Abstract
We report a preterm (35 4/7 weeks) male neonate with Down's syndrome (DS) diagnosed with isolated pericardial
effusion (PE) at 20 weeks of gestation. He was born by precipitous delivery, needed
no resuscitation and presented within first 24 hours of life with respiratory distress,
anemia due to feto-maternal bleed, hypotension, hepatomegaly, and coagulopathy. Postnatal
echocardiography confirmed a 5 mm rim of PE without tamponade, normal cardiac structure,
and function. He was stabilized with ventilation, packed red cell, fresh frozen plasma,
inotropes (dopamine, dobutamine, and adrenaline), and steroid (hydrocortisone). Subsequent
evaluation confirmed hypothyroidism, transient myeloproliferative disorder (TMD),
hepatic failure due to fibrosis/cirrhosis with portal hypertension, and steroid sensitive
hypotension on two occasions possibly due to adrenal insufficiency. PE completely
resolved over 2 weeks. In view of progressively worsening liver failure with ascites
and portal hypertension, the family opted for palliation. Literature review has been
discussed regarding perinatal onset of PE in DS.
Keywords
Down's syndrome - hypothyroidism - pericardial effusion - steroid - transient myeloproliferative
disorder