Deceased Donor Uterus Transplantation: A Narrative Review of the First 24 Published Cases.

Uterus transplantation is the surgical treatment for absolute uterine factor infertility (AUFI), a congenital or acquired condition characterized by the absence of a uterus. More than 80 transplants have been performed worldwide, resulting in more than 30 live births, originating both from living and deceased donors. The collection of published articles on deceased donor uterus transplantations was performed in PubMed and SCOPUS by searching for the terms "Uterus transplantation" AND "deceased donor"; from the 107 articles obtained, only case reports and systematic reviews of deceased donor uterus transplantations and the resulting live births were considered for the present manuscript. The extracted data included the date of surgery (year), country, recipient (age and cause of AUFI) and donor (age and parity) details, outcome of recipient surgery (hysterectomy), and live births (date and gestational age). The search of peer-reviewed publications showed 24 deceased donor uterus transplantations and 12 live births (a birth rate of 66%) with a 25% occurrence of graft loss during follow-up (6 of 24). Among this series, twelve transplants were performed in the USA (seven births), five in the Czech Republic (one birth), three in Italy (one birth), two in Turkey (two births), and two in Brazil (one birth). The median recipient age was 29.8 years (range 21-36), while the median donor age was 36.1 years (range 20-57). Of 24 recipients, 100% were affected by MRKH (Mayer-Rokitanski-Kuster-Hauser) syndrome. Two live births were reported from nulliparous donors. Deceased donor uterus transplantation birth rates are very similar to the living donor rates reported in the literature, but ethical implications could be less important in the first group. It is necessary to register every case in the International Registry for Uterus Transplantation in order to perform a systematic review and comparison with living donor rates.

Experiences of Pelvic and Generalized Persistent Pain Syndromes in MRKH: A Scoping Review.

STUDY OBJECTIVE: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by the congenital absence of the uterus and vagina, sometimes with associated extragenital anomalies. Currently, there is limited literature on pelvic pain and comorbid pain syndromes in people with MRKH. The aims of this scoping review were to summarize existing literature on pelvic and generalized persistent pain syndromes associated with MRKH and to identify knowledge gaps for further research into this field. METHODS: This scoping review followed the Joanna Briggs Institute framework. The population of interest was patients with a diagnosis of MRKH. MEDLINE, CINAHL, Scopus, Cochrane, Embase, and Emcare databases were searched. Articles that did not meet the inclusion criteria or critical appraisal standards were excluded. The resultant articles were reviewed by 2 independent researchers, and a third was used in cases of disagreement. A descriptive analytical method was used for data analysis. RESULTS: We screened 3348 articles for eligibility. Of these, 39 articles, which described 1353 cases of MRKH, met the criteria. Four studies described baseline pelvic pain in MRKH, 19 described acute presentations, and 13 described postintervention pain levels. CONCLUSION: Despite the paucity of research, this review found that cyclic pelvic pain was mostly present in women with uterine remnants, whereas pelvic pain in those without remnants was poorly understood. There were no studies exploring generalized persistent pain syndromes in MRKH. Further cross-sectional studies are needed to elucidate the prevalence and levels of pain syndromes in MRKH.

A rare variant of mullerian agenesis: a case report and review of the literature.

INTRODUCTION: Menstruation is a developmental milestone and usually marks healthy and normal pubertal changes in females. Menarche refers to the onset of first menstruation in a female. The causes of primary amenorrhea include outflow tract abnormalities, resistant endometrium, primary ovarian insufficiency, and disorders of the hypothalamus, pituitary, or other endocrine glands. A rare variant of mullerian agenesis, which warrants an individualized approach to management, is presented here. CASE REPORT: We present here the case of a 25-year-old Indian female with pain in the lower abdomen and primary amenorrhea. After a thorough history, clinical examination, imaging, and diagnostic laparoscopy, two small uteri, a blind upper half vagina, bilateral polycystic ovaries, and a blind transverse connection between the two uteri-a horseshoe band cervix-were detected, which confirmed the diagnosis of mullerian agenesis. There was evidence of adenomyosis in the mullerian duct element. This is a rare form of Müllerian abnormality with an unusual presentation. CONCLUSION: Mullerian agenesis is the most common cause of primary amenorrhea with well-developed secondary sexual characteristics. There are various forms of mullerian agenesis. Most of the cases are managed by a multidisciplinary team. Rare variants warrant an individualized approach to management.

A case report of laparoscopic surgery for Mayer-Rokitansky-Küster-Hauser syndrome with preservation of functional primordial uterus.

