Ipsilateral Renal Anomaly Syndrome Research Articles

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.

Abdominal Vaginoplasty for Management of High Vaginal Septum in Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome

Definitive management of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome involves surgical excision of the vaginal septum. A minimally invasive transvaginal approach is typically preferred. However, a transvaginal approach is not always feasible in a patient with a high vaginal septum. We describe two cases of OHVIRA with high vaginal septum that were successfully managed with abdominal vaginoplasty.

Acute urinary retention caused by OVHIRA syndrome

Obstructed hemivagina and ipsilateral renal anomaly (OVHIRA) syndrome is a congenital urogenital malformation that associates a bifid uterus with a longitudinal vaginal septum, resulting in a blind hemivagina and an ipsilateral renal agenesis. The clinical presentation is highly variable, delaying diagnosis and leading to important complications. An 18-year-old woman was diagnosed with OVHIRA syndrome following acute urinary retention. An agenesis in the left-sided renal system and an enormous pelvic mass compressing the adjacent structures were revealed in the intravenous urography. Nuclear magnetic resonance was the imaging modality that provided most diagnostic information, defining the pelvic mass as a giant left hematometrocolpos contained with a longitudinal vaginal septum. Resection of the septum was performed draining all reduced hematic content and both hemiuteri communicated with a single vagina, resulting in an asymptomatic patient. OVHIRA syndrome is a little known entity that can occur atypically, which makes the diagnosis difficult and delays the treatment. It is important to maintain a high degree of clinical suspicion to avoid irreversible complications.

Obstructed hemivagina and ipsilateral renal agenesis with intestinal malrotation

The combination of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis represents a rare congenital anomaly called Herlyn-Werner-Wunderlich syndrome (HWWS) or obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Several anomalies have recently been reported to be associated with this syndrome. The present patient with HHWS had multiple anomalies: intestinal non-rotation, anomalies of the large vessels of the abdomen including duplication of the inferior vena cava and a high-riding aortic bifurcation, and hypodontia. Hypodontia has never been reported in a patient with HWWS. The patient underwent a preventative Ladd's procedure and vaginal reconstruction. To prevent serious complications from concomitant anomalies such as intestinal malrotation, a patient with HWWS should be evaluated in detail for associated malformations.

Using the Tracheobronchial Stent to Achieve Vaginal Patency After Repeated Failed Attempts in a Patient With OHVIRA

OHVIRA syndrome (obstructed hemivagina and ipsilateral renal agenesis) also known as Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare Mullerian duct anomaly. Surgical management can be difficult, especially if the level of obstruction is high, and there is a chance of re-obstruction requiring further surgical procedures. Successful use of a tracheobronchial stent to maintain vaginal patency has been previously described in a patient with OHVIRA undergoing a primary procedure.1 However, this is the first reported case of successfully achieving vaginal patency with the tracheobronchial stent in a patient with OHIVRA who had previously re-obstructed twice. A 13 year-old G0 female presented to the E.R. with a chief complaint of increasing intermittent lower abdominal and low back pain for the past few months. Patient had menarche at age 11 with normal cyclic menses lasting 5-7 days. On exam, a moderately sized palpable mass was noted 4 cm below her umbilicus. Pelvic ultrasound revealed an 11x7x9cm mass and the CT scan identified an absent left kidney, marked left hematosalpinx, uterine didelphys and left hematometrocolpos. The patient underwent resection and marsupialization of the left vaginal septum with evacuation of the hematometrocolpos. However, 3 weeks postoperatively, she developed significant lower abdominal pain and low-grade fever with leukocytosis. Imaging at that time suggested a tubo-ovarian abscess as a cause of her symptoms. She then underwent a laparoscopy, which was converted to a laparotomy when it was discovered she had a 10cm left tubo-ovarian abscess and complete re-obstruction of her left vagina. A left salpingo-oophorectomy and reopening of the left vaginal cuff with placement of a 24 French foley catheter was performed. The catheter was kept in place for 6 weeks and then removed in the office. Two weeks after removal, patient developed lower abdominal pain and imaging showed a 2.5 cm hematometra. Patient underwent re-opening of her obstructed left vagina and an 18x40mm AERO tracheobronchial stent was placed. Two months later, the stent was removed. One-year after removal of the stent, she is having normal menses, no pain and no evidence of re-obstruction. Obstructed hemivagina and ipsilateral renal agenesis(OHVIRA) constitutes about 0.16-10% of all Mullerian anomalies.2 The arrest of the ipsilateral mesonephric duct at 8 weeks of gestation is the believed to be the cause of the obstructed hemivagina.2 The mean age of presentation is 14 years and patients typically present with vague symptoms such as dysmenorrhea, abdominal pain and occasionally a pelvic mass. Initial management with imaging such as ultrasonography can help aid in diagnosis of OHVIRA. A retrospective case series of 27 patients concluded that OHVIRA syndrome can be surgically management solely with single-stage vaginoplasty.3 However, complications such as vaginal stenosis can arise especially if the level of obstruction is high in the vagina. This case demonstrates that tracheobronchial stents can be successful in achieving vaginal patency in patients who have failed their primary vaginoplasty. 1.Cooper, Amber and Merritt, Diane F. “Novel use of a tracheobronchial stent in a patient with uterine didelphys and obstructed hemivagina.” Fertility and Sterility. 93(3). 2010 900-03. Print2.Cox, Deven, and Ching, Brian."Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos." Radiology Case. 6.3 (2012): 9-15. Print.3.Smith, Nicole and Laufer, Marc. “ Obstructed hemivagina and ipsilateral renal anomaly(OHVIRA) syndrome: management and follow up.” Fertility and Sterility. 87(4). 2007. 918-22. Print

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

Abstract To report a case of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA syndrome) with uterus didelphys that has been diagnosed successfully with ultrasound and managed with a single stage vaginoplasty.

