Obstructed Hemivagina And Ipsilateral Renal Anomaly Syndrome Research Articles

Minimally Invasive Surgical Approach to Tubo-Ovarian Abscess in Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome

<h3>Study Objective</h3> We present a case of an acute presentation of tubo-ovarian abscess in the setting of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome in a non-sexually active adolescent, and demonstrate the surgical principles and techniques used int the management of the tubo-ovarian abscess and obstructed hemivagina/septum. <h3>Design</h3> Case report and surgical video. <h3>Setting</h3> Vaginoscopy and four port laparoscopy. <h3>Patients or Participants</h3> An adolescent female who has never been sexually active, presenting with a tubo-ovarian abscess on a background of OHVIRA syndrome. <h3>Interventions</h3> Vaginoscopy for identification of patent cervix and vaginal septum, laparoscopic drainage of tubo-ovarian abscess, adhesiolysis, and right salpingectomy, vaginoscopic septal resection with monopolar Collins knife. <h3>Measurements and Main Results</h3> A 15-year-old female, who had never been sexually active, presented with lower abdominal pain, fevers and malodorous vaginal discharge. Imaging of the pelvis demonstrated a right tubo-ovarian abscess of 5.8 × 4.7 × 5.8cm, uterine didelphys, obstructed right vaginal cavity, and right renal agenesis, suggesting OHVIRA syndrome. Medical management with intravenous antibiotics was unsuccessful, and the patient was managed surgically in a minimally invasive manner. Vaginoscopy identified a normal left cervix, and a small perforation was noted in the vaginal septum. Laparoscopy revealed a right sided tubo-ovarian complex adherent to the right pelvic side wall, bowel, and omentum. Blunt dissection and electrosurgical techniques were used to adhere to tissue planes. Drainage of multiple collections and a subsequent right salpingectomy was performed. A monopolar Collins knife was used with the vaginoscopy to resect the vaginal septum. The patient recovered well and was discharged shortly after her procedure. <h3>Conclusion</h3> Vaginoscopic septal resection is feasible in the setting of OHVIRA syndrome and in non-sexually active patients. Pre-operative imaging allows correct diagnosis of OHVIRA and assist in surgical planning. Care must be taken in tissue dissection to avoid injury.

UTERINE DIDELPHYS WITH OBSTRUCTED HEMIVGINA AND IPSILATERAL RENAL ANOMALY (OHVIRA) SYNDROME: A RARE CASE STUDY

Uternie didelphys with obstructed Hemivagina and ipsilateral Renal anomaly (OHVIRA) syndrome is a rare congenital anomaly. These patients may present with varied symptoms. Hence may often get misdiagnosed or may go undiagnosed till a later age as the patient may be asymptomatic. We present here such an unusual case of a 36yrs old married female who came to our OPD with diffuse pain abdomen associated with dysmenorrhoea and was later diagnosed on MRI with OHVIRA syndrome who underwent septal resection and was relieved from the symptoms.

Herlyn-Werner-Wunderlich Syndrome in a young female mimicking acute appendicitis

Abstract Objective Herlyn-Werner-Wunderlich (HWW) Syndrome, also known as Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome, is a very rare congenital urogenital tract anomaly. The incidence of the syndrome is less than 1/1,000,000 females. OHVIRA syndrome radiologically presents as the Wunderlich triad: uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Clinical presentation is progressive dysmenorrhea beginning with the menarche. If unilateral renal agenesis is diagnosed in a female newborn, gynecological exams should be performed to exclude corresponding genital anomalies. We present here a case of OHVIRA syndrome mimicking acute appendicitis. Methods A 12-year-old recently menarched female presented to our emergency department with lower abdominal pain and nausea without vomiting. The pain started two days earlier and it was the first such episode. The abdomen was tender in the right lower quadrant with no alarming signs. WBC count and CRP were normal. Abdominal ultrasound suggested acute appendicitis (without clear visualization of the appendix) and also revealed left renal agenesis. Performed abdominal MRI confirmed renal agenesis and uterine malformation with 2 uterine bodies, 2 cervical uteri and a closed hemi-vagina with hematocolpos. Results With the diagnosis of the rare OHVIRA syndrome and the visualization of a healthy appendix the cause of the pain was identified. The patient was hospitalized for one night to control pain and a treatment with oral contraception was introduced to suppress the hormonal cycle. Since pain was not well controlled the patient returned to the emergency department two days later. Indication for surgical treatment was made and the blood filled hemivagina incised, drained and marsupialised. Oral contraception (Dienogest) was prescribed. Further gynecological follow-up and corrective surgery are planned. Conclusion OHVIRA syndrome is very rare and may mislead physicians presuming other pathologies such as acute appendicitis. In young women presenting with abdominal pain, OHVIRA should be taken into account. Ultrasound and MRI are the imagery of choice, especially in fertile young women. Rapid and complete diagnosis is necessary to treat correctly and prevent complications. Moreover, detected genitourinary malformations should always lead to further investigations of concomitants pathologies.