BACKGROUND: In the past, the primary treatment for MRKH syndrome (Mayer-Rokitansky-Küster-Hauser syndrome) with a functional primordial uterus was surgical removal of the functional primordial uterus. In rare instances, the endometrium of the functional primordial uterus is well developed, and surgical preservation of the functional primordial uterus provides the possibility of preserving reproductive function for these patients. CASE PRESENTATION: A 14-year-old female was diagnosed with type I MRKH syndrome with a functional primordial uterus through physical examination and imaging investigations. We freed the functional primordial uterus through laparoscopic surgery and excised a portion of the lower myometrium to create an outlet at a lower uterine segment, which we then intermittently anastomosed to the tip of the artificial vagina. The patient recovered well after the surgery, and a re-examination showed no significant abnormalities. CONCLUSION: We were successful in preserving the functional primordial uterus using laparoscopic surgery in a patient with MRKH syndrome and connecting it to an artificial vagina through reconstructive surgery to ensure unobstructed menstrual drainage and preserve the reproductive potential of the patient.

Evaluation of Sexual Function Outcomes in Patients with Rokitansky Syndrome: A Systematic Review and Meta-analysis.

OBJECTIVE: In patients with Rokitansky syndrome, vaginal agenesis can be treated using various surgical techniques, and various factors can affect each surgery outcome in the long term. This meta-analysis aimed to evaluate sexual function outcomes in patients with Rokitansky syndrome after various surgery techniques. DATA SOURCES: Searches were conducted in Google Scholar, PubMed, Cochrane database, ScienceDirect, Web of Science, and ClinicalTrials.gov. Systematic searches were conducted on studies published until November 2022 (CRD42022370735). METHODS OF STUDY SELECTION: During the first stage of database scanning and reference check, 1820 results were identified, and an evaluation of the total 10 studies was finally conducted. The inclusion criteria involved selecting randomized controlled trials that focused on assessing sexual function after surgical treatment in patients of all ages with Rokitansky syndrome. TABULATION, INTEGRATION, AND RESULTS: EndNote version 20 software was used to organize and identify duplicate articles through screening. The Joanna Briggs Institute's critical appraisal tool was used to evaluate each study's quality for bias potential. The results showed that the total scores of female sexual functions in patients with Rokitansky syndrome after vaginoplasty were significantly lower than in healthy women (standardized mean difference, -0.233; p <.05; range, -0.376 to -0.090). The 6-domain analysis of the Female Sexual Function Index questionnaire revealed that lubrication (p <.05) and satisfaction (p <.05) were significantly lower in patients undergoing vaginoplasty. CONCLUSION: All surgical techniques to create a neovagina for patients with Rokitansky syndrome have successfully affected the sexual function outcomes. Considering all other factors affecting sexual function outcomes in the long term, more quantitative and qualitative studies are needed to assess sexual satisfaction in patients treated with surgical techniques.

Laparoscopic intervention for solid pelvic tumours in Mayer-Rokitansky-Küster-Hauser syndrome: a case of bilateral uterine adenomyomas of the rudimentary uterus.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a condition caused by Müllerian anomalies, is characterised by congenital vaginal aplasia and a rudimentary uterus. Case reports concerning uterine fibroids associated with MRKH syndrome are limited, and differentiating between uterine fibroids and ovarian solid tumours prior to surgical intervention is often challenging. Here, we present the case of a patient with MRKH syndrome and asymptomatic bilateral pelvic solid tumours located close to both ovaries. Based on intraoperative and histopathological findings, the tumours were diagnosed as adenomyomas of the rudimentary uterus. This is the first reported case of a uterine adenomyoma associated with MRKH syndrome. Moreover, our report highlights the fact that diagnostic laparoscopy is a valuable method to evaluate pelvic tumours in MRKH syndrome.

Intestinal Obstruction Secondary to Malformation in a Child with Mayer-Rokitansky-Küster-Hauser Syndrome.

BACKGROUND: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by underdevelopment of the uterus and vagina. CASE: A 4-year-old girl was declared dead on arrival to the hospital with a history of chronic constipation since birth. Postmortem examination showed Müllerian remnants attached to abnormal fibrous tissue. The fibrous tissue extended from the descending colon to the rectum and connected to a urinary bladder duplication cyst. The fibrous tissue formed into a constricted band around the rectum. The cause of death was Streptococcus pyogenes sepsis with intestinal obstruction secondary to the rectal fibrotic band and urinary bladder duplication cyst. SUMMARY AND CONCLUSION: MRKH syndrome is a rare congenital disorder, and the malformations rarely cause death. We describe the features of abnormal fibrous tissue and urinary bladder duplication cyst in a patient with MRKH syndrome.

