Abstract：
To show a conservative surgical treatment for a female adolescent affected by Wunderlich syndrome with didelphys uterus and obstructed hemivagina.
Stepwise demonstration of the technique with narrated video footage.
In the context of obstructive congenital müllerian anomalies, involving a stagnation of menstrual blood, the Wunderlich syndrome is the most common and constantly characterized by the duplicity of the uterine body, by the presence of an imperforate hemivagina, and by renal agenesis ipsilateral to the obstructed hemivagina. The imperforate hemivagina leads to dysmenorrhea and abdominal pain, owing to the hematocolpos and the hematometra, which arose immediately after the menarche. This is the case of a 14-year-old female adolescent affected by Wunderlich syndrome referred to the San Raffaele Hospital adolescent center (Milan, Italy) for dysmenorrhea and abdominal pain. At vaginal exploration, a right imperforated hemivagina and hematocolpos were highlighted. A diagnostic magnetic resonance imaging (MRI) found the duplicity of the uterine body, the hematometra, and the right renal agenesis.
A combination of explorative laparoscopy and surgical vaginal time leads to the excision of the vaginal septum that allows the drainage of the hematocolpos and of the hematometra. Three surgical steps are described: 1. First laparoscopic step: exploration of the abdominal cavity with the detection of a double uterine body, an enlarged hemiuterus, and an enlarged hemivagina caused by the hematometra and the hematocolpos. Careful evaluation of adnexa for eventual presence of hematosalpinx. 2. Vaginal step divided into the following: (A) puncture of the vaginal tumescence (corresponding to the imperforated hemivagina) with a 19-gauge needle mounted on a syringe. Aspiration results in thick creamy black material (old menstrual blood). (B) In correspondence with the needle puncture, a full-thickness incision of the vaginal wall widely opening the second uterine cervix and (C) stabilization of the opening by the marsupialization of the edge of the obstructed hemivagina were performed. 3. Second laparoscopic step: having emptied the hematocolpos of the left hemiuterus, the didelphys uterus and the disappearance of the hematocolpos can be clearly seen.
Here, we demonstrate a conservative surgical approach for the treatment of Wunderlich syndrome. This rare malformation is characterized by an extreme variability of the anatomic presentation, and the precise identification of the variety together with the early diagnosis is of fundamental importance for the surgical correction.
Stepwise demonstration of the technique with narrated video footage.
In the context of obstructive congenital müllerian anomalies, involving a stagnation of menstrual blood, the Wunderlich syndrome is the most common and constantly characterized by the duplicity of the uterine body, by the presence of an imperforate hemivagina, and by renal agenesis ipsilateral to the obstructed hemivagina. The imperforate hemivagina leads to dysmenorrhea and abdominal pain, owing to the hematocolpos and the hematometra, which arose immediately after the menarche. This is the case of a 14-year-old female adolescent affected by Wunderlich syndrome referred to the San Raffaele Hospital adolescent center (Milan, Italy) for dysmenorrhea and abdominal pain. At vaginal exploration, a right imperforated hemivagina and hematocolpos were highlighted. A diagnostic magnetic resonance imaging (MRI) found the duplicity of the uterine body, the hematometra, and the right renal agenesis.
A combination of explorative laparoscopy and surgical vaginal time leads to the excision of the vaginal septum that allows the drainage of the hematocolpos and of the hematometra. Three surgical steps are described: 1. First laparoscopic step: exploration of the abdominal cavity with the detection of a double uterine body, an enlarged hemiuterus, and an enlarged hemivagina caused by the hematometra and the hematocolpos. Careful evaluation of adnexa for eventual presence of hematosalpinx. 2. Vaginal step divided into the following: (A) puncture of the vaginal tumescence (corresponding to the imperforated hemivagina) with a 19-gauge needle mounted on a syringe. Aspiration results in thick creamy black material (old menstrual blood). (B) In correspondence with the needle puncture, a full-thickness incision of the vaginal wall widely opening the second uterine cervix and (C) stabilization of the opening by the marsupialization of the edge of the obstructed hemivagina were performed. 3. Second laparoscopic step: having emptied the hematocolpos of the left hemiuterus, the didelphys uterus and the disappearance of the hematocolpos can be clearly seen.