Obstructed Hemivagina with Ipsilateral Renal Anomaly(OHVIRA) Syndrome - A Rare Congenital Anomaly

Obstructed Hemivagina with Ipsilateral Renal Anomaly(OHVIRA) Syndrome - A Rare Congenital Anomaly Objective: We present a rare case of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome with a single septate uterus. This syndrome is shrouded in controversies concerning its nomenclature and development. We attempt to highlight the different viewpoints surrounding this unusual condition, which can be quite distressing for the affected young girls and confusing the treating doctors.

Cervical cancer in a patient with uterus didelphys and obstructive hemivagina, ipsilateral renal anomaly (OHVIRA) syndrome

We report a rare case of cervical adenocarcinoma diagnosed and treated in a woman with uterine didelphys, obstructive hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. A 49-year-old multiparous woman applied to our institution with menorrhagia and intermenstrual bleeding since 6 months. On pelvic examination, a grossly normal cervix was noted. A 7-cm firm mass was palpated in the left pelvic region extending to the vaginal level. Also, a 4-cm fluctuant mass was palpated on the left vaginal wall. On pelvic ultrasound, a 40-mm cystic mass with internal echoes resembling an endometrioma was visualized adjacent to the left superior vaginal wall. Upper abdominal ultrasound revealed left unilateral renal dysplasia, which was further confirmed by magnetic resonance imaging (MRI) (Fig. 1). Laboratory, pap-smear and endocervical curettage (ECC) results were normal. Endometrial biopsy taken with a pipelle endometrial sampling cannula was reported as endometrioid adenocarcinoma. Laparotomy was performed and peritoneal washings were obtained for cytology. Upon exploration, uterine didelphys (UD) with normal adnexa on both sides were discovered (Fig. 2). After complete resection of the uterus with bilateral tubes and ovaries, a blind vaginal stump on the left side was visualized. A brown-colored fluid was drained from the left vaginal lumen. There was a small opening between the left and right vaginal lumens. The operation was completed with bilateral pelvic-paraaortic lymph node dissection (LND) and omentectomy. Final pathology was reported as UD with a 10 9 8 mm well-differentiated primary endometrioid adenocarcinoma of the right cervix. Endometrial tissues on both uteruses were free of any tumoral involvement. Retroperitoneal lymph nodes, omentum and peritoneal cytology were negative for any tumoral spread. The patient was informed about the pathology results. Further treatment options including surgical treatment or chemoradiation were discussed. The patient preferred surgical treatment. Radical parametrectomy and proximal vaginectomy were performed. Postoperative follow-up period was uneventful. On pathological examination, bilateral parametria and vaginal tissues were free of tumor. The patient was consulted to the radiation oncology department of an affiliated hospital. Regular follow-up without adjuvant treatment was recommended, and she was discharged from the hospital. Short-term (15 months) follow-up visits were normal without any treatment related complications or signs of recurrence. Obstructive hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome (Herlyn–Werner–Wunderlich syndrome) is a rare type of congenital Mullerian anomaly (CMA), which is frequently associated with UD [1, 2]. This syndrome results from lateral fusion defect of the Mullerian ducts with asymmetric vaginal obstruction. The classical urinary manifestation of OHVIRA syndrome is ipsilateral renal agenesis. However, as in our case, dysplastic or duplicated kidneys have also been reported [3]. Women with CMA are generally diagnosed in adolescence or reproductive period. Symptoms and clinical findings vary greatly, depending on the present anomaly. The woman presented here did not report any symptoms M. Kaba T. Gungor E. Baser (&) B. Ozdal Department of Gynecologic Oncology, Zekai Tahir Burak Women’s Health Education and Research Hospital, Talatpasa Bulvari, Samanpazari, Altindag, Ankara, Turkey e-mail: eralpbaser@gmail.com

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.

An unusual presentation of obstructed hemivagina and ipsilateral renal anomaly syndrome: A case report

Obstructed hemivagina and ipsilateral renal anomaly 증후군은 중복자궁에 동반하여 편측 질폐쇄 및 동측 신장의 기형이 함께 나타나는 증후군으로 대개의 경우 초경 이후 심해지는 월경통 및 하복부 종괴, 질벽의 팽윤이 전형적인 증상으로 나타난다. 조기 진단 및 치료를 통해 증상을 완화하고 생식기의 손상을 최소화하며 이후의 가임력을 유지할 수 있으므로 이는 예후에 중요한 영향을 미친다. 저자들은 초경 이후 특별한 증상 없이 지내오다가 질출혈로 검사하던 중 우연히 발견된, 비전형적 증상 발현을 보이는 일측 질폐쇄와 동측 신장무발생을 동반한 중복자궁 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Surgical Entry and Repair of Hematocolpos Associated with Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome, a Novel Technique

Surgical Entry and Repair of Hematocolpos Associated with Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome, a Novel Technique

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.

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.