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

Aims. The aim of this study was to identify any progression between benign osteofibrous dysplasia (OFD), OFD-like adamantinoma and malignant adamantinoma, and to investigate the rates of local recurrence, metastases and survival, in order to develop treatment algorithms for each. Patients and Methods. A single institution retrospective review of all patients presenting with OFD, OFD-like adamantinoma and adamantinoma between 1973 and 2012 was undertaken. Complete data were available for 73 patients (42 with OFD; ten with an OFD-like adamantinoma and 21 with an adamantinoma). The mean follow-up was 10.3 years (3 to 25) for OFD, 9.2 years (3.0 to 26.3) for OFD-like and 11.6 years (0.25 to 33) for adamantinoma. Results. The mean age at diagnosis for OFD was 13.5 years (1 to 49), 10.5 years (6 to 28) for OFD-like and 34 years (14 to 86) for adamantinoma. A total of 24 of the 42 patients with OFD (57%) have not required any treatment and have been managed with observation. A total of 18 of the 42 patients with OFD underwent surgery, 13 with curettage and five with resection. In all, three patients developed recurrence following curettage (23%) but none following resection. All these patients were cured with further limited surgery. A total of six patients initially diagnosed with OFD were subsequently found to have OFD-like adamantinoma. Of the ten patients initially diagnosed with OFD-like adamantinoma, three (30%) were managed with observation alone and seven underwent surgery, two with curettage and five with resection. Local recurrence arose in two patients, one each after curettage and resection. No patients with either OFD or an OFD-like adamantinoma developed metastases or had progression to adamantinoma. All patients with an adamantinoma were treated by surgery, three with curettage, six with amputation and 12 with excision. In all, two of the three treated with curettage developed local recurrence, requiring further surgery. Late development of both local recurrence and metastases led to a ten year disease specific survival of 93% which had dropped to 39% by 20 years. Conclusion. We found no evidence of progression from OFD to adamantinoma. Conservative management with observation or curettage is often successful for patients with OFD and OFD-like adamantinoma. Resection with clear margins is required for patients with adamantinoma. Late tumour recurrence is not uncommon in adamantinoma and prolonged follow-up should be considered. Cite this article: Bone Joint J 2017;99-B:409–16

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

Osteofibrous dysplasia is a rare condition usually affecting the tibiae and fibulae of males in the first two decades of life. Involvement at the ulna has also been reported in some cases. We describe an extensive, rapidly progressive lesion of the ulna in a three-year-old girl. The progression of the disease accelerated after a local marginal removal, and required extensive resection of the ulna with reconstruction using a fibular autograft. The particular features of this case are the change in the aggressive nature of the condition, which has not been previously reported. We believe that this is only the second recorded case of histologically proven involvement of the ulna

Introduction: The purpose of this study is to demonstrate that definitive surgery (extraperiosteal excision) is required in patients with osteofibrous dysplasia (OFD) due to the risk of recurrence and co-existent adamantinoma. The possible link with adamantinoma is controversial with some authors believing that they are part of one histological process. This therefore provides difficulty in recommending treatment options. Methods: Using our database 22 cases of OFD were identified. Management was diverse. Results: Definitive (extraperiosteal) surgery, in the majority of our patients, was localized excision for small lesions (less than 50% of the bony circumference) and segmental excision followed by reconstructive surgery for more extensive ones. Seven patients had a “shark-bite” excision and a further seven were treated with fibula autografting. Of the latter group, one required further excision and bone transport due to recurrence of OFD. Five underwent bone transport & distraction osteogenesis and one had a proximal tibial replacement. Nine initially underwent curettage, but eight recurred. No recurrences occurred following localized extraperiosteal excisions and bone transport. There were three confirmed cases of adamantinoma. Discussion: In view of the risk of association of OFD with adamantinoma, and to some extent the continuous morbidity of OFD if left untreated, we believe that radical extraperiosteal excision is indicated in most if not all cases of OFD

