Autosomal recessive polycystic kidney disease associated with severe hydrocephalus

Case contributed by Dr Abdulrahman Abdo Ali Abbas

Presentation

Pregnant female presented for anomalies screening

Patient Data

Age: 40 years
Gender: Female

Fetal MRI has done at 26 weeks of gestational age shows :

  • bilateral enlarged fetal kidneys filling the abdominal cavity, they show hyperintense cortex with an innumerable small cysts
  • severe oligohydramnios
  • decrease thoracic size
  • enlarged fetal head largely replaced by a disproportionate severe ventriculomegaly, thinned cortical mantle, effaced peri-cerebral space, cortical mantle dehiscence, and occipital meningocele

Case Discussion

The coincidence development of the central nervous system and the renal system lead to some association between the CNS and kidneys anomalies. ARPKD may be associated with many CNS anomalies such as holoprosencephaly, hydranencephaly, and internal hydrocephalus.

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