Journal Information
Vol. 28. Issue. 5.October 2008
Pages 475-573
Vol. 28. Issue. 5.October 2008
Pages 475-573
DOI:
Full text access
Biliptysis as the initial symptom of a rare complication of autosomal dominant polycystic kidney disease
Biliptisis, síntoma de presentación de una rara complicación en el paciente con enfermedad renal poliquística autosómica dominante.
Visits
5623
Lázaro L. Capote Pereiraa, Sigifredo Montero Ferrera, Ana E. Mera Fernándeza, Eliseo Capote Leyvaa, Mileydis Pozo Calderóna, Yanet Artimes Hernándeza
a Servicio de Nefrología, Diálisis y Trasplante, ISMM Dr. Luis Díaz Soto, La Habana, La Habana, Cuba,
This item has received
Article information
Abstract
Full Text
Bibliography
Download PDF
Statistics
La enfermedad renal poliquística autosómica dominante (ERP) es una enfermedad hereditaria que se manifiesta usualmente en los adultos y que se caracteriza por el desarrollo de múltiples quistes renales y extrarrenales que se desarrollan paulatinamente a lo largo de la vida, con ritmos de crecimiento distintos e impredecibles1. Esta enfermedad afecta cerca de 1 persona por 1 000 y es la más común de las enfermedades poliquísticas renales.
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.
Full Text
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.

Bibliography
[1]
Sanz S, Coto E. Enfermedad poliquística: clasificación, etiopatogenia, diagnóstico y actitudes terapéuticas. Cap 12.1. Enfermedades quísticas. En: Avendaño LH. Nefrología Clínica. 2da ed. Madrid: Ed. Médica Panamericana; 2003.
[2]
Bird R, Fagen K, Taysom D, Silverman ED. A case of bronchobiliary fistula in the setting of adult polycystic kidney and liver disease, with a review of the literature.Clin Nucl Med. 2005 May; 30(5):326-8. [Pubmed]
[3]
Sahin E, Enon S, Cangir AK, Kutlay H, Kavukcu S, Akay H, et al. Single-stage transthoracic approach for right lung and liver hydatid disease. J Thorac Cardiovasc Surg. 2003 Sep; 126(3):769-73. [Pubmed]
[4]
Partrinou V, Dougenis D, Kritikos N, Polydorou A, Vagianos C. Treatment of postoperative bronchobiliary fistula by nasobiliary drainage. Surg Endosc. 2001 Jul; 15(7):758. [Pubmed]
Idiomas
Nefrología (English Edition)
Article options
Tools
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?