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Pelvic dural ectasia leading to bilateral ureteric obstruction in the pediatric patient

Henry Tran1, Alex Kavanagh, Paul Steinbok

  • 1Department of Urological Sciences, University of British Columbia, Vancouver, BC;

Canadian Urological Association Journal = Journal De L'Association Des Urologues Du Canada
|May 15, 2013
PubMed

Insights

A rare case of a teenager with Marfan syndrome experienced severe urinary obstruction due to pelvic cysts. Surgical intervention successfully managed this complex condition, highlighting the importance of multidisciplinary care.

Area of Science:

  • Neurosurgery
  • Urology
  • Pediatric Medicine
  • Genetics

Background:

  • Marfan syndrome is a genetic disorder affecting connective tissue.
  • Pelvic dural ectasia is a rare complication of Marfan syndrome.
  • Dural ectasia can cause mass effect on pelvic organs.

Purpose of the Study:

  • To report a rare case of bilateral ureteric obstruction in a pediatric patient with Marfan syndrome and pelvic dural ectasia.
  • To discuss the diagnostic and management strategies for this unique presentation.

Main Methods:

  • Case report detailing a 16-year-old patient with Marfan syndrome.
  • Utilized magnetic resonance imaging (MRI) for diagnosis.
  • Involved combined neurosurgical and urologic surgical resection.

Main Results:

  • Identified large pelvic cysts causing bilateral ureteric obstruction and severe hydroureteronephrosis.
  • Observed extensive sacral dural ectasia compressing pelvic structures.
  • Successful surgical resection of pelvic masses relieved obstruction.

Conclusions:

  • Pelvic dural ectasia can lead to significant genitourinary complications.
  • Multidisciplinary management is crucial for rare conditions like this.
  • Early diagnosis and intervention can prevent irreversible kidney damage.

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