A twenty-year history of alveolar echinococcosis: analysis of a series of 117 patients from eastern France

Objectives Alveolar echinococcosis of the liver is a very rare and severe p arasitic disease due to the growth of the larva of Echinococcus multilocula ris. The aim of this paper was to describe a 20-year study of the epidemiol ogical, clinical and therapeutic aspects of alveolar echinococcosis in east ern France. Design One hundred and seventeen consecutive cases, diagnosed and followed in our liver unit, were studied from 1972 to 1993. Methods Data from 85 patients followed since 1983 (period B) were compared to data from a first series of 32 patients (period A) collected from 1972 t o 1982; 1983 was chosen as the cut-off year because of the numerous changes that occurred in the diagnosis, follow-up and treatment of the disease at this time, in particular the introduction of parasitostatic benzimidazoles. Results The results of patient follow-up were evaluated in December 1997. T he cumulative prevalence was 2.5 per 100 000 persons in period A whereas it reached 6.6 per 100 000 in period B. The annual incidence in period B was 7.3 on average, compared with 2.7 in period A. Twenty-nine pet cent of pati ents from period B were asymptomatic at the time of diagnosis compared with 10% in period A. This change was correlated with less advanced liver lesio ns, and was related to the extensive use of abdominal ultrasound, and from 1987, serological screening. Curative resections were performed in 24% of t he cases in period B versus only 3% in period A. From 1986, liver transplan tations were performed in eight patients from period A and 13 patients from period B. In period B, palliative surgery was frequently replaced by radio logical non-operative procedures to treat abscesses and jaundice. From 1982 , 73 patients received benzimidazoles for a period of time ranging from 4 t o 138 months. Stabilization of the lesions was observed in two-thirds of th e patients. Episodes of jaundice or digestive haemorrhage due to portal hyp ertension were 31.5 and 11 times less frequent respectively in patients fro m period B compared with period A. Actuarial survival at 5 years improved f rom 67% in period A to 88% in period B in patients of similar age. Conclusions Radical changes in the diagnosis and the management of alveolar echinococcosis have occurred during the last decade. Together they have co ntributed to an improvement in the status of the patients affected by this very severe parasitic disease. Eur I Gastroenterol Hepatol 12:327 - 336 (C) 2000 Lippincott Williams & Wilkins.