Unusual sonographic features of ARPKD.
The classic sonographic appearance of the kidneys in fetuses with autosomal recessive polycystic kidney disease (ARPKD) has been well described. We report a case of enlarged kidneys with pyramidal hyperechogenicity quite similar to medullary nephrocalcinosis found in a fetus at 34 weeks' gestation. At 39 weeks, a female neonate was delivered and died after 22 h due to pulmonary insufficiency secondary to severe oligohydramnios. On pathological analysis, the gross and microscopic findings were typical of ARPKD with diffuse dilatation of tubules throughout. The fetal renal lobulation was prominent and on section, the pyramids were delineated within each lobule, accounting for the clear image of the pyramids observed on sonography.
Department of Obstetrics, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. mariaokumura@hotmail.com
Maria
M
Okumura
Victor
V
Bunduki
Christina
C
Shiang
Regina
R
Schultz
Marcelo
M
Zugaib
D000328:Adult; D017809:Fatal Outcome; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging*; D005865:Gestational Age; D006801:Humans; D007668:Kidney / Q000000981:diagnostic imaging / Q000196:embryology / Q000473:pathology; D016104:Oligohydramnios / Q000000981:diagnostic imaging; D017044:Polycystic Kidney, Autosomal Recessive / Q000000981:diagnostic imaging* / Q000473:pathology; D011247:Pregnancy; D016216:Ultrasonography, Prenatal