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      Uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with ipsilateral renal agenesis

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          Abstract

          Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn Werner-Wunderlich Syndrome) is a rare congenital anomaly. It mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos. A patient with dysmenorrhea from a double uterus and an obstructed hemivagina is a diagnostic dilemma because the menses are regular. We report a case of a 14-year-old girl with this condition who was diagnosed as uterus didelphys with unilateral hematocolpos and hydrosalpinx with ipsilateral renal agenesis on the basis of sonography and confirmed by laparoscopic examination.

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          Most cited references 11

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          Müllerian anomalies: a proposed classification. (An analysis of 144 cases).

          Hospital and clinic charts for 100 patients with diagnosed Müllerian anomalies were reviewed. Reproductive history, type of therapy, and treatment results for those patients and for another 44 with a history of exposure to diethylstilbestrol and abnormal hysterosalpingographic findings were recorded and evaluated. The anomaly most frequently associated with reproductive failure was the septate uterus. A new classification, based on degree of failure of normal development, was used in separating the anomalies into groups with similar clinical manifestations, treatment, and prognosis for fetal salvage.
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            Uterine anomalies. How common are they, and what is their distribution among subtypes?

             Gerard Nahum (1998)
            To establish the prevalence and distribution of uterine anomalies in the general population and to enumerate them among septate, bicornuate, didelphic, unicornuate, hypoplastic/aplastic and solid forms. A Medline search and standard reference tracing were employed to locate 47 studies from 14 countries regarding the prevalence and distribution of uterine anomalies. Twenty-two studies involving 573,138 women undergoing universal screening for uterine malformations were analyzed to establish the prevalence of uterine anomalies among fertile women. A separate analysis of uterine anomalies among 6,512 infertile women was also performed. Nineteen studies incorporating 1,092 fertile and 456 infertile women with unselected uterine anomalies were reviewed to establish the distribution of müllerian malformations among major subtypes. Seventeen studies incorporating 161 women with unicornuate uteri were analyzed to establish the distribution of unicornuate müllerian defects among various subtypes, including those with and without cavitary contralateral uterine horns. Uterine anomalies were identified in 1 in 594 fertile women (0.17%) and in 1 in 29 infertile women (3.5%). This difference was statistically significant (chi 2 = 3,424, df = 1, P < .00001). The prevalence of uterine anomalies in the general population was 1 in 201 women (0.50%). Their distribution was: 7% arcuate, 34% septate, 39% bicornuate, 11% didelphic, 5% unicornuate, and 4% hypoplastic/aplastic/solid and other forms. Congenital uterine malformations are more common than generally recognized. Knowledge concerning their prevalence and varieties is important in recognizing and managing the obstetric and gynecologic complications that may result.
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              Obstructive Müllerian anomalies: case report, diagnosis, and management.

               J Burgis (2001)
              Many factors affect the development of the female reproductive tract. Obstructive anomalies prevent normal menstruation, allow for collection of blood in the uterus and the vagina, and may increase the incidence of retrograde menstruation. A high index of suspicion is necessary to diagnose these disorders, and an adequate workup is essential. This report will present a case of obstructive longitudinal vaginal septum. The workup and operative findings will be described, followed by a classification and discussion of other obstructive Müllerian anomalies. The incidence, workup, and management will be reviewed.
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                Author and article information

                Journal
                J Hum Reprod Sci
                JHRS
                Journal of Human Reproductive Sciences
                Medknow Publications (India )
                0974-1208
                1998-4766
                Jul-Dec 2009
                : 2
                : 2
                : 87-89
                Affiliations
                Department of Radiodiagnosis, Sardar Patel Medical College, Bikaner, Rajasthan - 334 001, India
                Author notes
                Address for correspondence: Dr. Gaurav Jindal, Room No. 8, PG Boys Hostel, Sardar Patel Medical College, Bikaner, Rajasthan - 334 001, India. E-mail: gauravjindal11@ 123456hotmail.com
                Article
                JHRS-02-87
                10.4103/0974-1208.57230
                2800935
                19881156
                © Journal of Human Reproductive Sciences

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Case Report

                Human biology

                renal agenesis, unilateral hematometrocolpos, uterus didelphys

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