Current paediatric orthopaedic practice in hereditary multiple osteochondromas of the forearm: a systematic review

This systematic review aims to answer three research questions concerning the management of hereditary multiple osteochondromas of forearm in children: What is the best available evidence for the currently employed surgical procedures? What patient characteristics are associated with better prognosis? What disease characteristics are associated with better prognosis?

We searched the literature using three major databases with no publication date restrictions. To enhance search sensitivity and maintain precision we used keywords/subject terms correlating with patient population, problem and interventions. We used strict inclusion/exclusion criteria to improve validity evidence.

The search process yielded 34 eligible studies with a total of 282 patients (315 forearms). We comprehensively analysed study and patient demographics and interventions and outcomes. Eleven studies (32%) had a long-term follow-up and 31 studies (91%) were retrospective. Of the total number of forearms, ulnar lengthening +/- associated procedures was used in 210 forearms (66.7%), isolated osteochondroma excision in 65 forearms (20.6%) and isolated distal radius hemiepiphysiodesis in 15 forearms (4.7%) among others.

Ulnar lengthening can restore radiologic anatomy, improve appearance and to a lesser extent objective clinical parameters like joint range of motion on the short/intermediate term. Isolated osteochondroma excision can relief pain and satisfy cosmetic concerns occasionally. There is poor evidence to suggest that surgery improves quality of life or function. Predictors of surgical success in regard to patient and disease characteristics remain elusive. Natural history and prospective randomized control studies where the control group receives no treatment should be rethought. They have the potential for bias control and identification of the ideal surgical candidate. The complex interplay between the confounding variables has undermined the capability of most studies to provide well-grounded evidence to support and generalize their conclusions. Valid quality of life scales should supplement objective outcome measures.