Fetal surgery for cystic adenomatoid malformation of the lung

https://doi.org/10.1016/0022-3468(93)90332-FGet rights and content

Abstract

We reviewed our experience with fetal therapy for congenital cystic adenomatoid malformation of the lung (CCAM) at the University of California, San Francisco Fetal Treatment Center. Fetuses with life-threatening CCAM were selected for prenatal treatment according to predetermined guidelines, including the gestational age of the fetus, the size of the intrathoracic lesion, maternal health, and the development of fetal hydrops. The knowledge that fetuses with hydrops are at high risk for fetal or neonatal death led to fetal surgical resection of the massively enlarged pulmonary lobe (fetal lobectomy) in six cases. In the first case, resection was too late, since preoperative labor and maternal preeclampsia could not be reversed, leading to premature delivery of a nonviable infant. In the next four cases, CCAM resection led to resolution of the hydrops, impressive in utero lung growth, and neonatal survival. Right middle and lower lobe resection in the sixth fetus at 21 weeks was successful, but subsequent inexplicable fetal death highlights the need for better postoperative fetal monitoring and treatment. Three other fetuses with a single predominant cyst underwent thoracoamniotic shunt placement alone; two survived after delivery and prompt neonatal surgery with the assistance of high-frequency ventilation or extracorporeal membrane oxygenation. Fetal therapy can now be considered for otherwise fatal space-occupying intrathoracic lesions in the fetus.

References (32)

  • E Morris et al.

    Cystic adenomatoid malformation of the lung: An obstetric and ultrasound perspective

    Eur J Obstet Gynecol Reprod Biol

    (1991)
  • KH Nicolaides et al.

    Chronic drainage of fetal pulmonary cyst

    Lancet

    (1987)
  • RW Jennings et al.

    New techniques in fetal surgery

    J Pediatr Surg

    (1992)
  • JM Roberts et al.

    Preeclampsia: An endothelial cell disorder

    Am J Obstet Gynecol

    (1989)
  • NS Adzick et al.

    Fetal cystic adenomatoid malformation: Prenatal diagnosis and natural history

    J Pediatr Surg

    (1985)
  • NS Adzick et al.

    Compensatory growth after pneumonectomy in fetal lambs: A morphologic study

  • Cited by (0)

    Presented at the 25th Annual Meeting of the Pacific Association of Pediatric Surgeons, Albuquerque, New Mexico, May 17–21, 1992.

    1

    The members of the UCSF Fetal Treatment Center include the following: Program Coordinator: Lori J. Howell, RN, MS; Anesthesiology: Charles B. Cauldwell, MD, PhD, Mark A. Rosen, MD; Neonatology: Joseph A. Kitterman, MD, Augusto Sola, MD; Obstetrics/Genetics: Jane Chueh, MD, Mitchell S. Golbus, MD, James D. Goldberg, MD; Pediatric Surgery: N. Scott Adzick, MD, Alan W. Flake, MD, Michael R. Harrison, MD; Sonography: Peter W. Callen, MD, Roy A. Filly, MD, Ruth B. Goldstein, MD; Obstetrical Nurse Specialis: Maribeth Inturrisi, RN, MS; Operating Room Nurse Specialist: Anne H. Levine, RN; Social Worker: Stephanie A. Berman, LCSW.

    View full text