy for chronic dislocation of the radial head.

Cite this article: Bone Joint J 2015;97-B:1582–7.


The Bone & Joint Journal
Vol. 97-B, Issue 12 | Pages 1726 - 1731
1 Dec 2015
Kim HT Lim KP Jang JH Ahn TY

The traditional techniques involving an oblique tunnel or triangular wedge resection to approach a central or mixed-type physeal bar are hindered by poor visualisation of the bar. This may be overcome by a complete transverse osteotomy at the metaphysis near the growth plate or a direct vertical approach to the bar. Ilizarov external fixation using small wires allows firm fixation of the short physis-bearing fragment, and can also correct an associated angular deformity and permit limb lengthening.

We accurately approached and successfully excised ten central- or mixed-type bars; six in the distal femur, two in the proximal tibia and two in the distal tibia, without damaging the uninvolved physis, and corrected the associated angular deformity and leg-length discrepancy. Callus formation was slightly delayed because of periosteal elevation and stretching during resection of the bar. The resultant resection of the bar was satisfactory in seven patients and fair in three as assessed using a by a modified Williamson–Staheli classification.

Cite this article: Bone Joint J 2015;97-B:1726–31.


The Journal of Bone & Joint Surgery British Volume
Vol. 94-B, Issue 10 | Pages 1421 - 1426
1 Oct 2012
Makarov MR Samchukov ML Birch JG Cherkashin AM Sparagana SP Delgado MR

We undertook a retrospective analysis of 306 procedures on 233 patients, with a mean age of 12 years (1 to 21), in order to evaluate the use of somatosensory evoked potential (SSEP) monitoring for the early detection of nerve compromise during external fixation procedures for limb lengthening and correction of deformity. Significant SSEP changes were identified during 58 procedures (19%). In 32 instances (10.5%) the changes were transient, and resolved once the surgical cause had been removed. The remaining 26 (8.5%) were analysed in two groups, depending on whether or not corrective action had been performed in response to critical changes in the SSEP recordings. In 16 cases in which no corrective action was taken, 13 (81.2%, 4.2% overall) developed a post-operative neurological deficit, six of which were permanent and seven temporary, persisting for five to 18 months. In the ten procedures in which corrective action was taken, four patients (40%, 1.3% overall) had a temporary (one to eight months) post-operative neuropathy and six had no deficit.

After appropriate intervention in response to SSEP changes, the incidence and severity of neurological deficits were significantly reduced, with no cases of permanent neuropathy. SSEP monitoring showed 100% sensitivity and 91% specificity for the detection of nerve injury during external fixation. It is an excellent diagnostic technique for identifying nerve lesions when they are still highly reversible.


The Bone & Joint Journal
Vol. 97-B, Issue 4 | Pages 442 - 448
1 Apr 2015
Kosuge D Barry M

The management of children’s fractures has evolved as a result of better health education, changes in lifestyle, improved implant technology and the changing expectations of society. This review focuses on the changes seen in paediatric fractures, including epidemiology, the increasing problems of obesity, the mechanisms of injury, non-accidental injuries and litigation. We also examine the changes in the management of fractures at three specific sites: the supracondylar humerus, femoral shaft and forearm. There has been an increasing trend towards surgical stabilisation of these fractures. The reasons for this are multifactorial, including societal expectations of a perfect result and reduced hospital stay. Reduced hospital stay is beneficial to the social, educational and psychological needs of the child and beneficial to society as a whole, due to reduced costs.

Cite this article: Bone Joint J 2015; 97-B:442–8.


