Herlyn-Werner-Wunderlinch: An unusual presentation in a patient with Prader-Willi syndrome.

Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear.

Peripartal management of dichorial twin pregnancy in a bicornuate bicollis uterus: a case report and review of the literature.

INTRODUCTION: The management of a pregnancy in a bicornuate uterus is particularly challenging. A bicornuate uterus is a rare occurrence and a twin pregnancy in a bicornuate uterus even more rare. These pregnancies call for intensive diagnostic investigation and interdisciplinary care. CASE PRESENTATION: We report on a 27-year-old European woman patient (gravida I, para 0) with a simultaneous pregnancy in each cavity of a bicornuate bicollis uterus after embryo transfer. The condition was confirmed by hysteroscopy and laparoscopy. Several unsuccessful in vitro fertilization (IVF) attempts had been performed earlier before embryo transfer in each cornus. After a physiological course of pregnancy with differential screening at 12 + 6 weeks and 22 + 0 weeks of gestation, the patient presented with therapy-resistant contractions at 27 + 2 weeks. This culminated in the uncomplicated spontaneous delivery of the leading fetus and delayed spontaneous delivery of the second fetus. DISCUSSION: Only 16 cases of twin pregnancy in a bicornuate unicollis uterus have been reported worldwide and only 6 in a bicornuate bicollis uterus. The principal risks in such pregnancies are preterm labor, intrauterine growth restriction, malpresentation and preeclampsia. These typical risk factors of a twin pregnancy are greatly potentiated in the above mentioned setting. CONCLUSION: A twin pregnancy in the presence of a uterine malformation is rare and difficult to manage. These rare cases must be collected and reported in order to work out algorithms of monitoring and therapy as well as issue appropriate recommendations for their management.

An unusual case of severe asphyxia with the fetal position unexpectedly inverted in a malformed uterus: a case report.

BACKGROUND: We present a severe neonatal consequence due to the unexpected and crucial inversion of the fetal position after sudden termination of tocolysis during early labor of a woman with congenital uterine anomaly. It has been reported that congenital uterine anomalies latently affect the fetal position. The clinical pitfalls in childbirth with uterine anomalies are discussed here on the basis of clinical evidence. CASE PRESENTATION: At a perinatal medical center in Japan, a 29-year-old Japanese mother who had a history of bicornuate uterus, received tocolysis to prolong her pregnancy for 5 days during the late preterm period after preterm-premature rupture of the membrane. She gave birth to a 2304 g male neonate of the gestational age of 35 weeks and 5 days with severe asphyxia by means of crash cesarean section for fetal sustained bradycardia after sudden termination of tocolysis. We found the fetal position to reverse from cephalic to breech position during early labor. He ended up having severe cerebral palsy after brain cooling against hypoxic-ischemic encephalopathy for 3 days. The mechanism of inversion from cephalic to breech position without amnionic fluid remains unclear, although women with a known diagnosis of a uterine anomaly have higher risk of adverse outcomes such as malpresentation. CONCLUSIONS: When considering the clinical course of this case on the basis of the medical reports, we suspected that uterine anomalies and changes in intrauterine pressure could cause fetal malpresentation and adverse neonatal outcomes.

Nonpuerperal uterine inversion caused by an adenosarcoma: A case report.

INTRODUCTION: Eighty-five per cent of uterine inversions are puerperal. Non-puerperal uterine inversion is usually caused by tumours that exert a traction force on the fundus of the uterus. This causes the uterus to be partially or completely inverted. It is commonly related to benign tumours like submucosal leiomyomas. Nevertheless, malignancies are an infrequent association. CASE PRESENTATION: We report a case of a 35-year-old female patient, medically and surgically free, gravida0 para0, complaining of menometrorrhagia associated with pelvic pain for 2 years. A suprapubic ultrasound scan showed an enlarged, globular uterus with a heterogeneous, undefined mass of 49 mm in size. MRI scan showed the appearance of a U-shaped uterine cavity and a thickened inverted uterine fundus with an endometrial infiltrating mass of 25 mm. Intraoperative exploration showed uterine inversion involving the ovaries; the fallopian tubes and the round ligaments and a necrotic intracavitary mass. The malignancy of the tumor was confirmed through anatomopathological examination as Adenosarcoma. CONCLUSIONS: Uterine inversion is rare outside the puerperal period, and malignant etiology must not be overlooked. Therefore, comprehensive care with meticulous etiological investigation is crucial.

