1- Radiology Department, Hospital de São José, Centro Hospitalar de Lisboa Central, EPE, Lisboa
2- Pediatric Hematology Unit, Department of Pediatrics, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa
3- Radiology Department, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa
- Artigo publicado no Journal of Advances in Radiology and Medical Imaging, Volume 2, Issue 1, 2017
Introdução – Sickle cell disease (SCD) is the most frequent of a group of conditions known as haemoglobinopathies. The disease is an autosomal recessive genetic disorder, characterized by abnormal haemoglobin (haemoglobin S) that under certain conditions polymerizes resulting in microvascular occlusions. Bones are the second most affected organ by SCD, after the spleen. Despite the importance of osteo-articular involvement in SCD, these complications are still understudied and the pathophysiology of these changes is not fully understood. The most frequent complications that require hospitalization in SCD patients are painful vaso-occlusive crises (VOC) and osteomyelitis.
Objectivos – This review addresses acute and long-term osteo-articular complications in SCD, with particular emphasis on differentiating infection from infarction (osteonecrosis), where imaging is playing a crescent role on the acute bone pain diagnosis algorithm.
Métodos – We performed a retrospective study to illustrate the osteo-articular manifestations associated with SCD in our institution, in children with at least one Haematology appointment in 2013 and 2014. All the imaging examinations were first analysed by senior doctors specialized on Paediatrics Radiology without following any specific protocol. During the study all the imaging findings were reviewed by a junior doctor and a senior doctor specialized in musculoskeletal radiology.
Resultados – Osteo-articular manifestations with imaging findings were reported in 28 out of 97 patients (23 homozygous – SS patients and 5 composite heterozygous - SC, Sβ) with equal gender distribution. These cases were reviewed and 11 major manifestations were depicted (infection and avascular necrosis). The remaining patients presented 17 minor manifestations such as effects of intramedullary marrow with replacement and hyperplasia as well as growth effects.
Conclusões – Awareness of the imaging features of SCD is important for early diagnosis and management of musculoskeletal complications. Acute osteomyelitis may be particularly difficult to distinguish from bone infarction, and MRI could be an important tool in these cases.