The Macedo ileum-based reservoir for the management of bladder involvement in epidermolysis bullosa.

INTRODUCTION: Epidermolysis bullosa (EB) can severely affect the urinary tract, leading to strictures and urine outflow obstruction, which pose significant risks to kidney function. Procedures involving the urinary mucosa often exacerbate these issues, making safe bladder emptying a major challenge. This study reviews surgical methods for managing urological complications in EB patients, with a focus on the Macedo procedure, which offers a promising alternative that avoids further bladder mucosa irritation and prevents disease exacerbation.

MATERIAL AND METHODS: We conducted a systematic review of urological surgeries in EB patients using PubMed, Web of Science, and Google Scholar, adhering to PRISMA guidelines. We propose the Macedo procedure as a novel, safe, and effective technique for managing these patients' urological issues.

RESULTS: The review revealed that most existing urological procedures for EB-related urethral obstruction often result in suboptimal outcomes, leading to permanent suprapubic catheters or vesicostomies. The Macedo ileum-based reservoir, with its continent stoma, significantly enhances the quality of life. By ensuring that catheters contact only the ileal mucosa, the procedure prevents exacerbation of EB lesions while maintaining efficient urine outflow and bladder drainage. We applied this technique to our EB patient with complete urethral obstruction following unsuccessful urethral dilations and a vesicostomy. The Macedo reservoir with a continent catheterizable stoma markedly improved the patient's quality of life, with promising initial outcomes despite limited follow-up.

CONCLUSION: The Macedo procedure represents a valuable surgical option for catheterization in EB patients, reducing urothelial irritation and preventing disease progression, and has the potential to improve patients' quality of life.