Herlyn–Werner–Wünderlich syndrome: A case series / Síndrome de Herlyn-Werner-Wünderlich: una serie de casos

Introduction: Herlyn–Werner–Wünderlich syndrome is a uterine malformation characterized by uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Clinical findings: The manifestation of the disease is widely diverse; it is usually diagnosed after menarche, with dysmenorrhea and abnormal uterine bleeding; it is also associated with infertility. Main diagnosis: Four clinical cases, their diagnosis are reported here. Therapeutic interventions and results: The treatment and results of these four patients are described here. Conclusion: When studying uterine malformation it is important to consider this rare disease to avoid possible complications and giving the patient a correct diagnose and treatment. The hysteroscopy resection of the longitudinal vaginal septum in those symptomatic patients with hematocolpos should be considered as a good option for treatment.(AU)

Introducción: El síndrome de Herlyn-Werner-Wünderlich es una malformación uterina que asocia útero didelfo, hemivagina obstruida total o parcialmente y agenesia renal ipsilateral. Hallazgos clínicos: La clínica que presenta este síndrome es muy diversa; se suele diagnosticar después de la menarquia cursando con dismenorrea y sangrado uterino anómalo; así mismo se asocia a infertilidad. Diagnósticos principales: Se presentan a continuación 4 casos clínicos, su diagnóstico y tratamiento mediante diversas técnicas. Intervenciones terapéuticas y resultados: Se describen en este manuscrito los tratamientos aplicados a estas pacientes y sus resultados. Conclusión: Ante el hallazgo de una malformación uterina es importante tener en cuenta esta entidad infrecuente, para evitar posibles complicaciones y proporcionar a la paciente un diagnóstico y tratamiento correctos. La resección histeroscópica del tabique vaginal longitudinal en aquellas pacientes sintomáticas con hematocolpos debe ser considerada como una buena opción de tratamiento.(AU)

Spontaneous uterine rupture with amniotic sac protrusion during the third trimester of a unicornuate uterus pregnancy: A rate case report.

RATIONALE: Uterine rupture is an obstetrical emergency associated with severe maternal and fetal mortality. It is rare in the unscarred uterus of a primipara. PATIENT CONCERNS: A 25-year-old woman in her 38th week of gestation presented with slight abdominal pain of sudden onset 10 hours before. An emergency cesarean section was done. After surgery, the patient and the infant survived. DIAGNOSES: With slight abdominal pain of clinical signs, ultrasound examination showed that the amniotic sac was found in the peritoneal cavity with a rupture of the uterine fundus. INTERVENTIONS: Uterine repair and right salpingectomy. OUTCOMES: After surgery, the patient and the infant survived. The newborn weighed 2600 g and had an Apgar score of 10 points per minute. Forty-two days after delivery, the uterus recovered well. LESSONS: Spontaneous uterine rupture should be considered in patients even without acute pain, regardless of gestational age, and pregnancy with abdominal cystic mass should consider the possibility of uterine rupture.

Retroperitoneal 'uterus-like mass' of cervical origin.

We report a rare case of a retroperitoneal uterus-like mass communicating with the endocervix, which presented as abdominal pain and bloating associated with severe irregular vaginal and postcoital bleeding. Our patient did not have any structural abnormalities of the urogenital system or otherwise, which makes a müllerian defect unlikely in our case. Based on the diagnostic criteria for the choristoma, that theory would be excluded here as the mass communicated with the endocervix. This strengthens the theory of metaplasia, under the effect of oestrogen and accelerated by the hyperoestrogenic state of pregnancy as the most likely postulate for our patient. Although the uterus-like mass is not commonly reported, it should be considered as a possible differential for pelvic masses.

Robotic-assisted single-site abdominal cerclage in the bicornuate uterus patient with cervical insufficiency.

