Laparoscopic-assisted retroperitoneal nephrectomy in autosomal recessive polycystic kidney disease

Abstract

Autosomal recessive polycystic kidney disease (ARPKD) occurs in 1 in 20000 live births. A mortality rate of 30-40% is reported, generally relating to pulmonary hypoplasia, and 60% require renal replacement therapy (RRT) by 10 years. In the neonatal period, the large kidneys can cause significant mass effect, with the need for prolonged respiratory support and difficulty in establishing feeds. Early postnatal peritoneal dialysis (PD) is required in up to 25%, and dialysis efficiency can similarly be compromised. In these situations, unilateral or bilateral nephrectomy may be recommended. All previous reports of nephrectomy in ARPKD have described an open approach, generally via a transperitoneal route, with the risk of compromising future PD. Here, we demonstrate laparoscopic-assisted retroperitoneal nephrectomy (LARN), which has been used successfully in two patients. Case 1: a 37/40, 3.2kg female infant. She was extubated to CPAP d6 and commenced haemodialysis d7. Left LARN was performed d34 and CPAP was discontinued within 48h. Right LARN was performed d49 as it was felt PD still would not be possible and PD was then successfully commenced d62. She unfortunately died aged 11/12. Case 2: a term 3.5kg male infant. He was ventilator-dependent and required PD catheter insertion on d1. PD was discontinued for leakage d7, and haemodialysis commenced. Left LARN was performed d18. He remained ventilator-dependent and given this, and the PD leakage, right LARN was performed d20. He was extubated 48h later, and PD successfully reintroduced after 1 week. He is currently aged 4/12. LARN is achievable in ARPKD. Although unilateral LARN may be sufficient, in these cases bilateral LARN was required. Relief of the mass effect allowed respiratory support to be discontinued and peritoneal dialysis to be established in both children.