Obstructed Hemivagina and Ipsilateral Renal Anomaly: Case Report

BACKGROUND: Hemivaginal obstruction syndrome and ipsilateral renal anomalies are known as Herlyn-Werner-Wunderlich syndrome (HWWs) or better known by the acronym obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). It occurs as a result of complete failure of the Müllerian ducts to fuse and accounts for about 5% of Müllerian duct anomalies. Initial manifestations usually appear as a result of secretions accumulating within the hemivaginal obstruction. Patients with HWWs can come with complaints of lower abdominal pain, severe dysmenorrhea, pelvic or vaginal mass, abnormal vaginal discharge, acute urinary retention, fever, or vomiting. Therefore, careful diagnosis and appropriate management of this condition are desirable. The objective of the study was to report on the management of the obstruction due to HWWs. Patient was suspected hematocolpos with uterus didelphys from ultrasound and suggested right renal agenesis from BNO IVP. Diagnosis then confirmed by laparoscopy diagnostic. CASE REPORT: A 12-year-old patient was admitted to the gynecology ward of Dr. M. Djamil Central General Hospital from polyclinic with abdominal pain since 4 months before being admitted to the hospital. The pain gets worse every day before menstruation and decreases when given painkillers. CONCLUSION: OHVIRA syndrome is a triad characterized by hemivaginal obstruction and agenesis of the ipsilateral kidney (OHVIRA) and uterus in the delphys. Clinical symptoms in general are cyclic dysmenorrhea, palpable mass due to accumulation of menstrual flow, and severe pelvic pain which can later develop into a persistent form as a result of prolonged retention of menstrual secretions and obstructed hemivagina. Management options are surgical resection of the obstructed vaginal septum and drainage.

Possibilities of Early Ultrasound Diagnostics of the Herlin — Werner — Wunderlich syndrome (Case Report)

Herlin — Werner — Wunderlich syndrome or OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly) is a rare type of uterine and vaginal doubling defect (0,1 – 10,0 % of all uterine abnormalities) which is characterized by a triad of symptoms: uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal dysgenesis. The manifestation of the disease usually present after menarche, is accompanied by severe pain syndrome, leads to redoubted complications and organ-carrying operations with decreasing the fertility. At an earlier age, it can manifest itself as an infection of the genitourinary system. The diagnosis is based on clinical and anamnestic data using instrumental methods, the most accessible and safe of which is ultrasound. This article presents three clinical cases of patients 5 – 6 years old with a description of the ultrasound picture of the OHVIRA syndrome.

Experiences Replay Accompanies Efficient Non-Local Learning: MEG Neurocomputational Dynamics

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare diagnosis; patients classically present with dysmenorrhea and increasing pelvic pain. Unusual manifestations of OHVIRA syndrome might occur as a result of patient anatomy and the rupture or relief of the obstructed hemivagina.We present a 15-year-old patient with OHVIRA syndrome who developed a spontaneous cervicovaginal fistula and subsequently underwent hemihysterectomy.

