Multiple hereditary exostoses is an autosomal dominant skeletal disorder in which there are numerous cartilage-capped excrescences in areas of actively growing bone. The condition is genetically heterogeneous, and at least three genes, ext1, ext2 and ext3 are involved. The reported risk for malignant transformation to chondrosarcoma has been from 0.6% to 2.8%. We have reviewed six generations of a family with 114 living adult members, 46 of them with multiple exostoses. Four have had operations for chondrosarcoma, giving the risk for malignant transformation as 8.3% in this family. Clinical and radiological examination revealed two additional patients with a suspicion of malignancy, but in whom the histological findings were benign. Reported elsewhere in detail, genetic linkage analysis mapped the causative gene to chromosome 11 and molecular studies revealed a guanine-to-thymine transversion in the ext2 gene. Patients with multiple hereditary exostoses carry a relatively high risk of malignant transformation. They should be informed of this possibility and regularly reviewed

Aims

We report a prospective cohort study of the midterm results of surgical dislocation of the hip (according to Ganz) to perform resection of osteochondromas involving the femoral neck in patients with multiple hereditary exostoses (MHE).

We present our experience of forearm lengthening in children with various conditions performed by a single surgeon between 1995 and 2009. A total of 19 children with a mean age of 9.8 years (2.1 to 15.9) at the time of surgery had 22 forearm lengthenings using either an Ilizarov/spatial and Ilizarov circular frame or a monolateral external fixator. The patients were divided into two groups: group A, in whom the purpose of treatment was to restore the relationship between the radius and the ulna, and group B, in whom the objective was to gain forearm length. The mean follow-up after removal of the frame was 26 months (13 to 53).

There were ten patients (11 forearms) in group A with a mean radioulnar discrepancy of 2.4 cm (1.5 to 3.3) and nine patients (11 forearms) in group B. In group A, the mean lengthening achieved was 2.7 cm (1.0 to 5.5), with a lengthening index of 11.1 weeks/cm. Equalisation or overcorrection of the discrepancy was achieved in seven of 11 forearms, but lengthening was only partially successful at preventing subluxation or dislocation of the radial head. In group B, the mean lengthening achieved was 3.8 cm (1.9 to 6.8), with a lengthening index of 7.25 weeks/cm. Common complications in both groups were pin-site infection and poor regenerate formation.

Forearm lengthening by distraction osteogenesis is a worthwhile procedure in children that can improve cosmesis and function, particularly in patients with shortening of both radius and ulna.

Aims

Few studies dealing with chondrosarcoma of the pelvis are currently available. Different data about the overall survival and prognostic factors have been published but without a detailed analysis of surgery-related complications. We aimed to analyse the outcome of a series of pelvic chondrosarcomas treated at a single institution, with particular attention to the prognostic factors. Based on a competing risk model, our objective was to identify risk factors for the development of complications.

We present the validation of a translation into Danish of the Oxford ankle foot questionnaire (OxAFQ). We followed the Isis Pros guidelines for translation and pilot-tested the questionnaire on ten children and their parents. Following modifications we tested the validity of the final questionnaire on 82 children (36 boys and 45 girls) with a mean age of 11.7 years (5.5 to 16.0) and their parents. We tested the reliability (repeatability (test–retest), child–parent agreement, internal consistency), feasibility (response rate, time to completion, floor and ceiling effects) and construct validity. The generic child health questionnaire was used for comparison. We found good internal consistency for the physical and the school and play domains, but lower internal consistency for the emotional domain. Overall, good repeatability was found within children and parents as well as agreement between children and parents. The OxAFQ was fast and easy to complete, but we observed a tendency towards ceiling effects in the school and play and emotional domains. To our knowledge this is the first independent validation of the OxAFQ in any language. We found it valid and feasible for use in the clinic to assess the impact on children’s lives of foot and/or ankle disorders. It is a valuable research tool.

Cite this article: Bone Joint J 2015;97-B:420–6.

Osteochondroma is the most common benign bone tumour. The risk of sarcomatous change in an isolated lesion is approximately 1%. We report a case of an isolated osteochondroma which appeared benign on clinical and plain radiographic examination but routine histological analysis revealed non-Hodgkin’s lymphoma in the underlying bone. This association has not previously been reported and the case emphasises the importance of routine histological analysis, even if a lesion appears benign.