OBJECTIVE To describe the treatments of a patient using the laparoscopic Davydov's method for Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and ureteral reimplantation for hydronephrosis and hydroureter. DESIGN Surgical video article. A consent form from the patient was obtained as appropriate; the nature of the study did not necessitate ethics committee approval. There were no conflicts of interest. SETTING University hospital. PATIENT(S) A 28-year-old woman who presented at our gynecology department with the symptoms of primary amenorrhea and difficult intercourse. She had repaired congenital rectovestibular fistula and imperforate anus at the age of 8. At physical examination, she had a phenotypically normal vulva with a vaginal small pouch (0.5 cm). Magnetic resonance imaging of the pelvis revealed normal ovaries, a primordial uterus, absence of vaginal canal, and a 4.0 × 4.2 × 4.0 cm cystic structure posterior to the bladder. Magnetic resonance urography showed right to left renal crossed-ectopia with inferior fusion, and hydronephrosis and hydroureter from the superior kidney with Grade Ⅳ vesicoureteral reflux. Karyotype was 46, XX. INTERVENTION(S) Saline solution 300 mL was injected into the rectovesical space with an infusion of diluted adrenaline (1:200,000). The goal of this injection was to aid in the identification of tissue planes and reduce blood loss. The space between urethra/bladder and rectum progressively was dissected. Blunt dissection was performed initially with digital separation of tissues. Then, an 8-cm-long neovaginal vault of about 3 cm in diameter was created. The mobilized peritoneum was pulled downward with eight Vicryl sutures and connected to the vaginal epithelium. By cystoscope, we found the left orifice but could not find the right orifice of the hydroureter. Then we ligated the hydroureter by 2-0 absorbable suture near the cyst and cut off the hydroureter, and then incised of all the layers at the top of the bladder to make a bladder flap. We placed the 5 Fr double J stent in the hydroureter and the bladder and anastomozed with the ureteral stump (3-0 Vicryl). Then we removed the cyst laparoscopically. We performed a purse-string stitch to create the apex of the neovagina by taking posterior serosa of the bladder, the pelvic peritoneum between the ovary and rectum, primordial uterus, and anterior rectal serosa. MAIN OUTCOME MEASURE(S) Measurement of the final canal length, sexual function (Female Sexual Function Index), and degree of hydronephrosis. RESULT(S) Three days later, we started to change the vaginal mold and the patient was advised to wear it day and night for the first postoperative month. The vaginal mold had to be worn each night until normal sexual intercourse was possible. Findings confirmed the cyst was Gartner's duct cyst. One year after the surgery, the final canal length was 9 cm and Female Sexual Function Index score was 28. The ultrasound showed that the degree of hydronephrosis of upper moiety was mild. CONCLUSION(S) The distal Wolffian ducts in the female are absorbed but may persist as vestigial remnants (Gartner's duct cysts). A few cases of the combined urogenital-Wolffian anomalies are reported; most of them are associated with the anomalies of müllerian duct fusion, such as Herlyn-Werner-Wunderlich syndrome (uterus didelphys, obstructed hemivagina, and mesonephric duct anomalies). The embryogenesis of the combined anomalies is not completely understood. With comprehensive preoperative assessments, laparoscopic surgery could be a safe and effective treatment to these cases.

中文翻译：

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Mayer-Rokitansky-Küster-Hauser 综合征伴下交叉融合肾异位和 Gartner 导管囊肿的罕见发现：视频病例报告

目的 描述使用腹腔镜 Davydov 方法治疗 Mayer-Rokitansky-Küster-Hauser (MRKH) 综合征和输尿管再植术治疗肾积水和输尿管积水的患者。设计外科视频文章。已酌情获得患者的同意书；研究的性质不需要伦理委员会的批准。没有利益冲突。设置大学医院。患者 一名 28 岁女性，因原发性闭经和性交困难就诊于我们的妇科。她在 8 岁时修复了先天性直肠前庭瘘和肛门闭锁。体检时，她的外阴表型正常，阴道有一个小袋（0.5 cm）。骨盆的磁共振成像显示正常卵巢、原始子宫、没有阴道管，膀胱后有一个 4.0 × 4.2 × 4.0 cm 的囊性结构。磁共振尿路造影显示右至左肾交叉异位，下融合，肾积水和上肾输尿管积水，Ⅳ级膀胱输尿管反流。核型为 46, XX。干预措施 将 300 mL 盐水注入直肠膀胱空间，同时输注稀释的肾上腺素 (1:200,000)。这种注射的目的是帮助识别组织平面并减少失血。逐渐解剖尿道/膀胱和直肠之间的空间。钝性解剖最初是用组织的数字分离进行的。然后，创建了一个 8 厘米长、直径约 3 厘米的新阴道穹窿。用八条 Vicryl 缝线将活动的腹膜向下拉并连接到阴道上皮。通过膀胱镜，我们找到了输尿管的左侧口，但找不到输尿管的右侧口。然后我们在囊肿附近用2-0可吸收缝线结扎输尿管，切断输尿管，然后切开膀胱顶部的所有层，制成膀胱瓣。我们将 5 Fr 双 J 支架放置在输尿管和膀胱中，并与输尿管残端 (3-0 Vicryl) 吻合。然后我们通过腹腔镜切除了囊肿。我们通过取膀胱后浆膜、卵巢和直肠之间的盆腔腹膜、原始子宫和直肠前浆膜进行荷包缝合以创建新阴道的顶点。主要结果测量最终根管长度的测量，性功能（女性性功能指数）和肾积水程度。结果 三天后，我们开始更换阴道模具，并建议患者在术后第一个月日夜佩戴。每天晚上必须佩戴阴道模具，直到可以进行正常的性交。结果证实囊肿是Gartner的导管囊肿。术后一年，终根管长9cm，女性性功能指数28分。超声显示上半部分肾积水程度较轻。结论(S) 女性的远端 Wolffian 导管被吸收，但可能作为残留物（Gartner's 导管囊肿）持续存在。报告了一些合并泌尿生殖-沃尔夫畸形的病例；大多数与苗勒管融合异常有关，例如 Herlyn-Werner-Wunderlich 综合征（双子宫、半阴道阻塞和中肾管异常）。组合异常的胚胎发生尚不完全清楚。通过全面的术前评估，腹腔镜手术可能是这些病例的安全有效的治疗方法。