[Cystic adenomatoid malformation of the lung, bilateral renal agenesis and left heart hypoplasia. An unusual association in Potter's syndrome].
We describe an autopsy case of congenital cystic adenomatoid malformation of the lung (CCAM) associated with bilateral renal agenesis. Prenatal ultrasound examination showed additional left heart hypoplastic syndrome. A therapeutic abortion was induced at 23 weeks of gestation. The association CCAM-bilateral renal agenesis is a rare condition (5 cases previously described) which has to be known because of the mitigation effect of the CCAM on the oligohydramnios determined by bilateral renal agenesis. However, this instance is usually associated with oligohydramnios. The pathogenesis of polyhydramnios in isolated CCAM is discussed in regard with these data.
Carles
D
D
Unité de Foetopathologie, Groupe Hospitalier Pellegrin, Bordeaux.
Dallay
D
D
Serville
F
F
Maugey-Laulom
B
B
Alberti
E M
EM
Tissot
H
H
Weichhold
W
W
Wursten-Guitton
F
F
D000015:Abnormalities, Multiple / Q000473:pathology*; D000032:Abortion, Therapeutic; D000328:Adult; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000473:pathology*; D005260:Female; D006330:Heart Defects, Congenital / Q000473:pathology*; D006801:Humans; D007668:Kidney / Q000002:abnormalities*; D008297:Male; D011247:Pregnancy; D013577:Syndrome; D014683:Vena Cava, Superior / Q000002:abnormalities
Malformation adénomatoïde kystique du poumon, agénésie rénale bilatérale et hypoplasie du coeur gauche. Une association inhabituelle dans un syndrome de Potter.