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.

Bliss procedure for undescended ovaries.

OBJECTIVE: To highlight a novel surgical approach for the management of undescended ovaries in those presenting with infertility, to allow for potential transvaginal egg retrieval. The video demonstrates a novel surgical approach for mobilization and oophoropexy of undescended ovaries to allow for future transvaginal egg retrieval in the context of artificial reproductive technology (ART). DESIGN: Case report. Institutional Review Board approval is not required because this was not a human study. Patient consent was obtained for video footage. SETTING: Hospital. PATIENTS: We present a 26-year-old nulligravid woman with a unicornuate uterus, a high riding-right ovary, and an undescended left ovary with prior laparoscopic remnant uterine horn resection. Because of her 9 years of infertility and a prior unsuccessful ovarian mobilization and oophoropexy, she was referred for consideration of a repeat laparoscopic bilateral ovarian mobilization and oophoropexy. INTERVENTION: Surgical intervention for undescended ovaries. MAIN OUTCOME MEASURES: Postoperative ovarian location and postoperative pain. RESULTS: The patient reported minimal pain postoperatively at 6 weeks. Multiple follow-up imaging revealed both ovaries behind the uterus (antral follicle counts 15), with easy transvaginal access for future ART. CONCLUSION: Undescended ovary is uncommon and usually requires no treatment. However, intervention may be required in the context of infertility and ART, where transvaginal egg retrieval is impossible because of the location of the ovaries. This is the first educational video to our knowledge highlighting a novel surgical approach for the management of undescended ovaries.

Müllerian Anomalies: Presentation, Diagnosis, and Counseling.

Müllerian anomalies represent a complex collection of developmental defects occurring in up to 5% of the general population. They are increasingly more common in individuals with infertility (8.0%) and in those with a history of pregnancy loss (13.3%); they have the highest prevalence in individuals with a history of both (24.5%). A wide spectrum of anomalies can occur based on the stage at which müllerian development ceases in utero, ranging from mild (eg, a partial uterine septum) to severe, with complete absence of the cervix, uterus, and fallopian tubes (eg, müllerian agenesis). The components of the reproductive tract involved and, importantly, whether an obstruction of the tract is involved correlates with the timing of presentation, the constellation of associated symptoms, and the necessity for either medical or surgical management. Individuals, regardless of the severity of the defect, should be counseled on the gynecologic, reproductive, and obstetric risks associated with their specific müllerian anomaly to minimize adverse sequela and outcomes. We will review the clinical presentation, diagnostic evaluation, and clinical counseling of individuals with müllerian anomalies.

A Retrospective Analysis of 83 Patients with Testicular Mass Who Underwent Testis-Sparing Surgery: The Eurasian Uro-oncology Association Multicenter Study.

INTRODUCTION: Herein, we analyzed the histopathological, oncological and functional outcomes of testis-sparing surgery (TSS) in patients with distinct risk for testicular cancer. METHODS: This is a multicenter retrospective study on consecutive patients who underwent TSS. Patients were categorized in high- or low-risk testicular germ cell tumor (TGCT) according to the presence/absence of features compatible with testicular dysgenesis syndrome. Histology was categorized per size and risk groups. RESULTS: TSS was performed in 83 patients (86 tumors) of them, 27 in the high-risk group. Fifty-nine patients had a non-tumoral contralateral testis present. Sixty masses and 26 masses were benign and TGCTs, respectively. No statistical differences were observed in mean age (30.9 ± 10.32 years), pathological tumor size (14.67 ± 6.7 mm) between risk groups or between benign and malignant tumors (p = 0.608). When categorized per risk groups, 22 (73.3%) and 4 (7.1%) of the TSS specimens were malignant in the high- and low-risk patient groups, respectively. Univariate analysis showed that the only independent variable significantly related to malignant outcome was previous history of TGCT. During a mean follow-up of 25.5 ± 22.7 months, no patient developed systemic disease. Local recurrence was detected in 5 patients and received radical orchiectomy. Postoperative testosterone levels remained normal in 88% of those patients with normal preoperative level. No erectile dysfunction was reported in patients with benign lesions. CONCLUSION: TSS is a safe and feasible approach with adequate cancer control, and preservation of sexual function is possible in 2/3 of patients harboring malignancy. Incidence of TGCT varies extremely between patients at high and low risk for TGCT requiring a careful consideration and counseling.

Diagnostic and surgical approaches to congenital uterine anomalies for the minimally invasive gynecologic surgeon.

PURPOSE OF REVIEW: Congenital uterine anomalies (CUAs) impact the physical and psychosocial wellbeing of affected patients. Managing these conditions depends on the clinical scenario, and in some cases, can involve the use of minimally invasive surgical techniques. The purpose of this review is to provide an update of the diagnosis, perioperative considerations, and treatment of CUAs. RECENT FINDINGS: The American Society for Reproductive Medicine (ASRM) updated the guidelines for classification of CUAs to provide practitioners with a standardized classification system and have created an interactive tool designed for provider use. SUMMARY: Gynecologic surgeons are likely to encounter CUAs during their career. This review provides updated guidance for the workup and treatment of CUAs.

