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Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Uterus didelphys is complete duplication of the uterine horns leading to two separate uterine cavities and cervices. A unicornuate uterus is where there is just one uterine horn and fallopian tube and there may be a rudimentary horn on the contralateral side. Renal anomalies are most common with this subtype. Uterine agenesis is when the uterus has not formed at all and the upper two-thirds of the vagina will also be absent; however, the ovaries and fallopian tubes may or may not be present.
Pediatric Hematocolpos
Published in Botros Rizk, A. Mostafa Borahay, Abdel Maguid Ramzy, Clinical Diagnosis and Management of Gynecologic Emergencies, 2020
Omar M. Abuzeid, Mostafa I. Abuzeid
Uterus didelphys occurs when there is complete failure of fusion of the two Müllerian ducts. As a result, there is duplication of the corpus and cervix uteri with two endometrial cavities and two cervices fused in the lower uterine segment. Usually there is a longitudinal vaginal septum, which is situated between the two cervices. Longitudinal vaginal septum occurs when there is incomplete fusion of the Müllerian ducts or incomplete regression of the medial aspect of the Müllerian ducts in the lower genital tract. Obstructed hemivagina will occur if a longitudinal vaginal septum fuses with the vaginal wall on one side (Figure 17.4).
Habitual Abortion
Published in E. Nigel Harris, Thomas Exner, Graham R. V. Hughes, Ronald A. Asherson, Phospholipid-Binding Antibodies, 2020
Dwight D. Pridham, Christine L. Cook
Congenital uterine anomalies result from failed fusion of the mullerian ducts. Anomalies include arcuate, septate, unicornuate, bicornuate and didelphic configurations (Figure 1). These are found in 3% of all women undergoing postpartum intrauterine examination.12 Several series have reported such anomalies to be present in 10 to 15% of habitual abort-ers.13,14 Arcuate uterus, in which there is only a mild alteration of cavity contour, probably is not associated with adverse reproductive outcome. Uterus didelphys also will most frequently be associated with acceptable reproductive outcome. Prematurity may be more common, but association with HAB is unlikely; in one series 13 of 14 pregnancies had a successful outcome.15 Unicornuate uteri have a similar effect, with the greatest concern being prematurity rather than HAB. Only one in five bicornuate uteri are associated with recurrent loss.16 Uterine septa may have the greatest association with HAB, but are nonetheless associated with a 78% viable pregnancy rate. Theoretically, uterine abnormalities may cause adverse outcomes in one of two ways. Space constraints may yield abnormal presentations (breech, transverse), premature labor and/or positional neonatal deformities. Poor vascularity of septate or bicornuate uteri may lead to poor placentation.15 Since poor vascularity should be most likely in septate uteri, less in bicornuate and least common in unicornuate or didelphic uteri, this theory fits well with the observed association of these deformities with HAB.
OHVIRA syndrome presenting with acute abdomen findings treated with minimally invasive method: three case reports
Published in Acta Chirurgica Belgica, 2022
Reyhan Gündüz, Elif Ağaçayak, Mehmet Sıddık Evsen
The combination of obstructed hemi-vagina and uterus didelphys was first reported in 1922 [1]. Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital defect of the Müllerian ducts, which are developmental precursors to the fallopian tubes, uterus, cervix, and upper vagina [1]. Because of the obstructed hemi-vaginal and ipsilateral renal agenesis (OHVIRA) which are parts of its characteristics, HWWS is also known as OHVIRA syndrome [1]. Obstructive Müllerian anomalies are estimated to affect approximately 0.1–3.8% of women [2]. Anatomically, HWWS is characterized by three anomalies of the female reproductive system: uterus didelphys, obstructed hemi-vagina, and ipsilateral renal agenesis. The presence of normal external genitalia generally delay the diagnosis until after menarche [3]. It is most commonly diagnosed at puberty due to pelvic and abdominal pain, worsening dysmenorrhoea, pelvic mass, and the ipsilateral renal agenesis. More rarely, it can be found in neonates or adults with primary infertility, pyometra, urinary obstruction, and ischiorectal swelling [4]. The diagnosis is made with history and physical examination together with appropriate imaging tests; diagnostic laparoscopy often is not required [5]. Early diagnosis and surgical intervention are important to relieve symptoms, prevent complications and preserve future fertility in OHVIRA syndrome [6].
Clinical and radiological findings for early diagnosis of Herlyn–Werner–Wunderlich syndrome in pediatric age: experience of a single center
Published in Gynecological Endocrinology, 2018
Carmine Noviello, Mercedes Romano, Fabiano Nino, Ascanio Martino, Giovanni Cobellis
The mean age of patients was 9 years (2 months–15 years). Presenting signs were a pelvic structure (Figure 1) with ipsilateral renal agenesis in two cases (age 2 months and 6 years), abdominal pain and hematocolpos (Figure 2) in the other four cases (all started their menstrual period). MRI confirmed in all cases the renal agenesis, uterus didelphys without communication between the cervices and blind hemivagina. Blood study did not show any signs of infection, and none had chromosomal abnormality. Cystoscopy showed the absence of ureteral remnants in the two patients with pelvic structure. Colposcopy allowed to identify the type of septum obstruction, and a one-stage surgical treatment was performed in all cases of complete obstruction, but in one case a second look was necessary 4 days later for a better resection of the septum. In two patients (without menstruation), a mucous collection was drain from the blind hemivagina. According to the characteristics of the HWWS, we had 5 patients in the group A, 1 in the group B, and none in the group C. The only one case of incomplete obstruction of the hemivagina was treated first by dilatation of the communication, and then a resection of the septum was necessary because of persistence of the hematocolpos. The mean follow-up was 18 months for all patients: nobody had abdominal pain, non-hemato-metrocolpos was present, but hypertrophy of the kidney in 5 cases.
A case report of first hearing loss, then painful menarche: a young girl with Herlyn–Werner–Wunderlich syndrome (OHVIRA syndrome) and concomitant inner ear anomalies
Published in Journal of Obstetrics and Gynaecology, 2021
Ayşe Ö. Balık, Buket Yağcı, Murat Özoğul, Fisun Vural
Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare anomaly of Mullerian ducts. The syndrome consists of obstructed hemivagina, uterus didelphys and ipsilateral kidney agenesis was first reported in 1922 (Tong et al. 2013). This syndrome is also called an OHVIRA, an acronym of obstructed hemivagina/hemicervix and ipsilateral renal disorder.