Congenital Pouch Colon: Further Histopathological Perspectives.

Congenital pouch colon is an uncommon anomaly worldwide and is usually associated with anorectal malformations. Imperforate anus with a large air fluid level on the abdominal x ray suggests the diagnosis. Most cases are diagnosed in neonates and an early management limit complications. Few studies have documented the histopathological features of congenital pouch colon.We present two cases with varied associated anomalies (Case 1 with rectovesical fistula, Case 2 with Mayer Rokitansky Kuster Hauser syndrome) and their histopathological features. Immunohistochemistry for calretinin showed paucity of ganglion cells and intrinsic fibers with occasional punctate positivity. The c-Kit immunostain documented fewer interstitial cells of Cajal. Cystitis glandularis with intestinal metaplasia (Case 1) and an additional muscle layer (Case 2) are described.These novel histopathological features characterize the entity further and may be related to genesis of the pouch and its clinical manifestations.

Clinical characteristics of 1,055 Chinese patients with Mayer-Rokitansky-Küster-Hauser syndrome: a nationwide multicentric study.

OBJECTIVE: To reveal the proportion of concomitant extragenital malformations in a large cohort of Chinese patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. STUDY DESIGN: Retrospective study. SETTING: Tertiary teaching hospitals in China. PATIENT(S): A total of 1,055 Chinese Han women with MRKH syndrome diagnosed and treated at 11 Chinese tertiary teaching hospitals from January 2015 to January 2020. INTERVENTION(S): Karyotype analysis, hormone profiling, pelvic ultrasonography, spinal roentgenograms, urologic ultrasonography, and Chinese female reproductive tract malformation registry platform (https://ecrf.linklab.com/). MAIN OUTCOME MEASURE(S): Patients' demographic and clinical characteristics, concurrent malformations, and family histories. RESULT(S): Of the 1,055 Chinese Han patients with MRKH, 69.6% had type I MRKH syndrome and the remaining 30.4% had type II MRKH syndrome. Among the type II patients, 12.6% had müllerian duct aplasia, unilateral renal aplasia/ectopic kidney, and cervicothoracic somite dysplasia association. Skeletal malformations were the most common associated extragenital malformations in the study (22.0%, 232/1,055), of which idiopathic scoliosis and congenital vertebral malformations were the 2 main skeletal malformations (80.6% and 14.2%, respectively). Renal malformations were the second-highest associated extragenital malformations (9.7%, 102/1,055), with unilateral renal agenesis and ectopic kidney being the most common renal malformations (48.0% and 22.5%, respectively). CONCLUSION(S): Type II disease was less common among Chinese patients with MRKH syndrome compared with European patients. Skeletal malformations were more common extragenital malformations than renal malformations in our cohort.

Modified Vecchietti vaginoplasty using self-made single-port laparoscopy in Mayer-Rokitansky-Küster-Hauser syndrome.

OBJECTIVE: To introduce the minimally invasive Vecchietti procedure based on single-port laparoscopy with self-made surgical instruments for the surgical management of Mayer-Rokitansky-Küster-Hauser syndrome (MRKH). DESIGN: Surgical video article. The approval of the Institutional Review Board and written consent from the patient were obtained. SETTING: University hospital. PATIENT(S): A 22-year-old woman presented with primary amenorrhea and inability to participate in penetrative sexual intercourse. A gynecological examination revealed a phenotypically normal vulva and absence of the vagina. A normal 46,XX karyotype was expressed. Magnetic resonance imaging and ultrasonic imaging of the abdomen and pelvis indicated that the ovaries were normal in size and there was vaginal agenesis with rudimentary uterus. No other congenital malformations were present. INTERVENTION(S): The modified Vecchietti procedure involved a transvaginal operation and laparoscopic surgery. The epidural catheter was stretched and made into two wires that were tucked into the core of the Veress needle and then folded into four strings for perineal puncture. A transverse incision of about 3 cm was made in the center of the navel, layer by layer into the abdomen, and placed in a self-made single port (made of a small wound protector and an 8-inch glove), introducing the laparoscope. Under laparoscopic surveillance, the Veress needle was inserted through the vesicorectal space guided by the index finger, which was placed in the rectum. The epidural catheter was pumped from the core into the abdominal cavity under laparoscopy. Subsequently, cystoscopy was performed to ensure that no bladder perforation occurred during the needle insertion. The ball-shaped acrylic device and the two rubber stoppers were attached to the epidural catheter of the vulva. An epidural puncture needle with wire perforated the peritoneum through McBurney's point and the opposite McBurney's point, pulling the epidural catheter out of the abdominal cavity. The epidural catheter was curled around the gauze rolls until the ball-shaped device could be accommodated into the newly created cavity at a sufficient depth. After the surgery, the top of neovagina was lifted about 1 cm every day by tightening gauze rolls to increase the traction, until a neovagina 9 cm long was achieved. MAIN OUTCOME MEASURE(S): The clinical and anatomical data such as the operative time, intraoperative bleeding, duration of hospitalization, and measurement of the final length of the newly created canal 30 days after surgery and 3 months after surgery. RESULT(S): The operative time was 30 minutes, and the intraoperative blood loss was 10 mL. The duration of hospitalization was 10 days. Before discharge, the vaginal depth was 9 cm at 5 days after the surgery, and the self-made traction system was removed. A plastic mold was then inserted using povidone-iodine. The vaginal dilator had to be worn day and night. The patient was advised to sit at the corner of a hard bed or chair from time to time to enhance the dilated effect of the vaginal mold. Three months after the surgery, it could be worn each night until regular sexual intercourse was initiated. The canal length 30 days and 3 months after the surgery was nearly 9 cm. CONCLUSION(S): The modified Vecchietti vaginoplasty is a simple, safe, cost-effective, and minimally invasive procedure, offering an anatomic and functional neovagina for MRKH patients.

