and tricuspid valve incompetence and left ventricular hypertrophy that are
most probably secondary to hypertension.
- Gastrointestinal involvement
Hepatic cysts are the commonest extrarenal manifestations of
ADPKD as they occur in 40% of cases. Other gastrointestinal
manifestations include diverticulosis (may be complicated by diverticulitis,
abscess formation or perforation), pancreatic and splenic cysts and inguinal
hernias. - Neurological involvement
Intracranial aneurysm occurs in 10% of cases. It may rupture
leading to subarachnoid haemorrhage.
Pathology:
The two kidneys are massively enlarged (Fig. 7.1), in 80% of
cases, the enlargement is symmetrical. Cross section will show hundreds of
cysts occupying the cortex and medulla and compressing the normal renal
tissue in between.
Diagnosis:- By detecting renal cysts by US or CT scanning.
- Gene linkage analysis for the detection of responsible gene on
chromosome 16.
Management:- Abdominal and flank pain which is due to enlarging cyst is
managed by non-narcotic analgesics, rarely percutaneous cyst
rupture may be indicated for persistent severe pain.