Presentation
Patients with OHVIRA usually present soon after menarche with dysmenorrhoea, pelvic pain or pelvic mass. Rarely, a patient may present in early adulthood with infertility. [birpublications.org]
First, this was a retrospective study, which presents potentials for selection bias. The study also included a small number of subjects, although it is presently the largest single-institution study. [journals.plos.org]
It usually presents at puberty with pelvic pain, but rarely can present in neonates or in adulthood in the form of pyometra, ischiorectal swelling, urinary obstruction, and primary infertility. [1], [2], [3], [4] Since this condition can be treated by [ijri.org]
Experts in all pertinent areas present concise, practical, evidence-based summaries of relevant topics, producing a key resource for physicians and scientists engaged in this exciting field of medicine. [books.google.de]
Therefore, in all patients who present an MDA, it is imperative to investigate the urinary tract. [link.springer.com]
Entire Body System
- Swelling
It is characterized by five signs, An acronym that may be used to remember the key symptoms is PRISH, for pain, redness, immobility, swelling. [wikivisually.com]
Other symptoms may include swelling of the abdomen, nausea and vomiting during menstruation, and pelvic pain. Fertility may also be affected. The condition often leads to an increased need for C-sections due to the smaller uteruses. [en.wikipedia.org]
[…] duplication, intestinal malrotation, and ovarian malposition. [6] Patients with this syndrome may present with a wide variety of symptoms such as secondary amenorrhea, dysmenorrhea, chronic pelvic pain which may be cyclical in nature, or palpable pelvic swelling [mamcjms.in]
It usually presents at puberty with pelvic pain, but rarely can present in neonates or in adulthood in the form of pyometra, ischiorectal swelling, urinary obstruction, and primary infertility. [1], [2], [3], [4] Since this condition can be treated by [ijri.org]
A 14-year-old girl was admitted to our institute with complaints of recurrent pelvic pain, mainly at the time of menses, and a gradually increasing lower abdominal swelling since menarche, which was attained at the age of 12 years. [jhrsonline.org]
- Recurrent Urinary Tract Infection
Among those, five patients (13.9%) presented complicated symptomatic presentations such as protruding vaginal mass, recurrent urinary tract infection, urinary incontinence, and abdominal pain, requiring surgery ( Table 2 ). [journals.plos.org]
- Anorexia
One month ago she presented to the emergency room complaining of lower abdominal pain mainly on the right iliac fossa with history of anorexia, nausea and vomiting. On examination secondary sexual characters were normal for age. [mejfm.com]
- Anemia
Massive fetomaternal bleed is very rare and even rarer is the resultant severe anemia causing early neonatal death, despite an uneventful normal pregnancy until the end. [ingentaconnect.com]
Respiratoric
- Aspiration
(C) Aspirated hemorrhagic vaginal fluid. (D) Resolution of hydronephroureterosis after vaginal fluid aspiration. [journals.plos.org]
The septum of the bulging mass was punctured with a 16 G needle and old altered blood was aspirated (Fig 3A). [jpss.eu]
After needle aspiration of 60 mL of jelly-like brownish fluid from the mass, a didelphys uterus with an obstructed right hemivagina was observed. A communication was performed between the right obstructed and left patent hemivaginas. [vdocuments.us]
Histopathology from the left adnexal lesion showed endometriotic cysts and aspirated pelvic fluid showed hemosiderin-laden macrophages. Postoperatively, she was kept on medical management for endometriosis. [ijri.org]
Gastrointestinal
- Abdominal Pain
Among these 20 cases, abdominal pain occurred in three patients (15%), abdominal or vaginal mass occurred in 12 (60%), and pyocolpos and AUR each occurred in two cases (10%). [journals.plos.org]
We describe imaging findings in a 14-year-old female patient who presented with cyclical lower abdominal pain. [ijamhrjournal.org]
However MRI has been considered as the imaging modality of choice by various authors (6) A 15-year-old girl presented with chief complaint of lower abdominal pain during menses for last 6 months. [mejfm.com]
Three patients presented with abdominal pain during puberty. Four patients were detected by prenatal ultrasonography. One two-year-old patient was accidentally found by an imaging study of another disease. [jstage.jst.go.jp]
- Acute Abdomen
Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis: a rare cause of acute abdomen in pubertal girls. J Pediatr Surg. 1991; 26 1200-1201 17 Stassart J P, Nagel T C, Prem K A. et al. [thieme-connect.de]
Journal of Gynecological Endoscopy and Surgery. 2011; 2(2): 105-108 [Pubmed] 12 Herlyn- werner- wunderlich syndrome: A rare urogenital anomaly masquerading as acute abdomen Ashu Rastogi, Anshu Khamesra The Indian Journal of Pediatrics. 2010; 77(8): 917 [jhrsonline.org]
Since the patient presented with an acute abdomen, appendicitis and ovarian torsion were a likely possibility following trans-abdominal ultrasound. The patient was taken to the theater, unnecessary laparotomy was performed. [mejfm.com]
Mostyn Embrey syndrome should be considered in the differential diagnoses in young females with unilateral renal agenesis presenting with pelvic mass, symptoms of acute abdomen and acute urinary retention [ 1 ]. [imedpub.com]
They complained of various symptoms: dysmenorrhea (94%), spotting (41%), chronic pelvic pain (24%), vaginal discharge (14%), dyspareunia (14%), fever (3%) and acute abdomen pain (2%). [academic.oup.com]