A nine-year-old girl presented with a four-year history of progressive bowing of the left tibia. She had been seen in our clinic three and four years earlier, when no treatment was advised. She had been complaining of mild pain in the left leg for one month but was otherwise not very perturbed about her deformed leg. Examination showed bowing of the left tibia, no leg length discrepancy, no limp, and a normal left knee and ankle. Radiological examination showed features of osteo-fibrous dysplasia of the left tibia, with eccentric expansion of the cortex, intracortical osteolysis, marginal sclerosis encroaching on the medulla and diffuse lesional calcification. No other bones were involved. Because of the progressive bowing of the tibia and the mild pain, the recommended loose observation of the patient was abandoned. Daily treatment with 30 mg IVI pamidronate for three days resolved the pain. One month after the latest presentation the lesion was biopsied. Histological examination confirmed the diagnosis of osteofibrous dysplasia of the tibia. There was no evidence of adamantinoma. The literature on this rare bone lesion in childhood supports the use of open biopsy if the deformity becomes painful, the bowing is progressive and the patient presents after the age of nine years. Important differential diagnoses include fibrous dysplasia and adamantinoma

The purpose of this study is to demonstrate that definitive surgery (extraperiosteal excision) is required in patients with osteofibrous dysplasia (OFD) due to the risk of recurrence and co-existent adamantinoma. OFD is an unusual childhood condition, 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 with some authors believing that they are part of one histological process. This therefore provides difficulty in recommending treatment options. A retrospective review of OFD was conducted. Using the Stanmore Bone Tumour Unit database 22 cases were identified who were initially diagnosed with OFD or were diagnosed on final histology. All cases were tibial except one lesion in the ulna and one in the fibula. Management was diverse depending on the severity of symptoms and the extent of the lesions encountered. Definitive (extraperiosteal) surgery in the majority of our patients was localized excision for small lesions (less than 50% of the bony circumference) and segmental excision followed by reconstructive surgery for more extensive ones. Seven patients had a sharkbite excision and a further seven were treated with fibula autografting. Of the latter group, one required further excision and bone transport due to recurrence of OFD. An additional five underwent bone transport & distraction osteogenesis using the Ilizarov technique and one had a proximal tibial replacement. Nine initially underwent curettage, but eight recurred (recurrence rate 88.9%). No recurrences occurred following localized extraperiosteal excisions and bone transport. There were three confirmed cases of adamantinoma. In view of the risk of association of OFD with adamantinoma, and to some extent the continuous morbidity of OFD if left untreated, we believe that radical extraperiosteal excision is indicated in most if not all cases of OFD

Introduction and Aim: The purpose of this study is to demonstrate that definitive surgery (extraperiosteal excision) is required in patients with osteofibrous dysplasia (OFD) due to the risk of recurrence and co-existent adamantinoma. OFD is an unusual childhood condition, 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, with some authors believing that they are part of one histological process. This therefore provides difficulty in recommending treatment options. Method and Results: A retrospective review of OFD was conducted. Using the Stanmore Bone Tumor Unit database, 22 cases were identified who were initially diagnosed with OFD or were diagnosed on final histology. All cases were tibial except one lesion in the ulna and one in the fibula. Management was diverse, depending on the severity of symptoms and the extent of the lesions encountered. Definitive (extraperiosteal) surgery in the majority of our patients was localised excision for small lesions (less than 50% of the bony circumference) and segmental excision followed by reconstructive surgery for more extensive ones. Seven patients had a ‘sharkbite’ excision and a further seven were treated with fibula autografting. Of the latter group, one required further excision and bone transport due to recurrence of OFD. An additional five underwent bone transport and distraction osteogenesis using the Ilizarov technique and one had a proximal tibial replacement. Nine initially underwent curettage, but eight recurred (recurrence rate 88.9%). No recurrences occurred following localised extraperiosteal excisions and bone transport. There were three confirmed cases of adamantinoma. Conclusion: In view of the risk of association of OFD with adamantinoma, and to some extent the continuous morbidity of OFD if left untreated, we believe that radical extraperiosteal excision is indicated in most if not all cases of OFD

Aims: Purpose of this study was to obtain long term follow-up in patients with Osteofibrous Dysplasia (OFD), in order to investigate natural history of the disease, late results of treatment, and potential risk of Adaman-tinoma development in this setting. Methods: A retrospective study of 48 patients with histologically proven OFD observed at our Institution between 1900 and 1997 was undertaken. Clinico-pathologic features of all cases were reviewed and found consistent with OFD. A clinical status update and current radiographs were obtained in all patients. A subgroup of 21 patients with minimum follow-up of 20 years (21 to 44 years, average 27) was analysed for functional result and adamantinoma development. Functional result according to MTS-ISOLS score correlated with surgical aggressiveness. Results: Best results were observed in patients that received a single biopsy or curettage; worse results were seen after multiple resections or osteotomies and associated with complications as infection or compartment syndrome. No patients had current symptoms or significant symptoms changes nor physical findings, radiographic clues or subsequent radiographic changes suggesting adamantinoma development. Conclusions: OFD is a benign condition; the natural history of the disease has minimal consequences in the adult life. Surgical treatment is usually not necessary and may actually worsen the result because of the potential for severe complications. The relationship with adamantinoma remains unclear, follow-up is suggested

