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Urinary bladder diverticula and the Ehlers-Danlos syndrome in children
G Levard1, Y Aigrain, L Ferkadji
1Clinique Chirurgicale Infantile, Hôpital Bretonneau, Paris, France.
Insights
Bladder diverticula in Ehlers-Danlos syndrome (EDS) often recur after surgery. These distinct EDS diverticula likely stem from bladder wall anomalies, necessitating surgery only for symptomatic cases.
Area of Science:
- Urology
- Pediatric Surgery
- Genetics
Background:
- Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
- Bladder diverticula can cause significant micturition disturbances.
Observation:
- A 5-year-old boy with EDS underwent surgical removal of two large bladder diverticula causing severe micturition issues.
- A new, asymptomatic diverticulum developed one year post-surgery.
Findings:
- EDS-associated bladder diverticula represent a distinct group with a high rate of postoperative recurrence.
- Recurrence is likely due to an intrinsic anomaly of the vesical wall, not obstruction.
- These diverticula are associated with micturition difficulties and urinary tract infections.
Implications:
- The inherent recurrence of EDS diverticula suggests surgical intervention should be reserved for symptomatic patients.
- Further research into the underlying vesical wall anomaly in EDS is warranted.
- This finding impacts the long-term management strategy for pediatric patients with EDS and bladder diverticula.
Abstract:
Two large bladder diverticula that induced severe micturition disturbances were surgically removed in a 5-year-old boy with Ehlers-Danlos syndrome (EDS). One year after surgery, a new, asymptomatic diverticulum developed. This case is discussed in the light of nine similar cases that have already been published. It is concluded that EDS diverticula form a distinct group characterized by postoperative recurrence. They are not due to vesicourethral obstruction but probably result from an anomaly of the vesical wall. They are responsible for micturition difficulties and urinary tract infections. Their unavoidable recurrence means that surgery should be restricted only to symptomatic patients.