Men's Health and Reproductive Health Research Center, Shahid Beheshti University of Medical Sciences and Shahid Beheshti University, Tehran, Iran.
Student Research Committee, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.
Am J Mens Health. 2023 Mar-Apr;17(2):15579883231156663. doi: 10.1177/15579883231156663.
Substitution urethroplasty with either a flap or graft is the gold standard for treating long segment urethral strictures. In 1992, Burger and colleagues rediscovered and popularized buccal mucosal graft (BMG). After that El-Kassaby and colleagues, in 1993, used BMG to repair anterior urethral stricture. De la Chapelle syndrome or 46 XX male syndrome is a rare genetic disorder found in 1 in 20,000-25,000 men. This condition described as a presentation of male phenotype along a 46 xx karyotype. In this case report, we report a reconstructive surgery of a 46 XX male syndrome with ambiguous genitalia who presented with the chief complaint of bulbar urethral fistula opened in the perineal space. In this case, we used a buccal mucous graft with the ventral-onlay urethroplasty technique for reconstructing the failed bulbar urethra and closure of the fistula.
替代尿道成形术，无论是皮瓣还是移植物，都是治疗长段尿道狭窄的金标准。1992 年，Burger 和同事重新发现并推广了颊黏膜移植物（BMG）。此后，1993 年 El-Kassaby 及其同事使用 BMG 修复前尿道狭窄。De la Chapelle 综合征或 46XX 男性综合征是一种罕见的遗传性疾病，在 20000-25000 名男性中发现 1 例。这种情况表现为男性表型，伴有 46XX 核型。在本病例报告中，我们报告了 1 例 46XX 男性综合征伴生殖器模糊的重建手术，该患者主要诉诉球部尿道瘘开口于会阴部。在这种情况下，我们使用颊黏膜移植物进行腹侧尿道成形术技术来重建失败的球部尿道并闭合瘘管。
