Manifestations of Sickle Cell Disorder at Abdominal and Pelvic Imaging

Abstract

Sickle cell disorder (SCD) refers to a spectrum of hematologic disorders that cause a characteristic clinical syndrome affecting the entire body. It is the most prevalent monogenetic hemoglobinopathy worldwide, with a wide range of focal and systemic expressions. Hemoglobin gene mutation leads to the formation of abnormal sickle-shaped red blood cells, which cause vascular occlusion and result in tissue and organ ischemia and infarction. Recurrent episodes of acute illness lead to progressive multisystem organ damage and dysfunction. Vaso-occlusion, hemolysis, and infection as a result of functional asplenia are at the core of the disease manifestations. Imaging plays an essential role in the diagnosis and management of SCD-related complications in the abdomen and pelvis. A thorough understanding of the key imaging findings of SCD complications involving hepatobiliary, gastrointestinal, genitourinary, and musculoskeletal systems is crucial to timely recognition and accurate diagnosis. The authors aim to familiarize the radiologist with the SCD spectrum, focusing on the detection and evaluation of manifestations that may appear at imaging of the abdomen and pelvis. The topics the authors address include (a) the pathophysiology of the disease, (b) the placement of SCD among hemoglobinopathies, (c) the clinical presentation of SCD, (d) the role of imaging in the evaluation and diagnosis of patients with SCD who present with abdominal and pelvic manifestations in addition to extraperitoneal manifestations detectable at abdominal or pelvic imaging, (e) imaging features associated with common and uncommon sequelae of SCD in abdominal and pelvic imaging studies, and (f) a brief overview of management and treatment of patients with SCD. Online supplemental material is available for this article. ^©RSNA, 2022.