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Pedro Pegado(1); Sara Serpa(3); Rosario Perry(4); Carina Ruano(2); Ana Nunes(6); Ana Petinga(6); Pedro Alves(5); Eugenia Soares(7)

(1) Internato Complementar de Radiodianóstico - H. S José;
(2) HSAC;
(3) H Divino Espirito Santo;
(4) Internato Complementar de Pediatria;
(5);Assistente Hospitalar HDE;
(6) Assistente Hospitalar graduado Consultor;
(7) Coordenadora do serviço de Radiologia HDE.

- ESSR York, 2015 - Link:

Introdução: To illustrate the imaging of musculoskeletal manifestations of sickle cell disease (SCD) in 98 children followed in our center. To demonstrate usefulness of imaging in the diagnosis of the various musculoskeletal manifestations in SCD. SCD is the most frequent of a group of conditions known as hemoglobinopathies. The condition is an autosomal recessive genetic disorder, characterized by the reduced or abnormal production of a haemoglobin protein that results in stiff and sticky red blood cells leading to unpredictable episodes of microvascular occlusions. Several organs can be affected with alterations in the central nervous system, bone and joints, cardiovascular system, respiratory system, gastrointestinal tract, and kidneys, which increases morbidity and mortality in this group of patients. The physiopathology of osteo articular involvement in SCD is still not completely understood, with the most important event being the occlusion of microvessels with consequent ischemia. Over time, the disease produces various musculoskeletal abnormalities as a result of chronic anemia; these include marrow hyperplasia, reversion of yellow marrow to red marrow, and, occasionally, extramedullary hematopoiesis. A combination of tissue infarction, immunodeficiency due to splenic dysfunction and excess iron leads to increase risk of osteomyelitis in SCD. Other complications such as septic arthritis, medullary and epiphyseal bone infarcts, growth defects, and soft tissue changes are also described.
Caso clínico: This retrospective study reports the osteo-articular manifestations associated with sickle-cell disease encountered in our institution from 2013 to 2014. Osteo-articular manifestations with imaging findings were reported in 18 patients out of 98. The most frequent complications and those that required hospital care were painful vaso-occlusive crises and osteomyelitis at femoral head. Simple X-ray, Ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI) are current modalities that contribute to diagnosis of osteo-articular disorders in SCD.  Marrow hyperplasia, osteomyelitis, medullary and epiphyseal osteonecrosis, growth defects and dactilitis are illustrated.
Discussão/conclusão: Familiarity with the imaging features of SCD is important for the diagnosis and management of complications. Acute osteomyelitis may be particularly difficult to distinguish from those of bone infarction, MRI could be an important tool in those cases

Palavras Chave: MRI, CT, US, sickle cell disease