Patients with cystic echinococcosis in the three national referral centers of Mongolia: A model for CE management assessment

Background: Mongolia is one of the endemic countries for cystic echinococcosis (CE), a zoonotic disease caused by the larval stage of Echinococcus granulosus. The goal of this study is to describe the current clinical management of CE in Mongolia, to capture the distribution of cyst stages of patients treated, and to contrast current practice with WHO-IWGE expert consensus. Methods: Hospital records of CE patients treated between 2008 and 2015 at the three state hospitals and fulfilling the inclusion criterion ‘discharge diagnosis CE’ (ICD 10 code B.67.0–67.9) were reviewed. Demographical, geographical, clinical and ultrasonography (US) data were extracted and analyzed. The annual surgical incidence was estimated. The digital copies of US cyst images were independently staged by three international experts following the WHO CE cyst classification to determine the proportions of patients which ideally would have been assigned to the WHO recommended treatment modalities surgery, percutaneous, medical (benzimidazole) treatment and watch & wait. Results: A total of 290 patient records fulfilled the inclusion criteria of the study. 45.7% of patients were below 15 years of age. 73.7% of CE cysts were located in abdominal organs, predominantly liver. US images of 84 patients were staged and assessed for interrater-agreement. The average raw agreement was 77.2%. Unweighted Kappa coefficient and weighted Kappa was 0.57 and 0.59, respectively. Mean proportions of images judged as stages CE1, CE2, CE3a, CE3b, CE4 and CL were 0.59, 0.01, 0.19, 0.08, 0.03 and 0.11, respectively. 40 cysts met the inclusion criteria of treatment modality analysis. The mean proportions of cases with a single cyst assigned to medical, percutaneous treatment, surgery and watch & wait were 52.5% (95% CI 42–65), 25.8% (95% CI 15–30), 5.1% (95% CI 0–10) and 3.3% (95% CI 0–10), respectively. 13.3% (95% CI 5–25) of cysts were staged as CL and therefore assigned to further diagnostic requirement. Conclusion: WHO CE cyst classification and WHO-IWGE expert consensus on clinical CE management is not implemented in Mongolia. This results in exclusively surgical treatment, an unnecessary high risk approach for the majority of patients who could receive medical, percutaneous treatment or observation (watch & wait). Introduction of WHO-IWGE expert consensus and training in ultrasound CE cyst staging would be highly beneficial for patients and the health care services. Author summary: Cystic Echinococcosis (CE) is a zoonotic disease, commonly known as dog tapeworm. The disease is distributed globally and predominantly affects rural populations with limited access to health care. Following the expert consensus of the WHO-Informal Working Group on Echinococcosis (WHO—IWGE) patients with uncomplicated cysts are assigned on the basis of WHO cyst classification to four treatment modalities: medical (benzimidazoles), percutaneous, surgical treatment, and ‘watch & wait’. In Mongolia, one third of the population practices nomadic farming. These populations are heavily affected by CE. However, cyst staging and WHO-IWGE recommendations are not implemented and patients referred to the three national treatment centres receive surgical treatment. This exposes a large proportion of patients to an unnecessary high risk approach who could be treated–depending on cyst stage—with benzimidazoles, percutaneously or observed (watch & wait). We reviewed the hospital records of patients with CE and admitted between 2008 and 2015 to the three national CE treatment centres, retrospectively staged the cysts and assigned the patients to the four WHO-IWGE recommended treatment modalities. We found a high proportion of patients in the study population who would have most likely benefitted from non-surgical treatment options.