Background Sickle cell disease (SCD) is a common inherited hemoglobin disorder with significant clinical impact worldwide. In Saudi Arabia, its prevalence varies by region, reaching up to 1.2% in the Eastern Province and 0.8% in the Southwest.

Acute splenic sequestration (ASS) remains a major cause of morbidity and mortality in young children with SCD. It is defined by sudden splenic enlargement, a drop in hemoglobin >2 g/dL from baseline, and reticulocytosis, often triggered by infections. ASS typically occurs between 3 months and 5 years of age, with highest risk in HbSS genotypes. Clinical signs include abdominal distension, pallor, tachycardia, and lethargy. Recurrence is common, and management includes rapid stabilization with transfusion. However, there is no consensus on long-term preventive strategies.

This study aims to describe the prevalence, clinical presentation, and outcomes of ASS in pediatric SCD patients in a single center in Saudi Arabia, and to propose a context-specific management approach.

Methods This retrospective cohort study was conducted at Alaziziyah Children Hospital, Jeddah, between January 2022 and october 2024. It included pediatric patients with sickle cell disease who developed acute splenic sequestration. Data were collected using a structured form and included demographics, prior episodes of ASS, clinical presentation, hemoglobin electrophoresis, spleen size (clinical and ultrasound), laboratory results, treatment received, splenectomy status, hospital stay duration, complications, and outcomes.

Results A total of 444 children with SCD were enrolled in the study, of whom 37 had acute splenic sequestration (ASS), yielding a prevalence of 8.3%. Among patients with ASS, 54.1% were aged less than 3 years, with a mean age of 3.24 ± 1.19 years. More than half of them (59.5%) had a prior history of splenic sequestration. The most common clinical features included fever (51.4%), sudden splenic enlargement (48.6%), jaundice (37.8%), infection (35.1%), and pallor (27%).

The mean Spleen size during clinical examination was 6.09 ± 2.94 cm and the mean previous US abdomen finding (spleen size) was 10.7 ± 2.07 cm. As for management, all patients received fluids and blood transfusion. Only 16.2% had oxygen supply, 45.9% had antipyretics, 56.8% had antibiotics and 16.2% had analgesics. Only one patient (2.7%) underwent splenectomy and 43.2% had a hospital stay more than 3 days with a mean duration of hospital stay of 3.46 ± 1.59 days. All patients had a good prognosis.

The prevalence of previous splenic sequestration was significantly higher among patients who presented with jaundice (54.5% vs. 13.3%) or sudden splenic enlargement (68.2% vs. 20%) (p=<0.05). On the other hand, a non-significant association was found between previous splenic sequestration and all patients' demographics, other clinical presentation or hemoglobin electrophoresis types (p=>0.05).

Patients who had recurrent episodes of splenic sequestration had significantly larger spleen size (7.25 ± 3.03 vs. 4.4 ± 1.84 cm), higher mean Hb level (6.29 ± 1.02 vs. 5.178 ± 1.35 g/dl) and higher mean total Bilirubin level (3.74 ± 3.13 vs. 2.05 ± 0.72 (p=<0.05). additionally, these patients were less likely to recieve antibiotics (40.9% vs, 80%; p=<0.05).

59.5% had previous splenic sequestration. A pervious study found that 35.4% had at least one episode of SSC.

Conclusion Acute splenic sequestration occurred in 8.3% of children with SCD, mostly below the age of three. Fever, jaundice, and sudden splenic enlargement were the most common clinical features. Prior sequestration was reported in 59.5% and was significantly associated with larger spleen size and higher bilirubin and hemoglobin levels. All patients recovered after receiving fluids and transfusions, with no reported complications or deaths. These findings support the importance of early recognition, regular spleen monitoring, and caregiver education to prevent recurrence and optimize management.

This content is only available as a PDF.
2025
Sign in via your Institution