Morr Ann-Katrin, Mosimann Beatrice, Tschumi Sibylle, Surbek Daniel, Raio Luigi
Department of Obstetrics, University Women's Hospital, Inselspital, Bern University Hospital, Bern, Switzerland.
Department of Pediatric Nephrology, University Children's Hospital, Inselspital, Bern University Hospital, Bern, Switzerland.
Z Geburtshilfe Neonatol. 2022 Apr;226(2):98-103. doi: 10.1055/a-1586-5493. Epub 2021 Sep 7.
This study's objective was to identify prenatal criteria helping differential diagnosis of bilateral enlarged, hyperechogenic kidneys, especially looking at development of renal volume and amniotic fluid volume with increasing gestational age.
Retrospective analysis (single-center database) of all bilateral enlarged, hyperechogenic kidneys between 2000-2018. Renal enlargement was defined as renal volume>90 percentile. Evaluation included development of renal and amniotic fluid volume during pregnancy and fetal outcome.
23 cases fulfilled the inclusion criteria. 12 pregnancies were terminated. For 11 continued pregnancies, longitudinal information on amniotic fluid volume and renal volume were available. 4 cases with oligohydramnios showed a progressive reduction; 6 cases with normal/increased amniotic fluid volume remained stable; in 1 case amniotic fluid volume normalized from initially being oligohydramnios. Regarding renal volume, 4 cases showed exponential enlargement, 3 cases linear progression; in 2 cases renal volume stabilized after initial progression; 2 cases showed initial progression and secondary regression. 4 fetuses survived: 3 autosomal dominant polycystic kidney diseases, 1 Bardet-Biedl syndrome.
Progressive reduction of amniotic fluid volume with exponential increase of renal volume is highly suggestive for autosomal recessive polycystic kidney disease. Cases of autosomal dominant polycystic kidney disease show a linear progression of renal volume>90 percentile and mostly normal amniotic fluid volume.
本研究的目的是确定有助于双侧肾脏增大、回声增强的鉴别诊断的产前标准，尤其关注肾体积和羊水量随孕周增加的变化情况。
对2000年至2018年间所有双侧肾脏增大、回声增强的病例进行回顾性分析（单中心数据库）。肾增大定义为肾体积>第90百分位数。评估包括孕期肾体积和羊水量的变化以及胎儿结局。
23例符合纳入标准。12例妊娠终止。对于11例继续妊娠的病例，可获得羊水量和肾体积的纵向信息。4例羊水过少者呈进行性减少；6例羊水正常/增加者保持稳定；1例羊水最初过少但后来恢复正常。关于肾体积，4例呈指数性增大，3例呈线性进展；2例肾体积在最初进展后稳定；2例最初进展随后消退。4例胎儿存活：3例为常染色体显性多囊肾病，1例为巴德-比德尔综合征。
肾体积呈指数性增加而羊水量进行性减少高度提示常染色体隐性多囊肾病。常染色体显性多囊肾病病例显示肾体积>第90百分位数呈线性进展且大多羊水正常。
