Abnormalities of the adult shoulder due to sickle cell osteonecrosis during childhood

Abstract

Patients and methods: A retrospective study of 110 adults with sickle cell anemia (SS genotype) was conducted to determine the frequency of complications of sickle cell osteonecrosis of the shoulder in childhood. The glenohumeral joint was evaluated on plain anteroposterior and lateral radiographs of the shoulder.

Results: Mean time since osteonecrosis of the shoulder was 24 years. Radiographic abnormalities were seen in 106 of the 220 shoulders (48.2%). Both shoulders were affected in 86% of cases and at least one hip in 96%. Radiographic abnormalities included isolated caput magna, caput plana, a short humeral neck and ascension of the tuberosities. Twelve shoulders exhibited glenoid fossa abnormalities, which consisted in complete or partial hypoplasia. Evidence of glenohumeral osteoarthritis was seen in 11 shoulders.

Discussion: The shoulder abnormalities seen in our patients were probably due to growth disturbances in the proximal humerus secondary to osteonecrosis of the humeral head during childhood. They were common but less likely to produce functional impairment than osteonecrosis-related lesions of the hip. However, some patients had premature osteoarthritis and geometric shoulder abnormalities responsible for functional loss in adulthood.