Here, we demonstrate a conservative surgical approach for the treatment of Wunderlich syndrome. This rare malformation is characterized by an extreme variability of the anatomic presentation, and the precise identification of the variety together with the early diagnosis is of fundamental importance for the surgical correction.
摘要：
目的探讨1例女性青少年Wunderlich综合征合并双子宫和半阴道阻塞的保守手术治疗方法。
使用叙述的视频片段逐步演示该技术。
在梗阻性先天性穆勒异常的背景下，包括月经血停滞，Wunderlich综合征是最常见的，经常以子宫体的双重性为特征，由于半阴道无孔，以及阻塞的半阴道同侧的肾脏发育不全。半阴道无孔导致痛经和腹痛，由于血细胞计数和血细胞计数，这是在初潮后立即出现的。这是一名受Wunderlich综合征影响的14岁女性青少年转诊到圣拉斐尔医院青少年中心(米兰，意大利）用于痛经和腹痛。在阴道探查时，突出显示右侧半阴道无孔和血肿。诊断磁共振成像（MRI）发现子宫体的重复性，血肿，和右肾发育不全。
探查性腹腔镜检查和手术阴道时间的组合导致切除阴道隔，从而可以引流血结肠和血结肠。描述了三个手术步骤:1.腹腔镜第一步：探查腹腔，检测双子宫体，增大的半子宫,以及由血肿和血肿引起的半阴道扩大。仔细评估附件是否存在输卵管积血。2.阴道步骤分为以下步骤：（A）用安装在注射器上的19号针穿刺阴道肿胀（对应于无孔的半阴道）。抽吸导致厚厚的乳脂状黑色物质(旧的经血)。（b）与针头穿刺相对应，对阴道壁进行全层切口,广泛地打开第二子宫颈和(C)通过阻塞的半阴道边缘的袋状化使开口稳定。3.腹腔镜第二步：排空左半子宫的血结肠，可以清楚地看到子宫和血结肠的消失。
这里,我们证明了治疗Wunderlich综合征的保守手术方法.这种罕见的畸形的特点是解剖表现的极端变异性，品种的准确识别和早期诊断对于手术矫正至关重要。
使用叙述的视频片段逐步演示该技术。
在梗阻性先天性穆勒异常的背景下，包括月经血停滞，Wunderlich综合征是最常见的，经常以子宫体的双重性为特征，由于半阴道无孔，以及阻塞的半阴道同侧的肾脏发育不全。半阴道无孔导致痛经和腹痛，由于血细胞计数和血细胞计数，这是在初潮后立即出现的。这是一名受Wunderlich综合征影响的14岁女性青少年转诊到圣拉斐尔医院青少年中心(米兰，意大利）用于痛经和腹痛。在阴道探查时，突出显示右侧半阴道无孔和血肿。诊断磁共振成像（MRI）发现子宫体的重复性，血肿，和右肾发育不全。
探查性腹腔镜检查和手术阴道时间的组合导致切除阴道隔，从而可以引流血结肠和血结肠。描述了三个手术步骤:1.腹腔镜第一步：探查腹腔，检测双子宫体，增大的半子宫,以及由血肿和血肿引起的半阴道扩大。仔细评估附件是否存在输卵管积血。2.阴道步骤分为以下步骤：（A）用安装在注射器上的19号针穿刺阴道肿胀（对应于无孔的半阴道）。抽吸导致厚厚的乳脂状黑色物质(旧的经血)。（b）与针头穿刺相对应，对阴道壁进行全层切口,广泛地打开第二子宫颈和(C)通过阻塞的半阴道边缘的袋状化使开口稳定。3.腹腔镜第二步：排空左半子宫的血结肠，可以清楚地看到子宫和血结肠的消失。
这里,我们证明了治疗Wunderlich综合征的保守手术方法.这种罕见的畸形的特点是解剖表现的极端变异性，品种的准确识别和早期诊断对于手术矫正至关重要。
