High-risk fetal congenital pulmonary airway malformations have a variable response to steroids.
OBJECTIVE
Anecdotal reports suggest that maternal steroids may arrest the growth of congenital pulmonary airway malformations (CPAMs), preventing or reversing hydrops. We reviewed our experience with CPAMs to determine the fetal response to steroid therapy.


METHODS
This study is a retrospective review of all fetal CPAMs from 2004 to 2008. Fetuses with high-risk CPAMs that received at least one course of steroids were identified. Fetal magnetic resonance imaging and ultrasound data were used to classify the CPAMs, identify hydrops fetalis and follow the fetuses poststeroid dosing.


RESULTS
Forty-four fetuses with CPAM were identified. Fifteen patients were found to have received at least one course of steroids. Thirteen were hydropic and 2 were nonhydropic. Seven of the 13 hydropic fetuses (54%) showed an initial response to steroid administration, whereas the 2 nonhydropic high-risk fetuses progressed to birth without developing hydrops. Seven of the 15 patients, however, resulted in fetal demise or early postnatal death, giving a survival rate of 53%.


CONCLUSIONS
High-risk CPAMs have a variable response to steroids. This variable response demonstrates the need for a placebo-controlled randomized study to more accurately determine the effect of steroids on hydrops and CPAM growth rates. Repeated steroid courses may not be helpful, and progression in CPAM volume to head circumference ratio (CVR) or hydrops should prompt open fetal surgery to prevent irreversible fetal insult.
Morris
Lee M
LM
The Fetal Care Center of Cincinnati, Division of Pediatric General Surgery, Cincinnati Children's Hospital, OH 45229-3039, USA.
Lim
Foong-Yen
FY
Livingston
Jeffrey C
JC
Polzin
William J
WJ
Crombleholme
Timothy M
TM
D001623:Betamethasone / Q000627:therapeutic use*; D016009:Chi-Square Distribution; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000188:drug therapy*; D005260:Female; D005315:Fetal Diseases / Q000188:drug therapy*; D006801:Humans; D015160:Hydrops Fetalis / Q000188:drug therapy*; D011247:Pregnancy; D012189:Retrospective Studies; D016896:Treatment Outcome
D001623:Betamethasone