Sickle cell disease (SCD) is characterized by repeated episodes of vaso-occlusion and hemolysis beginning in the pediatric period that result in serious multi-organ system complications. In particular, renal complications are often the cause of morbidity and reduced life expectancy of patients with SCD. Therefore, it is essential that radiologists be able to identify the imaging features early to help guide prompt and appropriate treatment. Sequelae in pediatric patients include sickle cell nephropathy, infarction and papillary necrosis, and assorted glomerulopathies. These in turn can lead to altered hemodynamics, impaired urinary concentrating ability, hematuria, proteinuria, and acute and chronic kidney injury. Children with SCD are also at increased risk of asymptomatic bacteriuria and urinary tract infection. Even those children and young adults who only have sickle cell trait (SCT) rather than SCD may develop chronic kidney disease later in life and carry markedly increased risk for renal medullary carcinoma. Segmental testicular infarction can compromise fertility in patients with both SCD and SCT. The genitourinary manifestations of SCD and SCT in the pediatric patient will be reviewed in this educational exhibit, with an emphasis on radiologic appearances. For each entity, the clinical presentation, pathophysiology, and differential diagnosis of the imaging findings will also be briefly reviewed. The renal complications covered will include renal infarction, papillary necrosis, renal vein thrombosis (as a complication of nephrotic syndrome), urinary tract infection, hematuria, renal medullary carcinoma, and acute and chronic kidney disease. Testicular and penile sequelae of SCD including segmental testicular infarction and priapism will also be discussed. A variety of imaging modalities will be used to illustrate the various complications, including ultrasonography, computed tomography, and magnetic resonance imaging.
SPR 2018 Annual Meeting & Postgraduate Course