Mayer-Rokitansky-Küster-Hauser Syndrome with a Solitary Duplex Kidney and Anal Stenosis: Report of a Rare Case.

BACKGROUND: To date, only 23 cases of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with duplex kidney have been reported. We present the first reported case of MRKH syndrome with solitary duplex kidney and anal stenosis. CASE: A 17-year-old Chinese girl presented with primary amenorrhea and fully developed secondary sex characteristics. Ultrasonography of the abdomen and pelvis revealed the absence of the right kidney, a left duplex kidney, and a primordial uterus. Surgery for anal stenosis was performed when she was 1 year of age. The patient had a normal 46, XX karyotype.

Mayer-Rokitansky-Küster-Hauser syndrome with rare findings of inferior crossed-fused renal ectopia and Gartner's duct cyst: a video case report.

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.

Laparoscopic lateral suspension to treat neovaginal prolapse in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome: A rare case report.

Various methods have been described to treat neovaginal prolapse in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. In this case report, we describe neovaginal prolapse of a 21-year-old patient with MRKH syndrome which had been created by sexual intercourse dilation. Herein, the laparoscopic lateral suspension was performed for the surgical correction of neovaginal prolapse which is not available in the literature as far as we search. Prolapse was successfully corrected and vaginal length was provided at a sufficient length of 7 cm. Since after 1-year of operation, she has remained satisfied with her surgical outcome anatomically, sexually and psychologically. Laparoscopic lateral suspension is a safe and effective treatment in a patient who has neovaginal prolapse with MRKH syndrome and also can be used as a potentially alternative management in the treatment of neovaginal prolapse in patients with MRKH syndrome.

Vaginal Reconstruction in Patients with Mayer-Rokitansky-Küster-Hauser Syndrome-One Centre Experience.

Background and Objectives: The Mayer-Rokitansky-Küster-Hauser syndrome is a congenital condition in which patients are born with vaginal and uterus agenesis, affecting the ability to have a normal sexual life and to bear children. Vaginal reconstruction is a challenging procedure for plastic surgeons. The aim of this study is to report our experience in the management of twelve patients with congenital absence of the vagina due to the MRKH syndrome. Materials and Methods: We performed a retrospective study on 12 patients admitted to the Plastic Surgery Department of the Clinical Emergency Hospital "Prof. Dr. Agrippa Ionescu", Bucharest, Romania, for vaginal reconstruction within a period of eleven years (January 2009-December 2019). All patients were diagnosed by the gynaecologists with vaginal agenesis, as part of the Mayer-Rokitansky-Küster-Hauser syndrome. The Abbe'-McIndoe technique with an autologous skin graft was performed in all cases. Results: The average age of our patients was 20.16 (16-28) years. All patients were 46 XX. The average surgical timing was 3.05 h (range 2.85-4h). Postoperative rectovaginal fistula was encountered in 1 patient. Postoperative average vaginal length was 10.4 cm (range 9.8-12.1 cm). Regular sexual life was achieved in 10 patients. Conclusion: Nowadays, there is no established standard method of vaginal reconstruction. In Romania, the McIndoe technique is the most applied. Unfortunately, even if the MRKH syndrome is not uncommon, less and less surgeons are willing to perform the procedure to create a neovagina.

