Management of Splenic Sequestration Crisis

Clinical Presentation and Diagnosis

Splenic sequestration is characterized by a rapidly enlarging spleen and a decrease in hemoglobin level of more than 2 g/dL below the patient's baseline value, often presenting with mild to moderate thrombocytopenia 1
Although most common in children with HbSS younger than 5 years and adolescents with HbSC, it may occur at any age in patients with any form of SCD, including adults with sickle cell-hemoglobin C disease or sickle beta thalassemia 1

Prompt recognition and careful administration of red blood cell transfusions are lifesaving interventions, with transfusions of 3 to 5 mg/kg recommended, and post-transfusion hemoglobin checked before the next aliquot of red cells is ordered 1
Care must be taken to avoid acute overtransfusion to a hemoglobin greater than 10 g/dL, as sequestered red cells may be acutely released from the spleen as the event resolves 1
Aggressive hydration is crucial, with careful monitoring of fluid balance to prevent overhydration 2

For severe pain, parenteral opioids such as morphine are indicated and should be administered by scheduled around-the-clock dosing or patient-controlled analgesia 3

Continuous monitoring of vital signs, oxygen saturation, and hemoglobin levels is essential, with baseline oxygen saturation documented, and oxygen therapy administered to maintain SpO2 above baseline or 96% (whichever is higher) 2
Regular assessment by hematology specialists is recommended for patients with splenic sequestration 2

Maintain normothermia, as hypothermia can lead to shivering and peripheral stasis, which increases sickling 4
Thromboprophylaxis should be used for all peri- and post-pubertal patients, and mobilization encouraged when appropriate 4

Surgical splenectomy to prevent recurrence may be recommended after recovery from life-threatening or recurrent episodes, according to the American Academy of Pediatrics 1
Parents and caregivers of young children with SCD should be taught to check their child's spleen daily to recognize enlargement early and urgently seek medical attention 1

Delayed recognition of splenic sequestration can lead to rapid progression to shock and death 1
Overtransfusion can lead to complications when sequestered red cells are released from the spleen 1

Before initiating blood transfusion, administer up to three boluses of 20 mL/kg of crystalloid replacement for initial resuscitation, and blood replacement should only be considered after crystalloid resuscitation, as recommended by the World Journal of Emergency Surgery guidelines 5
While general pediatric transfusion triggers suggest considering transfusion when hemoglobin is less than 7 g/dL, splenic sequestration represents an acute emergency requiring immediate intervention, with a defining feature of a decrease in hemoglobin of more than 2 g/dL below the patient's baseline value 5

Reduction in heart rate, improved mental status, return of peripheral pulses and normal skin color, increase in blood pressure, adequate urinary output, and increased extremity warmth are signs of effective resuscitation 5
If massive transfusion becomes necessary, apply a 1:1:1 ratio of blood products in children, as recommended by the World Journal of Emergency Surgery guidelines 5