Resection of Vaginal Septum in Obstructed Hemivagina and Ipsilateral Renal Anomaly: Utility of Three Dimensional Intraoperative Ultrasound

Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital condition of the female urogenital tract, presenting intraoperative challenges. We demonstrate the utility of three dimensional intraoperative ultrasound to better delineate anatomy and aid in optimal resection of the vaginal septum. Case: A 12-year-old female was referred to pediatric and adolescent gynecology for irregular periods and evaluation of her gynecologic organs. Imaging studies confirmed OHVIRA syndrome. She underwent uncomplicated vaginal septum resection, guided by three dimensional intraoperative ultrasound. Summary and Conclusion: Intraoperative ultrasound can better delineate challenging anatomy. We show that three dimensional intraoperative ultrasound offers significant advantages over traditional two dimensional ultrasound and is a supplement to MRI, which may be helpful in complex anatomical cases like OHVIRA syndrome.

A case of obstructed hemivagina and ipsilateral renal anomaly syndrome complicated with pyometra: tips and tricks for laparoscopic hemihysterectomy

A case of obstructed hemivagina and ipsilateral renal anomaly syndrome complicated with pyometra: tips and tricks for laparoscopic hemihysterectomy

Virginity-sparing management of blind hemivagina in obstructed hemivagina and ipsilateral renal anomaly syndrome

To demonstrate the hymen-sparing management of a blind hemivagina in obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with the use of transrectal ultrasound (TRUS)-guided vaginoscopic septoplasty supported by pre- and postoperative diagnostics with the use of a novel ultrasound technique: 3-dimensional saline-solution infusion contrast sonovaginocervicography (3D-SVC) with virtual speculoscopy. Video presentation of surgical and ultrasound techniques. University hospital and two private centers. We are demonstrating four cases with blind hemivagina as a component of OHVIRA syndrome and varying level and features of obstruction including: 1) hemihydrocolpos; 2) hemihematocolpos; 3) "old blood" deposits in small hemivagina; and 4) narrow hymenal opening. The patients were diagnosed preoperatively by means of 3D-SVC with the use of TRUS. Surgery was planned according to available data from ultrasound and 3D-SVC, and the place of incision of the vaginal septum and blinded hemivagina with cervix were performed with the use of TRUS guidance. Wide septal incision was performed with the use of a monopolar or bipolar resectoscope with needle Collin electrode, and after incision the occult second of double cervix or part of septate cervix was visualized, and the septum was excised with the use of a loop electrode. In narrow hymenal opening, a small diagnostic sheath was used for wide septal incision. Anatomic results in the vagina were assessed with the use of 3D-SVC 2months after surgery. Agreement between imaging from preoperative diagnostics with the use of 3D-SVC and intraoperative findings, and anatomic (hymenal integrity, obstruction, status of vagina and cervix) and clinical outcomes (pain). In these four cases, 3D-SVC accurately recognized the morphology of blind hemivagina, oblique vaginal septa, and double or septate cervix. Successful minimally invasive wide septoplasty with preservation of hymen were performed with the use of hysteroscope and TRUS guidance. Concomitant laparoscopy was performed if endometriosis and hematosalpinx were present. No peri- or late postoperative complications occurred. Patients were discharged within 3hours or within 12hours in case of laparoscopy. Anatomic results were optimal (lack of septum) or suboptimal (wide opening) after septum resection and incision, respectively, without recurrence of obstruction according to 3D-SVC. Pain was not noticed 2months after the primary surgery. 3D-SVC is a useful and accurate technique in diagnosis, surgery planning, and postoperative assessment in women with blind hemivagina and intact hymen. TRUS-guided vaginoscopic septoplasty is a reasonable alternative to traditional vaginal surgery and allows hymen preservation.

Clinical Implications of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in the Prepubertal Age Group.

PurposeObstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare syndrome characterized by Müllerian duct and renal anomalies. It is usually regarded as a disease of adolescence; however, due to a number of possible problems, the management of patients before puberty should not be overlooked. We assessed the clinical course of prepubertal patients to propose appropriate management.Materials and MethodsWe retrospectively assessed 43 prepubertal OHVIRA syndrome patients who were diagnosed and followed up at our institution from July 2004 to June 2015. We reviewed medical records, focusing on presentation, radiologic findings, surgical management, and the overall clinical course.ResultsMedian age at diagnosis was 1.3 months and median follow-up period was 25.5 months. The most common accompanying ipsilateral urologic anomalies were ectopic ureter and ureterocele, while the most common contralateral anomaly was vesicoureteral reflux. During the follow-up period, six patients (14.0%) required surgery at a median age of 31.2 months due to recurrent urinary tract infection, uncontrolled vaginal distention compressing adjacent organs, urinary incontinence, or intractable abdominal pain.ConclusionsWhile OHVIRA syndrome is known as a postpubertal disease, about 13% of prepubertal patients in our study required surgery. When ectopic ureter insertion into the vagina is present, further treatment may be needed to address the complications caused by continuous urine production. Patients should be monitored for complications arising from either obstructed hemivagina or renal anomalies with regular follow-up, especially before the age of five years.

MRI Findings of Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA syndrome) with a Blind Megaureter: Case Report

The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital anomaly of the Mullerian (paramesonephric) and Wolffian (mesonephric) ducts. It was initially reported in 1922 by Purslow and also referred to as the HerlynWerner-Wunderlich syndrome. It typically occurs concurrently with uterine didelphys and ipsilateral renal agenesis (1-5). Ipsilateral renal agenesis can be accompanied with other urologic anomalies simultaneously. A blind ectopic ureter combined with ipsilateral agenesis and Mullerian duct anomalies is extremely rare (6, 7). To our knowledge, a blind ectopic ureter associated with OHVIRA syndrome has not been reported in English literature. The diagnosis of ureteral anomalies can be challenging due to their rare incidence and cystic features that can be mistaken for an adnexal lesion in female patients. Regarding this matter, MRI is considerably helpful not only for diagnosing Mullerian duct anomalies, but also for detecting and characterizing associated non-uterine anomalies (1-5). In this case report, we present an unusual case of a 13-year-old girl with OHVIRA syndrome and a blind ectopic megaureter, which was This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Received: July 19, 2015 Revised: August 19, 2015 Accepted: August 27, 2015

Uterine Didelphys Associated With Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome: a Case Report

A 30 year old nulligravida female reported to the fertility centre of AHD with the complaints of primary infertility for three and half years and spasmodic dysmenorrhoea. There is also history of progressively increasing right lower abdominal pain as well as discomfort which was cyclically associated with the onset of menses. Transabdominal sonography showed-‘Endometrial splitting into two at the fundus–suggesting bicornuate uterus. Echogenic soft tissue in the cervical canal due to blood clots. Non visualized right kidney. Mildly enlarged left kidney’. HSG done outside AHD suggestive of unicornuate uterus with single fallopian tube. IVU showed non visualized right kidney. Normally excreting left kidney. TVS showed normal sized septated nulliparus uterus with homogeneous myometrium and thick endometrium with proliferative phase echo. Mildly enlarged right ovary with mildly distended right tube. Mild collection adjacent to the vagina. Then the patient came to the gynaecology dept of AHD from where she was sent to our Radiology department to undergo MRI of pelvis. The MRI showed uterine didelphys. Obstructed hemivagina (right) with hematocolpos extended upto pelvic brim along right and posterior aspect of uterus through anomalous dilated remnant of right lower ureter with ipsilateral renal agenesis. Patient was diagnosed as OHVIRA syndrome radiologically. DOI: http://dx.doi.org/10.3329/pulse.v6i1-2.20358 Pulse Vol.6 January-December 2013 p.66-69

Uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA syndrome): A case report

Abstract Uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital urogenital anomaly. We report a case of a 14-year-old female who presented with acute lower abdominal pain and tender pelvic mass. The initial diagnosis of twisted left ovarian cyst was confirmed by abdominal ultrasonography (US), which showed an associated absent left kidney. The findings at laparoscopy were that of uterus didelphys with obstructed left hemivagina. Parents were concerned about hymnal integrity and refused hemivaginal septal resection.

