Abstract
A 34-year-old multiparous woman with a breech presentation, intrauterine growth restriction
and premature rupture of membranes was transferred to our referral unit at 33 weeks of gestation. She was diagnosed with Alagille syndrome soon after birth because
of cholestasis and pruritus. Her condition was later complicated by esophageal varices,
treated with propranolol, thrombocytopenia, and insulin-dependent diabetes. She had
characteristic facies, posterior embryotoxon, “butterfly” vertebrae but had no cardiac
or renal abnormalities. Due to the early onset of spontaneous labor, emergency cesarean
section under general anesthesia was performed 48 h after admission. This is the first case describing anesthetic care during delivery
in a patient with Alagille syndrome. We discuss the anesthetic implications of the
syndrome, emphasizing problems associated with portal hypertension and cholestasis,
thrombocytopenia and cardiac abnormalities such as pulmonary artery stenosis.
Keywords
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Article info
Publication history
Published online: September 19, 2011
Accepted:
July 21,
2011
Identification
Copyright
© 2011 Elsevier Ltd. Published by Elsevier Inc. All rights reserved.
