Prenatal diagnosis and fetal therapy of congenital cystic adenomatoid malformation type I of the lung: a report of five cases

• Published in: Congenital anomalies 2003-03, Vol.43 (1), p.72-78
• Main Authors: Morikawa, Mamoru, Yamada, Hideto, Okuyama, Kazuhiko, Kato, Emi Hirayama, Watari, Michiko, Kataoka, Soromon, Cho, Kazutoshi, Minakami, Hisanori
• Format: Article
• Language: English
• Subjects: Adult; congenital cystic adenomatoid malformation; Cystic Adenomatoid Malformation of Lung, Congenital - diagnosis; Cystic Adenomatoid Malformation of Lung, Congenital - diagnostic imaging; Cystic Adenomatoid Malformation of Lung, Congenital - genetics; Cystic Adenomatoid Malformation of Lung, Congenital - therapy; Female; Fetal Diseases; fetal therapy; Humans; Infant, Newborn; Lung - abnormalities; Lung Diseases - therapy; Magnetic Resonance Imaging; Male; Pregnancy; Prenatal Care; Prenatal Diagnosis; thoracoamniotic shunt; Time Factors; Ultrasonography
• ISSN: 0914-3505; 1741-4520
• DOI: 10.1111/j.1741-4520.2003.tb01029.x

ABSTRACT  We experienced five pregnancy cases with type I congenital cystic adenomatoid malformation (CCAM) of fetuses and summarized here their clinical characteristics, pregnancy outcomes, and fetal therapies. Four of five cases were prenatally diagnosed using magnetic resonance imaging (MRI) as having lung abnormality, and the remaining case was prenatally diagnosed as having congenital diaphragmatic herniation (CDH). One fetus underwent the puncture of cysts in the lung, and two fetuses received in utero thoracoshunts between cysts and the amniotic fluid cavity (thoracoamniotic shunt). One pregnancy ended in artificial termination at 17 gestational weeks, and 4 pregnancies succeeded in live births. All these 4 infants underwent surgical operations for CCAM, and 1 infant underwent an additional operation for CDH. The MRI examinations were useful to prenatally identify CCAM, and the in utero thoracoamniotic shunt appears to be beneficial in preventing lung hypoplasia in the affected fetuses.