Hydronephrosis: A Rare Presentation of Uterine Didelphys with Obstructed Hemivagina and Ipsilateral Renal Anomaly

BackgroundObstructed hemivagina and ipsilateral renal anomaly syndrome is a Müllerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal anomalies.CaseA 12-year-old girl with a history of right renal agenesis presented to the emergency department with abdominal pain, dysuria, and urinary retention. Imaging identified a uterine didelphys with a large obstructed right hemivagina compressing the left ureter, causing hydronephrosis. She underwent vaginal septum resection for curative treatment.Summary and ConclusionIn female patients who present with abdominal pain and a history of renal abnormalities, obstructed hemivagina and ipsilateral renal anomaly syndrome must be considered in the differential diagnosis. This consideration is important in preventing complications such as hydronephrosis seen in this patient.     

Obstructed Hemivagina with Ipsilateral Renal Anomaly

The association of obstructed hemivagina with ipisilateral renal anomaly (OHVIRA) is a well-described entity. While there has been an increased familiarity with this disorder, the exact incidence of OHVIRA is unknown. Our aim was to review the available literature on this topic, look at common presentations, and uncommon presentations. This condition is a well-described entity but requires careful evaluation, because unique presentations do occur. Diagnostic challenges include time of presentation and symptoms associated with presentation. Surgical challenges include microperforation of the hemivaginal septum, pelvic inflammatory disease, thick septum, or high septum. Each of these must be managed carefully. Although a solitary kidney is frequently thought to be associated with OHVIRA, dysplastic kidneys, pelvic kidneys, or ectopic ureters can occur. Prompt and accurate diagnosis is essential for relief of symptoms and prevention of complications. To minimize problems associated with delayed diagnosis, magnetic resonance imaging evaluation is recommended along with referral to a center with expertise in these conditions.     

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

Background  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. Case  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. Conclusion  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.     

Herlyn-Werner-Wunderlich syndrome: 3D ultrasonographic diagnosis in premenarche

IntroductionUterine malformations are a heterogeneous group of congenital anomalies resulting from abnormal development of Mullerian ducts. Herlyn-Werner-Wunderlich syndrome is a rare condition that includes the triad of uterus didelfus, blind hemivagina and ipsilateral renal agenesis. We report a case with premenarche diagnosis and treatment and make a review of the illness.Case reportA premenarche 11 year old female was referred for pediatric nephrology and neurodevelopment consultation due to prenatal diagnosis of left renal agenesis. A pelvic ultrasound was performed and an anomalous uterus and a cystic pelvic mass was incidentally discovered. A 3D trans-rectal ultrasound identified a bicolis didelfus uterus and mucocolpos. A unidigital vaginal exam revealed a single cervix with soft bulging of the left apical vaginal wall. Using monopolar knife, an incision was done allowing drainage of the cavity. A part of the apical vaginal septum was removed and a normal cervix was seen.CommentsA high degree of clinical suspicion is important for the diagnoses. 3D ultrasound or MRI are the imaging diagnostic exams of choice, helped by a careful bimanual pelvic examination. An early diagnosis is important to prevent chronic pelvic pain and perhaps severe endometriosis.     

Unique Diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) at the Time of First-Trimester Ultrasound Imaging

BackgroundObstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is usually diagnose during adolescence due to pain-related symptoms; however, this syndrome can go unrecognized for several years, and unique presentations may occur later in life.CaseWe describe a 24-year-old-woman diagnosed with previously unknown OHVIRA syndrome and a 7-week intrauterine pregnancy in the obstructed side by the unique ultrasound image obtained during routine first-trimester ultrasonography. The patient was managed with single-stage vaginoplasty, and the rest of the pregnancy were uneventful.Summary and ConclusionThis report is unique in terms of showing that the pregnancy could develop in the uterine cavity on the side of the obstruction despite the blind hemivagina.     

Resection of Longitudinal Vaginal Septum Using a Surgical Stapler

BackgroundA longitudinal vaginal septum is a rare congenital anomaly that can cause dyspareunia, difficulty with tampon insertion, persistent vaginal bleeding, and dysmenorrhea. Various surgical techniques have been described.CaseWe present the case of a 14-year-old girl with obesity and diabetes mellitus with uterine didelphys and double vagina with a longitudinal vaginal septum. The patient presented with dysmenorrhea. Resection of the longitudinal vaginal septum using a GIA and EndoGIA (Medtronic, Inc, Doral Fl) stapler device was performed.Summary and ConclusionWe introduce a safe and effective technique for resecting a longitudinal vaginal septum using stapler technology. This technique eliminates the potential risk of thermal injury to nearby structures from currently described methods.     

