Obstetrician patterns of steroid administration for the prenatal management of congenital pulmonary airway malformations

Abstract

BACKGROUND: Congenital pulmonary airway malformation (CPAM) is the most common prenatally-diagnosed lung malformation. This lesion, classified as macrocystic or microcystic, can lead to significant fetal compromise. Management options include observation, maternal antenatal steroid administration, and fetal surgical intervention. Current evidence suggests that microcystic (but not macrocystic) lesions and those with a cyst volume ratio (CVR) >1.6 are responsive to steroid therapy. The objective of this study was to identify patterns of prenatal steroid administration for the management of CPAMs and to identify characteristics of CPAMs prompting steroid administration. METHODS: An 18-question survey was distributed to obstetricians from the Pregnancy-Related Care Research Network (PRCRN) and the North American Fetal Therapy Network (NAFTNet), from January to April 2019, to capture antenatal steroid prescribing patterns. RESULTS: Response rates were 28.3% (138/487) for PRCRN and 63.3% (19/30) for NAFTNet. Among PRCRN members, 16.8% administered prenatal steroids, with most (77.2%) doing so for both microcystic and macrocystic CPAMs; corresponding percentages for NAFTNet members were 90.9% and 52.6%. Two thirds (65.6%) of obstetricians who administer steroids do so for a CVR > 1.6, without evidence of mediastinal shift or hydrops fetalis. CONCLUSIONS: There is a lack of consensus among obstetricians as to the CPAM characteristics that should prompt administration of prenatal steroids. Many surveyed obstetricians do not use cyst type or CVR to guide decision-making regarding steroid therapy.

Clinical Image Feature Analysis and Diagnostic Efficacy Evaluation of Pulmonary Ultrasound in the Diagnosis of Congenital Pulmonary Airway Malformations in Children: Based on a Single Center, Retrospective Cohort Study

“…e timing of CT examination for prenatal diagnosis of CPAM and asymptomatic children after birth is still controversial. Some suggest that enhanced CT should be done before 6 months, and some should be completed before operation [17][18][19][20].…”

The optimal timing of surgery for asymptomatic congenital pulmonary airway malformation in children: a meta analysis

“…The difference of Thyroid Transcription Factor 1, Fibroblast Growth Factor 7, Fibroblast Growth Factor 9, Clara cell marker 10, SOX 2, SOX10, COL2A1, Hoxb-5, hMSH-2 and other transcription factors and expression appear to play a role in the pathogenesis of CPAM 16,17 .In addition, the positive rate of Transcription Factor 1 in CPAM type I, II, and III is significantly different, and it can be used as an important marker for CPAM tissue typing 18 .In severe cases, the lesion continues to grow and ultimately may cause severe lung compression, mediastinal shift, hydrops fetalis, and/or fetal demise. Management options include observation, maternal antenatal steroid administration, and fetal surgical intervention 19,20 {Aziz, 2021 #37;Loh, 2012 #38;Aziz, 2021 #35}.In children with symptoms after birth, it is recommended to surgically remove the lesions as soon as possible. For asymptomatic children with prenatal diagnosis of CPAM, most experts still recommend early elective surgery, but the exact timing of surgery is still controversial.…”

Innovative Fetal Therapy for a Giant Congenital Pulmonary Airway Malformation with Hydrops

“…In our patient, maternal corticosteroids were given intramuscularly as some studies have suggested that this medication could be helpful to promote the reduction of fetal microcystic CPAM with minimal maternal harm and survival benefit to the fetus [24] (cite in comment). There is no consensus in the maternal-fetal community regarding the use of steroids, and both macro- and microcystic lesions may prompt the therapy when the CVR is 1.6 or higher [25].…”