Bilioptysis is a rare clinical entity characterized by the expectoration of biliary secretions through the respiratory tract. Its presence is pathognomonic of a biliopulmonary or bronchobiliary fistula. We present the case of a 69-year-old man with a history of ileal carcinoid tumor with metastatic liver disease, who underwent hepatectomy and had a history of abdominal biliary collections. The patient was admitted for bibasal cavitary pneumonia associated with acute respiratory failure. During the clinical course, the patient developed bilioptysis, whose final diagnosis could be confirmed by imaging and bronchoscopic studies. The etiopathogenesis, differential diagnosis, and therapeutic options of this rare complication are discussed.
La bilioptisis es una entidad clínica poco frecuente caracterizada por la expectoración de secreciones biliares a través del tracto respiratorio. Su presencia es patognomónica de una fístula biliopulmonar o broncobiliar. Presentamos el caso de un varón de 69 años con antecedentes de tumor carcinoide ileal con enfermedad metastásica hepática, sometido a hepatectomía y con historia previa de colecciones biliares abdominales. El paciente ingresó por una neumonía cavitada bibasal asociada a insuficiencia respiratoria aguda. Durante la evolución clínica, el paciente presentó bilioptisis, cuyo diagnóstico final pudo ser confirmado mediante estudios de imagen y broncoscopia. Se discuten la etiopatogenia, el diagnóstico diferencial y las opciones terapéuticas de esta complicación poco frecuente.
Bilioptysis, defined as the expectoration of bile through the respiratory tract, is an infrequent clinical manifestation but is always indicative of an abnormal communication between the biliary system and the bronchial tree. Early recognition is essential to guide appropriate treatment and to prevent severe complications such as recurrent pulmonary infections, sepsis, or irreversible lung damage.
We present the case of a 69-year-old male with a medical history of type 2 diabetes mellitus, arterial hypertension, dyslipidemia, and a 20 pack-year smoking history. Two years prior, the patient underwent a right hemicolectomy with extensive ileal resection for an ileal carcinoid tumor, followed by a subsequent major hepatectomy for metastatic liver disease. This latter procedure was complicated by a biliary fistula, which was managed via percutaneous drainage and biliary stent placement. The patient remained under surveillance with adjuvant somatostatin analogues and was considered free of disease during follow-up.
The patient presented with a five-day history of progressive dyspnea, fever, dry cough, and mild hemoptysis. Detailed records of his prior biliary complications – managed at an outside institution – were unavailable at the time of admission. On initial evaluation, he was in fair general condition and tachypneic, with bilateral crackles, decreased breath sounds at the right lung base, and signs of acute partial respiratory failure. Chest radiography showed bilateral lower and middle-lobe consolidations, while the non-contrast thoracic CT reported these as cavitating lesions suggestive of infectious necrotizing pneumonia, accompanied by a small right pleural effusion.
Empirical antibiotic therapy with a beta-lactam plus beta-lactamase inhibitor was initiated. On the second day of admission, the patient developed expectoration of large volumes of greenish, biliary-appearing material, occasionally reaching up to 300mL per day, raising the differential diagnosis between bilioptysis and bilious vomiting. Shortly thereafter, he experienced clinical deterioration with recurrent fever, increasing oxygen requirements, elevated acute-phase reactants, and clinical criteria for sepsis.
Blood cultures grew Bacteroides spp. sensitive to carbapenems, with no other relevant microbiological findings. A follow-up thoracic CT on the tenth day of admission demonstrated progression of cavitary lesions and revealed a subphrenic biliary collection (bilioma) with extension toward the thoracic cavity, although a clear bronchobiliary fistulous tract could not be identified (Fig. 1). A retrospective review of the admission CT confirmed that the bilioma was already present. However, it was initially overlooked due to the absence of bilioptysis at presentation, a radiological evaluation focused on acute thoracic findings, and the absence of detailed information regarding the patient's history of biliary complications to guide the initial report.
(A) Coronal CT image showing areas of decreased attenuation at the right lung base, similar to suprahepatic collections, suggesting communication between the abdominal biliary collection and the pulmonary parenchyma. (B) Characteristic greenish-yellow biliary expectoration (bilioptysis) collected in a sample container.
Given clinical and analytical worsening, antibiotic therapy was escalated to meropenem plus linezolid and diagnostic flexible bronchoscopy was performed under high-flow nasal oxygen therapy. Left vocal cord paralysis with incomplete glottic closure was observed. Additionally, the respiratory origin of the biliary secretions was confirmed, through the direct visualization and suctioning of large volumes of bile-stained material, predominantly localized in the right lower lobe.
Subsequently, and following the adjustment of antibiotic therapy, the patient showed clinical, laboratory, and oximetric improvement, with partial resolution of bilioptysis. After completing four weeks of antimicrobial therapy and once clinically stable, a multidisciplinary approach was taken. Percutaneous biliary drainage was established, followed by an ERCP that revealed migrated stent and multiple choledocholithiasis. After biliary clearance, a new metallic stent was successfully deployed, leading to a significant reduction in drainage output. The percutaneous drain was subsequently removed after imaging confirmed resolution of the collection, with no recurrence of bilioptysis to date.
