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Case Report

Challenges Faced During the Ventilation of a Child With Bronchobiliary Fistula: A Case Report

Zainal Abidin, Huda MD, MMed*; Mohd Lutfi, Nijar MBChB, MMed; Phang, Ye Yun MBBS, MMed; Zarina, Fakir Mohamed Intan MD, MMed; Hamidah, Ismail MD, MMed; Saedah, Ali MD, MMed*; Wan Hassan, Wan Mohd Nazaruddin MD, MMed*

Author Information
doi: 10.1213/XAA.0000000000001281
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The International Incidence of Childhood Cancer estimated the worldwide rates of hepatoblastoma were 0.5–2.0 cases per million in children between 0 and 14 years.1 United States Surveillance, Epidemiology, and End Results program estimated an occurrence of 100 cases per year in 1999 and reported an annual increase of 4% from 1992 to 2004.1 The 5-year survival rate was estimated to be 74%.

Given the high-incidence of hepatoblastoma, many investigations are focusing on the fetal development and on the similarities between embryonic liver cells and hepatoblastoma.2 The identified contributing factors include very low birth weights <1500 g, premature births, eclampsia, severe preeclampsia, maternal smoking, familial adenomatous polyposis, and the Beckwith–Weidmann Syndrome.1

The family of the patient has provided written consent for writing and publication of the case.


A 1-year-old boy (weight 9.2 kg) was born at 32 weeks of gestation with the birth weight of 1.6 kg. He was diagnosed with a congenital hepatoblastoma and had undergone right extended hepatectomy and partial omentectomy. Histological examination revealed a mixed epithelial and mesenchymal tumor. Postoperatively, he developed right lobar pneumonia and pleural effusion requiring noninvasive ventilation for 6 days. He was discharged in a stable condition after the completion of chemotherapy involving intravenous cisplatin.

Approximately 6 months after the treatment, he presented to the hospital with persistent biloptysis that worsened over the past 2 weeks (Figure). He was planned for a hepatobiliary iminodiacetic acid scan (HIDA) and was electively intubated for the procedure. Unexpectedly, continuous copious bilious fluid gushed from the endotracheal tube (ETT), and we had difficulty connecting him to a ventilator, as vigorous suction had to be performed frequently. He was planned to undergo emergency right thoracotomy with ventilator settings as follows: oxygen 80%, pressure control 20 cm H2O, positive end-expiratory pressure 5 cm H2O, generated tidal volume 60–80 mL, and respiratory rate of 24 breaths per minute.

Preoperative chest x-ray with right lower zone haziness and prominent left lobe of the liver. (The child underwent right extended hepatectomy.)

Anesthesia was induced with target-controlled infusion (TCI) of propofol at 6 mg·kg−1·h1 and remifentanil at 0.3·µg·kg−1·min1. The maintenance of saturation and ventilation was challenging because of copious bilious secretion from the ETT. Multiple attempts were made to clear the fluid by suctioning through the ETT. There were several episodes of desaturation to 67%.

In the middle of the surgery, he developed cardiac arrest, which lasted for about 2 minutes and was possibly due to hypoxia, direct myocardial irritation, and the cardiovascular depression effect of positive-pressure ventilation. He was resuscitated with 2 doses of 10 µg·kg−1 intravenous epinephrine. TCI propofol and remifentanil were discontinued. Sevoflurane was used for the maintenance of anesthesia, and his blood pressure increased to 120/60 mm Hg, his heart rate increased to 130 beats/min, and he achieved 100% oxygen saturation. His hemodynamic parameters were maintained with epinephrine infusion titrated up to 0.5 µg·kg−1·min−1.

Intraoperatively, a bile-stained tract was noted toward the central part of the diaphragm. Additionally, right lung lower lobectomy was performed. The estimated blood loss was 400 mL, and he was transfused with 300 mL blood and 100 mL fresh-frozen plasma. Thereafter, he was transported to the pediatric intensive care unit, ventilated, and sedated with intravenous (IV) midazolam and morphine infusion. He was supported with epinephrine infusion at 0.08–0.15 µg·kg−1·h−1.

He was transferred to the general pediatric ward after 8 days. Approximately 2 weeks postoperatively, he was breathing room air and was taking food orally. He was sitting comfortably on his mother’s lap when we interviewed her.


Thoracobiliary fistula may communicate with either pleural space (pleurabiliary) or bronchi (bronchobiliary), as in our case. This is a rare condition, and there is no consensus on the optimal medical management.3 It was first identified in 1850 by Peacock4 as a complication of hydatid cysts. Early identification is essential in view of the corrosive nature of bilirubin will ultimately cause acute necrotizing pneumonia.3

Thoracocentesis detects the presence of bilirubin with a ratio of pleural fluid:total bilirubin serum level range between 0.6 and 1.0.5 Primary investigation such as an ultrasound of the hepatobiliary system is useful as ultrasound is able to delineate the portal triad and6 could be conducted without sedation. More invasive procedures, such as HIDA cholescintigraphy, are considered more accurate in the identification of biliary leaks.3 However, in our case, we had to abandon this procedure as the condition deteriorated after he was electively intubated and ventilated.

Lung isolation, which is commonly performed using a double-lumen tube, is more suitable for patients above 8 years old. Other options include the use of bronchial blockers or 1 lung intubation with ETT at the desired bronchus.7 The procedure is technically challenging for pediatric patients. Ventilating the lungs separately via a technique described as independent lung ventilation is achievable if lung isolation is successful.8 Independent lung ventilation involves ventilating the right and left lungs differently using separate ventilators.8

The case could be managed differently if we could appreciate the possibility of bronchobiliary fistula. Besides, introducing mechanical ventilation without knowledge of the level of severity can be proven catastrophic. The condition deteriorated and the patient had to undergo an emergency thoracotomy. Since we were struggling with the ventilation, we did not isolate the lungs using the techniques discussed above. We hoped the case report served a lesson of the importance of suspecting the pathology earlier when a patient is presented with persistent biloptysis.


Name: Huda Zainal Abidin, MD, MMed.

Contribution: This author helped write the manuscript.

Name: Nijar Mohd Lutfi, MD, MMed.

Contribution: This author helped write the manuscript.

Name: Ye Yun Phang, MD, MMed.

Contribution: This author helped write the manuscript.

Name: Fakir Mohamed Intan Zarina, MD, MMed.

Contribution: This author helped review and revise the manuscript.

Name: Ismail Hamidah, MD, MMed.

Contribution: This author helped review and revise the manuscript.

Name: Ali Saedah, MD, MMed.

Contribution: This author helped review and revise the manuscript.

Name: Nazaruddin W. Hassan, MD, MMed.

Contribution: This author helped to coordinate and critically review the manuscript for important intellectual content.

This manuscript was handled by: BobbieJean Sweitzer, MD, FACP.


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