OBJECTIVE: To describe the intraoperative and postoperative implications arising from the unexpected diagnosis of a Müllerian anomaly during the surgical management of an ectopic pregnancy.
METHODS: Video article.
METHODS: Academic center.
METHODS: A 39-year-old nulligravid woman with anovulation and irregular menstrual cycles presented to the office. Her urine pregnancy test result was incidentally positive; the serum β-human chorionic gonadotropin level was 5,644 mIU/mL. Outpatient transvaginal ultrasonography demonstrated a 2.1 × 1.7 × 2.2-cm thick-walled structure in the left adnexa without an intrauterine pregnancy. These findings were highly suspicious for a left tubal ectopic pregnancy. The patient was consented for laparoscopy with planned left salpingectomy. The patient included in this video gave consent for publication of the video and posting of the video online including social media, the journal website, scientific literature websites (e.g., PubMed, ScienceDirect, and Scopus), and other applicable sites.
METHODS: Diagnostic laparoscopy did not show an obvious left tubal ectopic pregnancy. Instead, a right unicornuate uterus with a dilated rudimentary left uterine horn was seen. Both fallopian tubes and ovaries appeared normal. These laparoscopic findings were consistent with an ectopic pregnancy in the rudimentary horn. However, in the absence of informed consent for a hemihysterectomy and no evidence of ectopic rupture or bleeding within the pelvis, we decided to proceed with excision of the ectopic pregnancy from the uterine horn. An incision was made over the anterior surface of the uterine horn, and the pregnancy sac was dissected from the underlying myometrium and excised in its entirety. Left salpingectomy was also performed. The patient was discharged home the same day, and her β-human chorionic gonadotropin levels decreased to <5 mIU/mL within 28 days of surgery.
METHODS: Complete resolution of a left rudimentary uterine horn ectopic pregnancy through surgical excision of the pregnancy sac without hemihysterectomy.
RESULTS: Postoperative hysterosalpingography demonstrated a right unicornuate uterus with normal fill and spill of the right fallopian tube. Magnetic resonance imaging of the pelvis confirmed the findings of a right unicornuate uterus with a noncommunicating left rudimentary uterine horn that did not contain any endometrial tissue. Thus, the patient did not require an interval hemihysterectomy. She underwent letrozole and intrauterine insemination treatment 5 months after the initial surgery, which resulted in a clinical intrauterine pregnancy. However, this pregnancy was terminated in the early second trimester because of findings of trisomy 18. She conceived naturally 1 year later, and this pregnancy resulted in a full-term vaginal birth at 39 weeks of gestation.
CONCLUSIONS: Undiagnosed or unexpected Müllerian anomalies can impact the standard intraoperative and postoperative management of ectopic pregnancies.

UNASSIGNED: Septate uterus is the commonest of congenital structural uterine anomaly with highest failure rate reproductive. It is secondary to incomplete resorption of the Müllerian duct during embryogenesis. Pregnancy in septate uterus carries with it maternal-fetal risk. The importance of this report is to provide data and encourage report of similar conditions in this region.
METHODS: A 25-Year-old, female booked G7P3A3 at 39 weeks and 4 days with history of miscarriages and preterm delivery for second, fourth and fifth pregnancies. She had an emergency cesarean section on the last one due to a contracted pelvis on a term pregnancy. Delivered a male baby of 3000 g, APGAR score of 9, 10, 10 respectively at the first, fifth and tenth minutes. Intraoperative findings revealed a partial septate uterus. The abdominopelvic cavity organs were anatomically normal.
UNASSIGNED: Septate uterus is associated with adverse obstetric outcomes, like recurrent miscarriages, premature delivery, fetal malposition, intrauterine growth retardation, postpartum hemorrhage. 9-35 % of women with this malformation may experience infertility. Although the efficacy of septoplasty and preconception interventions has not been fully established in improving obstetric outcomes, in current medical practice these procedures are suggested.
CONCLUSIONS: Septate uterus as congenital uterus malformations, should be suspected in any women with obstetric complications high risk such as miscarriage, preterm delivery and malpresentation.