OBJECTIVE: To demonstrate the surgical techniques for improving safety in robotic-assisted abdominal cerclage in patients with bicornuate uteri complicated by recurrent pregnancy loss and cervical insufficiency. DESIGN: Stepwise demonstration with narrated video footage. SETTING: An academic tertiary care hospital. PATIENTS: Our patient is a 22-year-old G2P0020 with a history of recurrent pregnancy loss. During her first pregnancy, she was asymptomatic until 19 weeks and delivered because of a preterm premature rupture of membranes. A transvaginal cerclage was performed for her second pregnancy at 14 weeks, which ended at 16 weeks because of preterm premature rupture of membranes. The final magnetic resonance imaging report noted a "bicorporeal uterus with duplication of the uterine body, resulting in two markedly divergent uterine horns that are fused at the isthmus... unlike a typical didelphic uterus, a single, non septated cervix is noted, which shows normal appearances, measuring 3.8 cm in length." Given her history of a uterine anomaly and recurrent pregnancy loss in the absence of other biochemical factors, her maternal-fetal medicine specialist referred her to us as the patient strongly desired future viable pregnancies. The patient was counseled on multiple alternatives, including different methods of performing the cerclage, and ultimately decided on the robotic-assisted (Da Vinci Xi) prophylactic abdominal cerclage. INTERVENTIONS: The bicornuate uterus is a rare class IV mullerian duct anomaly caused by the impaired fusion of the mullerian ducts in the uterus, classically appearing in imaging studies as a heart-shaped uterus. This patient demographic reports a high incidence of obstetric complications. Pregnancy in such a uterus causes complications like first- and second-trimester pregnancy loss, preterm labor, low-birthweight infants, and malpresentation at delivery.1 Researchers have postulated that there is an abnormal ratio of muscle fibers to connective tissue in a congenitally abnormal cervix. During pregnancy, an inadequate uterine volume may lead to increased intrauterine pressure and stress on the lower uterine segment, which can lead to cervical incompetence.2 To address cervical incompetence, cervical cerclages are a commonly utilized procedure, as recent studies demonstrate that the incidence of term pregnancies in the group with documented cervical incompetence treated with cerclage placement increased from 26% to 63%.3 One observational study noted improved obstetrical outcomes occurred with interval placement, a cerclage placed in between pregnancies in the nongravid uterus, compared with cerclage placement between 9 and 10 weeks gestation, with the mean gestational age for delivery at 32.9 weeks and 34.5 weeks when a cerclage was placed in gravid and nongravid women, respectively.4 In addition, another retrospective study was done, which demonstrated a lower incidence of neonatal death with prophylactic cerclages.5 Operating on a nonpregnant uterus offers several benefits, including its reduced size, fewer and smaller blood vessels, and simplified handling. Moreover, there are clearly no concerns regarding the fetus. In the decision to use a robotic-assisted platform vs. laparoscopic, a systematic review showed the rates of third-trimester delivery and live birth (LB) using laparoscopy during pregnancy were found to be 70% and 70%-100%, respectively. The same review demonstrated slightly improved outcomes via the robotic route regarding gestational age at delivery (median, 37 weeks), rates of LB (90%), and third-trimester delivery (90%).6 Additional factors contributing to the preference for robotics in surgical procedures include incorporating advanced tools, which can enhance the robotic system's advantages compared with traditional laparoscopy. An invaluable tool in this context is the simultaneous utilization of Firefly mode, which employs a near-infrared camera system, achieved through injecting indocyanine green dye or integrating other light sources concurrently. The intravenous administration of indocyanine green is acknowledged widely for its safety and efficacy as a contrast agent in the evaluation of microvascular circulation and organ vascularization. This property equips surgeons with heightened precision when guiding the needle, proving especially advantageous when faced with challenges in visualizing vascular anatomy. In our specific case, we harnessed the capabilities of Firefly mode in conjunction with hysteroscopic light, enabling us to vividly illustrate the contours of a bicornuate uterus from both external and internal perspectives. We demonstrate a simplified technique of the abdominal cerclage, one cerclage around the internal cervical os of the uterus, using a robotic-assisted platform in a nongravid patient. The surgery began with the eversion of the umbilicus, and a 15-mm skin incision was made in the umbilicus. A Gelpoint mini advanced access site laparoscopy device was inserted into the incision, and CO2 was allowed to insufflate the abdominal cavity with careful attention given to intraabdominal pressure. Once the DaVinci was docked, the surgeon began the creation of a bladder flap. The bladder was carefully dissected from the lower uterine segment and both uteri using monopolar scissors. The anatomical differences of a bicornuate uterus prompted the surgeon to dissect a wider circumference for safety reasons, where a wider dissection offers a better view of the uterine vessels and ease of introducing the Mersilene tape later on. Bilateral uterine vessels were further skeletonized and exposed anteriorly using blunt dissection and monopolar scissors. After further dissection and lateralization, the final result creates a landmark medial to the right uterine vessels at the level of the internal cervical os with which the needle of the Mersilene tape will be able to pass through. The Mersilene tape was guided from anterior to posterior via a previously straightened needle. Similarly, a landmark was created on the left, and the Mersilene tape was directed from anterior to posterior. The Mersilene tape was placed circumferentially around the internal cervical os of the bicornuate uterus, medial to the uterine vessels. Both ends of the Mersilene tape were then gently pulled, ensuring that the tape was lying flat on the anterior of the internal cervical os with no bowels or uterine vessels within it. The tape was then tied posteriorly at the 6 o'clock position with appropriate tension. A 2-0 silk was then sutured to the tails of the tape using the purse-string technique to ensure that it would remain securely tied and in the correct position. Hemostasis was assured. Both a hysteroscopy and a cystoscopy were done after the completion of the cerclage to ensure that no tape or sutures were seen within the cervical canal or the uterine cavity. None were observed. MAIN OUTCOMES MEASURES: The success criteria for the surgery were identified as the patient's ability to attain a viable pregnancy after the cerclage placement, along with achieving LB. RESULTS: Subsequently, a spontaneous pregnancy was achieved. An infant weighing 3 pounds and 16 ounces was delivered by cesarean section at 36 weeks because of an oligohydramnios. The infant is currently healthy at 13 pounds. CONCLUSION: Robotic-assisted abdominal cerclage around the internal cervical os in a bicornuate uterus offers a possibly feasible and straightforward technique for surgeons seeking to reduce risks, although further research is needed.

