Mirandola Mariateresa, Gui Benedetta, Naldini Angelica, Foschi Nazario, Casula Viola, Testa Antonia Carla, Scambia Giovanni, Catena Ursula
Department of Woman and Child Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
Department of Urology, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy.
Front Surg. 2023 Mar 9;10:1105551. doi: 10.3389/fsurg.2023.1105551. eCollection 2023.
persistent urogenital sinus (PUGS) is a rare condition characterized by abnormal communication between the urethra and vagina, that can frequently be associated with other complex Mullerian malformation (33%). We present a case of PUGS associated with a complex Mullerian malformation diagnosed in adult age after the integration of gynecological ultrasound with hysteroscopy, both performed by expert operators.
27-year-old women was referred to our clinic because of frequent urinary tract infections and cyclic pelvic pain. She was virgo, with normal menstrual cycles and dysmenorrhea. A didelphys uterus and double vagina with bilateral hematocolpos was firstly diagnosed through transrectal and transabdominal ultrasound. An MRI was then performed and a monorenal and ipsilateral ureteral malformation were diagnosed; in addition, a complete absence of the lower third of the vagina and an abnormal origin of the urethra from the bladder were described. Patient underwent lower genital tract endoscopy: external vaginal orifice was obliterated, a PUGS was diagnosed and both vaginas' ostia were detected on the PUGS's posterior-lateral walls. The procedure was performed under transabdominal ultrasound guidance which confirmed the endoscopic anatomical suspicion, avoiding complications such as perforation and misdiagnosis.
ultrasound guided endoscopy plays an essential role in the evaluation of complex anatomic anomalies, such as persistent urogenital sinus (PUGS), leading to a dynamic one-stop diagnosis; it avoids delays and misdiagnosis in preoperative assessment possibly related to the separately application of different radiological and endoscopic techniques.
持续性泌尿生殖窦（PUGS）是一种罕见疾病，其特征为尿道与阴道之间存在异常连通，常与其他复杂的苗勒管畸形相关（33%）。我们报告一例成年后诊断出的与复杂苗勒管畸形相关的PUGS病例，该病例是在由专业操作人员进行的妇科超声与宫腔镜检查相结合后确诊的。
一名27岁女性因频繁尿路感染和周期性盆腔疼痛转诊至我院。她为处女，月经周期正常，有痛经。首先通过经直肠和经腹超声诊断出双子宫和双阴道伴双侧阴道积血。随后进行了磁共振成像（MRI）检查，诊断出单肾和同侧输尿管畸形；此外，还描述了阴道下三分之一完全缺失以及尿道从膀胱异常起源的情况。患者接受了下生殖道内镜检查：阴道外口闭锁，诊断为PUGS，并在PUGS的后侧壁检测到两个阴道开口。该操作在经腹超声引导下进行，证实了内镜检查的解剖学怀疑，避免了穿孔和误诊等并发症。
超声引导下的内镜检查在评估复杂解剖异常（如持续性泌尿生殖窦（PUGS））中起着至关重要的作用，可实现动态一站式诊断；它避免了术前评估中可能因分别应用不同的放射学和内镜技术而导致的延误和误诊。
