Background: Congenital bronchobiliary fistula (CBBF) is a rare disease with abnormal connection between the biliary tract and the bronchus. The misdiagnosis rate of CBBF is high, delayed surgery may induce poor outcome. Case presentation: We reported a girl with CBBF who was confirmed via bronchoscopy and chest computed tomography (CT). The girl was 7-month-old admitted to the hospital with “recurrent pneumonia”. Bilirubin crystallization detection was positive in the bronchoalveolar lavage fluid (BALF). CT images showed the abnormal traffic between the biliary tract and the right main bronchus, the girl was diagnosed with CBBF and recovered after surgical operation. Literature search: A total of 27 cases were collected, 14 (51.8%) cases were diagnosed in the neonatal period, 9 (33.3%) patients were diagnosed in infancy. CBBF was more common in female (19/27, 70.3%). The specific manifestations were bilious sputum (24/27, 88.9%) and bilious vomiting (7/27, 22.2%). Recurrent pneumonia (9/27, 33.3%) was common clinical manifestations. The most abnormal fistula originates different location around the carina was right main bronchus (21/27, 77.8%). There were 23(85.2%) cases recovered after surgical operation and 4(14.8%) cases with severe comorbidities/complications died. Conclusions: CBBF should be suspected in infants with bilious sputum. Bronchoscopy plays crucial role in preoperative evaluation and postoperative follow-up. With early diagnosis and surgery, the prognosis is quite well for CBBF patients without severe biliary malformation and/or severe postoperative complications.

Background: Clear cell sarcoma of the kidney (CCSK) is a rare but the second common renal malignant tumor mimicking Wilms’ tumor. Radiomics is helpful for differentiating CCSK from Wilms’ tumor preoperatively through analyzing the pixel distribution of lesions on medical images quantitatively. Procedure: In this study, the regions of interest (ROIs) of lesions were delineated on corticomedullary phase (CMP) and nephrographic phase (NP) images to extract radiomics features. Dimensionality reduction and Logistic Regression (LR) algorithm were used to construct the classification models. The area under the receiver operator characteristic curve (AUC), sensitivity and specificity were calculated for evaluation, and Delong test was used to compare the performance of the most meaningful features and LR models. Results: Lower skewness was observed in Wilms’ tumor, and higher skewness in CCSK. Skewness transformed by exponential and squareroot filters from NP images achieved moderate to good diagnostic performance for CCSK with AUCs of 0.707 (95%CI: 0.573, 0.840) and 0.705 (95%CI: 0.572, 0.839) in the training set, and 0.818 (95%CI: 0.608, 1.000) and 0.803 (95%CI: 0.585, 1.000) in the validation set, respectively. Delong test showed no significant difference between LR model, exponential-skewness and squareroot-skewness based on NP images in both training and validation sets. Conclusion: Skewness from nephrographic phase at exponential and squareroot filters is helpful to discriminate between CCSK and Wilms’ tumor in children, and higher skewness on NP images may be a potential imaging biomarker for diagnosing CCSK from Wilms’ tumor.