Effect of Maternal Betamethasone Administration on Prenatal Congenital Cystic Adenomatoid Malformation Growth and Fetal Survival

Abstract

Objective: To evaluate the effect of prenatal steroid treatment on the growth of congenital cystic adenomatoid malformations (CCAM) and survival in affected fetuses not amenable to other percutaneous ultrasound-guided prenatal interventions. Methods: A retrospective review of patients with a CCAM or hybrid lesion treated with two maternal prenatal betamethasone injections was performed. Patients receiving cyst aspiration or thoracoamniotic shunting at the time of or after steroid administration were excluded. Growth rates and survival data were compared to historical non-steroid treated controls. Results: Eleven patients were treated with prenatal steroids (10 microcystic and 1 macrocystic). Survival was 100% in fetuses with hydrops (5/5) or a CCAM volume ratio (CVR) >1.6 (7/7) at the time of steroid administration. This compares to a mortality of 100 and 56.2% respectively in historical non-treated controls. Resolution of hydrops was seen in 80% (4/5) of steroid-treated patients. CCAM growth rates were variable after steroid administration. However, when compared to historical data where CVR and CCAM volume have been documented to increase until 28 weeks’ gestation, the CVR and CCAM volume growth rates decreased in 72.73% and 50% of patients respectively from the time of steroid administration to 28 weeks’ gestation. Conclusions: In the fetus with a CCAM, the presence of hydrops fetalis or a CVR >1.6 is indicative of poor fetal outcome without prenatal intervention. The observed effect of antenatal steroid treatment on CCAM growth is variable, but its potential to improve survival in these high-risk groups is encouraging and warrants further controlled evaluations.

Genetic Analysis of Congenital Cystic Adenomatoid Malformation Reveals a Novel Pulmonary Gene: Fatty Acid Binding Protein-7 (Brain Type)

“…Patients with CCAM and nonimmune hydrops fetalis with predicted mortality did not undergo fetal intervention, and were treated instead with prenatal betamethasone to increase lung maturity. After a course of prenatal steroids during the second trimester, patients had resolution of hydrops and were delivered without respiratory distress in most cases (28,29). The exact mechanism of the improvement in these patients' pulmonary status and resolution of CCAM after steroid administration is unknown.…”

Fetal Pleural Effusion with Supraventricular Tachycardia; Coincidence? Cause?

“…Viseral ve pariyetal duvardan plevral boşluğa filtre edilen sıvı lenfatik sistem tarafından absorbe edilir. Pulmoner venöz hidrostatik basınçta veya lenfatik basınçta artış, kan onkotik basıncında azalma, lokal doku travması veya inflamasyon plevral efüzyonla sonuçlanır (4) (1). Asemptomatikten solunum yetmezliğine kadar değişen farklı klinik seyri olabilir.…”

“…Fetal plevral efüzyon primer veya sekonder olarak tüm gebelerin 1:10,000-1:15,000'inde görülen nadir bir durumdur (1). İzole olabileceği gibi immün ya da nonimmün hidropsun bir parçası olarak da ortaya çıkabilir.…”

“…Bu olgularda neden sıkılıkla ortaya konamaz. Akciğer kaynaklı nedenler primer lenfanjiektazi, konjenital kistik adenomatoid malformasyon, bronkopulmoner displazi, diafragmatik hernidir (1,2). Akciğer dışı nedenler arasında kromozomal anomaliler, anormal karyotip; Down sendromu, Turner sendrom, genetik sendromlar; Noonan, Sialidosis, enfeksiyonlar; konjenital TORCH, Parvovirus enfeksiyonları, metabolik hastalıklar; lizozomal, glikojen depo hastalıkları, hematolojik; nonimmün ya da immün hemolitik anemiler yer alır.…”

Congenital Malformations and Developmental Anomalies of the Lung

“…Large macrocystic CPAMs have been successfully treated with throacoamniotic shunting [34]. Maternal betamethasone therapy is useful in predominantly solid lesions with hydrops [35]. Patients who do not respond may be candidates for fetal surgery (at \30 weeks).…”