Molina E, Cerdá J, Sánchez-Martín R, Romero R, Estellés C, Aguilar F, Vázquez J
Servicio de Cirugía Pediátrica, Hospital General Universitario Gregorio Marañón, Madrid.
Cir Pediatr. 2000 Apr;13(2):54-7.
The urogenital sinus is an embriological anomaly which consists on a common channel from the urethra and vagina. The major incidence is produced in the congenital adrenal hyperplasia's context. In certain occasions it can be associated to an imperforate anus, then the malformation is called a cloacal defect. There are multiple surgical techniques to correct this malformation and different therapeutical approaches (without surgery, surgery at one or various times, early or delayed surgery) being the newest one the total urogenital mobilization. The purpose of this work is to reflect our experience with this technique. We present seven girls with urogenital sinus (3 with congenital adrenal hyperplasia, 2 with a cloacal defect, and the other 2 associated to ambiguous genitalia). Five patients were operated in the first year of life. The outcome has been favorable, and the cosmetic and functional results have been very good. The surgical technique consists on posterior sagittal incision, it can be done transanorectal if necessary, the urethrovaginal union is achieved and both structures are mobilized together, connecting them to the perineum, as a single unit. We believe that the total urogenital mobilization is actually the surgical technique to be chosen in every of urogenital sinus, for being easier, allowing early realization (girls under 1 year old), correcting simultaneously other anomalies, reducing the complications (urethrovaginal fistula, vaginal structure, or acquired vaginal atresia); and the result is excellent.
泌尿生殖窦是一种胚胎学异常，它由尿道和阴道的共同通道组成。主要发病于先天性肾上腺皮质增生症的背景下。在某些情况下，它可能与肛门闭锁相关，此时这种畸形被称为泄殖腔缺损。有多种手术技术可用于矫正这种畸形，以及不同的治疗方法（非手术、一次或多次手术、早期或延迟手术），最新的方法是全泌尿生殖系统游离术。这项工作的目的是反映我们在这项技术上的经验。我们报告了7例泌尿生殖窦女孩（3例患有先天性肾上腺皮质增生症，2例患有泄殖腔缺损，另外2例伴有生殖器模糊）。5例患者在出生后第一年接受了手术。结果良好，美容和功能效果都非常好。手术技术包括后矢状切口，必要时可经肛门直肠进行，实现尿道阴道联合，将两个结构一起游离，作为一个整体连接到会阴。我们认为，全泌尿生殖系统游离术实际上是每种泌尿生殖窦病例都应选择的手术技术，因为它更简单，可早期实施（1岁以下女孩），能同时矫正其他异常，减少并发症（尿道阴道瘘、阴道结构异常或后天性阴道闭锁）；而且效果极佳。
