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Congress: ECR24
Poster Number: C-15178
Type: EPOS Radiologist (educational)
DOI: 10.26044/ecr2024/C-15178
Authorblock: M. d. O. Costa, J. Costa, R. Godeiro Costa, F. P. N. De Macêdo, M. Moreira Neto; Natal/RN - Brasil/BR
Disclosures:
Mariana de Oliveira Costa: Nothing to disclose
Juliana Costa: Nothing to disclose
Ricardo Godeiro Costa: Nothing to disclose
Francisco Pires Negromonte De Macêdo: Nothing to disclose
Manuel Moreira Neto: Nothing to disclose
Keywords: Haematologic, Musculoskeletal bone, Paediatric, CT, MR, Ultrasound, Education, Education and training, Haematologic diseases
Findings and procedure details

The manifestations of the disease are expressed in the most diverse organs and systems, as detailed below:

 

MUSCULOSKELETAL

OSTEONECROSIS: also known as aseptic necrosis or avascular necrosis, results from infarction of bone trabeculae; usually occurs in the femoral head, proximal humerus and vertebral bodies (known as H-shaped vertebrae). On radiographs they may appear as lytic or blastic areas.

DACTYLITIS (hand-foot syndrome): edema and periosteal reaction on hands and feet due to ischemia, typically occurs in children.

SUBPERIOSTEAL SPONTANEOUS HEMORRHAGE

MARROW HYPERPLASIA (due to chronic anemia):  extramedullary hematopoiesis (liver, spleen and/or hematopoietic masses) and persistence of red bone marrow. This persistente may cause expansion of the bone, manifesting the "hair on end" sign, which is the enlargement of the diploc space.

OSTEOMYELITIS: bone infection involving the medullary cavity, typically bacterial etiology

MUSCLE AND/OR SOFT TISSUE NECROSIS

 

CRANIAL

CEREBRAL ISCHEMIA: on peripheral areas of the white matter

MOYAMOYA SYNDROME: development of thin collateral vessels secondary to occlusion of the internal carotid artery.

ARTERIAL TORTUOSITIES AND ANEURYSM FORMATION: 

 

CARDIOPULMONARY

ACUTE CHEST SYNDROME: consolidation in the lower lobes. Ischemic etiology, more serious.

RECURRING PNEUMONIA: consolidation of variable location. Infectious etiology.

CHRONIC LUNG DISEASE: sequelae of recurrent ischemia.

DILATED CARDIOMYOPATHY: caused by ischemic sequelae of coronary syndromes.

PULMONARY HYPERTENSION: due to chronic hypoxia and chronic pulmonary thromboembolism

 

ABDOMINAL

SPLEEN: Autosplenectomy: resulting from progressive infarcts and calcium deposition, results in impaired immune status that makes the patient susceptible to infection. Splenic sequestration syndrome: acute accumulation of blood in the spleen, increasing its volume, associated with hemodynamic shock.

KIDNEYS: enlarged (on initial stages; due to increased plasma flow) and chronic kidney disease (long-term; due to acute papillary necrosis and/or renal vein thrombosis).

LIVER: necrosis and cirrhosis of ischemic etiology, as well as sequestration syndrome.

BILIARY TRACT: pigmented gallstones (can cause cholecystitis, choledocholithiasis and pancreatitis)

INTESTINAL ISCHEMIA: propensity for thrombosis due to blood stasis

GALLERY