Ipsilateral Renal Agenesis Syndrome Research Articles

Ohvira syndrome with rare presentations – A case report

Herlyn Werner Wunderlich syndrome is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. It is synonymous with OHIVRA syndrome which is Obstructed Hemivagina with Ipsilateral Renal Agenesis syndrome. Very few case reports have been reported regarding the syndrome. Herein, we report a case of 20 year old unmarried female with unusual presentation of OHVIRA syndrome with complaints of spotting per vagina and with vaginal cyst on examination. Diagnosis was confirmed by MRI which showed features of OHVIRA with pyometra caused by the obstructed hemivagina. She was managed surgically by diagnostic laparoscopy and per vaginal resection of the vaginal septum and drainage of around 50cc of pyometra followed by visualisation of two separate cervical canals post procedure. As a rare entity, a high degree of clinical suspicion is necessary to diagnose and intervene at the right time to ensure optimum results and prevent complications and preserve fertility.

Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis Syndrome in Adulthood: A Diagnostic Challenge.

A patient in her early 20s presented with constant and progressive lower abdominal and back pain, mainly on the right side of the abdomen, purulent vaginal discharge and pyrexia. A radiological assessment revealed a possible tubo-ovarian abscess and the incidental diagnosis of ipsilateral renal agenesis. The patient was treated for pelvic inflammatory disease (PID); however, after antibiotic administration and since the symptoms did not resolve, an abdominal MRI was requested, which revealed uterus didelphys with two cervices, an obstructed haemivagina and evidence of haematocolpos. The diagnosis of Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome was confirmed, and the patient underwent the excision of the vaginal septum, the drainage of the haematopyocolpos and the laparoscopic drainage of the tubo-ovarian abscess. She achieved a good recovery.

Three cases of obstructed hemi-vagina and ipsilateral renal agenesis syndrome complicated with different severities of endometriosis

Three cases of obstructed hemi-vagina and ipsilateral renal agenesis syndrome complicated with different severities of endometriosis

OBSTRUCTED HEMIVAGINA AND IPSILATERAL RENAL ANOMALY SYNDROME: A NONCLASSICAL PRESENTATION WITH AN INTERCERVICAL COMMUNICATION

To describe a nonclassical presentation and infertility management of Obstructed Hemivagina with Ipsilateral Renal Agenesis Syndrome (OHVIRA), also known as Herlyn-Werner-Wunderlich Syndrome (HWWS). This is a case report of a 31-year-old female with OHVIRA syndrome who presented to a community-based medical center and fertility office. A 31-year-old female presented to her obstetric provider with a missed abortion (MAB) and decision was made for surgical management. Intraoperatively, a uterine didelphys was incidentally found due to an inability to evacuate the correct cavity during suction dilation and curettage. Additionally, the right cervix was dilated three centimeters, likely due to chronic hematocolpos and hematometria. MRI imaging suggested OHVIRA syndrome with a patent cervical connection at the level of the internal cervical os. Subsequently, she underwent complete vaginal septum excision and marsupialization. A prophylactic robotic-assisted transabdominal cerclage was then placed for concern of an incompetent cervix from chronic obstruction and intercervical communication. Finally, in vitro fertilization was undertaken, leading to a successful pregnancy outcome. OHVIRA syndrome classically presents in adolescence with nonspecific complaints of dysmenorrhea or pelvic pain. We report a nonclassical presentation of OHVIRA syndrome and illustrate a stepwise management strategy for women with this heterogenic Mullerian anomaly to achieve and maintain a pregnancy to term.

Clinical characteristics and management of patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis.

To analyze the clinical characteristics and management of patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis. This retrospective study reviewed the medical records of 17 patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis admitted to the Third Xiangya Hospital of Central South University between June 2007 and December 2019. The median age at surgery was 23 years. The most common presenting complaint was infertility. Seven (41.2%) patients were misdiagnosed previously. The obstruction was complete in five patients. All 17 patients underwent vaginoplasty, in which seven adolescent girls underwent vaginoscopic vaginal septum resections. Eight patients underwent hysteroscopic resections of the uterine septum for infertility or spontaneous miscarriage. Laparoscopy was performed in seven patients for specific indications, and only one patient was found to have pelvic endometriosis during the laparoscopy. During the follow-up, seven patients wished to conceive, and there were five living infants (four cesarean deliveries at term and one preterm vaginal delivery). Complete septate uterus with double cervix, obstructed hemivagina, and ipsilateral renal agenesis is an variant of obstructed hemivagina and ipsilateral renal agenesis syndrome. Consequently, healthcare providers should be aware of this potential variant.