OBJECTIVE: The objective of this video is to demonstrate the diagnosis, evaluation, and techniques for surgical management of a longitudinal vaginal septum, a rare müllerian anomaly.
METHODS: This is a stepwise demonstration of evaluation and surgical techniques with video narration.
METHODS: The incidence of müllerian defects, which can include any anomaly in the fallopian tube, uterus, cervix, or vagina, has been estimated to be 2% to 4% [1]; 30% to 40% of patients with müllerian defects also have associated renal anomalies [1,2]. In normal development, the müllerian ducts fuse at 10 weeks\' gestation and the septum between the 2 ducts is absorbed in a caudal to cephalad direction [3]. The exact incidence of complete longitudinal vaginal septa is unknown as they are very rare [4]. Longitudinal vaginal septa may cause dyspareunia, inability to have penetrative intercourse, labor dystocia, or hygiene issues and be very emotionally distressing for patients [5].
METHODS: Preoperative evaluation of an adult with longitudinal vaginal septum that included a careful physical examination and abdominal and pelvic imaging. Intraoperative resection with key strategies: (1) placing a Foley catheter to help avoid urinary tract injuries and (2) intermittent rectal examinations to retract the rectum away from the plane of dissection.
CONCLUSIONS: Patients who present with longitudinal vaginal septa should undergo evaluation for uterine and renal anomalies. Here, we show that resection of longitudinal vaginal septa in adults is feasible and appropriate for patients who present with inability to have penetrative intercourse. Intraoperatively, care should be taken to avoid injuring the rectum or urinary tract.

Uterine diverticulum is a rare congenital malformation caused by abnormal fusion of the Müllerian ducts. The diagnosis of uterine diverticulum is difficult, and it is often misdiagnosed as a Müllerian duct anomaly, degenerated uterine fibroid, or ovarian cyst. We herein report a case of uterine diverticulum mimicking an ovarian endometriotic cyst. A multiparous woman with a history of normal vaginal delivery underwent magnetic resonance imaging for investigation of lower abdominal pain and fever. A 155-mm cystic lesion was observed on the ventral side of the uterus. The content of the cyst showed high signal intensity on T1- and T2-weighted images with precipitates of low signal intensity on the dorsal side, suggesting an endometriotic cyst of the ovary. Surgical and pathological findings revealed that the cyst was pedunculated from the anterior uterine body and composed of 3 layers: CD10-positive endometrium, a smooth muscle layer, and serosa. A uterine diverticulum was definitively diagnosed.

OBJECTIVE: To summarize the available evidence on the laparoscopic Davydov and Vecchietti methods to create a neovagina and to compare these techniques with a focus on neovaginal length, sexual function, operative time, and complications.
METHODS: A systematic electronic search up to August 2022 using PubMed and Embase is performed.
METHODS: Not applicable.
METHODS: Women with Mayer-Rokistansky-Küster-Hauser syndrome.
METHODS: All published clinical studies concerning the laparoscopic Davydov and laparoscopic Vecchietti procedures as a surgical technique to create a neovagina in women with Mayer-Rokistansky-Küster-Hauser syndrome were obtained. The guidelines for the preferred reporting items for systematic reviews and meta-analysis were followed. The following data were extracted: operative time, hospital stay, major early complications (within 3 months postsurgery), dilation therapy, neovaginal length, vaginal discharge, vaginal stenosis, time to sexual activity, sexual satisfaction, penetrative sexual activity, dyspareunia, score on the Female Sexual Function Index (FSFI), and duration of follow-up. The Newcastle Ottawa Scale was used to assess the quality of articles.
METHODS: Neovaginal length, FSFI scores, operative time, and complications.
RESULTS: A total of 1,163 articles were identified, of which 33 studies were included in this systematic review. Of these, 12 studies (380 patients) are related to the Davydov method, 19 studies (1,126 patients) to the Vecchietti method, and 2 articles concern both. There is clinical heterogeneity and variety in the quality of the studies. Eighteen studies were included in the meta-analyses. The mean neovaginal length 12 months after the Davydov method is 8.3 cm (95% confidence interval [CI] 8.1-8.6), vs. 8.7 cm (95% CI 7.2-10.3) after the Vecchietti method. The mean FSFI score after the Davydov method is 28.9 (95% CI 26.8-31.1), compared with 27.5 (95% CI 25.0-30.1) after the Vecchietti method. The operative time of the Davydov method is 126 minutes (95% CI 109-143), compared with 40 minutes (95% CI 35-45) of the Vecchietti method.
CONCLUSIONS: The operations yield comparable neovaginal length, sexual function, and complication rates. The mean FSFI scores indicate no sexual dysfunction in either group. The operative time of the Davydov method is significantly longer. There is no superiority shown for one of the surgical techniques in functional terms.

