William H Peranteau1, Matthew M Boelig2, Nahla Khalek2, Julie S Moldenhauer2, Juan Martinez-Poyer2, Holly L Hedrick2, Alan W Flake2, Mark P Johnson2, N Scott Adzick2. 1. The Center for Fetal Diagnosis and Treatment at the Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States. Electronic address: peranteauw@email.chop.edu. 2. The Center for Fetal Diagnosis and Treatment at the Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, United States.
Abstract
PURPOSE: Administration of maternal betamethasone (BMZ) is a therapeutic option for fetuses with large microcystic congenital lung lesions at risk for, or causing, hydrops. Not all fetuses respond to a single course of BMZ. We review our experience with the use of single and multiple courses of maternal BMZ for the management of these patients. METHODS: A retrospective review of fetuses with congenital lung lesions managed with maternal BMZ from 2003 to 2014 was performed. RESULTS: Forty-three patients were managed with prenatal steroids (28 single course, 15 multiple courses). Single course recipients demonstrated a reduction in lesion size and resolution of hydrops in 82% and 88% of patients respectively compared to 47% and 56% in recipients of multiple steroid courses. Survival of multiple course patients (86%) was comparable to that of single course patients (93%) and improved compared to non-treated historical controls. Multiple course recipients demonstrated an increased need for open fetal surgery and postnatal surgery at a younger age. CONCLUSION: Fetuses who fail to respond to a single course of BMZ may benefit, as indicated by hydrops resolution and improved survival, from additional courses. However, failure to respond is indicative of a lesion which may require fetal or immediate neonatal resection.
PURPOSE: Administration of maternal betamethasone (BMZ) is a therapeutic option for fetuses with large microcystic congenital lung lesions at risk for, or causing, hydrops. Not all fetuses respond to a single course of BMZ. We review our experience with the use of single and multiple courses of maternal BMZ for the management of these patients. METHODS: A retrospective review of fetuses with congenital lung lesions managed with maternal BMZ from 2003 to 2014 was performed. RESULTS: Forty-three patients were managed with prenatal steroids (28 single course, 15 multiple courses). Single course recipients demonstrated a reduction in lesion size and resolution of hydrops in 82% and 88% of patients respectively compared to 47% and 56% in recipients of multiple steroid courses. Survival of multiple course patients (86%) was comparable to that of single course patients (93%) and improved compared to non-treated historical controls. Multiple course recipients demonstrated an increased need for open fetal surgery and postnatal surgery at a younger age. CONCLUSION: Fetuses who fail to respond to a single course of BMZ may benefit, as indicated by hydrops resolution and improved survival, from additional courses. However, failure to respond is indicative of a lesion which may require fetal or immediate neonatal resection.
Authors: Jennifer Pogoriler; Daniel Swarr; Portia Kreiger; N Scott Adzick; William Peranteau Journal: Am J Surg Pathol Date: 2019-01 Impact factor: 6.394
Authors: John S Riley; John W Urwin; Edward R Oliver; Beverly G Coleman; Nahla Khalek; Julie S Moldenhauer; Susan S Spinner; Holly L Hedrick; N Scott Adzick; William H Peranteau Journal: J Pediatr Surg Date: 2017-11-16 Impact factor: 2.545
Authors: Niamh C Adams; Teresa Victoria; Edward R Oliver; Julie S Moldenhauer; N Scott Adzick; Gabrielle C Colleran Journal: Pediatr Radiol Date: 2020-11-30
Authors: Natalie Divjak; Sabine Vasseur Maurer; Eric Giannoni; Yvan Vial; Anthony de Buys Roessingh; Barbara E Wildhaber Journal: Front Pediatr Date: 2017-12-04 Impact factor: 3.418
Authors: Ingrid Anne Mandy Schierz; Mario Giuffrè; Ettore Piro; Maria Clara Leone; Giuseppa Pinello; Giovanni Corsello Journal: Ital J Pediatr Date: 2018-08-22 Impact factor: 2.638
Authors: Jane E Gross; Michael Y McCown; Caroline Okorie; Lara C Bishay; Fei J Dy; Jordan S Rettig; Christopher D Baker; John R Balmes; Andrew T Barber; Sourav K Bose; Alicia Casey; Stephen M M Hawkins; Alexandra Kass; Garrett Keim; Nadine Mokhallati; Gregory Montgomery; William H Peranteau; Ryan Serrano; Timothy J Vece; Nadir Yehya; Debra Boyer; Margaret M Hayes Journal: ATS Sch Date: 2020-12-30