D B Glazier1, M R Zaontz. 1. Section of Pediatric Urology, The Children's Regional Hospital at Cooper Hospital/University Medical Center, Camden, New Jersey, USA.
Abstract
PURPOSE: Epidermolysis bullosa is a devastating rare disorder that rarely presents with urological complications. We report our experience with and review the literature on this disorder. MATERIALS AND METHODS: Two brothers with epidermolysis bullosa presented to our center with severe dysuria and urinary tract obstruction caused by meatal stenosis. The obstruction was temporarily relieved by meatotomy but recurrent obstructive skin blistering with severe dysuria required ureterosigmoidostomy for palliation of symptoms. RESULTS: Both children tolerated ureterosigmoidostomy well with 1 requiring bicarbonate supplementation for metabolic acidosis. Ureterosigmoidostomy greatly improved quality of life, and both children have complete symptom relief. CONCLUSIONS: Epidermolysis bullosa is usually a severe illness associated with a poor prognosis. Crippling urological symptoms may develop due to recurrent skin blistering causing severe dysuria and secondary obstruction. Ureterosigmoidostomy, despite its complications, provides significant palliation for patients with recalcitrant symptoms.
PURPOSE: Epidermolysis bullosa is a devastating rare disorder that rarely presents with urological complications. We report our experience with and review the literature on this disorder. MATERIALS AND METHODS: Two brothers with epidermolysis bullosa presented to our center with severe dysuria and urinary tract obstruction caused by meatal stenosis. The obstruction was temporarily relieved by meatotomy but recurrent obstructive skin blistering with severe dysuria required ureterosigmoidostomy for palliation of symptoms. RESULTS: Both childrentolerated ureterosigmoidostomy well with 1 requiring bicarbonate supplementation for metabolic acidosis. Ureterosigmoidostomy greatly improved quality of life, and both children have complete symptom relief. CONCLUSIONS: Epidermolysis bullosa is usually a severe illness associated with a poor prognosis. Crippling urological symptoms may develop due to recurrent skin blistering causing severe dysuria and secondary obstruction. Ureterosigmoidostomy, despite its complications, provides significant palliation for patients with recalcitrant symptoms.
Authors: Guangping Tai; Parisa Ranjzad; Fiona Marriage; Samrina Rehman; Helen Denley; Jill Dixon; Karen Mitchell; Philip J R Day; Adrian S Woolf Journal: PLoS One Date: 2013-11-18 Impact factor: 3.240