Hysteroscopic Transillumination as a Guide for Laparoscopic Excision of Rudimentary Uterine Horn: A Winning Duo.

STUDY OBJECTIVE: Anatomic anomalies of the female reproductive genital tract affect approximately 5.5% of women [1]. The hemiuterus or class U4 by the European Society of Human Reproduction and Embryology / European Society for Gynaecological Endoscopy 2013 classification is a rare congenital malformation defined as a unilateral uterine development, with a contralateral part that could be either incompletely formed or absent. This class is divided into 2 subclasses depending on the presence or not of a functional rudimentary cavity (U4a/U4b) [2]. This work aimed to share our experience performing an hysteroscopic and laparoscopic combined technique to surgically manage this uterine malformation exploiting the hysteroscopic transillumination. DESIGN: A step-by-step explanation of surgical technique with narrated video footage. SETTING: Tertiary Level Academic Hospital "IRCCS Azienda Ospedaliero - Universitaria di Bologna" Bologna, Italy. INTERVENTIONS: A 32-year-old woman with a symptomatic (severe dysmenorrhea and chronic pelvic pain) suspected U4a uterine malformation diagnosed at our center was scheduled for laparoscopic removal of the right uterine horn and ipsilateral salpingectomy exploiting the contemporary hysteroscopic transillumination guidance. We selected this approach to avoid possible complications owing to the anatomic anomalies that are very common in these cases [3,4]. After coagulation and section of the right round ligament at the uterine angle and opening of the right broad ligament, access to the retroperitoneum was obtained to directly visualize the entire course of ureter and the uterine artery. The right uterine artery was coagulated and sectioned at the uterus. Then, the hysteroscope was introduced to the uterine fundus and the light source brightness was increased up to 100% to allow an adequate transmural visualization of the uterine defect from the laparoscope. Once the defect edge was well highlighted, the right uterine horn was isolated and removed using a monopolar hook, taking care to preserve an adequate amount of myometrium. A double layer running suture with barbed absorbable thread (V-Loc) for reconstructive purposes was performed. The specimen was retrieved inside an endobag to allow a safe extraction. Right salpingectomy was then performed. CONCLUSION: Considering the great anatomic variability of this condition, this type of surgery is not always simple. Indeed, the borders between the uterus and the rudimentary uterine horn are often not perfectly recognizable; this can lead to accidental removal of healthy myometrium and increase the risk of perioperative bleeding [5]. In our experience, the combined hysteroscopic and laparoscopic combined technique allows the surgeon to better delimit the borders of the hemiuterus, providing a more conservative and safer surgery. Hysteroscopic transillumination offers the possibility to modulate the radicality in the resection of the rudimentary horn and in the final treatment of dysmorphism.

