Acute splenic sequestration crisis in children with Sickle Cell Disease

OBJECTIVE: The aim of this study was to demonstrate the clinical experience with acute splenic sequestration crisis in children with sickle cell disease, followed in Madina Maternity & Children's Hospital, Madina Al-Munawara, Kingdom of Saudi Arabia.

METHODS: A retrospective review of hospital case notes of all children with acute splenic sequestration in sickle cell disease, was carried out in the Pediatric Hematology unit at Madina Maternity & Children's Hospital between 1993 through to 2000.

RESULTS: One hundred and twenty children with sickle cell disease were registered and followed in Madina Maternity & Children's Hospital. Out of these, 8 had acute splenic sequestration crisis with a prevalence of 7%. Seven were Saudi and one was non-Saudi (Sudanese), 7 had sickle cell anemia and one had Sickle b-Thalassemia. The female to male ratio was 3:1, 2 patients presented with associated painful crisis. In 50% of the patients, the spleen was not palpable before the attack of acute splenic sequestration crisis. All patients had major splenic sequestration with circulatory collapse, 4 patients (50%) had recurrence and 3 (37.5%) had splenectomy carried out at the age of 2 years. The steady state hematological data did not show any risk factor for acute splenic sequestration crisis and none of our patients died.

CONCLUSION: We conclude that acute splenic sequestration crisis is of relatively high prevalence in the western region of the Kingdom of Saudi Arabia, and is of severe type. Management measures recommended are, prevention of sickle cell disease through health education, reduction of consanguineous marriage, implementation of premarital and neonatal screening programs for hereditrary blood disease, regular follow-up and education of parents to palpate the spleen in an established sickle cell case.