Ventral-Onlay Buccal Mucosal Graft Urethroplasty of a Perineal Fistula in a 26-Year-Old Patient With 46 XX Male Syndrome: A Case Report. Issue 2 (April 2023)

• Record Type: Journal Article
• Authors: Hosseini, Jalil; Zamani Hajiabadi, Arshia; Mirjalili, Ali Mohammad
• Is Part Of: American journal of men's health. Volume 17:Issue 2(2023)
• Publication Date: 2023-04
• Subjects: buccal mucosal graft -- 46 XX male syndrome -- reconstructive surgery; Men -- Diseases -- Periodicals; Sex factors in disease -- Periodicals; Men -- Health and hygiene -- Periodicals; Men -- Health and hygiene -- United States -- Periodicals; Men -- Medical care -- Periodicals; 362.10811
• DOI: 10.1177/15579883231156663
• Languages: English
• ISSNs: 1557-9883

Abstract

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.