Case Number 1
A late preterm infant girl at 36 6/7 estimated gestational age was born
via C-section due to concern for worsening placental insufficiency to a
38yo gravida 3 para 2 mother. APGARS were 3 and 8 at 1 and 5 minutes of
life respectively. Pregnancy was complicated by suspected trisomy 21
(confirmed postnatally), fetal growth restriction, complete AV canal
defect and enlarged liver noted on ultrasound (US) at 34 weeks GA.
Physical exam revealed small for gestational age, upward slanting eyes,
low set ears, and palpable hepatomegaly 4cm below right costal margin.
Infant was transferred to our institution for subspecialty care. Blood
work demonstrated metabolic acidosis, grossly altered coagulation
studies, significant hyperbilirubinemia, thrombocytosis, leukocytosis of
110×103/μL (110 ×109/L) with 15%
peripheral blasts confirmed on hematopathology review as myeloblasts.
Her coagulopathy was treated with fresh frozen plasma and
cryoprecipitate. Pediatric hematology-oncology was consulted for
treatment and management due to persistent severe leukocytosis and
peripheral blasts.
Despite full enteral feeds and the addition of ursodiol, her direct
bilirubin level continued to rise, peaking at 26.7 mg/dL (0.267 g/L)
around 3 weeks of life. Bilirubin slowly improved but, her liver failure
and portal hypertension led to intractable ascites requiring drain
placement, systemic hypotension requiring inotropic support, and
respiratory failure requiring mechanical ventilation. The decision was
made with family for comfort care and the patient passed away at DOL 75.