We report a case which highlights the progression of osteofibrous dysplasia to adamantinoma and questions whether intralesional curettage is the appropriate treatment. The role of a joint-sparing massive endoprosthesis using cortical fixation is demonstrated and we describe a unique biomedical design which resulted in the manufacture of an end cap to allow amputation through a custom-made proximal tibial replacement, rather than an above-knee amputation following recurrence

We present a retrospective study of patients suffering from a variety of benign tumours in whom external fixators were used to treat deformity and limb-length discrepancy, and for the reconstruction of bone defects. A total of 43 limbs in 31 patients (12 male and 19 female) with a mean age of 14 years (2 to 54) were treated. The diagnosis was Ollier’s disease in 12 limbs, fibrous dysplasia in 11, osteochondroma in eight, giant cell tumour in five, osteofibrous dysplasia in five and non-ossifying fibroma in two. The lesions were treated in the tibia in 19 limbs, in the femur in 16, and in the forearm in eight. The Ilizarov frame was used in 25 limbs, the Taylor Spatial Frame in seven, the Orthofix fixator in six, the Monotube in four and the Heidelberg fixator in one. The mean follow-up was 72 months (22 to 221). The mean external fixation period was 168 days (71 to 352). The mean external fixation index was 42 days/cm (22.2 to 102.0) in the 22 patients who required limb lengthening. The mean correction angle for those with angular deformity was 23° (7° to 45°). At final follow-up all patients had returned to normal activities. Four patients required a second operation for recurrent deformity of further limb lengthening. Local recurrence occurred in one patient, requiring further surgery

The authors aim to report the long-term follow-up experience in the reconstruction of bony defects by Ilizarov’s distraction osteogenesis using bone transport method following en bloc resection of bone tumors. En bloc resection was performed nine patients with bone tumors between October 1991–January 2000. The mean age of the patients was 19.3 years. Histological diagnosis was osteosarcoma in four cases, Ewing sarcoma in two cases, giant cell tumor (aggressive) in one, osteofibrous dysplasia (latent) in one and osteoblastoma (aggressive) in one case. The average follow-up period was 122 months and bone defect after resection was 14 cm. The function of the affected leg was excellent in four patients, good in two and poor in one patient according to the modified system of the MSTS. In the case where reconstruction of the ulna was done, MSTS score was excellent and DASH skoru was 2.5. We imply that in patients with long life expectancies, reconstruction with distraction osteogenesis seems to be an efficient method in the long-term follow up, on condition that its complications are promptly managed

Young active patients with malignant tumours arising in the distal fibula, requiring excision, present a challenge to the treating surgeon. Wide local excision is advocated, to achieve clearance, however, disruption of the ankle mortise results and fusion is often required to restore stability. The loss of movement is poorly tolerated in the younger patient and leads to progressive degenerative changes in surrounding joints. Excision of the distal fibula lesion followed by harvesting of the proximal fibula and using this graft to recreate the ankle mortise restores ankle stability and retains ankle movement. Between 1998 and 2007, we have performed this technique on 4 patients. Diagnoses were Ewing’s sarcoma, chondrosarcoma, parosteal osteosarcoma and osteofibrous dysplasia. To date there has been no evidence of distant or local recurrence. One case was complicated by infection, which resolved with radical debridement and antibiotics; the other three fibula grafts survived. Good to excellent results were achieved. We will present the technical aspects of this procedure, with particular reference to the most recent case, performed on a young female patient with parosteal osteosarcoma. We believe this technique provides good oncological and functional results and recommend this treatment option is considered in young active patients requiring distal fibula excisions for sarcoma. Correspondence should be addressed to Major M Butler RAMC, Princess Elizabeth Orthopaedic Centre, Royal Devon and Exeter Hospital, Exeter, Devon

