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Related Experiment Videos

Bladder obstruction in Hunter's syndrome.

D Rigante1, P Ferrara, R Ricci

  • 1Department of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy.

Scandinavian Journal of Urology and Nephrology
|August 19, 1999
PubMed
Summary

This case study details bladder obstruction in Hunter syndrome, a rare genetic disorder. Successful treatment involved intermittent catheterization and intravesical oxybutynin chloride, resolving the urinary blockage.

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Area of Science:

  • Urology
  • Medical Genetics
  • Biochemistry

Background:

  • Hunter syndrome (Mucopolysaccharidosis II) is a rare X-linked genetic disorder.
  • It results from a deficiency in the enzyme iduronate-2-sulfatase, leading to glycosaminoglycan accumulation.
  • This accumulation can affect multiple organ systems, including the urinary tract.

Observation:

  • A patient diagnosed with Hunter syndrome presented with acute, painful urinary retention.
  • Diagnostic imaging, including ultrasonography and cystometrography, confirmed bladder outlet obstruction.
  • The obstruction was characterized by the inability to void, causing significant discomfort.

Findings:

  • The patient underwent intermittent catheterization to relieve the immediate obstruction.

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  • Intravesical oxybutynin chloride was administered to manage bladder function.
  • This combined therapeutic approach resulted in the successful functional resolution of the bladder obstruction.

    • Implications:

    • This case highlights a potential urological complication of Hunter syndrome.
    • It suggests that intermittent catheterization combined with anticholinergic therapy can be an effective treatment strategy.
    • Further research may explore the prevalence and optimal management of bladder dysfunction in Hunter syndrome patients.