2 Discussion
CBBF is a rare disease with congenital abnormal connection between the
hepatic duct and trachea or bronchus. The age of onset and severity of
symptoms depend on the diameter of the fistula. Therefore, the symptoms
appear at any age from newborn to adults[9,10,29].
Typical clinical feature of CBBF is bilious sputum or bile-stained
sputum in tracheal intubation. Patients usually have chronic wet
coughing, recurrent wheezing, shortness of breath and growth
retardation[12]. CBBF has usually been
misdiagnosed as an esophagotracheal fistula, gastroesophageal reflux,
aspiration pneumonia, tracheoesophageal fistula or high intestinal
obstruction[30,31].
In 1952, Neuhauser et al[29] reported the first
case of CBBF. Up to now, 27 cases have been enrolled in English and
Chinese (Table 1). Among them, fourteen (51.8%) cases were diagnosed in
the neonatal period, nine (33.3%) were diagnosed in infancy, four
(14.8%) were diagnosed in puberty or adulthood. The common
manifestations were biliary sputum (24/27, 88.9%), recurrent pneumonia
(9/27, 33.3%) and bilious vomiting (7/27, 22.2%). CBBF was more common
in female (19/27, 70.3%). The abnormal fistula originates different
location around the carina: right main bronchus (21/27, 77.8%), left
main bronchus (3/27, 11.1%), right intermediate bronchus (2/27, 7.4%),
and bilateral main bronchus (1/27, 3.7%).
The mechanism of CBBF formation is not clearly clarified. However, there
are two possible speculated mechanisms. One is considered as the
duplication of the upper gastrointestinal tract, which growing between
the laryngotrachea and hepatic diverticulum; the other is believed to be
the fusion between abnormal bronchial buds and abnormal bile
ducts[20,26]. The pathological results in our case
consistent with the latter pathogenesis.
The detection of bilirubin crystallization in sputum or/and BALF plays
crucial role in CBBF diagnosis[4,5,11-14,29,31].
Furthermore, abnormal fistula was easily found via bronchoscopy. In this
case, the bilirubin detection was positive in the BALF and the location
of the fistula connection was confirmed via bronchoscopy and fistula
angiography, which provided more comprehensive information and was
verified intraoperatively.
Chest CT and airway reconstruction, MRI or isotope examination can find
an abnormal fistula from the trachea, through the diaphragm, into the
abdominal cavity, and liver lobes communicate with each other[15]. Intraoperative management of fistula could
base on preoperative isotope examination[32]. Liu
AH et al[31] indicated gas accumulation in the
lumen of the right hepatic duct and the gallbladder lumen by
hepatobiliary B-ultrasound, which provided clues for clinical diagnosis
of BBF.
Patients with CBBF are ineffective to routine treatment and require
operation to remove the fistula. If the bile drainage is normal, only
thoracic fistula resection can be performed[33,34]; there also has reports of resection of
thoracic and abdominal fistulas [13].
Fistula-jejunostomy Roux-en-Y anastomosis, hilar-jejunostomy, or
Cholecystojejunostomy can be performed to fully drain bile so as to
avoid fistula recurrence for cases with biliary malformation, such as
absence of common bile duct, abnormal bile-intestinal drainage[14].
As surgery has higher cost and more damage, interventional therapy is
alternative for CBBF patients without severe biliary malformation. The
bronchial occlusion with biological glue has achieved good results in
the treatment of adult respiratory
diseases[35,36]. Tissue glue occlusion via
bronchoscopy also have been used in the treatment of adult secondary
bronchobiliary fistulas[36-38]. Chen X et
al[7] successfully cured the pediatric patient
with congenital brachobiliary fistula for the first time via
bronchoscopy. The interventional treatment via bronchoscopy in patients
with bronchobiliary fistula requires respiratory intervention doctors to
achieve further breakthroughs in future.
Combined this case and literature review, twenty-three (85.2%) patients
recovered after operation, four (14.8%) patients died from severe
comorbidities (biliary dysplasia (n=1), biliary atresia(n=1), congenital
diaphragmatic hernia(n=1), esophageal atresia and/or tracheoesophageal
fistula (n=1)). Two cases had complications (pneumothorax (n=1),
septicemia(n=1)). one case with no surgery. CBBF patients with delayed
or no operation, severe biliary malformation, or severe postoperative
complications have poor prognosis[17,23,24,28].