Premium
Unusual sonographic features of ARPKD
Author(s) -
Okumura Maria,
Bunduki Victor,
Shiang Christina,
Schultz Regina,
Zugaib Marcelo
Publication year - 2006
Publication title -
prenatal diagnosis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.956
H-Index - 97
eISSN - 1097-0223
pISSN - 0197-3851
DOI - 10.1002/pd.1410
Subject(s) - autosomal recessive polycystic kidney disease , oligohydramnios , fetus , medicine , nephrocalcinosis , pathology , medullary cavity , pathological , kidney , anatomy , polycystic kidney , pregnancy , cyst , biology , genetics
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. Copyright © 2006 John Wiley & Sons, Ltd.