Single Euploid Embryo Transfer Outcomes After Uterine Septum Resection.

STUDY OBJECTIVE: To study pregnancy outcomes after single euploid embryo transfer (SEET) in patients who underwent prior uterine septum resection to those with uteri of normal contour, without Müllerian anomalies or uterine abnormalities including polyps or fibroids, and without a history of prior uterine surgeries. DESIGN: Retrospective cohort study. SETTING: Single academic affiliated center. PATIENTS: 60 cycles of patients with prior hysteroscopic uterine septum resection who underwent an autologous SEET between 2012 and 2020 were used as the investigational cohort. A 3:1 ratio propensity score matched control cohort of 180 single euploid embryo transfer cycles from patients without a history of uterine septa were used as the control group. INTERVENTIONS: No interventions administered. MEASUREMENTS AND MAIN RESULTS: Pregnancy, clinical pregnancy loss, ongoing clinical pregnancy, and live birth rates in patients with a history of uterine septum resection compared with matched patients without Müllerian anomalies or uterine surgeries. Patients with a prior uterine septum had significantly lower rates of chemical pregnancy (58.33% vs 77.2%, p = .004), implantation (41.67% vs 65.6%, p = .001), and live birth (33.33% vs 57.8%, p = .001) per transfer. No statistical difference in clinical pregnancy loss rates was found when comparing septum patients with controls (8.33% vs 7.8%, p = .89). CONCLUSION: Patients with a history of hysteroscopic resection who undergo in vitro fertilization are more susceptible to suboptimal clinical outcomes compared with patients with normal uteri. Early pregnancy loss rates in patients with a uterine septum are higher than in those without; however, after resection, the rates are comparable. Patients born with septate uteri require assessment of surgical intervention prior to SEET, and to optimize their reproductive outcomes.

Evaluation of Recurrent Pregnancy Loss.

Recurrent pregnancy loss (RPL) affects approximately 5% of couples. Although RPL definitions vary across professional societies, an evaluation after a second clinically recognized first-trimester pregnancy loss is recommended. Good quality evidence links parental chromosomal rearrangements, uterine anomalies, and antiphospholipid syndrome (APS) to RPL. In contrast, the relationship between RPL and other endocrine, hematologic, and immunologic disorders or environmental exposures is less clear. Anticoagulant therapy and low-dose aspirin are recommended for patients with RPL who have also been diagnosed with APS. Vaginal progesterone supplementation may be considered in patients experiencing vaginal bleeding during the first trimester. Surgical correction may be considered for patients with RPL in whom a uterine anomaly is identified. Evaluation and management of additional comorbidities should be guided by the patient's history rather than solely based on the diagnosis of RPL, with the goal of improving overall health to reduce complications in the event of pregnancy. Most people with RPL, including those without identifiable risk factors, are expected to achieve a live birth within 5 years from the initial evaluation. Nevertheless, clinicians should be sensitive to the psychological needs of individuals with this condition and provide compassionate and supportive care across all stages.

Hysteroscopic Metroplasty Using Holmium: YAG Laser for Treatment of Septate Uterus.

