Alagille syndrome and pregnancy: anesthetic management for cesarean section.

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.

journal_name

Int J Obstet Anesth

authors

Rahmoune FC,Bruyère M,Tecsy M,Benhamou D

doi

10.1016/j.ijoa.2011.07.012

subject

Has Abstract

pub_date

2011-10-01 00:00:00

pages

355-8

issue

4

eissn

0959-289X

issn

1532-3374

pii

S0959-289X(11)00089-6

journal_volume

20

pub_type

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