Table 3.
Other extrarenal manifestations of ADPKD
| Manifestation | Associated | % Affected | Screen | Comment |
|---|---|---|---|---|
| Cardiac valve abnormalities | Yes | Mitral valve prolapse 25% | No | Screen only if cardiovascular signs/symptoms |
| Pericardial effusion | Yes | Up to 35% | No | Screen only if cardiovascular signs/symptoms |
| Extracranial aneurysms | Yes, case reports | Unknown | No | Clinicians should be aware of vascular phenotype in some patients |
| Arachnoid cysts | Yes | 8–12% | No | Possible increased risk for subdural hematoma. |
| Spinal meningeal cysts | Yes | 1.7% | No | Rare cause of spontaneous intracranial hypotension |
| Pancreatic cysts | Yes | 10% | No | Usually asymptomatic |
| Diverticular disease | Possibly in association with ESRD | ~20–50% in ESRD | No | Increased incidence in patients who have reached ESRD |
| Abdominal hernias | Yes | Unknown | No | |
| Seminal vesicle cysts | Yes | ~40% | No | Does not correlate with abnormal semen parameters |
| Male infertility | Unknown | Unknown | No | Abnormal semen parameters reported |
| Bronchiectasis | Possibly | 37% in one series vs. 13% controls | No | One study only; mild, no clinical consequence |
| Congenital hepatic fibrosis | Yes, case reports, usually affecting only one generation within a family with ADPKD | Rare | No | Rare but potentially life-threatening; early diagnosis in siblings with ADPKD can be lifesaving with appropriate monitoring and treatment. |
Abbreviations: ADPKD, autosomal dominant polycystic kidney disease; ESRD, end-stage renal disease