Bladder exstrophy (BE)–epispadias complex is a rare congenital malformation that impacts the normal development of the lower abdominal wall, the bladder, and genitalia. The incidence of BE is approximately one per 10,000–50,000 births. There is an increased incidence in males, being twice as common in boys as compared to girls. Improved prenatal diagnosis has increased the ability to detect this condition and helps prepare the expectant parents for the appearance of their baby and for the clinical management journey ahead of them. The timing and technique of repair varies based on surgeon preference as well as clinical experience, and both play a role in the clinical outcome of the patient, burden of care, and cost of care. There has been a significant shift away from emergent repair of BE within the initial 72 h of delivery. Elective repair of BE permits multiple benefits including allowing the parents to bond with their child, shared decision-making about the nature and timing of intervention, and optimization of nutrition and growth of the bladder plate. The parental expectations of reconstructive surgery are that the procedure will restore the normal appearance of the abdominal wall and genitalia and that their child will achieve normal urinary continence and sexual function.

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Surgical Considerations: Indications and Timing for Surgery (Defining Outcomes and Expectations of Exstrophy Repair)

  • Pramod P. Reddy

摘要

Bladder exstrophy (BE)–epispadias complex is a rare congenital malformation that impacts the normal development of the lower abdominal wall, the bladder, and genitalia. The incidence of BE is approximately one per 10,000–50,000 births. There is an increased incidence in males, being twice as common in boys as compared to girls. Improved prenatal diagnosis has increased the ability to detect this condition and helps prepare the expectant parents for the appearance of their baby and for the clinical management journey ahead of them. The timing and technique of repair varies based on surgeon preference as well as clinical experience, and both play a role in the clinical outcome of the patient, burden of care, and cost of care. There has been a significant shift away from emergent repair of BE within the initial 72 h of delivery. Elective repair of BE permits multiple benefits including allowing the parents to bond with their child, shared decision-making about the nature and timing of intervention, and optimization of nutrition and growth of the bladder plate. The parental expectations of reconstructive surgery are that the procedure will restore the normal appearance of the abdominal wall and genitalia and that their child will achieve normal urinary continence and sexual function.