Uterine Didelphys With Obstructed Hemivagina and Ipsilateral Renal Agenesis Presenting With Hydronephrosis

Mullerian anomalies are present in 7% of the female population with a multifactorial pathogenesis thought to be associated with a midline fusion defect. OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) Syndrome comprises about 2-3% of Mullerian abnormalities. Case reports and small series have reported on this syndrome, which most commonly presents in young females with abdominal pain and a pelvic mass.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus

To describe an unusual case of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus. Case report. Major academic medical center. A 12-year-old girl with OHVIRA syndrome. None. None. A 12-year-old girl presented with presumed OHVIRA syndrome and had surgical correction of the obstructed hemivagina. Two years later she presented with increasing pelvic pain and underwent laparoscopy for presumed endometriosis. A single uterus with a broad flat fundus was found as well as endometriosis. OHVIRA syndrome is typically associated with a didelphys uterus with two cervices and two vaginas, one of which is obstructed. The obstruction usually occurs on the same side as the renal anomaly. We report a rare congenital anomaly of the female reproductive tract: OHVIRA syndrome with a single uterus. From a fertility standpoint, cases of a single uterus with two cervices are managed differently than two uteri with two cervices. Healthcare providers managing complex reproductive tract anomalies should be aware of this potential variant.

Percutaneous obstructed hemivagina access (POHVA) to the difficult obstructed hemivagina in the OHVIRA syndrome: a novel minimally invasive technique

OBJECTIVE: To illustrate a novel minimally invasive technique for the identification and safe entry to the hematocolpos associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. DESIGN: Surgical technique. MATERIALS AND METHODS: Retrospective review from 2005-2010 of OHVIRA syndrome cases in which POHVA was employed. Preoperative evaluation consisted of a thorough history, physical examination and a pelvic MRI. At exam under anesthesia, vaginoscopy is performed to identify the hematocolpos and unobstructed cervix. To avoid blind dissection, a 10 Fr Council tip Foley catheter and a percutaneus vascular catheter introducer set, containing an 18 gauge × 7 cm needle, a 16 Fr introducer with peel-away sheath and a 50 cm guide wire, are used in the POHVA technique. The hematocolpos is needled until old menses is aspirated, otherwise placement is confirmed under fluoroscopic guidance. Through the needle, the wire is then inserted and followed with the introducer and peel-away sheath. The introducer is then removed and the Foley catheter is inserted over the wire. After Foley inflation, the wire and sheath are removed. The hematocolpos is completely drained, the septum is widely resected and sutured to prevent reobstruction. RESULTS: From 2005-2010, ten patients with cyclical pain were found to have OHVIRA syndrome and 4 had difficult access to the obstructed hemivagina. The POHVA technique was successfully performed in all 4 cases, with rapid identification and access to the difficult obstructed hemivagina without need of concomitant laparoscopy. There were no intraoperative complications. One patient required repeat POHVA and vaginoplasty secondary to restenosis. CONCLUSION: Single-stage vaginoplasty in girls with OHVIRA syndrome can be challenging when due to a small vaginal caliber, a less accessible and high lateral vaginal bulge, and chronic inflammation. The POHVA technique uses a widely available vascular catheter introducer set, permitting a controlled entry with good early results.

Complete septate uterus, obstructed hemivagina, and ipsilateral renal anomaly: pregnancy course complicated by a rare urogenital anomaly

Abnormalities of the female reproductive tract are associated with a multitude of intrapartum complications. In the case that follows, we report the complicated pregnancy course of a woman diagnosed postpartum with the uncommon syndrome of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) associated with a complete septate uterus. A 19-year-old nulliparous woman with a history of a right nephrectomy secondary to multicystic dysplastic kidney disease presented with premature rupture of membranes at 30 weeks' gestation. After failed induction of labor at 34 weeks' gestation, a uterine anomaly was discovered at the time of cesarean section. Magnetic resonance imaging performed postpartum revealed a complete septate uterus, longitudinal vaginal septum, and obstructed right hemivagina. The OHVIRA syndrome is classically associated with uterus didelphys, not complete septate uterus as in this woman. This case illustrates the potential for intrapartum complications in women with abnormalities of the urogenital tract.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single septate uterus: first case report

