BACKGROUND/ PURPOSE: Congenital pulmonary airway malformations (CPAM) are rare lesions often diagnosed during routine prenatal ultrasound. The presence of hydrops fetalis is an indicator of poor prognosis. Here we present a retrospective review of fetuses undergoing either open fetal surgery or steroids for predominantly microcystic CPAM with hydrops fetalis. METHOD: A retrospective review of patients undergoing open fetal surgery or steroids for CPAM at our institution was performed. The primary outcome was survival. RESULTS: A retrospective review of all patients referred to our institution with the diagnosis of CPAM was performed. Fetuses with predominantly microcystic CPAM and the presence of hydrops fetalis treated with steroid or surgery were included. Thirteen patients were treated with steroids, and 11 patients underwent open fetal surgery. In the steroid group 12 (92%) of 13 fetuses survived to delivery versus 9 (82%) of 11 in the open fetal surgery group. Only 5 (56%) of 9 of the patients in the open fetal surgery group survived to neonatal discharge compared to 10 (83%) of 12 in the steroid group. CONCLUSIONS: In the present retrospective study, improved survival was seen in fetuses with hydrops fetalis and predominantly microcystic CPAM treated with steroids when compared with open fetal surgery. Steroids should be considered for first-line therapy in these cases.
BACKGROUND/ PURPOSE:Congenital pulmonary airway malformations (CPAM) are rare lesions often diagnosed during routine prenatal ultrasound. The presence of hydrops fetalis is an indicator of poor prognosis. Here we present a retrospective review of fetuses undergoing either open fetal surgery or steroids for predominantly microcystic CPAM with hydrops fetalis. METHOD: A retrospective review of patients undergoing open fetal surgery or steroids for CPAM at our institution was performed. The primary outcome was survival. RESULTS: A retrospective review of all patients referred to our institution with the diagnosis of CPAM was performed. Fetuses with predominantly microcystic CPAM and the presence of hydrops fetalis treated with steroid or surgery were included. Thirteen patients were treated with steroids, and 11 patients underwent open fetal surgery. In the steroid group 12 (92%) of 13 fetuses survived to delivery versus 9 (82%) of 11 in the open fetal surgery group. Only 5 (56%) of 9 of the patients in the open fetal surgery group survived to neonatal discharge compared to 10 (83%) of 12 in the steroid group. CONCLUSIONS: In the present retrospective study, improved survival was seen in fetuses with hydrops fetalis and predominantly microcystic CPAM treated with steroids when compared with open fetal surgery. Steroids should be considered for first-line therapy in these cases.
Authors: Angie C Jelin; Nara Sobreira; Elizabeth Wohler; Benjamin Solomon; Teresa Sparks; Katelynn G Sagaser; Katherine R Forster; Jena Miller; P Dane Witmer; Ada Hamosh; David Valle; Karin Blakemore Journal: Prenat Diagn Date: 2020-02-17 Impact factor: 3.050
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: Abigail Wilpers; Anna Y Lynn; Barbara Eichhorn; Amy B Powne; Megan Lagueux; Janene Batten; Mert Ozan Bahtiyar; Cary P Gross Journal: Fetal Diagn Ther Date: 2022-03-10 Impact factor: 2.208