BACKGROUND/ PURPOSE: 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.
BACKGROUND/ PURPOSE: 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.
Authors: Rachel Shulman; Teresa N Sparks; Kristen Gosnell; Cinthia Blat; Mary E Norton; Hanmin Lee; Juan Gonzalez-Velez; Ruth B Goldstein Journal: Am J Perinatol Date: 2018-09-10 Impact factor: 1.862
Authors: Cynthia D Downard; Casey M Calkins; Regan F Williams; Elizabeth J Renaud; Tim Jancelewicz; Julia Grabowski; Roshni Dasgupta; Milissa McKee; Robert Baird; Mary T Austin; Meghan A Arnold; Adam B Goldin; Julia Shelton; Saleem Islam Journal: Pediatr Surg Int Date: 2017-06-06 Impact factor: 1.827
Authors: S Christopher Derderian; Cerine Jeanty; Shannon R Fleck; Lily S Cheng; Shabnam Peyvandi; Anita J Moon-Grady; Jody Farrell; Shinjiro Hirose; Juan Gonzalez; Roberta L Keller; Tippi C MacKenzie Journal: J Pediatr Surg Date: 2014-10-29 Impact factor: 2.545