暂无摘要。

OBJECTIVE: To enhance evidence-based knowledge on long-term sequalae in patients with surgically corrected obstructing Müllerian anomalies.
METHODS: This long-term case-control study included patients with menstrual outflow obstruction due to congenital anomalies of the uterus or vagina, who were at least 18 years old, and for whom 2 years had elapsed since the first surgery at the start of this study. The control group consisted of women without current gynecological problems. Patients underwent a surgical correction at the Radboud University Medical Center Nijmegen between 1980 and 2013. Of 78 patients approached, 38 (49%) were included in this study. The control group consisted of 54 females. The main outcome measures were pain and health state. The following questionnaires were used: Visual Analogue Scale pain scores, European Quality of Life-5 Dimensions questionnaire (EQ-5D-3L) and the adapted Endometriosis Health Profile questionnaire (EHP-30).
RESULTS: Patients had higher actual and maximum abdominal pain scores compared with controls (11 vs 0 [P = 0.007] and 48 vs 21 [P = 0.035], respectively). Based on the EQ-5D-3L scores, patients had more pain and discomfort (P = 0.005), more mood problems (P = 0.023), and a poorer subjective health state (P = 0.002) and self-rated health state (P = 0.031). Based on the EHP-30, patients had a significant poorer self-rated health state on four out of five subscales.
CONCLUSIONS: In this study, following surgically corrected menstrual obstruction, patients had statistically significant higher abdominal pain scores and a poorer self-rated health state compared with controls.

UNASSIGNED: Accurate diagnosis of a müllerian anomaly is essential for appropriate management and prevention of complications. However, diagnosis is often missed or delayed.
UNASSIGNED: This is a case of a nulliparous woman with a müllerian anomaly diagnosed at the age of 36 despite prior evaluation with ultrasound and laparoscopy. Magnetic resonance imaging (MRI) suggested a unicornuate uterus with a right non-communicating rudimentary horn. Hysteroscopy and chromopertubation confirmed the diagnosis. The rudimentary horn was resected laparoscopically using bipolar energy.
UNASSIGNED: An undiagnosed müllerian anomaly should be considered in the setting of persistent dysmenorrhea. Three-dimensional ultrasound or MRI should be used judiciously in patients with refractory dysmenorrhea or when ultrasound raises concern for a müllerian anomaly.

BACKGROUND: There are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly.
OBJECTIVE: To describe another variant of UD and compare the presentation and management across different institutions METHODS: This was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used.
RESULTS: Five patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain.
CONCLUSIONS: Diagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.

BACKGROUND: Despite the well-established embryological relationship in the development of renal and Müllerian structures, no clear guidelines exist regarding screening for Müllerian anomalies (MA) in the setting of a renal anomaly (RA). Delayed diagnosis of MA can have significant reproductive consequences.
OBJECTIVE: To investigate the prevalence of coexisting MA in patients with congenital RA.
METHODS: This is a retrospective cohort study of females age 12-35 years with a diagnosis of RA, identified by diagnosis codes, who were followed for care between 2013 and 2020. Data were collected on demographics, medical history, clinical presentation, and imaging studies. Descriptive statistics were used to summarize the data. This study was IRB approved.
RESULTS: A total of 465 patients were included in this study, of whom 326 patients (70.3%) had a pelvic evaluation during the study period. Of these 326 patients, 125 (38.3%) were found to have coexistent MA. About one-third of patients who underwent pelvic evaluation due to pain were found to have MA. For 69.6% (87/125) of patients with MA, the RA was diagnosed prior to the MA. The average age at time of RA diagnosis was 6.4 ± 8.8 years and the average age of MA diagnosis was 16.4 ± 6.9 years. Forty-eight (38.4%) patients had obstructive anomalies. Of the Müllerian obstructions, 93.8% were treated with urgent surgery and the remainder started on hormonal suppression. The prevalence of MA was dependent on the RA diagnosis (Figure). Of patients with a solitary kidney, 67.1% were diagnosed with MA. For other parenchymal RA, the prevalence of MA was 20-23%. In patients with solitary kidney, uterus didelphys was the most common MA (52.1%). Thirty percent of patients with a solitary kidney were diagnosed with an obstructive MA.
CONCLUSIONS: In this study, 38% of patients with RA who underwent a pelvic evaluation were found to also have MA. Our study shows the strongest association between MA and solitary kidney, but also emphasizes a significant risk with other RA. Almost 40% of patients with diagnosed MA were found to have an obstruction that required urgent treatment. Delays in diagnosis and treatment of Müllerian obstructions can be detrimental for future reproductive health, due to risk of chronic pain, infertility, infection, and endometriosis. Given the high prevalence of MA in patients with RA, especially those with congenital solitary kidney, routine screening with pelvic ultrasound should be performed around the age of expected menarche.