Septate uterus discovery during an emergency cesarean section in the North Eastern of the Democratic Republic of Congo: A case report and review of the literature.

INTRODUCTION AND IMPORTANCE: 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. CASE PRESENTATION: 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. CLINICAL DISCUSSION: 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. CONCLUSION: Septate uterus as congenital uterus malformations, should be suspected in any women with obstetric complications high risk such as miscarriage, preterm delivery and malpresentation.

Novel Approaches to Patients with Differences of Sex Development.

Patients with differences of sex development (DSDs) have complex anatomy and surgical needs related to both Mullerian and non-Mullerian structures. Approaches to vaginal reconstruction for these conditions are guided by individual anatomy, with the goal of establishing unobstructed outflow for the reproductive, urinary, and gastrointestinal tracts. Patients may have anatomy requiring vaginoplasty for either outflow tract obstruction or chosen sexual function. In this article, the authors focus on management of differences in vaginal anatomy with delayed vaginoplasty for the newborn with DSD.

Examining Results of Post-Thelarche Screening Pelvic Ultrasound in Females with Anorectal Malformations.

STUDY OBJECTIVE: Mullerian duct anomalies are common in females with anorectal malformations (ARMs), although there are no universally recommended screening protocols for identification. Historically, at our institution, we have recommended a screening pelvic ultrasound (PUS) 6 months after thelarche and menarche. We aimed to evaluate outcomes associated with our post-thelarche screening PUS in females with ARMs. METHODS: An institutional review board-approved retrospective chart review was performed for all female patients 8 years old or older with ARMs and documented thelarche. Data were collected on demographic characteristics and clinical course. The primary outcome was adherence to the recommended PUS. Secondary outcomes included imaging correlation with suspected Mullerian anatomy and need for intervention on the basis of imaging findings. RESULTS: A total of 112 patients met the inclusion criteria. Of them, 87 (77.7%) completed a recommended post-thelarche screening PUS. There were no differences in completion on the basis of age, race, establishment with a primary care provider, insurance status, or type of ARM. Nine patients (10.3%) had findings on their PUS that did not correlate with their suspected Mullerian anatomy; five (5.7%) required intervention, with two requiring menstrual suppression, two requiring surgical intervention, and one requiring further imaging. CONCLUSION: Most patients completed the recommended post-thelarche screening PUS. In a small subset of patients, PUS did not correlate with suspected Mullerian anatomy and generated a need for intervention. Post-thelarche PUS can be a useful adjunct in patients with ARMs to identify gynecologic abnormalities.

A Rare Case of Hematometrocolpos due to Transverse Vaginal Septum along with Distal Vaginal Atresia in an Adolescent Girl.

Primary amenorrhoea due to Müllerian malformations is rare, with 1 in 4500 cases and 2%-8% of cases presenting as infertility. Obstructive Müllerian anomalies present as hematometra and hematocolpos during puberty. Timely surgical intervention is required to relieve acute pelvic pain and restore functional anatomy. A 15-year-old girl presented to OPD with complaints of severe pain in her lower abdomen and lower back for the last 2-3 weeks, not relieving on medication. She has not attained menarche and has been having cyclical pain and low backache for 7-8 days every month for the last year. Physical examination showed a suprapubic lump with vaginal agenesis. Magnetic resonance imaging revealed hematometrocolpos due to transverse vaginal septum and distal vaginal atresia. Pull-through vaginoplasty along with complete excision of transverse vaginal septum was performed. Vaginal dilator therapy was done after the healing of the sutures. In follow-up, the patient attained menstruation with a patent vagina. Obstructive Müllerian anomalies should be identified early by detailed clinical examination and targeted investigations to prevent long-term morbidity and infertility.

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)

Successful pregnancy outcome via in-vitro fertilization and laparoscopic resection of non-communicating rudimentary horn pregnancy containing early pregnancy: a case report.