A Mayer-Rokitansky-Kuster-Hauser patient with leiomyoma and dysplasia of neovagina: a case report.

BACKGROUND: Most patients with congenital uterus and vaginal aplasia (i.e., Mayer-Rokitansky-Kuster-Hauser [MRKH] syndrome) have rudimentary pelvic uterine structures that contain smooth muscle. Although leiomyomas and dysplasia of vaginal mucosa are relatively common in the general population, they are rare in MRKH patients. Data on the vulnerability of neovaginas to HPV-associated dysplasia are limited. CASE PRESENTATION: A rare case of an MRKH patient with two gynaecological conditions detected during long-term gynaecological follow-up is presented. At the age of 21, the patient was treated for HPV-associated neovaginal dysplasia. At the age of 47, a pelvic leiomyoma was detected with transvaginal ultrasound and confirmed with magnetic resonance imaging. CONCLUSION: A Pap smear or human papillomavirus testing is indicated in sexually active MRKH women. Uterine rudiments contain smooth muscle, which facilitates the development of oestrogen-dependent diseases, such as leiomyomas and adenomyosis. Although magnetic resonance imaging is recommended in cases of a pelvic mass, easily attainable and cost-efficient transvaginal ultrasound offers high diagnostic accuracy in patients with a surgically created neovagina and is suitable for the patients' follow-up. Guidelines for the gynaecological follow-up of MRKH patients are warranted.

Lower urinary tract symptoms in patients with Mayer-Rokitansky-Kuster-Hauser syndrome after neo-vagina creation by Davydov's procedure.

INTRODUCTION: Lower urinary tract symptoms (LUTS) in subjects with Mayer-Rokitansky-Küster-Hauser syndrome treated by Davydov's laparoscopic neo-vaginoplasty were measured. METHODS: Davydov's laparoscopic neo-vaginoplasty was undertaken at the Department of Obstetrics/gynecology, San Raffaele Hospital, Milan, Italy, from November 2015 to July 2017, by two highly qualified surgeons. All women completed the ICIQ-UI Short Form questionnaire before and after treatment. LUTS were scored at recruitment, hospitalization, and 1, 3, 6 and 12 months after surgery. RESULTS: Twenty-one women (mean age 21 years ± 5.9) were operated on with no major complications. Mean surgery duration was 79 min (± 55 min). Before the operation, one patient (1/21; 4.8%) reported occasional urinary loss that persisted after surgery and throughout 12 months of follow-up. After the operation, one patient (1/21; 4.8%) had urinary retention, requiring self-catheterization for 2 weeks. One month after surgery, stress incontinence was recorded in one case (1/19; 5.2%) and urge incontinence in two cases (2/19; 10.5%). At 3-month follow-up, these three patients were free of symptoms. CONCLUSION: The rate of LUTS is lower than that reported by others using alternative surgical procedures. To the best of our knowledge, this is the first report validating LUTS after Davydov's neo-vaginoplasty.

Extrahepatic biliary duplication with heterotopic gastric mucosa in a 46,XX male patient.

Extrahepatic biliary duplication is a rare congenital biliary malformation, even more so when associated with heterotopic gastric mucosa. This case report highlights the difficulty of diagnosing such biliary abnormalities, in particular when the duplicated extrahepatic bile duct is the only structure visible by imaging as it is masking the common bile duct. This report shows that extrahepatic bile duct duplication may be a cause of chronic biliary obstruction and secondary sclerosing cholangitis. It has to be considered as a differential diagnosis of primary sclerosing cholangitis in children and adolescents. Furthermore, a potential link between the 46,XX karyotype and biliary duplication is discussed.

Evolving ethical issues with advances in uterus transplantation.

While uterus transplantation was once considered only a theoretical possibility for patients with uterine factor infertility, researchers have now developed methods of transplantation that have led to successful pregnancies with multiple children born to date. Because of the unique and significant nature of this type of research, it has been undertaken with collaboration not only with scientists and physicians but also with bioethicists, who paved the initial path for research of uterus transplantation to take place. As the science of uterus transplantation continues to advance, so too must the public dialogue among obstetrician/gynecologists, transplant surgeons, bioethicists, and other key stakeholders in defining the continued direction of research in addition to planning for the clinical implementation of uterus transplantation as a therapeutic option. Given the rapid advances in this field, the time has come to revisit the fundamental questions raised at the inception of uterus transplantation and, looking forward, determine the future of this approach given emerging data on the procedure's impact on individuals, families, and society.