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The Natural Progression of Symptomatic Humeral Head Osteonecrosis in Adults with Sickle Cell Disease
Alexandre Poignard, MD1; Charles-Henri Flouzat-Lachaniette, MD1; Julien Amzallag, MD1; Frederic Galacteros, MD1; Philippe Hernigou, MD1
1 University Paris-Est Creteil, Hôpital Henri Mondor, 94010 Creteil, France. E-mail address for P. Hernigou: philippe.hernigou@wanadoo.fr
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Investigation performed at Hôpital Henri Mondor, Creteil, France

Disclosure: None of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of any aspect of this work. None of the authors, or their institution(s), have had any financial relationship, in the thirty-six months prior to submission of this work, with any entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. Also, no author has had any other relationships, or has engaged in any other activities, that could be perceived to influence or have the potential to influence what is written in this work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.

Copyright © 2012 by The Journal of Bone and Joint Surgery, Inc.
J Bone Joint Surg Am, 2012 Jan 18;94(2):156-162. doi: 10.2106/JBJS.J.00919
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Osteonecrosis of the humeral head is a frequent complication in adults with sickle cell disease. However, little is known about the rate of, and the factors influencing, progression of symptomatic shoulder osteonecrosis in patients with this disease.


Eighty-two adult patients with sickle cell disease and symptomatic osteonecrosis of the humeral head (104 shoulders) were identified with magnetic resonance imaging (MRI) between 1985 and 1993. Nineteen of the eighty-two patients were homozygous for hemoglobin S (S/S genotype), thirty-seven had hemoglobin S/hemoglobin C (S/C), and twenty-six had hemoglobin S/beta-thalassemia (S/T). Shoulder osteonecrosis was graded with the method of Cruess with an adaptation for MRI as proposed by Steinberg et al. for hip osteonecrosis. Annual radiographs were obtained. At the initial evaluation, thirty-eight symptomatic shoulders were designated as stage I (with osteonecrosis seen only on MRI), forty-two symptomatic shoulders were designated as stage II (radiographic evidence without collapse), and twenty-four symptomatic shoulders were designated as stage III or IV (a crescent line or collapse).


Partial or total repair with a decrease in the size of the osteonecrotic lesion or in the stage was never observed on MRI. At the time of the most recent follow-up (average, twenty years; range, fifteen to twenty-four years), collapse had occurred in eighty-nine shoulders (86%). The mean interval between the onset of pain and collapse was six years (range, six months to seventeen years; median, eight years). Of the 104 symptomatic shoulders, sixty-three (61%) with collapse worsened clinically until surgical treatment was needed. The principal risk factors for development of shoulder osteonecrosis in adults with sickle cell disease were the presence of hip osteonecrosis and the S/T or S/C genotype. The rate and risk of progression of the lesion until collapse occurred were significantly related to the S/S genotype, to a stage of II, to a large size of the osteonecrotic lesion, and to a medial or posterior location of the lesion.


Untreated symptomatic shoulder osteonecrosis related to sickle cell disease has a high likelihood of progressing to humeral head collapse, and the natural evolution in the long term requires surgical treatment for many of these patients.

Level of Evidence: 

Prognostic Level II. See Instructions for Authors for a complete description of levels of evidence.

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    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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