[Minkowski-Chauffard disease as a surgical problem]

Minkowski-Chauffard's disease keeps on being primarily a subject for hematologists, but surgeons play a definite role in its treatment, if we take into consideration the encouraging effect of splenectomy. Experience is recorded gained at the Department of Abdominal Surgery of the Research Institute of Gastroenterology and Hematology with 34 patients for a period of 35 years, 13 of them during the last 18 years. A patient is presented with a characteristic clinical pattern, associated with calculosis of the common bile duct, a very rare observation in this disease. A review is made on the current knowledge on the etiology, pathogenesis clinical manifestations and treatment of the disease. The need of splenectomy, with simultaneous search for accessory spleen and for biliary pathology, prompting revision of the biliary system as well, is emphasized.