Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience.

Abstract

UNLABELLED:

Congenital cystic adenomatoid malformation of the lung (CCAM) is diagnosed by prenatal ultrasonography with an increasing frequency but controversy persists as to its prognosis and prenatal management.

METHOD:

A multi-institutional study of cases of CCAM diagnosed antenatally identified by ultrasonographers and by a review of hospital charts.

RESULTS:

We obtained 48 cases from five centers. We estimate the incidence of CCAM at 1:25,000 to 1:35,000 pregnancies. The incidence of voluntary abortions was 15% (7/48), of spontaneous abortions 2% (1/41) and of postnatal death 10% (4/40). One of the postnatal deaths was from trisomy 18. Of the 7 aborted fetuses, 2 had multiple malformations and 1 had severe hydrops and oligohydramnios; the other 4 had a large mass with mediastinal displacement but without hydrops. When pregnancy was allowed to continue, 56% of the lesions regressed spontaneously, even though one third of these had initial progression. In 17 cases (42%) the mediastinal shift corrected itself, sometimes by simple growth of the fetus but most often by a decrease in the size of the lung mass. In 1 fetus, repeated needle decompressions followed by double-pigtail catheter drainage of large cysts allowed regression of hydrops. Despite this, neonatal death occurred from pulmonary hypoplasia.

CONCLUSION:

CCAM can lead to fetal or neonatal demise from hydrops, lung hypoplasia, prematurity or severe associated malformations, but has a good prognosis in the majority of cases.

• Authors: J M Laberge, H Flageole, D Pugash, S Khalife, G Blair, D Filiatrault, P Russo, G Lees, R D Wilson
• Journal: Fetal diagnosis and therapy (Fetal Diagn Ther) 2001 May-Jun Vol. 16 Issue 3 Pg. 178-86 ISSN: 1015-3837 [Print] Switzerland
• PMID: 11316935 (Publication Type: Journal Article, Multicenter Study)
• Copyright: Copyright 2001 S. Karger AG, Basel

Topics

• Abortion, Induced (statistics & numerical data)
• Abortion, Spontaneous (epidemiology)
• Canada (epidemiology)
• Cystic Adenomatoid Malformation of Lung, Congenital (diagnostic imaging, mortality)
• Female
• Humans
• Hydrops Fetalis (diagnostic imaging, mortality)
• Incidence
• Pregnancy
• Pregnancy Outcome
• Retrospective Studies
• Ultrasonography, Prenatal (mortality)