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Zinner Syndrome (ZS) and Herlyn-Werner-Wunderlich Syndrome (HWWS) are congenital genitourinary anomalies accompanied by ipsilateral renal agenesis. These syndromes, which can cause symptoms such as infertility, pelvic pain, and bladder irritation, can be diagnosed by digital rectal examination, transrectal and abdominal ultrasonography (US), Computer Tomography (CT) and more ideally Magnetic resonance imaging (MRI). Similar physiopathological processes are observed in both syndromes and urogenital malformations accompany renal agenesis. We aimed to discuss the three cases of ZS in men and two HWWS cases in women, which are rarely observed in the literature. [ABSTRACT FROM AUTHOR]

Objectives. To analyze the gynecological pathologies and extragenital anomalies associated with unilateral renal agenesis (URA) and the possible origin of these congenital anomalies. Design. Retrospective case-control study. Setting. University Hospital. Population. This study included 276 women with genitourinary malformations who had undergone hysterosalpingography (and/or laparoscopy) and pyelography with images available for review. Methods. There were 60 cases of women diagnosed with genital malformations and congenital URA and 216 control cases of women with genital tract malformations and both kidneys present. All cases were categorized according to an embryological-clinical classification and the type of Müllerian malformation (American Society for Reproductive Medicine (ASRM) classification) and then compared by type for the presence of gynecological and extragenital pathologies. Main outcome measures. Genital malformations, endometriosis, leiomyomas and skeletal anomalies. Results. URA was generally associated with either agenesis of all of the derivatives of the urogenital ridge on the same side of the body, which were usually found on the left, or distal mesonephric anomalies such as a double uterus with a blind hemivagina or unilateral cervico-vaginal atresia, which were most frequently on the right. The uterine malformations that were most commonly seen in women with renal agenesis were bicornis-bicollis, didelphys and unicornuate uteri. Women with bicornuate or didelphys uteri and renal agenesis had more gynecological pathologies, such as endometriosis, than those with both kidneys present. Conclusions. URA and major uterine malformations are frequently related, and individuals with bicornuate or didelphys uteri have endometriosis more often than those without renal agenesis. Those malformations that seem to be caused by the absence or anomaly of a mesonephric duct lead to renal agenesis, ipsilateral vaginal anomalies (blind or atretic hemivagina) and failure of the induction function of the Wolffian ducts on the Müllerian ducts, causing uterine malformations. [ABSTRACT FROM AUTHOR]

Didelphic uterus with blind hemivagina and ipsilateral renal anomaly is a rare congenital malformation. The pathogenesis of this syndrome and its embryologic origin are still the subject of discussion. Due to the variable clinical picture and low index of suspicion, diagnosis is often delayed or inaccurate. Conservative surgical treatment—excision of the obstructing vaginal septum and marsupialization of the blind hemivagina—is considered the most appropriate treatment. This report describes the case of a 16-year-old girl with didelphic uterus and double vagina, with obstruction of the left hemivagina. The patient was also diagnosed with left renal dysplasia and ipsilateral ureter communication with the obstructed vagina. Following various examinations and left uretero-nephrectomy, the patient was effectively treated with resection of the vaginal septum. [ABSTRACT FROM AUTHOR]

We present a case of an unusual diagnosis of a bicornuate unicollis uterus mimicking a uterus didelphys with blind hemivagina. Few cases of a bicornuate uterus have been reported with the primary symptom of a paracervical pyocolpos. The difficulties involved in the diagnosis and management of this particular congenital malformation are described in detail. The role of imaging techniques and adequate preoperative preparation is emphasised with a review of recent literature. [ABSTRACT FROM AUTHOR]

