Fetal lung lesions: Management and outcome,☆☆,

Presented at the Eighteenth Annual Meeting of The Society of Perinatal Obstetricians, Miami Beach, Florida, February 2-7, 1998.
https://doi.org/10.1016/S0002-9378(98)70183-8Get rights and content

Abstract

Objective: Our purpose was to review our experience with fetal congenital cystic adenomatoid malformation and extralobar pulmonary sequestration emphasizing natural history, management, and outcome. Study Design: We conducted a retrospective review of 175 fetal lung lesions diagnosed by antenatal ultrasonography at 2 fetal treatment centers. Results: There were 134 congenital cystic adenomatoid malformation cases. Fourteen women underwent elective abortion, 101 women were managed expectantly, 13 women had fetal surgery, and 6 women had placement of a thoracoamniotic shunt. For the congenital cystic adenomatoid malformation lesions that were not associated with nonimmune hydrops, all babies survived. Of 25 large congenital cystic adenomatoid malformations that had associated hydrops that were followed expectantly, all fetuses died before or shortly after birth. Fetal surgical resection of the tumor (fetal lobectomy) was performed at 21 to 29 weeks’ gestation in 13 hydropic fetuses with 8 fetuses continuing gestation with subsequent hydrops resolution, impressive in utero lung growth, and neonatal survival. Six fetuses with a very large solitary cyst underwent thoracoamniotic shunting and 5 survived. There were 41 extralobar pulmonary sequestration cases. Twenty-eight extralobar pulmonary sequestrations dramatically regressed on serial prenatal sonography, were asymptomatic after birth, and were only detectable by imaging studies postnatally (no resection required). Of the remaining 13 extralobar pulmonary sequestration cases, 2 underwent elective abortion, 7 symptomatic lesions were resected after birth with survival, 1 hydropic fetus died, and 3 fetuses had an associated tension hydrothorax with secondary hydrops that was successfully treated by either fetal thoracenteses or thoracoamniotic shunting followed by postnatal resection. Conclusions: The natural history of prenatally diagnosed lung masses is variable, and associated anomalies are rare. Most congenital cystic adenomatoid malformation lesions can be managed with maternal transport, planned term delivery, and postnatal resection. Many extralobar pulmonary sequestrations dramatically decrease in size before birth and may not need treatment after birth. Fetal therapy is now an option for lung lesions associated with nonimmune hydrops. (Am J Obstet Gynecol 1998;179:884-9.)

Section snippets

Material and methods

Case study review was performed for 175 fetal lung lesion patients (134 congenital cystic adenomatoid malformations and 41 extralobar pulmonary sequestrations) referred to the University of California, San Francisco Fetal Treatment Center from July 1, 1983, to June 30, 1995, and referred to the Center for Fetal Diagnosis and Treatment at the Children’s Hospital of Philadelphia from July 1, 1995, to December 31, 1997. All patients had evaluation of their sonographic findings at the University of

Congenital cystic adenomatoid malformation

There were 134 cases of congenital cystic adenomatoid malformation diagnosed between 17 weeks and 38 weeks’ gestation. Fourteen women underwent pregnancy termination: 12 fetuses had nonimmune hydrops, 1 had multiple structural abnormalities and hydrops, and 1 was otherwise normal. One hundred and twenty women elected to continue their pregnancies after the diagnosis was made: 101 women were managed expectantly, 13 women carrying hydropic fetuses underwent open fetal surgery, and 6 women

Comment

Congenital cystic adenomatoid malformation is characterized by an overgrowth of terminal respiratory bronchioles that form cysts of various sizes. Stocker et al11 defined 3 types of congenital cystic adenomatoid malformation (types I to III) primarily on the basis of cyst size. We have classified prenatally diagnosed congenital cystic adenomatoid malformation into 2 categories on the basis of gross anatomy and ultrasonographic findings.1 Macrocystic lesions contain single or multiple cysts that

Acknowledgements

We gratefully acknowledge the excellent patient care provided by the dedicated multidisciplinary teams at the Center for Fetal Diagnosis and Treatment at the Children’s Hospital of Philadelphia and the Fetal Treatment Center at the University of California, San Francisco.

References (25)

  • NS Adzick et al.

    Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history

    J Pediatr Surg

    (1985)
  • JG Thorpe-Veeston et al.

    Cystic adenomatoid malformation of the lung: prenatal diagnosis and outcome

    Prenatal Diag

    (1994)
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    From The Center for Fetal Diagnosis and Treatment at the Children’s Hospital of Philadelphiaa and The Fetal Treatment Center at the University of California, San Francisco.b

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    Reprint requests: N. Scott Adzick, MD, The Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104.

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