A Case Report of Frequent Familial Aggregation of Polycystic Liver Disease

Haruhisa Harada, Tooru Noguchi, Hiroaki Urayama, Atsushi Sugiyama, Tomoyuki Siga, Minoru Numata, Masatoshi Makuuchi

Department of Surgery, Shinsyu University, School of Medicine

A 48-year-old woman with complaints of epigastric fullness and upper abdominal tumor was admitted because of policystic liver disease (PLD). Deroofing and fenestration procedures were performed. Later, her 56-year-old sister had the same disease, and the same operation was carried out. We examined three generations of their family and seven including one suspicious case were found to have PLD. Reports of PLD with familial aggregation are rare. Three years after the first woman's operation, she was re-admitted and underwent radiological investigations because of recurrence of abdominal symptoms. The remnant cysts were found to be enlarged. She was treated by injection of absolute ethanol under ultrasound guidance. One to four ml of absolute ethanol was injected into 10 cysts without later aspiration of the cysts' content. No recurrence was observed 8 months after the ethanol injection therapy. Family members of a patient with PLD should be investigated to study familial aggregation of PLD and the pattern of genetic transmission. Absolute ethanol injection therapy will be the treatment of choice for similar cases of PLD.

Key words
polycystic liver disease, ethanol injection therapy, familial aggregation

Reprint requests
Haruhisa Harada Department of Surgery, Shinshu University School of Medicine
3-1-1, Matsumoto, 390 JAPAN

Accepted
April 17, 1991

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