To the editor: Autosomal dominant polycystic kidney disease (PKD) is an inherited disease usually occurring in adults and characterized by gradual development of multiple renal and extrarenal cysts during life, with different and unpredictable growth rates.1 This disease occurs in approximately 1 out of every 1,000 people, and is the most common polycystic kidney disease.



This letter discusses the case of a 62-year-old female patient, a Caucasian with chronic renal failure (CRF)  secondary to PKD who attended the office complaining of fever for the past 4 days, followed by right chest pain irradiating to hypochondrium and right flank, associated to cough and breathlessness. Some days later she suffered increased breathlessness and cough, which produced an intense yellow expectoration, was sudden, and left an unpleasant taste (biliptysis). The patient experienced right pleural effusion. Minimum undifferentiated pleurotomy was performed, and broad spectrum antimicrobial treatment was administered. She subsequently showed an image with the appearance of a walled abscess in the right base, in addition to pneumothorax, which prompted the decision to perform conventional surgery, at which a biliobronchial fistula from the lower pulmonary lobe and a hepatic cyst, joined together and fibrosed with the phrenic nerve in the fistulous tract area, were found (fig. 1).



This is the first case reported in Cuba of a patient with PKD having a biliobronchial fistula from a liver cyst that

communicated first with the pulmonary parenchyma and subsequently entered the pleural space. The main symptom was biliptysis. A single case has been reported in the literature reviewed.2 This occurred in a patient with PKD and liver cysts in whom diagnosis was made using high-resolution computed tomography with no contrast agent. Hepatic cysts most commonly reported as causing biliobronchial fistula are those from an infectious origin, such as hydatid and amebic cysts.3,4 Data on treatment of biliobronchial fistula in the setting of PKD are few or none, because of the rarity of this complication. Drainage through an endoscopically inserted nasobiliary catheter as reported by Partrinou4, achieving reverse drainage to the biliobronchial fistula by decreasing duodenal pressures as compared to biliary tract pressures, is a noninvasive method that would be the procedure of choice for treating patients at a high surgical risk. The interest of this case lies in that, while this type of complication is uncommon in PKD patients, it should be considered in order to make an accurate diagnosis and start adequate management. It is also recommended that this condition is added to the long list of complications of PKD patients with CRF.