Chief Complaint: Fever
HPI:
15-month-old female presents to ED with complaint of fever. She has a history of HgbSS and has had fever for 24 hours. Mom denied cough, congestion, vomiting, diarrhea, rash. No known sick contacts. Decreased po today but normal urine output.
Vitals:
T 103.3 R HR 147 Resp 32 PO2 99% RA (no BP available)
PE (as documented in the record):
General: very well appearing, playful with crusty nasal discharge
Chest: CTA, no wheezing/rales/crackles, no tachypnea
Abdomen: soft, NT, ND, normal bowel sounds.
Skin: normal color, warm, dry
Plan:
CBC, BCx, Retic, CMP
Results:
WBC 25.5 Hgb 5.1 Hct 15.9 Plt 94
Retic: 27.7 %
With this information alone, what is the diagnosis? What is the next diagnostic test? Plan?
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Look at the age..... then consider the PMHx
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Now look back at the labs
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In a 15 month old with Sickle Cell Disease, fever and anemia with thrombocytopenia, the diagnosis is Splenic Sequestration until proven otherwise.
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Did you get the diagnosis?
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Plan:
US Spleen
IV antibiotics
Type and Screen
Heme Onc Consult
Acute Splenic Sequestration Crisis is a life-threatening complication seem most commonly in infant with sickle cell under 4 years of age. These patients may rapidly progress to cardiovascular collapse and death.
Remember to feel for a large spleen (organomegaly).
Patients who present with severe Acute Splenic Sequestration Crisis often have massive splenomegaly, tachycardia and hypovolemia, hemoglobin levels below baseline, reticulocytosis and thrombocytopenia
Treatment: IV hydration and urgent transfusion to approach baseline hemoglobin. Do not over transfuse as cells trapped in the spleen often recirculate after transfusion (called reperfusion syndrome) and the hemoglobin level may then increase.
Recurrences are common: 50-90%
Splenectomy is recommended after a second episode of Acute Splenic Sequestration Crisis.
Parents may be quite proficient at splenic palpation. Their complaint of newly palpable or enlarged spleen should be taken seriously!
Main Learning Points:
Most children auto-infarct their spleen by age 5.
Within 1-2 hours the child's spleen can hold an extra 2 points of hemoglobin.
Parents advised to check spleen daily when they are little.
Splenic sequestration occurs between 5m - 2 yrs, median 18 months.
RBCs flow into, but not out of, the spleen, they pool and sickle. Rapidly enlarging spleen and an acute drop of hemoglobin by 2 gm/dL.
Splenic sequestration can result in shock and death.
Treatment:
Treat shock
Transfuse RBCs, to get Hgb 7-9 gm/dL
Beware of reperfusion syndrome, occurs hours to 1 day after resuscitation. The spleen, if salvageable, relaxes and can auto transfuse back up to 2-3 gm/dL.
Splenectomy done to prevent recurrence.
If untreated, up to 50% recurrence rate.
PODCAST below with a great overview of Sickle Cell Complication:
https://podcasts.apple.com/us/podcast/pediatric-emergency-playbook/id1035668219?i=1000549638324