The purpose of this study was to review the outcomes of patients treated with injectable calcium phosphate cement (Norian SRS, Norian Corporation, Cupertino, California) for contained bone defects after resection of benign or low-grade malignant bone tumours. The clinical records and radiographs of 17 patients who had been treated with calcium phosphate cement were reviewed, looking for incorporation into bone, reabsorption of the material and complications. The 17 patients had a mean age of 29.8 years (range 7 to 64). The diagnosis was giant cell tumour in 9 cases, fibrous dyplasia in 2, low grade chondrosarcoma in 2, and one each of enchondroma, chondromyxoid fibroma, osteofibrous dysplasia, and chondroblastoma. The tibia was involved in 9 cases, the femur in 6 and the radius in 2. The mean follow up was 11 months (range 3 to 25). The material is radioopaque and well visualised on plain radiographs. In most cases, incorporation of the material into the bone structure appeared good, but there was little absorption of the material during the followup available. The exceptions were 2 cases in which the material was absorbed following local recurrence of giant cell tumour. One fracture associated with a giant cell tumour healed well in the presence of the material. In three patients, there were clinical and radiological features at follow up suggestive of periostitis related to the material. In one case a florid effusion of the knee may have been due to the material. Injectable calcium phosphate cement may have a role in the management of contained defects requiring mechanical support following resection of benign or low-grade malignant tumours of bone. However, problems with periostitis, possibly synovitis and absorption in the presence of local recurrence should be considered

Aims

Intercalary allografts following resection of a primary diaphyseal tumour have high rates of complications and failures. At our institution intercalary allografts are augmented with intramedullary cement and fixed using compression plating. Our aim was to evaluate their long-term outcomes.

In 1999, we developed a technique for biological reconstruction after excision of a bone tumour, which involved using autografts of the bone containing the tumour treated with liquid nitrogen. We have previously reported the use of this technique in 28 patients at a mean follow up of 27 months (10 to 54).

In this study, we included 72 patients who underwent reconstruction using this technique. A total of 33 patients died and three were lost to follow-up, at a mean of 23 months (2 to 56) post-operatively, leaving 36 patients available for a assessment at a mean of 101 months 16 to 163) post-operatively. The methods of reconstruction included an osteo-articular graft in 16, an intercalary in 13 and, a composite graft with prosthesis in seven.

Post-operative function was excellent in 26 patients (72.2%), good in seven (19.4%), and fair in three (8.3%) according to the functional evaluation system of Enneking. No recurrent tumour occurred within the grafts. The autografts survived in 29 patients (80.6%), and the rates of survival at five and ten years were 86.1% and 80.6 %, respectively. Seven of 16 osteo-articular grafts (44%) failed because of fracture or infection, but all the composite and intercalary grafts survived.

The long-term outcomes of frozen autografting, particularly using composite and intercalary grafts, are satisfactory and thus represent a good method of treatment for patients with a sarcoma of bone or soft tissue.

Cite this article: Bone Joint J 2014;96-B:555–61.

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.

We compared the accuracy of the growth remaining method of assessing leg-length discrepancy (LLD) with the straight-line graph method, the multiplier method and their variants. We retrospectively reviewed the records of 44 patients treated by percutaneous epiphysiodesis for LLD. All were followed up until maturity. We used the modified Green–Anderson growth-remaining method (Method 1) to plan the timing of epiphysiodesis. Then we presumed that the other four methods described below were used pre-operatively for calculating the timing of epiphysiodesis. We then assumed that these four methods were used pre-operatively. Method 2 was the original Green–Anderson growth-remaining method; Method 3, Paley’s multiplier method using bone age; Method 4, Paley’s multiplier method using chronological age; and Method 5, Moseley’s straight-line graph method. We compared ‘Expected LLD at maturity with surgery’ with ‘Final LLD at maturity with surgery’ for each method. Statistical analysis revealed that ‘Expected LLD at maturity with surgery’ was significantly different from ‘Final LLD at maturity with surgery’. Method 2 was the most accurate. There was a significant correlation between ‘Expected LLD at maturity with surgery’ and ‘Final LLD at maturity with surgery’, the greatest correlation being with Method 2. Generally all the methods generated an overcorrected value. No method generates the precise ‘Expected LLD at maturity with surgery’. It is essential that an analysis of the pattern of growth is taken into account when predicting final LLD. As many additional data as possible are required.

Cite this article: Bone Joint J 2013;95-B:993–1000.