Related Experiment Video
Updated: Jun 24, 2026
Robot-Assisted Laparoscopic Splenectomy In Children: A Case Report with Literature Review
Published on: March 27, 2026
Acute splenic sequestration in a cohort of children with sickle cell anemia
Paulo V Rezende1, Marcos B Viana, Mitiko Murao
1Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
Insights
Acute splenic sequestration (ASS) is common in children with sickle cell anemia, especially before age two. Relapse is frequent, and ASS is a significant cause of death, highlighting the need for improved healthcare management.
Area of Science:
- Pediatric Hematology
- Sickle Cell Disease Management
- Public Health Systems
Background:
- Newborn screening for sickle cell anemia (Hb SS/Sbeta(0)) enables early diagnosis and intervention.
- Acute splenic sequestration (ASS) is a serious complication in children with sickle cell anemia.
- Understanding ASS incidence and outcomes is crucial for optimizing care in resource-limited settings.
Purpose of the Study:
- To analyze the incidence, recurrence, and outcomes of acute splenic sequestration (ASS) in children with sickle cell anemia.
- To evaluate management strategies, including splenectomy, for ASS in pediatric patients.
- To identify the impact of ASS on mortality and assess healthcare system performance in Minas Gerais, Brazil.
Main Methods:
- Retrospective cohort study of 255 children with sickle cell anemia (Hb SS/Sbeta(0)) diagnosed via newborn screening.
- Data collected from medical records for children born between 2000 and 2004, with follow-up until 2006.
- Analysis of ASS episodes, treatment decisions (splenectomy vs. conservative management), and case-fatality rates.
Main Results:
- 173 episodes of ASS occurred in 89 patients; 75% of first episodes happened before age two.
- The cumulative probability of a first ASS episode was 40%, with a recurrence rate of 57.3%.
- ASS was the second leading cause of death (26.3% of deaths), with a case-fatality rate of 7.8% for subsequent episodes.
Conclusions:
- Acute splenic sequestration is a common and recurring complication in pediatric sickle cell anemia, particularly in the first two years of life.
- Conservative management is often preferred over immediate splenectomy, but delays can lead to further episodes and mortality.
- The study highlights fragilities in the Minas Gerais health system and the need for enhanced professional education for managing ASS crises.
Objective:
To analyze acute splenic sequestration (ASS) in children with sickle cell anemia diagnosed through a newborn screening program in the state of Minas Gerais, Brazil, and followed up at the hematology center in the city of Belo Horizonte, Minas Gerais, Brazil.
Methods:
Retrospective cohort of 255 children with sickle cell anemia (Hb SS/Sbeta(0)) born between January 01, 2000, and December 31, 2004, and followed up until December 31, 2006. Data were abstracted from the patients' medical records.
Results:
A total of 89 patients had 173 episodes of ASS (10.2 first episodes per 100 patient-years); 75% of the first episodes occurred before 2 years of age. The estimated probability of occurrence of the first episode of ASS during the study period was 40%. Recurrence rate reached 57.3%. After the first episode, splenectomy was indicated in only 12.4% of the cases; after the second, in 60.4% of the cases. After the third episode, 41.7% of the patients remained under clinical observation. The median time between indication for splenectomy and the actual surgical procedure was 2 months. During the intervening period, 37.2% of the children suffered a new episode of ASS and one child died. Case-fatality rate was 1.1% for the first episode and 7.8% for the subsequent episodes. Among a total of 255 children, 19 died: 36.8% due to infections and 26.3% after ASS.
Conclusions:
ASS is relatively common in sickle cell anemia, mainly in the first 2 years of life; relapse occurs in more than half of the cases. Conservative management instead of immediate splenectomy was the method of choice. Although the case-fatality rate was low, ASS was the second most common cause of death. These results disclose some fragilities of the health system in the state of Minas Gerais and the need for better professional education to approach ASS crises.
