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

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.

• Authors: Koushi Asabe, Yoichiro Oka, Takayuki Shirakusa
• Journal: Congenital anomalies (Congenit Anom (Kyoto)) Vol. 45 Issue 3 Pg. 96-101 (Sep 2005) ISSN: 0914-3505 [Print] Australia
• PMID: 16131368 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Abnormalities, Multiple
• Adult
• Cystic Adenomatoid Malformation of Lung, Congenital (complications, diagnostic imaging, pathology, therapy)
• Female
• Fetal Therapies (methods)
• Fetus (abnormalities)
• Humans
• Hydrops Fetalis (diagnostic imaging)
• Infant
• Infant, Newborn
• Japan
• Pneumonectomy (adverse effects)
• Polyhydramnios (diagnostic imaging)
• Pregnancy
• Ultrasonography, Prenatal