Bilioptysis associated with ARDS and sepsis: A rare presentation and review of the literature

Abstract

Bilioptysis or the presence of bilirubin in sputum or bronchial secretions is thought to be secondary to the presence of a broncho-biliary fistula. Bilioptysis is not well described in; literature, likely due to the rare nature of its incidence. There have been reports of bilioptysis secondary to other conditions such as hyperbilirubinaemia associated with liver cirrhosis, alcohol-related hepatitis, and haemolysis associated with sickle cell disease without demonstrable evidence of the presence of a fistula . The clinical implications of bilioptysis as well as the effect on the prognosis of the patient are still unclear and warrant further investigation. Here we present the first case of bilioptysis demonstrated on a bronchoalveolar aspirate (BAL) fluid sample in a patient with a brain tumour and possible associated neurogenic ARDS and sepsis.

Introduction

Bilioptysis or the presence of bilirubin in sputum or bronchial secretions is thought to be secondary to the presence of a broncho-biliary fistula. ¹ However, there have been reports of bilioptysis secondary to other conditions such as hyperbilirubinaemia associated with liver cirrhosis, alcohol-related hepatitis, and sickle cell disease without demonstrable evidence of a broncho-biliary fistula.^1–3

There are no reports of bilioptysis associated with sepsis or acute respiratory distress syndrome (ARDS). Here we present the first case of bilioptysis demonstrated on a bronchoalveolar aspirate (BAL) fluid in a patient with a brain tumor and associated ARDS and sepsis.

Case report

A previously fit and well 30 year old man presented to the Emergency Department with continuous tonic-clonic seizures refractory to benzodiazepines. He had a low Glasgow coma score (GCS) score. A blood gas analysis showed metabolic acidosis with a pH of 6.7 and bicarbonate of 5 meq/L. An urgent CT head showed a space-occupying lesion involving the left frontal lobe. A diagnosis of status epilepticus secondary to a brain tumour was made and in view of the low GCS and metabolic acidosis, he was intubated, ventilated, and shifted to the intensive care unit.

Initial biochemistry showed leukocytosis with a white cell count of 17,700 (4000 to 11,000 cells/microL), normal haemoglobin of 15.6 (12 to 16 g/dL), and a marginally elevated serum creatinine of 1.6 (0.7 to 1.1 mg/dL). Other lab parameters were within normal limits. A Chest X-ray showed features suggestive of ARDS. SARS-CoV-2 PCR was negative.

On follow up Patient developed septic shock. An endotracheal (ET) aspirate culture showed the presence of Staphylococcus aureus and Klebsiella pneumoniae and antibiotics were changed accordingly. On day 4 of admission, the patient continued to have fever. Blood and sputum cultures were repeated and antibiotic was escalated to meropenem.

He continued to remain hypoxic with severe ARDS. He was prone ventilated for worsening hypoxia and poor lung compliance His condition continued to deteriorate with a worsening acute kidney injury, hence haemodialysis was initiated.

On day 12, he remained febrile with further deterioration of his hemodyanamic status. Antibiotics were escalated to vancomycin, anidulafungin, ceftazidime/avibactam & aztreonam as per the advice of Infection Disease (ID) consultant.

A CT pulmonary angiogram showed bilateral dense consolidation. There was no evidence of broncho-biliary fistula [Figure 1(a)].

Figure 1.

Golden yellow BAL. (a) There was no evidence of broncho biliary fistula.

Despite being on prone ventilation, he continued to be hypoxaemic and hence VV ECMO was initiated. A bronchoscopy and BAL were performed which showed a golden yellow frothy serous fluid which was aspirated from both lungs [Figure 1].

BAL culture was negative. In view of the unusual colour Special stains were performed on BAL fluid including Sudan black and Oil red O, which were negative. However, bile salts were positive and BAL bilirubin levels were 1.9 mg/dL with a concurrent serum direct bilirubin of 4 mg/dL with a normal indirect bilirubin level. Cytology predominantly showed neutrophils. Peripheral smear was done which showed no evidence of sickeling. Reticulocyte count was normal. A repeat CT head showed an unchanged space-occupying lesion. Unfortunately, the patient continued to deteriorate despite the ECMO support and best medical therapy and succumbed to his illness on day 16.

Discussion

This case was unique in the fact that our patient had a bilioptysis without readily obvious underlying pathophysiology conducive to developing a bilioptysis. Bilioptysis is in itself a rare condition and previous reports have almost always implicated a broncho-biliary fistula.^1,4 A contrast-enhanced CT failed to demonstrate the presence of a fistula. Definite tests like MRCP, Hepatobilliary scintigraphy/HIDA for ruling out bronchobiliary fistula could not be done in our patient as he was critically ill with very high support on ventilator and succumbed to his illness very soon following the bronchoscopy. However, the clinical presentation of flooding of airways with golden colored serous fluid and affecting both the lungs equally and all segments having same golden yellow fluid oozing out, made us consider alveolo-capillary leak as a more likely possibility as compared to the presence of bronchobiliary fistula for the golden yellow BAL.

Although bilioptysis has been associated with sickle cell disease and chest crisis, where haemolysis leads to an increased indirect bilirubin level, our case had the presence of high direct bilirubin with normal indirect bilirubin. ³ Ngo et al reported an isolated case of bilioptysis in a patient with severe alcohol-induced hepatitis with super-added healthcare-associated pneumonia and ARDS, both of which our patient had as well. ² They proposed that the bilioptysis likely occurred secondary to a disruption of the lung alveolar membrane with a subsequent increase in the capillary‐alveolar membrane permeability which could lead to the leakage of bilirubin from the blood. We think that our patient had a similar increase in capillary permeability secondary to his ARDS and sepsis.

Conclusion

Bilioptysis is not well described in literature, likely due to the rare nature of its incidence. This case serves to add information to the existing literature on this rare condition. Patients with severe ARDS and high level of hyperbilirubinemia may be at risk of developing bilioptysis. The clinical implications of bilioptysis as well as the effect on the prognosis of the patient are still unclear and warrant further investigation.