First described by Peacock in 1850,1 a bronchobiliary fistula is an abnormal communication between the biliary system and the bronchial tree. Its most striking clinical feature is bilioptysis, a rare finding considered virtually pathognomonic of the condition. While these fistulas can be congenital, they are more commonly acquired as a result of hepatic infections, trauma, underlying malignancy, or as a complication of hepatobiliary surgery.2
Bronchobiliary fistula represents an exceptional complication of hepatic resection; its prevalence is estimated at under 1%, and it remains largely documented through isolated case reports.2,3 It typically manifests within weeks after surgery, although delayed presentations months or even years later have been reported.4 The underlying mechanism usually involves iatrogenic bile duct injury, resulting in bilioma formation. Infection, sustained biliary pressure, and progressive erosion of the diaphragm facilitate fistulous communication with the thoracic cavity.3
The true incidence of bronchobiliary fistula in the general population is unknown and likely underestimated, as most evidence derives from isolated case reports and small case series. Moreover, a significant proportion of fistulas are not directly visualized on imaging studies, even with advanced techniques, due to their small size or intermittent patency.5
Bilioptysis and bronchobiliary fistula frequently lead to pulmonary complications, including chemical pneumonitis, secondary bacterial infection, cavitary pneumonia, and pleural effusion, all of which contribute to substantial morbidity. Lung injury results from the irritative, inflammatory, and cytotoxic effects of bile acids on the bronchial epithelium and alveolar parenchyma, compounded by bacterial infection from the biliary tract,3,6 which is typically polymicrobial.
In a recent retrospective series of bronchobiliary fistulas following hepatic tumor ablation, all patients developed pneumonia and pleural effusion of varying severity.7 The right lower lobe is most commonly affected, owing to its anatomical proximity to the liver and diaphragm.2 In contrast, bilateral pulmonary involvement, as observed in our patient, is exceptional and has not been previously reported to our knowledge.
Mortality rates fluctuate significantly based on etiology and study series, with reported figures reaching as high as 47% in some historical cohorts.8 Increased mortality risk correlates strongly with diagnostic delay, extensive pulmonary involvement, and the presence of hepatic or neoplastic comorbidities.
Diagnosis of bronchobiliary fistula requires a high index of suspicion and a multimodal aproach. Thoracoabdominal CT and magnetic resonance cholangiography are the most commonly used imaging modalities, although they may fail to demonstrate a clear fistulous tract.2,5,7
Bronchoscopy plays a key diagnostic role by confirming the presence of bile within the respiratory tract, facilitating microbiological sampling, and occasionally allowing for the direct visualization of the fistulous orifice.9 In the present case, while bronchoalveolar lavage (BAL) was performed, quantitative bilirubin measurement was not obtained from the laboratory; however, the macroscopic observation of bile-stained fluid during the procedure provided strong clinical evidence of the communication. Nevertheless, the detection of bilirubin in BAL fluid remains highly advisable in suspected cases, as it provides definitive biochemical confirmation of a bronchobiliary fistula.
ERCP and hepatobiliary scintigraphy can further assist in identifying and characterizing the biliary communication.3 Additionally, ERCP offers a definitive therapeutic pathway through biliary decompression, which is essential to reverse the pressure gradient maintaining the fistula patency.
Management should be multidisciplinary and tailored to disease severity. Conservative treatment, including targeted antibiotic therapy, respiratory support, nutritional optimization, and somatostatin analogues to reduce biliary secretion, has shown favorable outcomes in selected cases.2 In refractory cases or those with persistent biliary leakage, invasive approaches such as endoscopic biliary decompression or surgical fistula closure with drainage of biliary collections may be required to reduce the pressure gradient maintaining fistula patency.8
In the present case, a small bronchobiliary fistula likely developed secondary to progressive diaphragmatic erosion caused by a suprahepatic bilioma. Concurrently, chemical and bacterial pneumonitis – resulting from both direct bile translocation and hematogenous spread of biliary microorganisms – contributed to fistula maturation and bilateral pulmonary involvement. Left vocal cord paralysis, as observed during bronchoscopy, may have facilitated inadvertent bile aspiration, further contributing to the left lower lung compromise.
The delayed onset after hepatectomy and the bilateral pulmonary involvement through distinct etiopathogenic mechanisms make this case truly exceptional and highlight the complex clinical spectrum associated with bilioptysis.
Ultimately, this case underscores that recognizing bilioptysis is a clinical cornerstone for uncovering an otherwise occult bronchobiliary fistula. Given the severe morbidity of bile-induced lung injury, prompt identification remains the most decisive factor in preventing mortality and ensuring the timely, life-saving multidisciplinary intervention required for these complex patients.
Declaration of generative AI and AI-assisted technologies in the writing processDuring the preparation of this work the author used Gemini in order to improve the language, polish the technical writing, and assist with the translation of the manuscript. After using this tool/service, the author reviewed and edited the content as needed and takes full responsibility for the content of the publication.
Informed consentWritten informed consent was obtained from the patient for the publication of this case report and any associated images.
FundingThis research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Authors’ contributionsMarwan Mohamed Choukri is the sole author of this manuscript and was responsible for the clinical management of the patient, the conception and design of the case report, the literature review, and the writing and final approval of the manuscript.
Conflicts of interestNone declared.