Prenatal Rupture of Hydrocolpos in a Cloacal Malformation.

INTRODUCTION: Cloacal dysgenesis occurs from failure of embryological division of urogenital sinus and hindgut, leading to a single common perineal opening for genitourinary and gastrointestinal tracts. The prenatal diagnosis of cloacal malformation is imprecise, but the clinical correlation of postnatal findings to prenatal history can help reveal explanations for unusual pathological findings in patients with urogenital abnormalities. CASE PRESENTATION: A 21-year-old woman was referred after her 20-week ultrasound demonstrated anhydramnios and concern for dilated fetal bowel. Fetal MRI confirmed anhydramnios and a dilated fetal colon, in addition to hydronephrosis and a pelvic cyst. Repeat ultrasound at 27 weeks showed unexpected complete resolution of her anhydramnios but new fetal ascites. The newborn girl was postnatally diagnosed with a cloacal malformation and an unusual near-complete fusion of her labia. She underwent proximal sigmoid colostomy and a tube vaginostomy at birth followed by cloacal reconstruction at 1.5 years old. CONCLUSION: In female fetus with a pelvic cyst, one should have a high index of suspicion for cloacal anomaly and consider the possibility of urinary obstruction leading to alteration in amniotic fluid.

Kidney transplant access for children and young adults with congenital anomalies of the kidney and urinary tract.

PURPOSE: Although congenital anomalies of the kidney and urinary tract (CAKUT) are among the leading causes of end-stage kidney disease (ESKD) in children and young adults, kidney transplantation access for this population has not been well studied in the US. We compared transplantation access in the US based on whether the etiology of kidney disease was secondary to CAKUT, and additionally by CAKUT subgroups (anatomic vs. inherited causes of CAKUT). METHODS: Using the United States Renal Data System, we conducted a retrospective cohort study of 80,531 children and young adults who started dialysis between 1995 and 2015. We used adjusted Cox models to examine the association between etiology of kidney disease (CAKUT vs. non-CAKUT, anatomic vs. inherited) and receipt of kidney transplantation, and secondarily, receipt of a living vs. deceased donor kidney transplant. RESULTS: Overall, we found an increased likelihood of kidney transplantation access for participants with CAKUT compared to those without CAKUT (HR 1.23; 95% CI 1.20-1.27). Among the subset of individuals with CAKUT as the attributed cause of ESKD, we found a lower likelihood of kidney transplantation in those with anatomic causes of CAKUT compared to those with inherited causes of CAKUT (adjusted HR 0.85; 0.81-0.90). CONCLUSION: There are notable disparities in kidney transplantation rates among CAKUT subgroups. Those with anatomic causes of CAKUT started on dialysis have significantly reduced access to kidney transplantations compared to individuals with inherited causes of CAKUT who were initiated on dialysis. Further studies are needed to understand barriers to transplantation access in this population.

Urogenital Sinus Diagnosed During Workup of Recurrent Urinary Tract Infections: A Case Report.

Urogenital sinus (UGS) is a rare anomaly characterized by a common cavity involving the reproductive and urinary tracts. We describe a patient with VACTERL syndrome who presented for urologic care at 11 years of age due to supposed "recurrent urinary tract infections" and was subsequently found to have UGS in which the vagina connected directly to the bladder. She underwent robotic UGS mobilization to disconnect the vagina from the bladder and vaginoplasty to mature the vagina to the perineum. The objective of this report is to describe the presentation, diagnosis, and management of a patient with rare high confluence UGS.

Successful management of cervical dysgenesis: Case report and review.

Cervical agenesis or dysgenesis is a rare Mullerian anomaly that is usually associated with vaginal aplasia. A literature review revealed reports of 83 cases including ours, of which 57 (68.6%) presented with obstruction of the external OS, 11 (13.2%) had the cervix replaced by a fibrous cord and 5 (6.02%) had a fragmented cervix. A total of 24 (28.9%) were managed by core and drilling technique (CDT), 16(19.2%) patients underwent uterovaginal anastomosis (UVA), 7(8.4%) underwent total abdominal hysterectomy preserving the ovaries and 5 (6.02%) were managed by cervical reconstruction. Unfortunately, 31 failed to return after their clinical and radiological diagnosis was confirmed. Early diagnosis and treatment are necessary to avoid long-term complications.

Forty-two normomenstruating adolescents with Müllerian obstructive anomalies: Presentation, pitfalls in the dagnosis and surgical management.