BACKGROUND: Non-communicating rudimentary horn pregnancy (NCRHP) lead to life-threatening condition for both mother and fetus. Early diagnosis of NCRHP and laparoscopic resection is important to prevent catastrophic conditions. However, delayed diagnosis until the second or third trimester makes it difficult to accurately diagnose between NCRHP and bicornuate uterine pregnancy, as both conditions present uterine rupture and massive hemoperitoneum. Furthermore, these rare cases are challenging in pregnancy trials and associated with adverse outcomes in subsequent pregnancies. CASE PRESENTATION: A 31-year-old gravida 1 para 0 Korean woman visited our infertility center with a confirmed positive urine pregnancy test after timed intercourse. Before she was scheduled to have timed intercourse, a unicornuate uterus with a non-communicating right uterine horn was suspected based on an ultrasound scan and hysterosalpingography during the initial infertility workup. A gestational sac was observed in the right non-communicating rudimentary horn at 5 weeks of gestation. Serum beta-human chorionic gonadotropin (b-hCG) level was 2052.0mIU/mL. An elective laparoscopic resection of the right rudimentary horn containing a gestational sac, along with ipsilateral salpingectomy, was performed with no adverse event. After 3-month of recovery period and three cycles of conceptional trials involving timed intercourse and intrauterine insemination, in-vitro fertilization (IVF) was performed using the antagonist protocol, and successful pregnancy was confirmed. The patient had been hospitalized from 21 + 6 weeks to 35 + 6 weeks of gestation, underwent cerclage placement and tocolytics with corticosteroid treatment. She delivered an early-term male baby by cesarean section. CONCLUSION: In this rare case, the successful pregnancy achieved through IVF following the appropriate management of NCRHP under laparoscopy underscores the critical importance of early diagnosis and intervention in cases of NCRHP. Timely identification and management of NCRHP are vital to prevent the occurrence of catastrophic conditions and to enhance the prognosis of a successful pregnancy through assisted reproductive technology (ART). Therefore, a high index of suspicion for NCRHP is important and employs a range of diagnostic modalities.

Evaluation and Resection of a Longitudinal Vaginal Septum in an Adult.

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. DESIGN: This is a stepwise demonstration of evaluation and surgical techniques with video narration. SETTING: 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]. INTERVENTIONS: 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. CONCLUSION: 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.

Case Series of Reproductive Outcomes after Surgical Correction of Obstructed Hemivagina in OHVIRA.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital developmental syndrome manifested by uterine duplication, lower genital tract obstruction, and unilateral renal anomaly. Literature on reproductive outcomes in this patient population is limited. The aim of this study is to describe obstetric outcomes after surgical correction of obstructed hemivagina in a longitudinal cohort of patients with a diagnosis of OHVIRA. All cases of OHVIRA presenting to a single tertiary care children's hospital from 1990 to 2021 were retrospectively reviewed. Three cases demonstrating a variety of clinically important reproductive outcomes are described in detail including risks such as retained products, endometritis, preterm labor, and malpresentation. Understanding the reproductive outcomes associated with this diagnosis is important for practitioners seeking to counsel and care for patients with this diagnosis. This case series demonstrates a wide array of potential gynecologic and obstetric risks, though ultimately with successful term and near-term pregnancies.

Uterine diverticulum mimicking endometriotic cyst of the ovary.

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.

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.

Long-term effects of surgically corrected menstrual outflow obstruction: A case-control study.

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. CONCLUSION: 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.

Laparoscopic-assisted neo-cervico-vaginal anastomosis and neo-vagina creation in congenital atresia: A retrospective study measuring the outcome and success.

