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Hyperammonemic Encephalopathy Approximately 40 Years after Urinary Diversion for Bladder Exstrophy: A Case Report

Hyperammonemic Encephalopathy Approximately 40 Years after Urinary Diversion for Bladder... Urinary diversion using intestine is a common surgical procedure applied in a variety of clinical situations. Patients are prone to metabolic complications due to the inherent differences between bowel and urothelium, which lead to altered absorption of solutes. A rare yet serious metabolic consequence is hyperammonemia, which can present with agitation and coma secondary to encephalopathy. Herein, we report the acute presentation of hyperammonemia in a patient with a ureterosigmoidostomy, highlighting the need for rapid recognition and appropriate management to prevent the development of an encephalopathic coma and possible death. A 39-year-old white male presented acutely unwell, having had a ureterosigmoidostomy for bladder exstrophy as an infant. He deteriorated rapidly on the second day of admission, necessitating sedation and intubation, and was found to have a significantly elevated serum ammonia level of 151 µmol/l (normal 9–35 µmol/l). A diagnosis of hyperammonemic coma was made and he was appropriately treated with a combination of antibiotics, neomycin, lactulose, sodium bicarbonate and rectal catheter drainage. Unusually, his liver function was normal and he had no clinical or microbiological evidence of a urinary tract infection. We discuss our hypothesis for the etiology of hyperammonemia in this case. The above case presentation illustrates a rare yet potentially fatal complication of what is a well-established and commonly performed surgical procedure, namely urinary diversion. It should alert the readers to consider the diagnosis of hyperammonemia in any patient with a urinary diversion who presents acutely unwell, particularly if they have signs of confusion, agitation, stupor and decreased levels of consciousness. Rapid recognition and treatment can prevent serious complications such as encephalopathic coma and death. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Current Urology Karger

Hyperammonemic Encephalopathy Approximately 40 Years after Urinary Diversion for Bladder Exstrophy: A Case Report

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Publisher
Karger
Copyright
© 2011 S. Karger AG, Basel
ISSN
1661-7649
eISSN
1661-7657
DOI
10.1159/000327462
Publisher site
See Article on Publisher Site

Abstract

Urinary diversion using intestine is a common surgical procedure applied in a variety of clinical situations. Patients are prone to metabolic complications due to the inherent differences between bowel and urothelium, which lead to altered absorption of solutes. A rare yet serious metabolic consequence is hyperammonemia, which can present with agitation and coma secondary to encephalopathy. Herein, we report the acute presentation of hyperammonemia in a patient with a ureterosigmoidostomy, highlighting the need for rapid recognition and appropriate management to prevent the development of an encephalopathic coma and possible death. A 39-year-old white male presented acutely unwell, having had a ureterosigmoidostomy for bladder exstrophy as an infant. He deteriorated rapidly on the second day of admission, necessitating sedation and intubation, and was found to have a significantly elevated serum ammonia level of 151 µmol/l (normal 9–35 µmol/l). A diagnosis of hyperammonemic coma was made and he was appropriately treated with a combination of antibiotics, neomycin, lactulose, sodium bicarbonate and rectal catheter drainage. Unusually, his liver function was normal and he had no clinical or microbiological evidence of a urinary tract infection. We discuss our hypothesis for the etiology of hyperammonemia in this case. The above case presentation illustrates a rare yet potentially fatal complication of what is a well-established and commonly performed surgical procedure, namely urinary diversion. It should alert the readers to consider the diagnosis of hyperammonemia in any patient with a urinary diversion who presents acutely unwell, particularly if they have signs of confusion, agitation, stupor and decreased levels of consciousness. Rapid recognition and treatment can prevent serious complications such as encephalopathic coma and death.

Journal

Current UrologyKarger

Published: Jan 1, 2011

Keywords: Bladder exstrophy; Hyperammonemia; Encephalopathy; Urinary diversion

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