The Bone & Joint Journal
Vol. 96-B, Issue 5 | Pages 691 - 700
1 May 2014
Solfelt DA Hill BW Anderson CP Cole PA

Cubitus varus is the most frequent complication following the treatment of supracondylar humeral fractures in children. We investigated data from publications reporting on the surgical management of cubitus varus found in electronic searches of Ovid/MEDLINE and Cochrane Library databases. In 894 children from 40 included studies, the mean age at initial injury was 5.7 years (3 to 8.6) and 9.8 years (4 to 15.7) at the time of secondary correction. The four osteotomy techniques were classified as lateral closing wedge, dome, complex (multiplanar) and distraction osteogenesis. A mean angular correction of 27.6º (18.5° to 37.0°) was achieved across all classes of osteotomy. The meta-analytical summary estimate for overall rate of good to excellent results was 87.8% (95% CI 84.4 to 91.2). No technique was shown to significantly affect the surgical outcome, and the risk of complications across all osteotomy classes was 14.5% (95% CI 10.6 to 18.5). Nerve palsies occurred in 2.53% of cases (95% CI 1.4 to 3.6), although 78.4% were transient. No one technique was found to be statistically safer or more effective than any other.

Cite this article: Bone Joint J 2014;96-B:691–700.


The Bone & Joint Journal
Vol. 96-B, Issue 5 | Pages 701 - 706
1 May 2014
Dartnell J Gough M Paterson JMH Norman-Taylor F

Proximal femoral resection (PFR) is a proven pain-relieving procedure for the management of patients with severe cerebral palsy and a painful displaced hip. Previous authors have recommended post-operative traction or immobilisation to prevent a recurrence of pain due to proximal migration of the femoral stump. We present a series of 79 PFRs in 63 patients, age 14.7 years (10 to 26; 35 male, 28 female), none of whom had post-operative traction or immobilisation.

A total of 71 hips (89.6%) were reported to be pain free or to have mild pain following surgery. Four children underwent further resection for persistent pain; of these, three had successful resolution of pain and one had no benefit. A total of 16 hips (20.2%) showed radiographic evidence of heterotopic ossification, all of which had formed within one year of surgery. Four patients had a wound infection, one of which needed debridement; all recovered fully. A total of 59 patients (94%) reported improvements in seating and hygiene.

The results are as good as or better than the historical results of using traction or immobilisation. We recommend that following PFR, children can be managed without traction or immobilisation, and can be discharged earlier and with fewer complications. However, care should be taken with severely dystonic patients, in whom more extensive femoral resection should be considered in combination with management of the increased tone.

Cite this article: Bone Joint J 2014; 96-B:701–6.


The Bone & Joint Journal
Vol. 95-B, Issue 8 | Pages 1027 - 1034
1 Aug 2013
Khan T Joseph B

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.


The Journal of Bone & Joint Surgery British Volume
Vol. 94-B, Issue 9 | Pages 1288 - 1291
1 Sep 2012
Sanghrajka AP Hill RA Murnaghan CF Simpson AHRW Bellemore MC

We describe three cases of infantile tibia vara resulting from an atraumatic slip of the proximal tibial epiphysis upon the metaphysis. There appears to be an association between this condition and severe obesity. Radiologically, the condition is characterised by a dome-shaped metaphysis, an open growth plate and disruption of the continuity between the lateral borders of the epiphysis and metaphysis, with inferomedial translation of the proximal tibial epiphysis. All patients were treated by realignment of the proximal tibia by distraction osteogenesis with an external circulator fixator, and it is suggested that this is the optimal method for correction of this complex deformity. There are differences in the radiological features and management between conventional infantile Blount’s disease and this ‘slipped upper tibial epiphysis’ variant.


The Journal of Bone & Joint Surgery British Volume
Vol. 87-B, Issue 2 | Pages 236 - 240
1 Feb 2005
Belthur MV Bradish CF Gibbons PJ

Between 1990 and 2001, 24 children aged between 15 months and 11 years presented with late orthopaedic sequelae after meningococcal septicaemia. The median time to presentation was 32 months (12 to 119) after the acute phase of the disease. The reasons for referral included angular deformity, limb-length discrepancy, joint contracture and problems with prosthetic fitting. Angular deformity with or without limb-length discrepancy was the most common presentation. Partial growth arrest was the cause of the angular deformity. Multiple growth-plate involvement occurred in 14 children. The lower limbs were affected much more often than the upper. Twenty-three children underwent operations for realignment of the mechanical axis and limb-length equalisation. In 15 patients with angular deformity around the knee the deformity recurred. As a result we recommend performing a realignment procedure with epiphysiodesis of the remaining growth plate when correcting angular deformities.