Cervico-vaginal agenesis is a developmental disorder classified as a Mullerian duct anomaly. STUDY OBJECTIVE: We aimed to study the surgical outcomes of vaginoplasty and laparoscopic-assisted cervical-vaginoplasty carried out using our set-up from June 2016 to December 2022. Additionally, we measured the success of our modified laparoscopy-assisted neo-cervical creation. DESIGN: A retrospective study. SETTING: The study was conducted in a tertiary care center and included all patients who underwent vaginoplasty and neo-cervico-vaginal creation using the rail-road method. PATIENTS: A total of 33 patients were followed-up meticulously and their outcomes were noted. The study was retrospective and largely depended on patient compliance during follow-up. INTERVENTION: Of the 33 patients, 22 underwent vaginoplasty (McIndoe technique) and 11 underwent neo-cervico-vaginoplasty (rail-road method using both open and laparoscopic methods). MEASUREMENTS AND MAIN RESULTS: The most common presentation was primary amenorrhea (cyclic abdominal pain with primary amenorrhea) and urinary retention. Four patients underwent hysteroscopy-guided hematometra drainage and none underwent hysterectomy. The average vaginal length measured during the follow-up was 5.2 ± 1.90 cm. Significant symptom relief was observed. Longest follow-up duration was 4.7 years with a mean duration of 210 (180) days. CONCLUSION: Although the procedure is strenuous, postoperative results showed satisfactory outcomes in improving the quality of life of patients. With expertise in the technique, the associated comorbidity is reduced, which provides relief for the patients.

Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series.

INTRODUCTION: 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. STUDY 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. CONCLUSION: 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.

Laparoscopic Davydov vs. laparoscopic Vecchietti neovaginoplasty in women with Mayer-Rokitansky-Küster-Hauser syndrome; a systematic review and meta-analysis.

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. DESIGN: A systematic electronic search up to August 2022 using PubMed and Embase is performed. SETTING: Not applicable. PATIENTS: Women with Mayer-Rokistansky-Küster-Hauser syndrome. INTERVENTIONS: 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. MAIN OUTCOME MEASURES: 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.

MRI Findings in a Rare Case of Left-Sided Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in a 21-Year-Old Female.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a complex Mullerian and Wolffian duct anomaly, which is difficult to diagnose before puberty. We present a rare case of a congenital syndrome known as OHVIRA in a 21-year-old female who came with complaints of intermittent type of lower abdominal pain, dysmenorrhea, and oligomenorrhea with frequent visits to different hospitals without any radiological investigations done. Early magnetic resonance imaging (MRI) investigations helped her in diagnosing and managing this syndrome.

Delayed diagnosis of a non-communicating right uterine horn: A case report and literature review.

Background: Accurate diagnosis of a müllerian anomaly is essential for appropriate management and prevention of complications. However, diagnosis is often missed or delayed. Case: 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. Conclusion: 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.

Spontaneous successful term delivery in a unicornuate uterus: A case report and literature review.

INTRODUCTION AND IMPORTANCE: Malformations of the uterus are generally rare and are due to defective fusion of Mullerian duct. These include the unicorn uterus, with or without a rudimentary horn. Pregnancy in a unicornuate uterus is a rare event and carries with it maternal-fetal risks. CASE PRESENTATION: In this report, we present an exceptional case of a pregnancy reaching full term in a unicornuate uterus without an accessory horn, which was serendipitously discovered during an emergency cesarean section. Intraoperatively, the uterus exhibited a distinct cylindrical shape with a flattened left wall, with the absence of both the left fallopian tube and left ovary. No additional abnormalities within the abdominopelvic cavity were noted. Notably, the postoperative recovery was uneventful for both the mother and newborn, without any notable complications. CLINICAL DISCUSSION: The presence of a unicornuate uterus is associated with adverse obstetric outcomes, including recurrent miscarriage, premature delivery, fetal malposition, intrauterine growth retardation, and uterine rupture. In addition, around 40 % of women with this malformation may experience infertility. Although the efficacy of preconception or early interventions, such as rudimentary uterine horn resection and prophylactic cervical cerclage has not been fully established in improving obstetric outcomes, their usefulness is suggested by current medical practice. CONCLUSION: Women with a unicornuate uterus are known to be at increased risk of obstetrical complications, such as spontaneous abortion, premature delivery, post-partum hemorrhage, abnormal fetal presentation and cesarean delivery. It is important to consider women with this anomaly as being at high obstetrical risk.

Dichorionic-Diamniotic Twin Pregnancy in a Bicornuate Uterus With Twin A Affected by Ebstein's Anomaly.

