Diagnosis of congenital bronchobiliary fistula in children by
bilirubin crystallization in the bronchoalveolar lavage fluid: A case
report and literature review
Guangli Zhang 1, Chongjie Wang1,
Zhili,Wang1, Xiaoyin Tian1, Yuanyuan
Li1, Qinyuan Li1, Xiujuan
Xu1, Hao Ding2, Jin
Zhu3, Jian Luo1, Zhengxiu
Luo1*
*Correspondence: luozhengxiu816@hospital.cqmu.edu.cn
Abstract: 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.
Keywords: Congenital bronchobiliary fistula (CBBF);
bronchoalveolar lavage fluid; bilirubin crystallization; children; case
report