The Journal of Bone & Joint Surgery British Volume
Vol. 87-B, Issue 1 | Pages 88 - 94
1 Jan 2005
Hasler CC Von Laer L Hell AK

We reviewed 15 patients, nine girls and six boys, with chronic anterior dislocation of the radial head which was treated by ulnar osteotomy, external fixation and open reconstruction of the elbow joint but without repair of the annular ligament. Their mean age was 9.5 years (5 to 15) and the mean interval between the injury and reconstruction was 22 months (2 months to 7 years).

All radial heads remained reduced at a mean follow-up of 20 months (6 months to 5 years). Normal ranges of movement for flexion, extension, pronation and supination were unchanged in 96.1% (49/51) and worse in 3.9% (2/51). Limited ranges of movement were improved in 77.8% (7/9), unchanged in 11% (1/9) and further decreased in 11% (1/9).There were two superficial pin-track infections and two cases of delayed union but with no serious complications. Reconstruction of the radiocapitellar joint is easier using external fixation since accurate correction of the ulna can be determined empirically and active functional exercises started immediately. Only patients with a radial head of normal shape were selected for treatment by this method.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 5 | Pages 695 - 699
1 May 2011
Spiro AS Babin K Lipovac S Stenger P Mladenov K Rupprecht M Rueger JM Stuecker R

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.


The Journal of Bone & Joint Surgery British Volume
Vol. 92-B, Issue 8 | Pages 1138 - 1143
1 Aug 2010
Beckles VLL Jones HW Harrison WJ

We present a retrospective review of 167 patients aged 18 years and under who were treated for chronic haematogenous osteomyelitis at our elective orthopaedic hospital in Malawi over a period of four years. The median age at presentation was eight years (1 to 18). There were 239 hospital admissions for treatment during the period of the study. In 117 patients one admission was necessary, in 35 two, and in 15 more than two.

A surgical strategy of infection control followed by reconstruction and stabilisation was employed, based on the Beit CURE radiological classification of chronic haematogenous osteomyelitis as a guide to treatment. At a minimum follow-up of one year after the end of the study none of the patients had returned to our hospital with recurrent infection.

A total of 350 operations were performed on the 167 patients. This represented 6.7% of all children’s operations performed in our hospital during this period. One operation only was required in 110 patients and none required more than three. Below-knee amputation was performed in two patients with chronic calcaneal osteomyelitis as the best surgical option for function. The most common organism cultured from operative specimens was Staphylococcus aureus, and the tibia was the bone most commonly affected. Polyostotic osteomyelitis occurred in four patients. We believe this is the largest reported series of patients treated for chronic haematogenous osteomyelitis.


The Journal of Bone & Joint Surgery British Volume
Vol. 92-B, Issue 7 | Pages 1017 - 1022
1 Jul 2010
Kelley SP Bache CE Graham HK Donnan LT

We report the outcome of 28 patients with spina bifida who between 1989 and 2006 underwent 43 lower extremity deformity corrections using the Ilizarov technique. The indications were a flexion deformity of the knee in 13 limbs, tibial rotational deformity in 11 and foot deformity in 19. The mean age at operation was 12.3 years (5.2 to 20.6). Patients had a mean of 1.6 previous operations (0 to 5) on the affected limb. The mean duration of treatment with a frame was 9.4 weeks (3 to 26) and the mean follow-up was 4.4 years (1 to 9). There were 12 problems (27.9%), five obstacles (11.6%) and 13 complications (30.2%) in the 43 procedures. Further operations were needed in seven patients. Three knees had significant recurrence of deformity. Two tibiae required further surgery for recurrence. All feet were plantigrade and braceable.