STUDY OBJECTIVE: To demonstrate the safety, efficacy, and ease of hysteroscopic metroplasty using holmium:YAG (Ho:YAG) laser for treatment of septate uterus. DESIGN: Stepwise demonstration of surgical technique with narrated video footage. SETTING: Septate uterus is the most common type of uterine anomaly. The incidence of uterine septum in women presenting with infertility and recurrent abortions is 15.4% [1,2]. Hysteroscopic septal incision is associated with improvement in live-birth rate in these women [3]. Hysteroscopic metroplasty for septate uterus can be done with the use of scissors and energy sources such as monopolar and bipolar electrosurgery and lasers. Ho:YAG laser is commonly used by urologists for various surgeries because of its "Swiss Army Knife" action of cutting, coagulation, and vaporization [4]. Ho:YAG laser is known for its precision. It causes lesser depth of tissue injury and necrosis and minimal collateral thermal damage compared with the electrosurgical devices and other lasers used for hysteroscopic surgery [5-8]. This is advantageous in hysteroscopic metroplasty given that it reduces the risk of uterine perforation during surgery and hence uterine rupture in the subsequent pregnancy. Reduced collateral damage to the surrounding endometrium helps promote early endometrial healing and prevent postoperative intrauterine adhesions. A 28-year-old patient with history of 2 spontaneous abortions came to our hospital for investigations. 3D transvaginal sonography of the patient showed presence of partial septate uterus with a fundal indentation of 1.5 cm (Supplemental video 1). INTERVENTION: Diagnostic hysteroscopy followed by septal incision using Ho: YAG laser was planned. We used a 2.9 mm BETTOCCHI Hysteroscope (Karl Storz SE & Co.) with a 5 mm operative sheath. Normal saline was used as the distending medium and the intrauterine pressure was maintained at 80 to 100 mm Hg. The procedure was done under total intravenous anesthesia using propofol injection. Vaginoscopic entry into the uterus (without any cervical dilatation) showed evidence of a partial uterine septum with tubal ostia on either side of the septum. A 400 micron quartz fiber was passed through a laser guide into the 5-Fr working channel of the operative hysteroscope. Ho:YAG laser (Auriga XL 50-Watt, Boston Scientific) with power settings of 15 watts (1500 mJ energy at 10 Hz) was used. Incision of the septum was started at the apex of the septum in the midline and continued in a horizontal manner from side to side toward the base (Supplemental video 2). Incision of the septum is continued till the tip of the hysteroscope can move freely from one ostium to the other (Supplemental video 3). The operative time was 12 minutes. There were no intra- or postoperative complications. Postoperative estrogen therapy was given for 2 months in the form of estradiol valerate 2 mg (tablet, Progynova, Zydus Cadila) 12 hourly orally for 25 days and medroxyprogesterone acetate 10 mg (tablet, Meprate, Serum Institute of India, Ltd) 12 hourly orally added in the last 5 days [9]. 3D transvaginal ultrasound was done on day 8 of menses. It showed a triangular uterine cavity with a very small fundal indentation of 0.37 cm. A second look hysteroscopy that was done on day 9 of menses showed an uterine cavity of good shape and size [10]. Few fundal adhesions were seen and they were incised using Ho:YAG laser. The patient conceived 5 months after the primary surgery and delivered by cesarean section at 38 weeks, giving birth to a healthy baby of 2860 grams. There were no complications during her pregnancy and delivery. A comparative study is essential to prove its advantages over other energy sources for this surgery. CONCLUSION: Hysteroscopic metroplasty using Ho:YAG laser for treatment of septate uterus is a simple, precise, safe, and effective procedure. VIDEO ABSTRACT.

Three-dimensional transvaginal ultrasound diagnosis of interstitial ectopic pregnancy in a unicornuate uterus: A case report.

A rare case of unicornuate uterus with interstitial ectopic pregnancy was diagnosed using three-dimensional transvaginal ultrasound (3D-TVUS). The ultrasound revealed a "lancet-shaped" endometrial corona, a gestational sac near the uterus base extending toward the uterine serosa, and visible interstitial lines. The patient underwent laparoscopic surgery for a lesion in the right fallopian tube. 3D-TVUS was crucial in precisely locating the gestational sac, aiding in effective treatment. Interstitial ectopic pregnancies risk severe hemorrhaging upon rupture. Rapid, accurate diagnosis is vital for lifesaving treatment and preventing critical complications.

Histological Features of Neovaginal Epithelium after Vaginoplasty in Mayer-Rokitansky-Küster-Hauser Syndrome.