INTRODUCTION: We analyzed the frequency, presentation and pitfalls in the diagnosis and surgical management of a large group of normomenstruating adolescents with obstructive reproductive tract anomalies. MATERIAL AND METHODS: Retrospective analysis of prospectively collected data. Of the 143 outpatients referred for severe dysmenorrhea and persistent pelvic pain, 42 (29.3%) young women with obstructive Müllerian anomalies and regular menstrual flow were identified. These patients were divided into four groups: (1) patients with duplicate uterine cavities, obstructed hemivagina and ipsilateral renal agenesis (n = 34); (2) patients with unicornuate uterus and noncommunicating cavitated rudimentary horn (n = 5); (3) patients with accessory cavitated uterine mass (n = 2); (4) patients with partially obstructed transverse vaginal septum (n = 1). All 42 patients were conservatively treated via laparoscopy and 35/42 patients had also vaginal surgery. RESULTS: Of the four groups, patients in groups 2 and 3 (n = 7) were conservatively managed by laparoscopy alone; for patients in groups 1 and 4 (n = 35), laparoscopy and the vaginal approach were used. Patients of group 1 were treated by resecting the obstructed vaginal septum with drainage of retained collections. In patients in group 2, surgery consisted of the removal of the rudimentary horn. Patients of group 3 were treated by the removal of myometrial neoformations. In the patient in group 4, treatment consisted of removal of the septum. All surgical procedures were successful and no major complications were recorded. Follow-up reports highlighted the disappearance of obstruction and clear improvement in pain symptoms. CONCLUSIONS: Unilateral obstructive anomalies of the female genital tract are difficult to identify. Early diagnosis allows the preservation of reproductive activity and avoids potential complications.

A uterine malformation diagnosed in the shock room: a case report which helps to identify how to avoid a potentially preventable life-threatening event.

An Indian wonen at her second pregnancy, with a previous preterm labour at 34.5 weeks, presented to the emergency room at 15.6 weeks, shocked due to massive hemoperitoneum. During the urgent surgery, a Mullerian malformation was detected, and a uterine rupture was diagnosed as the cause of the hemoperitoneum. Ultrasound and pelvic magnetic resonance results confirmed the malformation, determining that a unicornuate uterus with a rudimental horn within the pregnancy had taken place. We decided to report our case to underline that Mullerian malformation must be taken into consideration and evaluated at each routinary gynaecological visit or, at least, at the first pregnancy appointment, especially in the cases of women with previous adverse obstetric outcomes. The 2D abdominal ultrasound associated with trans-vaginal evaluation is an adequate procedure to test for suspected uterine malformations (if the operator keeps it in mind and is trained to check this kind of alteration), which is fundamental to reducing the risk of life-threatening events.

Laparoscopic Management of Rudimentary Uterine Horns in Patients with Unicornuate Uterus: A Systematic Review.

INTRODUCTION: Unicornuate uterus is a rare Müllerian anomaly. Its potential association with a rudimentary uterine horn can cause a diagnostic delay. The most common consequences are pelvic pain, hematometra, and endometriosis. Diagnosis of a unicornuate uterus is usually done by imaging combining ultrasound and magnetic resonance imaging. The aim of this systematic review is to assess the role of laparoscopic approach in the management of this rare condition. METHODS: A comprehensive search was performed in PubMed, EMBASE, SCOPUS, and Web of Science databases prior to 1 May 2022 according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement (PRISMA). The inclusion criteria were: cases of rudimentary horn managed through laparoscopy only; laparoscopic treatment of communicating or noncommunicating uterine horn. RESULTS: The search strategy identified 45 articles. After this first screening, 37 studies were evaluated. The full text of remaining articles was examined. 35 studies were finally included in this article. All included studies were case reports, due to the rarity of this condition. Rudimentary horns were noncommunicating in all cases. CONCLUSION: The laparoscopic removal of a rudimentary uterine horn could be considered a feasible therapeutic option. An accurate preoperative evaluation is mandatory to assess anatomic variants and to select the optimal and tailored surgical approach, based also on the symptoms complained by the patient.

Anatomy and embryology of congenital surgical anomalies: Congenital Anomalies of the Kidney and Urinary Tract.

Congenital anomalies of the kidney and urinary tract or "CAKUT" describes a spectrum of developmental disorders with a range of associated clinical presentations and functional consequences. CAKUT underlies the majority of chronic kidney disease and kidney replacement therapy requirement in children, but functional deterioration can also emerge in adulthood. Understanding the normal embryological processes involved in kidney development allows us to appreciate the timing and sequence of critical events implicated when things go wrong. In this review, we will describe the normal developmental mechanisms and relate this to what we currently know about the pathological processes involved in various forms of CAKUT. We will also review the proposed etiological factors, in particular genetics, involved in CAKUT.

Rare case of an isolated scaphoid congenital megalourethra: before and after reconstruction.

Congenital megalourethra, first described in 1955, is a rare urethral anomaly resulting from dysgenesis of the penile corpus spongiosum, with or without corpus cavernosum involvement, leading to dilatation of the penile urethra. Presentations come in two forms, scaphoid and fusiform, with the former being more common and resulting from deficient or absent corpus spongiosum. Fusiform types are much rarer, and consist of absence of both the corpus spongiosum and cavernosum.3 Here, we present a case involving the surgical correction of an isolated scaphoid-type congenital megalourethra with significantly improved postoperative cosmetic and functional outcomes.