The Obstructed Hemivagina,Ipsilateral Renal Anomaly,and Uterine Didelphys Triad and the Subsequent Manifestation of Cervical Aplasia

Study ObjectiveTo compare a case series of the obstructed hemivagina, ipsilateral renal anomaly and uterine didelphys triad with the literature, with a focus on a subset of patients with cervical aplasia.Design, Setting, and ParticipantsA retrospective case series was conducted of all patients with the triad managed between 2005-2013 at a tertiary center for adolescent gynecology.ResultsSimilarities in this cohort, compared to the literature, included heterogeneity of presentation, presence of endometriosis, and asymmetry of affected side. Notable differences included 1 patient with a 16p11.2 microdeletion and 2 patients with subsequent unilateral cervical aplasia. All patients underwent magnetic resonance imaging for diagnosis. Vaginal septum division was performed in 8 cases and excision in 1 case. Both cases with cervical aplasia ultimately underwent hemi-hysterectomy, and highlight the implications of this rare variant in regards to its existence, limitations of magnetic resonance imaging in this context, and suggestions for improvement in diagnosis and management.ConclusionThe complexity of these cases, especially the evolving manifestation of cervical aplasia postoperatively, illustrates the need to recognize limitations in imaging and divergence in definitive management.     

An unusual diagnosis of a bicornuate unicollis uterus

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.     

Obstructive Reproductive Tract Anomalies

BackgroundApproximately 7% of girls will have an anatomic abnormality in their reproductive tract, diagnosed before or after puberty.ObjectiveIt is important for providers to be aware of the obstructive reproductive tract conditions, the way in which various conditions present, and the way in which such conditions should be managed.DesignSystematic review of the literature using the GRADE evidence system.ResultsThere is limited data in most areas of obstructive reproductive tract anomalies; however, some retrospective and prospective series with small numbers are still useful to guide clinical practice.ConclusionsRecommendations are based on limited or inconsistent scientific evidence. Recommendations are based primarily on consensus and expert opinion.     

An Atypical Presentation of Ectopic Pregnancy With Unicornuate Uterus and Undescended Fallopian Tube

Background

Müllerian duct abnormalities are common and may be related to pregnancy complications. Few cases of ectopic pregnancy within an undescended Fallopian tube have been reported, and they present a diagnostic challenge.

Urinary Incontinence in Puberty: A Rare Clinical Presentation of the Herlyn-Werner-Wunderlich Syndrome

BackgroundWe report on an unusual presentation of Herlyn-Werner-Wunderlich syndrome in two 11-year-old girls within a year of menarche. The setting was a training and research hospital.CaseWe present two patients in the pubertal period with cyclic abdominal pain and urinary incontinence who received hysteroscopic septal resection. Menstrual flow was resumed and the complaints of incontinence were eliminated after the hysteroscopic resection of the vaginal septum.Summary and ConclusionOverflow incontinence was completely resolved after septum resection in two patients. The risk of stricture is high in Herlyn-Werner-Wunderlich syndrome if the septum is partially excised to open the obstruction, whereas the risk of stricture is low if a complete or wide excision is performed. In the presence of abdominal pain and urinary incontinence in puberty, Herlyn-Werner-Wunderlich syndrome must be considered in the differential diagnosis, and a detailed evaluation of the urinary system and pelvic anatomy must be performed.     

