Fig. 1

Diagnostic timelines of 16 CS patients. Important time points including onset of clinical presentation, first clinical visit, and diagnosis are depicted for each CS patient along the timeline. The course of the disease was divided into three phases: phase 1 (blue), defined as no proof of complication of portal hypertension (i.e., hypersplenism); phase 2 (purple), defined as discovered complication of portal hypertension without gastrointestinal variceal bleeding; and phase 3 (red), defined as at least one recorded variceal bleed. Imaging modalities and/or biopsy used before diagnosis are marked along the timelines. US: ultrasonography; CT: computed tomography; CT + C: computed tomography with contrast; MRI: magnetic resonance imaging; MRCP: magnetic resonance cholangiopancreatography; PET/CT: positron emission tomography/computed tomography