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Giant bladder diverticulum in Ehlers-Danlos syndrome type I causing outflow obstruction
N P Burrows1, B E Monk, J B Harrison
1Department of Dermatology, Addenbrooke's NHS Trust, Cambridge, UK.
Clinical and Experimental Dermatology
|December 23, 1998
Summary
A teenager with Ehlers-Danlos syndrome (EDS) type I experienced urinary retention due to a large bladder diverticulum. This case highlights a rare complication associated with this connective tissue disorder.
Area of Science:
- Urology
- Genetics
- Connective Tissue Diseases
Background:
- Ehlers-Danlos syndrome (EDS) is a group of inherited disorders affecting connective tissues.
- Urinary retention is a potential, though uncommon, complication in EDS patients.
- Giant bladder diverticula are rare outpouchings of the bladder wall.
Observation:
- A 16-year-old patient with diagnosed Ehlers-Danlos syndrome type I presented with recurrent urinary retention.
- Diagnostic evaluation revealed a giant bladder diverticulum as the cause of the urinary retention.
- Literature review was conducted to explore the association between EDS and bladder diverticula.
Findings:
- The case demonstrates a significant association between Ehlers-Danlos syndrome type I and the development of a giant bladder diverticulum.
- Recurrent urinary retention in this patient was directly attributed to the mass effect of the large diverticulum.
- The literature review identified limited but existing evidence linking EDS to urinary tract abnormalities, including diverticula.
Implications:
- This case underscores the importance of considering genitourinary complications in the management of Ehlers-Danlos syndrome.
- Early recognition and management of bladder diverticula are crucial for preventing complications like urinary retention in EDS patients.
- Further research is warranted to elucidate the pathophysiology and prevalence of bladder diverticula in Ehlers-Danlos syndrome.
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