The presentation of an acute abdomen has a wide range of differentials. Developmental anomalies are rarely considered as part of the clinical reasoning process due to it being a mimicker of other commoner diseases. The importance of considering such anomalies and recognizing them early is essential in order to prevent complications, such as infertility. Our case report presents an approach to a female of pediatric age complaining of right lower abdominal pain mimicking appendicitis. The physical examination and clinical findings were also in line with appendicitis. Thus, the initial work-up focused on that as the top differential. Due to the patient being female, other work-ups were conducted in order to rule out gynecological conditions such as a pelvic ultrasound. The ultrasound findings changed the approach, warranting a CT and MRI. The subsequent diagnosis was Herlyn-Werner-Wunderlich Syndrome. Intervention involved incision and dilation of the vaginal septum as it is the most effective and least traumatic method. On follow-up, the patient's symptoms resolved. Rare diseases can mimic common diseases. Clinically, findings of other work-ups indicated a completely different diagnosis than originally suspected. Clinical reasoning thus shifted the focus to another approach and intervention. Clinical features of appendicitis, a clinical diagnosis, were most evident. An inappropriate management may have ensued if investigations were not conducted. Ultimately, the case report illustrated the importance of radiological investigations in an acute abdomen. • An unnecessary appendectomy was avoided by considering acute abdomen mimickers. • The usual 'acute abdomen' should include unusual developmental anomalies. • Early recognition of rare syndromes, such as HWW syndrome, prevents complications. • Preserving fertility in HWW syndrome is key and is preventable if detected early. [ABSTRACT FROM AUTHOR]

Uterovaginal duplication with obstructed hemivagina and ipsilateral renal agenesis is referred to as the Herlyn-Werner-Wunderlich (HWW) syndrome. A 17 year old woman presented with right pelvic pain and dysmenorrhea, present since menarche at 13 and worsening over the past year. Ultrasound examination revealed a right pelvic mass (5x5 cm), double endometrial echoes, and hematocolpos. A right pelvic mass, agenesis of the right kidney, double uterus, and blind hemivagina with hematocolpos were detected by magnetic resonance imaging and intravenous pyelography. A right tubo-ovarian abscess with dense adhesions and a double uterus were observed on diagnostic laparoscopy. Adhesiolysis was carried out and purulent material irrigated. After a course of antibiotics, a vaginal septum resection was performed and the pyocolpos drained. She remained symptom free after four months of follow-up. Prompt and accurate diagnosis and treatment of this syndrome can significantly improve the lives of sufferers and prevent future complications. [ABSTRACT FROM AUTHOR]

Background: To evaluate fertility and pregnancy outcomes in patients with uterus bicorporeal and blind hemivagina. Our second objective was to investigate factors predicting fertility and pregnancy outcomes in those patients., Material and Methods: We conducted a retrospective analysis in a tertiary referring gynaecological department, in France. We included all patients with uterus bicorporeal and blind hemivagina who underwent at least one surgery in our centre. Initial characteristics of the patients included were extracted from their medical charts and patients were contacted to assess their fertility and pregnancies outcomes upon their consent to participate to the study., Results: Between 1989 and 2010, 79 patients fulfilled inclusion criteria and were selected for analysis. Mean follow up of those patients was of 16.15 (QI 10-21) years. Forty-six patients (58.2%) returned the survey and among them, 21 (45.7%) were fertile, 8 (17.4%) were infertile and 17 (37%) never attempted to get pregnant following initial management. Forty-nine pregnancies were included to assess pregnancies outcomes: 11 (22.5%) early miscarriages, 1 (2.0%) extra-uterine pregnancy, 2 (4.1%) second semester miscarriages and 35 (71.4%) leaded to living birth. Nineteen (54.3%) deliveries occurred by caesarean section and 14 (40.0%) by vaginal delivery. Fifteen pregnancies (42.9%) were complicated. In univariate analysis, adhesiolysis performed at the time of initial surgery was the only factor associated with infertility (p=0.004)., Conclusions: Fertility seems to be perfectly conserved in those patients and they do not have increased rate of adverse pregnancies outcomes., Competing Interests: Declaration of Competing Interest CT has occasionally served as a consultant for TWA – ADELPHI. The remaining authors have nothing to disclose., (Copyright © 2019. Published by Elsevier Masson SAS.)