title: 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.affiliation: Division of Pediatric Surgery, Maternity and Perinatal Care Center, Fukuoka University Hospital, 7-45-1 Nanakuma, Jonan-ku, Fukuoka, Japan. asabe@fukuoka-u.ac.jp
authors.forename: Koushi
authors.initials: K
authors.lastname: Asabe
authors.forename: Yoichiro
authors.initials: Y
authors.lastname: Oka
authors.forename: Takayuki
authors.initials: T
authors.lastname: Shirakusa
mesh_terms: D000015:Abnormalities, Multiple; D000328:Adult; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy; D005260:Female; D046128:Fetal Therapies / Q000379:methods; D005333:Fetus / Q000002:abnormalities*; D006801:Humans; D015160:Hydrops Fetalis / Q000000981:diagnostic imaging; D007223:Infant; D007231:Infant, Newborn; D007564:Japan; D011013:Pneumonectomy / Q000009:adverse effects; D006831:Polyhydramnios / Q000000981:diagnostic imaging; D011247:Pregnancy; D016216:Ultrasonography, Prenatal