Abdominal wall hernia in autosomal dominant polycystic kidney disease

Background Autosomal dominant polycystic kidney disease (ADPKD) is a common cause of renal impairment with a number of well recognized extrarenal associations. A high incidence of abdominal wall hernia was noted in patients with ADPKD. Methods A retrospective review of the notes of all patients with ADPKD on the South Wales renal replacement therapy database was performed. These patients were compared with age- and sex-matched patients with renal failure but without ADPKD and with general surgical patients. Results The prevalence of hernia in patients with ADPKD was 38 of 85 (45 per cent) compared with seven of 85 (8 per cent) for other forms of renal failure and three of 85 (4 per cent) for general surgical controls (P < 0.001). There were significantly greater numbers of inguinal (P < 0.001), incisional (P = 0.019) and paraumbilical (P = 0.007) hernias in patients with ADPKD compared with the other two groups. Conclusion These results show a significantly greater incidence of hernia, which could be an additional manifestation of the underlying defect in extracellular matrix production identified in patients with ADPKD.