A complex case of congenital cystic renal disease

Abstract

This case outlines the potential complexity of autosomal recessive polycystic kidney disease (ARPKD). It highlights the challenges involved in managing this condition, some of the complications faced and areas of uncertainty in the decision making process. With a paucity of published paediatric cases on this subject, this should add to the pool of information currently available.

Figure 1

Kidneys 10 cm in length (normal around 5.5 cm). Loss of normal cortico-medullary differentiation. Hyperechogenic medulla, hypoechogenic areas in keeping with small cysts. Some focal increase in echogenicity in keeping with nephrocalcinosis.

Figure 2

Liver US shows variable echogenicity in keeping with fibrosis.

Figure 3

A nodular liver with broad bands of fibrous tissue containing numerous bile duct profiles. x4 power, the stain is a trichrome stain.

Figure 4

Graphs A and B demonstrate the steady decline in renal function in terms of serum creatinine and glomerular filtration rate respectively.

Figure 5

Microscopic staining of sections from explanted graft kidney. (A) The infarcted parenchyma shows necrosis of the renal tubules in the affected area together with some signs of longstanding ischaemic damage including calcification (large picture: H&E x2. Insert left: H&E x10. Insert right H&E x4); (B): thrombus with recanalisation in a large hilar vessel highlights the acute on chronic vascular compromise. (H&E x2 large picture. Inserts: Elastic van Gieson x2 and H&E x10).