Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy,☆☆

Presented at the 33rd Annual Meeting of the American Pediatric Surgical Association, Phoenix, Arizona, May 19-23, 2002.
https://doi.org/10.1053/jpsu.2003.50089Get rights and content

Abstract

Background/Purpose: Development of hydrops fetalis in fetuses with congenital cystic adenomatoid malformations (CCAM) is a significant risk factor for fetal or neonatal demise. In rare cases, resolution of CCAM has occurred, presumably owing to lesion maturation or involution. Steroid therapy, utilized for lung maturity, has been postulated to accelerate this process. The natural history of hydropic fetuses with CCAM after receiving steroid therapy is presented. Methods: The authors prospectively followed up with 3 patients who had antenatally diagnosed CCAM and nonimmune hydrops fetalis with predicted mortality. All patients declined or were not candidates for fetal intervention and were treated with standard prenatal betamethasone to increase lung maturity. Results: Three fetuses had CCAM and nonimmune hydrops fetalis diagnosed prenatally. After a course of prenatal steroids during the second trimester, all 3 patients had resolution of their hydrops and were delivered at term without respiratory distress. Conclusions: Nonimmune hydrops fetalis in fetuses with congenital cystic adenomatoid malformation is a harbinger for fetal demise. The resolution of hydrops in these patients after receiving steroid therapy is an interesting and compelling observation. Because the mechanism of this process is speculative, further studies are needed to elucidate the relationship between antenatal steroids and maturation of congenital cystic adenomatoid malformation. J Pediatr Surg 38:508-510. Copyright 2003, Elsevier Science (USA). All rights reserved.

Section snippets

Materials and methods

The fetal treatment and diagnosis database for the Fetal Treatment Center at the University of California, San Francisco was reviewed for all mothers carrying fetuses with CCAM. Between 1997 and 2000, 3 women carrying fetuses with prenatally diagnosed CCAM were identified. All 3 fetuses exhibited signs of nonimmune hydrops fetalis based on antenatal sonograms at time of referral. Each woman was given a standard regimen of prenatal steroids (betamethasone, 12 mg intramuscularly for 2 doses, 24

Results

Patient 1 presented with a right-sided CCAM. Prenatal sonograms showed the lesion to be large and predominantly solid. At 23 3/7 weeks' gestation, the fetus exhibited sign of NIHF including severe mediastinal shift, mild pleural effusion, small ascites, mild scalp edema, and inverted diaphragm. A prenatal course of betamethasone was administered at 24 weeks' gestation. Follow-up sonograms showed resolution of ascites, pleural effusion, and diaphragmatic inversion by 27 4/7 weeks' gestation. The

Discussion

Advances in prenatal imaging have elucidated the natural progression of CCAM.7 However, the embryo- and pathogenesis of CCAM are unknown. CCAM represents an abnormality in lung development characterized by the lack of normal alveoli and the abnormal proliferation of terminal respiratory bronchioles.12 Some have speculated that CCAM represents an arrested state of normal lung development which has been characterized by increased cell proliferation and decreased apoptosis.13

Lung development is a

References (24)

  • NS Adzick et al.

    Fetal cystic adenomatoid malformation: Prenatal diagnosis and natural history

    J Pediatr Surg

    (1985)
  • T Taguchi et al.

    Antenatal diagnosis and surgical management of congenital cystic adenomatoid malformation of the lung

    Fetal Diagn Ther

    (1995)
  • Cited by (0)

    This study was partially funded by the Sean P. Nicholson Foundation.

    ☆☆

    Address reprint requests to Hanmin Lee, MD, The Fetal Treatment Center, University of California, San Francisco, 513 Parnassus Ave, HSW-1601, San Francisco, CA 94143-0570.

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