Idiopathic facial swelling secondary to sickle cell anaemia

Abstract

Sickle cell disease is a common inherited autosomal disease that is characterised by abnormally shaped (sickle-shaped) red blood cells (RBCs). It can involve virtually any organ system. The clinical manifestations of sickle cell disease vary and are classified as vaso-occlusion, chronic anaemia and infection. The imaging appearances of central nervous system and musculoskeletal involvement by sickle cell disease have been well documented; however, involvement of the head and neck region is often unreported, although it is not uncommon. In the head and neck, sickle cell disease can involve the inner ears, orbits, paranasal sinuses, bones, lymph nodes and vessels. This paper describes a case of idiopathic facial swelling associated with sickle cell disease in a young patient.

Acute Soft Skull Syndrome in an Adult Male with Sickle Cell Anemia in Sudan: A Case Report

“…14 As such, chronic anemia is linked with elevated cardiac output and increased blood flow velocity, resulting in arterial tortuosity in many cases. 15,16 Acute soft skull syndrome is a rare manifestation of SCD, and the pathogenesis is not clearly understood, although it is believed to be related to infraction of the skull due to expansion of intramedullary hematopoietic tissues leading to disruption of the outer and inner skull margins and in turn resulting in softening of cortical bones. 17 The differential diagnosis of a patient with acute soft head syndrome must include other causes of hematoma, such as trauma (which our patient had no history of), bleeding diathesis (in the present case, the platelet count and bleeding profile were within the normal range), and intracranial pressure (we excluded by the absence of any related features, such as altered level of consciousness, vomiting or visual changes).…”

Acute Soft Head Syndrome in Children with Sickle Cell Anaemia in Lagos, Nigeria

“…In this condition, chronic anaemia leads to an increased need for blood production and results in intra-medullary or extramedullary haematopoiesis [2]. Because of this increased need for blood, chronic anaemia is associated with increased cardiac output and high blood flow velocity, and it may lead to arterial tortuosity [3].…”