Tubulocystic anomalies of the mesonephric duct associated with ipsilateral renal dysgenesis

Renal agenesis and multicystic dysplastic kidney (MCDK) are usually associated with either an absent or atretic ureter. Occasionally, these renal anomalies may be associated with a dilated tortuous ureter, ureterocele or other cystic malformation of mesonephric duct (MND) remnants. The objective of this study was to identify and classify anatomical variants of tubulocystic remnants of the MND, with a secondary focus on natural history and management outcomes. A retrospective cohort study of patients seen in the study institution between 2007 and 2014 with a tubulocystic abnormality of MND structures associated with either MCDK or renal agenesis was conducted. Medical imaging and progress notes were reviewed for all patients. Data collected included anatomical information, surgical intervention, histology and outcomes of both conservatively managed and surgically resected MND remnant structures. Nineteen patients were identified, 5 girls and 14 boys. Median age at presentation was 4.6 years. Anomalies of the MND occurred on the left in 9 patients and on the right in 10 patients. Mean follow-up was 3.4 years. Patients fell into 3 distinct anatomical groups: Type I, including orthotopic remnants corresponding to ureteric bud structures (ureter and trigone); Type II, including ectopic MND remnants of ureteric bud structures, and Type III, including complex remnants corresponding to MND structures other than those from ureteric bud (vas, epididymis and seminal vesicles). Anomalies of structures arising from urogenital sinus and paramesonephric ducts were also identified. Most patients were asymptomatic and successfully managed conservatively. Transvesical puncture of trigonal cysts provided effective decompression in 5 patients. Partial or complete MCDK regression was seen in 7 patients, whereas MND cystic anomalies did not regress spontaneously. When MND tubulocystic structures persist along with renal agenesis or MCDK, most arise from ureteric bud structures in an orthotopic positionas a ureterocele with or without a blind-ending ureter-like structure. Less commonly, ureteric bud structures insert ectopically into the urogenital tract, or tubulocystic structures arising from the remainder of the MND occur. Embryogenesis of other urogenital structures may also be abnormal, and conditions such as Zinner syndrome and obstructed hemivagina and ipsilateral renal agenesis syndrome should be considered. Complications are uncommon, and surgical intervention should be limited to symptomatic patients. Remnants of metanephric blastema may involute, but MND remnants persist.

Management of Hematometra in Single Horn of Didelphic Uterus with Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome: A Case Report with Brief Review of Literature

ABSTRACT The triad of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare entity. These cases are difficult to diagnose due to heterogeneous presentation and rarity of the condition. Ultrasonography and magnetic resonance imaging are important for initial diagnosis; however, laparoscopy is required for the confirmation of the diagnosis. Early and accurate diagnosis of this condition followed by prompt surgical therapy is essential to prevent complications and to preserve future fertility. Here we report a case of uterus didelphys with hemivagina and ipsilateral renal agenesis with hematometra and hematosalpinx in a young girl. How to cite this article Mishra VV, Agarwal R, Bandwal P, Choudhary S, Gandhi K. Management of Hematometra in Single Horn of Didelphic Uterus with Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome: A Case Report with Brief Review of Literature. J South Asian Feder Obst Gynae 2016;8(3):231-235.

Laparoscopic Strassman Metroplasty in a Postmenarcheal Adolescent Girl With Herlyn-Werner-Wunderlich Müllerian Anomaly Variant, Obstructed Noncommunicating Didelphic Uterus Without Gartner Duct Pseudocyst

Asymmetric obstructed uterus didelphys (Herlyn-Werner-Wunderlich syndrome), also known as obstructed hemivagina with ipsilateral renal agenesis syndrome, is a rare congenital müllerian duct anomaly. Herein we present a case report of incomplete Herlyn-Werner-Wunderlich syndrome, with absence of the hemivaginal septum, diagnosed in a 12-year-old girl. Treatment of the severe pain using an analgesic agent was ineffective. Therefore, laparoscopic metroplastic surgery via the modified Strassman procedure was performed. After surgery, the patient no longer reported dysmenorrhea.