ObjectiveTo describe an unusual case of an Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome with a single septate uterus.DesignCase report.Materials and methodsCase review and review of literature.ResultsConclusionsCases of a single septate uterus with two cervices are rare. The OHVIRA Syndrome has been reported and described as an obstructed hemivagina and ipsilateral renal anomaly. This Syndrome is associated with a double or bicornuate uterus and has two cervices and two vaginas, one of which is obstructed. The obstruction usually occurs on the same side as the renal anomaly. We report a not previously described congenital anomaly of the female reproductive tract which is a variant of the OHVIRA Syndrome: the OHVIRA Syndrome with a single septate uterus. From a fertility standpoint, cases of a single septate uterus with two cervices are managed differently than two uteri with two cervices. This is the first case report of a single uterus with two uterine cavities, two cervices and an obstructed hemivagina with ipsilateral renal agenesis. Healthcare providers managing complex reproductive tract anomalies should be aware of this potential variant. ObjectiveTo describe an unusual case of an Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome with a single septate uterus. To describe an unusual case of an Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome with a single septate uterus. DesignCase report. Case report. Materials and methodsCase review and review of literature. Case review and review of literature. Results ConclusionsCases of a single septate uterus with two cervices are rare. The OHVIRA Syndrome has been reported and described as an obstructed hemivagina and ipsilateral renal anomaly. This Syndrome is associated with a double or bicornuate uterus and has two cervices and two vaginas, one of which is obstructed. The obstruction usually occurs on the same side as the renal anomaly. We report a not previously described congenital anomaly of the female reproductive tract which is a variant of the OHVIRA Syndrome: the OHVIRA Syndrome with a single septate uterus. From a fertility standpoint, cases of a single septate uterus with two cervices are managed differently than two uteri with two cervices. This is the first case report of a single uterus with two uterine cavities, two cervices and an obstructed hemivagina with ipsilateral renal agenesis. Healthcare providers managing complex reproductive tract anomalies should be aware of this potential variant. Cases of a single septate uterus with two cervices are rare. The OHVIRA Syndrome has been reported and described as an obstructed hemivagina and ipsilateral renal anomaly. This Syndrome is associated with a double or bicornuate uterus and has two cervices and two vaginas, one of which is obstructed. The obstruction usually occurs on the same side as the renal anomaly. We report a not previously described congenital anomaly of the female reproductive tract which is a variant of the OHVIRA Syndrome: the OHVIRA Syndrome with a single septate uterus. From a fertility standpoint, cases of a single septate uterus with two cervices are managed differently than two uteri with two cervices. This is the first case report of a single uterus with two uterine cavities, two cervices and an obstructed hemivagina with ipsilateral renal agenesis. Healthcare providers managing complex reproductive tract anomalies should be aware of this potential variant.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up

To review the experience at two large referral centers with the syndrome of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), and to review the risks, benefits, and complications of surgical management options. Retrospective case series. Children's Hospital Boston and Brigham and Women's Hospital, Boston, Massachusetts. Twenty-seven cases of obstructed hemivagina over a 12-year period. For each patient who presented symptoms, diagnostic studies, anatomic findings, surgical management, outcomes, and complications were reviewed. The mean age of diagnosis was 14 years. Twenty-three patients had ipsilateral renal anomalies, including 20 patients who had renal agenesis. Two had dysplastic ipsilateral kidneys requiring nephrectomy in infancy. Twenty-six patients underwent vaginal reconstruction, and eight of those additionally underwent laparoscopy for clarification of diagnosis. Six required a two-stage vaginoplasty because of incomplete previous resection (n = 1), infection or anatomic distortion (n = 4), or restenosis (n = 2). Vaginal septum adenosis was seen in eight patients. Most patients with OHVIRA syndrome can be treated solely with single-stage vaginoplasty. Routine laparoscopy is not essential to management. Vaginal stenosis is a postoperative possibility, and may be associated with vaginal adenosis.