A case of recurrent infantile polycystic kidney associated with hydrops fetalis

Nonimmune hydrops fetalis is becoming a predominant form of fetal hydrops due to the declining incidence of immune hydrops fetalis triggered by Rh isoimmunization. Infantile polycystic kidney appeared to be related to hydrops fetalis whether it is causal or merely coincidental and may represent another entry to differential diagnoses. Infantile polycystic kidney was diagnosed by an elevated maternal serum alpha-fetoprotein (AFP) value coupled with an ultrasonographic abnormality scanned as a multicystic mass with ascites in the fetal abdomen antenatally. This study presents a case of infantile polycystic kidney that resulted in a stillborn baby with hydrops fetalis and extensive placental calcification; it was the first case in Korea in which nonimmune hydrops fetalis was associated with infantile polycystic kidney in consecutive siblings by autosomal recessive inheritance in one family. In addition, this paper comprehensively reviews the incidence, etiology, prenatal diagnosis and proper management of nonimmune hydrops fetalis.

Estenosis del cordón umbilical e hydrops fetalis no inmunológico: presentación de un caso / Stenosis of the umbilical cord and non-immunologic hydrops fetalis: a case report

Se presenta un caso de hydrops fetalis no inmunológico (HFNI) asociado a constricción del cordón umbilical. La presencia de edema fetal generalizado y acumulación de líquido en los compartimientos serosos del feto sin evidencia de isoinmunización, especialmente isoinmunización al factor Rh, fue el criterio empleado para el diagnóstico de HFNI. El cordón umbilical mostró constricción y oclusión vascular con anormalidad de la gelatina de Wharton. Se sugiere la estenosis del cordón umbilical como la causa subyacente del HFNI y muerte fetal