OBJECTIVE: To analyze the features of the epithelia coating neovaginas after vaginoplasty in women affected by Mayer-Rokitansky-Küster-Hauser syndrome STUDY DESIGN: We conducted a retrospective analysis of prospectively collected data. Women affected by Rokitansky syndrome who underwent neovaginal biopsy after vaginoplasty (McIndoe surgery, intestinal vaginoplasty, Vecchietti surgery, and Davydov surgery) were included. Macroscopic mucosal features were assessed through clinical examination and the Schilling test. Each biopsy specimen was prepared for examination by light microscopy and in some cases by scanning electron microscopy (SEM). RESULTS: Thirty-six patients (4 McIndoe, 2 intestinal vaginoplasty, 14 Vecchietti, and 16 Davydov) were included. All biopsies were performed without complications. In McIndoe's neovaginas, the mucosal microscopic features were similar to normal skin, with large areas of preserved epithelium, heterogeneous presence of dermal papillae, and superficial keratinization. The characteristics of the intestinal neovagina's surface were similar to those of a sigmoid colon, with well-shaped glands, cylindrical cells, and a secreting mucosa. In Vecchietti neovaginas, the surface the epithelium was flat and multilayered, highly similar to that of a normal vagina, with the presence of glycogen and superficial desquamation. On medium SEM magnification evaluation, the epithelium presented flattened polygonal cells. Finally, in Davydov neovaginas, none of the specimens had persistent mesothelial elements. The squamous neo-epithelium had regular aspects of differentiation with the presence of glycogen. At greater SEM magnification, microridges were evident, with a regular distribution. CONCLUSION: Each different technique of vaginoplasty leads to unique histological and structural features of the neovagina's mucosa. Knowledge of these elements must be the basis for the choice of the most appropriate intervention.

Successful pneumovaginoscopic surgery for recurrent abscess of OHVIRA syndrome, preventing further recurrence and subsequent pregnancy.

INTRODUCTION: OHVIRA syndrome is a rare congenital anomaly of Müllerian duct development characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The primary treatment is surgical excision of the obstructed hemivaginal septum and hematometrial drainage. In recent years, minimally invasive approaches such as hysteroscopic or vaginoscopic septum resection have been reported. Furthermore, we originally developed some novel pneumovaginoscopic gynecologic surgeries for years using a device that consists of a cylinder that fits into the vagina and a lid that mounts multiple ports, allowing the vagina to be dilated with carbon dioxide gas, similar to a single-port laparoscope. MATERIALS AND SURGICAL TECHNIQUE: We report a successful pneumovaginoscopic surgery for a complicated recurrent abscess in a patient with OHVIRA syndrome. Conventional surgery was performed with a single forceps in a liquid, as in cystoscopy or hysteroscopy. However, this new surgery allowed multiple forceps in a gas, as in laparoscopy. So pus and blood were aspirated and washed away without leaking into the abdominal cavity via fallopian tubes. The surgical smoke generated by thermal coagulation also aspirated to clean the field of vision immediately. And thick, complicated abscesses were drained successfully. The patient conceived through IVF with ICSI and delivered safely at full term. DISCUSSION: Pneumovaginoscopy could benefit complex vaginal surgery cases, such as abscess formation in patients with OHVIRA syndrome.

Impact of congenital uterine anomalies on reproductive outcomes of IVF/ICSI-embryo transfer: a retrospective study.

OBJECTIVE: To study the impact of congenital uterine anomalies on reproductive outcomes after in vitro fertilization (IVF)/intracytoplasmic sperm injection (ICSI)-embryo transfer (ET). METHODS: A retrospective study including a total of 865women with congenital uterine anomalies and 865 age and admission time matched controls who underwent the first IVF/ICSI-ET cycle between January 2010 and December 2019 was conducted. Women with uterine anomalies were classified into canalization defect (complete septate uterus and subseptate uterus) and unification defect (unicornuate uterus, bicornuate uterus, and didelphus uterus) according to the processes of abnormal embryological development. Control women were selected by age (± 1.0 year) and admission time (± 6 months) matched with a 1:1 ratio. The reproductive outcomes were compared between women with uterine anomalies and the controls. The primary outcome was live birth; secondary outcomes were clinical pregnancy, ectopic pregnancy, preterm delivery, and spontaneous pregnancy loss. RESULTS: Compared with women with a normal uterus, women with canalization defects were less likely to experience live birth [84/332 (25.3%) vs 128/332 (38.6%), RR: 0.647, 95% CI 0.513-0.815, P < 0.001]. They also had a lower clinical pregnancy rate [126/332 (38.0%) vs 206/332 (62.0%), RR: 0.829, 95% CI 0.690-0.997, P = 0.046] and experienced a higher first-trimester pregnancy loss rate [25/126 (19.8%) vs 11/206 (5.3%), RR: 2.716, 95% CI 1.393-5.295, P = 0.003]. Compared with women with a normal uterus, women with a unification defect were also less likely to experience live birth [132/533 (24.8%) vs 219/533 (41.1%), RR: 0.713, 95% CI 0.586-0.868, P = 0.001]. Women with a unification defect had lower clinical pregnancy rates [182/533 (34.1%) vs 263/533 (49.1%), RR: 0.813, 95% CI 0.695-0.952, P = 0.010] and increased first-trimester pregnancy loss [36/182 (19.8%) vs 20/263 (7.6%), RR: 3.288, 95% CI 1.776-6.085, P < 0.001]. While uterine anomaly seemed not increase the risk of preterm birth, ectopic pregnancy and second-trimester pregnancy loss. CONCLUSIONS: Both canalization defects and unification defects were associated with lower fertility outcomes, including lower live birth rates, lower clinical pregnancy rates, and higher early miscarriage rates.

