Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone

S. C. Derderian, A. M. Coleman, C. Jeanty, F. Y. Lim, A. M. Shaaban, J. A. Farrell, Shinjiro Hirose, T. C. Mackenzie, H. Lee

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Background/purpose Congenital pulmonary airway malformations (CPAMs) are rare congenital lung lesions often diagnosed by prenatal ultrasound. High-risk cases can result in hydrops and prenatal or postnatal demise. Antenatal betamethasone has resulted in improved survival but it is unclear how to manage patients who do not respond to a single course. Methods We present a bi-institutional retrospective review of patients treated with multiple courses of prenatal steroids for high-risk CPAMs between 2007 and 2013. Results Nine patients met inclusion criteria. All but one either had an increased CPAM volume ratio (CVR) or number of fluid-containing compartments involved after a single course of antenatal betamethasone, prompting additional courses. Four patients stabilized, three improved and two progressed after the second course. The two cases with disease progression underwent an in utero resection. There were one in utero fetal demise and two deaths within the delivery room. Both fetuses that underwent a fetal resection died. All but one mother who delivered a viable fetus had complications of pregnancy. Conclusions Multiple courses of antenatal betamethasone for high-risk fetal CPAMs often result in favorable short-term outcomes without the need for open fetal resection. Pregnancy complications are common and women within this cohort should be monitored closely.

Original languageEnglish (US)
Pages (from-to)515-518
Number of pages4
JournalJournal of Pediatric Surgery
Volume50
Issue number4
DOIs
StatePublished - Jan 1 2015
Externally publishedYes

Fingerprint

Betamethasone
Mothers
Lung
Pregnancy Complications
Fetus
Delivery Rooms
Fetal Death
Disease Progression
Edema
Steroids
Survival

Keywords

  • Congenital cystic adenomatous malformation CCAM
  • Congenital pulmonary airway malformation
  • CPAM
  • Maternal betamethasone
  • Prenatal steroids

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone. / Derderian, S. C.; Coleman, A. M.; Jeanty, C.; Lim, F. Y.; Shaaban, A. M.; Farrell, J. A.; Hirose, Shinjiro; Mackenzie, T. C.; Lee, H.

In: Journal of Pediatric Surgery, Vol. 50, No. 4, 01.01.2015, p. 515-518.

Research output: Contribution to journalArticle

Derderian, S. C. ; Coleman, A. M. ; Jeanty, C. ; Lim, F. Y. ; Shaaban, A. M. ; Farrell, J. A. ; Hirose, Shinjiro ; Mackenzie, T. C. ; Lee, H. / Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone. In: Journal of Pediatric Surgery. 2015 ; Vol. 50, No. 4. pp. 515-518.
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