Achalasia is rare motor disorder of esophageal smooth muscle. It has been l
inked to malnutrition during pregnancy leading to maternal and fetal mortal
ity. We report a case of achalasia with intrauterine fetal death who succee
ded in following pregnancy with good fetal and maternal outcome after opera
tive treatment. A 34-year-old pregnant woman had intrauterine fetal death a
t 27 gestational weeks due to severe and persistent maternal malnutrition d
uring pregnancy. Achalasia was diagnosed postpartum and myotomy-fundoplasty
was performed. She succeeded in normal delivery with a healthy baby 21 mon
ths after the operation. This case suggests the effect of severe achalasia
of early onset on poor fetal outcome, and the efficacy of surgical myotomy
for the improvement of following pregnant outcome.