A life course perspective on Mayer-Rokitansky-Küster-Hauser syndrome: women's experiences and negotiations of living with an underdeveloped uterus and vagina.

PURPOSE: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by the absence or underdevelopment of the uterus and upper part of the vagina. It is usually diagnosed during adolescence, and the present study investigates how women experience and negotiate to live with MRKH syndrome long-term. METHODS: From January to March 2021, eighteen Danish women with MRKH syndrome participated in semi-structured interviews via video conference. The mean time since diagnosis was 11.5 years. A thematic analysis using the life course framework as a theoretical approach was applied. RESULTS: The analysis identified the diagnosis as a turning point, that dramatically altered the women's imagined futures. Not conforming to dominant social norms regarding sexuality and pregnancy meant that the women continuously managed and negotiated the meaning and impact of MRKH syndrome in relation to the five principles of the life course perspective: (1) Lifelong development, (2) Timing, (3) Human agency, (4) Linked lives, and (5) Historical time and place. CONCLUSION: Using the life course framework contributed to a holistic understanding of life with MRKH syndrome by showing how the meaning and consequences of the congenital condition changed over time and in adaptation to gendered and age-related social norms and expectations.IMPLICATIONS FOR REHABILITATIONThe perceived meaning and impact of living with Mayer-Rokitansky-Küster-Hauser syndrome change over time, and women's information and support needs thus change accordinglySensitive, clinical communication is essential when discussing treatment optionsOnline communities may provide support and reduce feelings of loneliness.

Surgical Management of OHVIRA and Outcomes.

STUDY OBJECTIVE: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) includes uterine didelphys, unilateral obstructed hemivagina, and ipsilateral renal anomaly. Surgical management of this condition relies on accurate diagnosis to excise the obstructed longitudinal vaginal septum (OLVS). Vital considerations involve identifying the side affected, ipsilateral renal anomaly (IRA), thickness of the septum (TS), septal axis (SA), and distance of the septum to perineum (DSP). The study aimed to evaluate the preoperative characteristics, imaging findings, and surgical outcomes of OHVIRA. METHODS: Institutional review board approval was obtained for this retrospective chart review. ICD-10 codes identified OHVIRA cases between 2012 and 2019 at a single children's hospital. Patient demographic characteristics, magnetic resonance imaging findings, surgical management, outcomes, and complications were reviewed. Descriptive statistics were utilized. RESULTS: Twenty-six patients met inclusion criteria. Most were diagnosed at puberty (92%). Abdominal pain (50%) was the most common presenting complaint. The mean age of diagnosis was 13.2 years overall and 11.2 years for those with regular cycles vs 13.4 years for those with irregular cycles. Preoperative imaging showed predominant right-sided OLVS (50%), IRA (77%), and oblique SA (65%). All patients underwent vaginoscopy, septum resection, and vaginoplasty, except 1 who was managed with an abdominal drain as a neonate. Four required postoperative vaginal stent or Foley, with DSP greater than 5 cm in all cases. One intraabdominal abscess complication occurred. No instances of hematocolpos re-accumulation or reoperation were observed during the 3-year follow-up period. CONCLUSION: This study demonstrates that detailed preoperative planning and a systematic surgical approach lead to favorable outcomes in OHVIRA irrespective of the OLVS laterality, TS, SA, or DSP.