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A population-based unselected registry material involving 1,038 unidentified multiple congenital anomalies had 16 unrelated cases with polycystic kidney and congenital cardiovascular malformation. The syntropy index of this combination was 4. The family study detected one specific and two half-concordant sib occurrences. The so-called "polycystic kidney and cardiovascular malformation" association with variable expression may be a MCA entity.

Maximum conservativism was the watchword up to 1979 in dealing with polycystic disease. In the period 1979 to 1985 a total of 45 patients were subjected to 80 instances of polycystic resection whereby larger cysts were removed by excision, smaller ones by ignipuncture, with particular attention to extirpation of the perihilar cysts. Post-operative incidence of hypertension dropped from 33 to 3%. The method described is rated as one of the feasible expedients to deal with polycystic disease in the early compensated stage.