Congenital Mullerian anomalies are rare developmental defects that result in malformation of the fallopian tubes, uterus, cervix, and vagina. The bicornuate uterus is one of the many variants of Mullerian anomalies, defined as having an external fundal indentation of greater than one centimeter. Pelvic ultrasound has a sensitivity of 99% for identifying bicornuate uteruses and is the predominant imaging device used for diagnosis. The cervical and uterine cavity anatomy in patients with a bicornuate uterus varies. The effect of maternal uterine structure on offspring development has been poorly documented. This report details a rare case of dichorionic-diamniotic twin pregnancy in a bicornuate uterus with one fetus affected by Ebstein's anomaly. Twin A was diagnosed by first-trimester ultrasound with right renal agenesis and Ebstein's anomaly. Twin B did not have any anatomical defects identified on ultrasound. Both twins were delivered via emergency repeat cesarean section at 34 weeks and four days due to nonreassuring fetal heart tracings and twin A in the breech presentation. Twin A and twin B were found to be in separate horns within the uterus during low transverse cesarean section. Twin A required endotracheal intubation in the delivery room due to respiratory distress. Both twins required neonatal intensive care treatment. Twin A was found to have a right pelvic kidney, rather than right renal agenesis, while in the neonatal intensive care unit. Females with germline mutations in the Mullerian duct and urogenital sinus development have resulted in concomitant malformations in the uterus and kidneys. This is a rare case of an infant with a cardiac anomaly born to a mother with a germline mutation. The relationship between congenital heart defects and uterine anomalies has not been identified. As seen in this case, maternal malformations impacting fetal cardiac development can be sporadic or result from germline mutations in mesoderm that have not been reported yet.

Retention of a Foreign Body in the Vagina of an Adult for 13 Years: A Case Report.

Cases in which foreign bodies have been inserted into the vagina predominately occur in the pediatric population. This report presents the case of an adult woman with a retained foreign body for 13 years. A duplicated ureter suggestive of a Mullerian anomaly was incidentally identified on intraoperative cystoscopy. Mullerian anomalies may be associated with complex patient presentations and are associated with reproductive implications that should be discussed based on patient-specific characteristics.

Management of Hematometra and Hematocolpos in Obstructive Mullerian Anomalies by Image-Guided Drainage with Interventional Radiology: A Multi-Institutional Case Series.

STUDY OBJECTIVE: To describe cases of image-guided drainage of symptomatic hematometrocolpos from obstructive Müllerian anomalies as a temporizing measure to manage acute pain symptoms and delay definitive management of the obstructive Müllerian anomalies that require complex reconstruction METHODS: Institutional Review Board exemption from all included institutions was obtained. A retrospective case series from 3 academic children's hospitals of 8 females under the age of 21 with symptomatic hematometrocolpos due to obstructive Müllerian anomalies drained by image-guided percutaneous transabdominal vaginal or uterine drainage with interventional radiology was reviewed and described. RESULTS: Eight pubertal patients with obstructive Müllerian anomalies (6 patients with distal vaginal agenesis, 1 patient with an obstructed uterine horn, and 1 patient with a high obstructed hemi-vagina) and symptomatic hematometrocolpos are reported. All patients with distal vaginal agenesis had greater than 3 cm lower vaginal agenesis, which would usually require complex vaginoplasty and use of postoperative stents. Given their immaturity and inability to use stents or dilators postoperatively or medical complexity, they subsequently underwent ultrasound-guided drainage of hematometrocolpos with interventional radiology to relieve pain symptoms, followed by menstrual suppression. The patients with obstructed uterine horns had complex medical and surgical histories requiring perioperative planning; they also underwent ultrasound-guided drainage of hematometra as a temporizing measure to manage acute symptoms. CONCLUSION: Patients presenting with symptomatic hematometrocolpos due to obstructive Müllerian anomalies might not be psychologically mature enough to undergo definitive complex reconstruction, which requires vaginal stent or dilator use postoperatively to prevent stenosis and other complications. Image-guided percutaneous drainage of symptomatic hematometrocolpos serves as a temporizing measure by offering pain relief until patients are ready to undergo surgical management and/or to allow time for complex surgical planning.