Complexities of Müllerian Anatomy in 46XX Cloacal Exstrophy Patients

Study ObjectiveTo characterize Müllerian anatomy in 46,XX cloacal exstrophy patients.DesignRetrospective review of prospectively maintained, institutionally approved exstrophy-epispadias-cloacal exstrophy database.SettingTertiary care, high-volume exstrophy center (Division of Pediatric Urology, The Johns Hopkins Hospital, Baltimore, Maryland).ParticipantsWe included 31 patients who were genetically female with cloacal exstrophy for whom records included detailed evaluation of Müllerian anatomy.InterventionsNone.Main Outcome MeasuresMüllerian structures, method of evaluation, management, and sexual activity.ResultsOf our patients, 12.9% (3/31) had no identified abnormalities. Vaginal anatomy was described for 30 patients; 3/30 had vaginal agenesis, 14/30 had a single vagina, and 13/30 had vaginal duplication. Of 14 patients with 1 vagina, 5 had atresia/hypoplasia, and 1 had a lateral displacement. One patient with 2 vaginas also had distal atresia. Of the cervices evaluated, 9/14 were duplicated (2/9 with a solitary vagina), and 19/27 of the uteri were duplicated (6/22 with 1 vagina, 1/22 with no vagina). Five patients required imaging to fully characterize their anatomy, and 7 patients had studies that failed to identify Müllerian structures seen in the operating room or on physical examination. Common reconstructive surgeries included vaginoplasties, incisions of vaginal septa, colporrhaphies, and hysterectomies. Sexual activity was confirmed for 3 patients, 2 of whom had conceived.ConclusionMost female cloacal exstrophy patients exhibit abnormalities of the Müllerian system. Axial imaging and ultrasound are helpful diagnostic adjuncts but do not replace careful physical examination and assessment in the operating room. Further studies of sexual activity and fertility are warranted.     

Case Report of the Successful Use of Mirena Levonorgestrel Intrauterine System in a Patient with Didelphys Uterus and Obstructed Hemivagina

Background

The Mirena levonorgestrel intrauterine system (IUS; Bayer HealthCare Pharmaceuticals) is frequently used for menstrual suppression in adolescents with special needs. However, the presence of a uterine anomaly is generally considered a contraindication to IUS insertion, thereby excluding a potentially highly effective option for this patient population.

Patient Quality of Life and Symptoms after Surgical Treatment for Endometriosis

Study Objective

To assess the impact of surgical treatment of endometriosis on quality of life and pain over a 3-year period of postoperative follow-up.

The association between Müllerian anomalies and perinatal outcome

Introduction: Deviation in the development of the female reproductive organs from the normal anatomy has been shown to have an impact on obstetrical outcomes and neonatal morbidity.

Material and methods: In this retrospective population-based cohort study, short-term neonatal morbidity and mortality were compared in pregnancies of women with and without uterine anomalies. The analysis included deliveries that occurred between the years 1991 and 2013 in a tertiary medical center. Statistical analysis included multiple logistic regression models.

Results: During the study period, 256,299 deliveries met the inclusion criteria; 0.49% (n?=?1251) of which occurred in women diagnosed with Müllerian anomalies. In the regression model, Müllerian anomalies were noted as an independent risk factor for placental abruption (adjusted odds ratio, 1.9; 95% confidence interval, 1.3–2.8; p?=?.001), intrauterine growth restriction (adjusted odds ratio, 1.9; 95% confidence interval, 1.5–2.4; p?p?p?p?=?.061).

Conclusion: Women with Müllerian anomalies are at an increased risk for multiple adverse pregnancy outcomes, including preterm delivery and intrauterine growth restriction. Perinatal mortality, however, is not increased when controlled for gestational age and weight suggesting that mortality in these pregnancies is mediated by preterm delivery and small for gestational age.     

A case of empty follicle syndrome who conceived after aspiration of an endometrial cyst

Empty follicle syndrome (EFS) has been defined as a condition where no oocytes can be retrieved for in vitro fertilization (IVF) even though ultrasound findings and estradiol (E₂) levels suggest the presence of potential follicles. The EFS is a rare condition with an incidence of 0.5–7 % of women undergoing IVF treatments. Although there are many hypotheses as to the cause of EFS, including advanced ovarian age, drug-related problems, and dysfunctional folliculogenesis, its cause remains unknown. A 37-year-old woman with endometriosis and a 5-year history of primary infertility underwent IVF treatment for 4 cycles. No oocytes were retrieved in 2 cycles and no fertilized eggs were obtained in the other 2 cycles. We assumed that endometriosis adversely affected folliculogenesis and fertilization. Aspiration of an endometrial cyst in the right ovary and subsequent administration of oral contraceptives resulted in successful folliculogenesis and fertilization. Thereafter, she conceived and delivered a 2,662 g female infant at 38 weeks of gestation. Here, we report a case of EFS who conceived in the 5th IVF cycle after aspiration of an endometrial cyst. We assumed that endometriosis might have been involved in the dysfunction of folliculogenesis and EFS.