We conclude that the Ilizarov technique offers a refreshing approach to the complex lower-limb deformity in spina bifida.


The Journal of Bone & Joint Surgery British Volume
Vol. 89-B, Issue 3 | Pages 378 - 381
1 Mar 2007
Lourenço AF Morcuende JA

The Ponseti method of treating club foot has been shown to be effective in children up to two years of age. However, it is not known whether it is successful in older children. We retrospectively reviewed 17 children (24 feet) with congenital idiopathic club foot who presented after walking age and had undergone no previous treatment. All were treated by the method described by Ponseti, with minor modifications. The mean age at presentation was 3.9 years (1.2 to 9.0) and the mean follow-up was for 3.1 years (2.1 to 5.6). The mean time of immobilisation in a cast was 3.9 months (1.5 to 6.0).

A painless plantigrade foot was obtained in 16 feet without the need for extensive soft-tissue release and/or bony procedures. Four patients (7 feet) had recurrent equinus which required a second tenotomy. Failure was observed in five patients (8 feet) who required a posterior release for full correction of the equinus deformity.

We conclude that the Ponseti method is a safe, effective and low-cost treatment for neglected idiopathic club foot presenting after walking age.


The Journal of Bone & Joint Surgery British Volume
Vol. 87-B, Issue 5 | Pages 716 - 719
1 May 2005
Davies R Holt N Nayagam S

Two protocols for the operative technique and care of the pin-site with external fixation were compared prospectively. There was a total of 120 patients with 46 in group A and 74 in group B. Infection was defined as an episode of pain or inflammation at a pin site, accompanied by a discharge which was either positive on bacterial culture or responded to a course of antibiotics.

Patients in group B had a lower proportion of infected pin sites (p = 0.003) and the time to the first episode of infection was longer (p < 0.001). The risk of pin-site infection is lower if attention is paid to avoiding thermal injury and local formation of haematoma during surgery and if after-care includes the use of an alcoholic antiseptic and occlusive pressure dressings.


The Journal of Bone & Joint Surgery British Volume
Vol. 88-B, Issue 2 | Pages 255 - 260
1 Feb 2006
Shin EK Jones NF Lawrence JF

We have evaluated the clinical outcomes of simple excision, ulnar lengthening and the Sauvé-Kapandji procedure in the treatment of deformities of the forearm in patients with multiple hereditary osteochondromas. The medical records of 29 patients (33 forearms) were reviewed; 22 patients (22 forearms) underwent simple excision (four with ulnar lengthening) and seven the Sauvé-Kapandji procedure.

Simple excision increased the mean supination of the forearm from 63.2° to 75.0° (p = 0.049). Ulnar lengthening did not significantly affect the clinical outcome. The Sauvé-Kapandji technique improved the mean pronation from 33.6° to 55.0° (p = 0.047) and supination from 70.0° to 81.4° (p = 0.045). Simple excision may improve the range of movement of the forearm but will not halt the progression of disease, particularly in younger patients. No discernable clinical or radiological improvement was noted with ulnar lengthening. The Sauvé-Kapandji procedure combined with simple excision of osteochondromas can improve stability of the wrist, movement of the forearm and the radiological appearance.


The Journal of Bone & Joint Surgery British Volume
Vol. 87-B, Issue 2 | Pages 226 - 230
1 Feb 2005
Manner HM Radler C Ganger R Grossbötzl G Petje G Grill F

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.


The Journal of Bone & Joint Surgery British Volume
Vol. 88-B, Issue 5 | Pages 658 - 664
1 May 2006
Lee RS Weitzel S Eastwood DM Monsell F Pringle J Cannon SR Briggs TWR

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.