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. 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.Aims
Methods
The aim of this study was to investigate the changes in femoral
trochlear morphology following surgical correction of recurrent
patellar dislocation associated with trochlear dysplasia in children. A total of 23 patients with a mean age of 9.6 years (7 to 11)
were included All had bilateral recurrent patellar dislocation associated
with femoral trochlear dysplasia. The knee with traumatic dislocation
at the time of presentation or that had dislocated most frequently
was treated with medial patellar retinacular plasty (Group S). The
contralateral knee served as a control and was treated conservatively
(Group C). All patients were treated between October 2008 and August
2013. The mean follow-up was 48.7 months (43 to 56). Axial CT scans
were undertaken in all patients to assess the trochlear morphological
characteristics on a particular axial image which was established
at the point with the greatest epicondylar width based on measurements
preoperatively and at the final follow-up.Aims
Patients and Methods
The medial rotation contracture caused by weak external rotation secondary to obstetric brachial plexus injury leads to deformation of the bones of the shoulder. Scapular hypoplasia, elevation and rotation deformity are accompanied by progressive dislocation of the humeral head. Between February and August 2005, 44 children underwent a new surgical procedure called the ‘triangle tilt’ operation to correct this bony shoulder deformity. Surgical levelling of the distal acromioclavicular triangle combined with tightening of the posterior glenohumeral capsule (capsulorrhaphy) improved shoulder function and corrected the glenohumeral axis in these patients. The posture of the arm at rest was improved and active external rotation increased by a mean of 53° (0° to 115°) in the 40 children who were followed up for more than one year. There was a mean improvement of 4.9 points (1.7 to 8.3) of the Mallet shoulder function score after surgical correction of the bony deformity.
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.
