Fetal case of congenital cystic adenomatoid malformation of the lung: Fetal therapy and a review of the published reports in Japan

Authors: Asabe, Koushi; Oka, Yoichiro; Shirakusa, Takayuki¹

Source: Congenital Anomalies, Volume 45, Number 3, September 2005 , pp. 96-101(6)

Publisher: Blackwell Publishing

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Abstract:

We herein report a case of type I congenital cystic adenomatoid malformation of the lung (CCAML) with non-immune hydrops fetalis (NIHF), a mediastinal shift and polyhydramnios diagnosed at 24 weeks’ gestation by ultrasonography. The fetus was treated with a cyst-amniotic shunt at 29 weeks’ gestation. Following a postnatal whole resection of the right lung, postpneumonectomy syndrome appeared and, as a result, the infant died 13 months after delivery due to respiratory failure. Only 19 cases demonstrating CCAML associated with NIHF have been reported previously in Japan. Four cases showed a spontaneous resolution of NIHF, while 5 cases with type I CCAML, which all underwent fetal intervention, demonstrated an excellent outcome.

Keywords: congenital cystic adenomatoid malformation of the; hydrops fetalis; polyhydramnios

Document Type: Research article

DOI: 10.1111/j.1741-4520.2005.00075.x

Affiliations: 1: Second Department of Surgery, Fukuoka University School of Medicine, Fukuoka, Japan

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