Cardiovascular
- Hypertension
Hypertension was diagnosed when high blood pressure equal to or greater than the 95 th percentile was observed at more than three visits [ 8 ]. [journals.plos.org]
Original Contribution / Clinical Investigation Improving Hypertension Control via a Team-based Educational and Refill Monitoring (TERM) Intervention, Sharjah, United Arab Emirates [pdf version] Sameh F. Ahmed, Hany M. Aiash, Hassan A. [mejfm.com]
Psychiatrical
- Suggestibility
Multiloculated left adnexal cystic lesions with blood products were seen suggestive of endometriotic cysts [Figure 5]. [ijri.org]
The findings suggest a specific developmental anomaly of the mullerian ductal system, probably secondary to a wolffian duct anomaly. [experts.umn.edu]
This suggests that all the cases of unilateral renal agenesis should be screened for mullerian anomalies. [ijamhrjournal.org]
One patient demonstrated persistently elevated creatinine adjusted for age, suggesting continued renal function deterioration. [journals.plos.org]
The left horn of the uterus, fallopian tube and left vagina was distended with hyper intense fluid (suggestive of blood) along with a bulky left ovary. [jpss.eu]
Urogenital
- Vaginal Bleeding
Vaginal aspiration was performed at 2 months ( Table 2 ); the aspirated fluid was hemorrhagic fluid originating from neonatal vaginal bleeding. [journals.plos.org]
The patient denied any recent abdominal trauma, abnormal vaginal bleeding, nausea, vomiting or diarrhea. A bimanual physical examination indicated a right-sided cystic and tender pelvic mass, movable, mildly tender to palpation. [link.springer.com]
- Absent Kidney
Irrespective of imaging modality, the typical findings are: duplication of the uterus, cervix and vagina unilateral hematocolpos or haematometrocolpos absent kidney on the same side as the uterovaginal obstruction Gartner duct cysts Treatment and prognosis [radiopaedia.org]
Because prenatal ultrasonography is now routinely performed, a large portion of OHVIRA patients are first encountered by urologists evaluating a dysplastic, atrophic, or absent kidney after detection during ultrasonography. [journals.plos.org]
Treatment
The clinical diagnosis is very challenging and requires imaging studies in which ultrasound and MRI play an essential role in the diagnosis, classification and treatment plan. [ncbi.nlm.nih.gov]
Treatment is surgical, with resection of the septum dividing the two hemivaginas in order to relieve the obstruction. [radiopaedia.org]
Table I Anatomic variants and surgical treatment. [academic.oup.com]
Treatment & Care Treatment depends on the individual patient and what symptoms are present. Treatment strategies typically include surgery to remove the wall of tissue that is blocking the vagina. [texaschildrens.org]
Main Outcome Measures: For each patient, the clinical course, specific anatomic findings, treatment(s) offered, and obstetrical outcome are presented. [experts.umn.edu]
Prognosis
Irrespective of imaging modality, the typical findings are: duplication of the uterus, cervix and vagina unilateral hematocolpos or haematometrocolpos absent kidney on the same side as the uterovaginal obstruction Gartner duct cysts Treatment and prognosis [radiopaedia.org]
Opportune diagnosis and treatment achieve complete improvement of symptoms, adequate reproductive prognosis and avoid major complications such as endometriosis, pelvic adhesions and infertility. [ncbi.nlm.nih.gov]
Batt RE, Mitwally MF (2003) Endometriosis from thelarche to midteens: pathogenesis and prognosis, prevention and pedagogy. J Pediatr Adolesc Gynecol 16:337–347 PubMed CrossRef 38. [springermedizin.de]
Uterus didelphys with an imperforate hemivagina is the least common abnormality of the Mullerian ducts, but has the best prognosis in terms of alleviation of symptoms and future fertility of these patients. [omicsonline.org]
Future research should analyze the relationship between the different anatomic variants found in this syndrome and their clinical findings (referring particularly to obstetrical prognosis) to improve counselling to patients at the moment of diagnosis. [academic.oup.com]
Etiology
The clinical and etiologic implications are discussed. 9 1988 byGrune & Stratton. Inc. INDEX WORDS: Double uterus; didelphys uterus. [vdocuments.us]
In the right paramedian side, at the level of the Douglas, posterior to the ideal point of continuity between uterus and rudimentary horn, there were some adjacent nodules most likely of endometrial etiology (14 mm, 38 mm in total) (Figure 9 ). [link.springer.com]
Congenital developmental anomalies of the Müllerian ducts are a significant etiological factor of infertility, and are associated with an increment in obstetrical complication. [thieme-connect.com]
Epidemiology
Hypertension in children and adolescents: epidemiology and natural history. Pediatr Nephrol. 2010;25: 1219–1224. pmid:19421783 View Article PubMed/NCBI Google Scholar 9. Mandava A, Prabhakar RR, Smitha S. [journals.plos.org]
Pathophysiology
The purpose of this review is to demonstrate the pathophysiology, clinical manifestations, diagnostic methods and treatment of the obstructed hemivagina and ipsilateral renal anomaly syndrome. [ncbi.nlm.nih.gov]
The underlying pathophysiological mechanism is potentially the increased risk of retrograde menstruation: because of the obstructed vaginal outflow, the risk of endometrial tissue reflux through the fallopian tube, which can result in retrograde endometriosis [link.springer.com]
Prevention
Early diagnosis of OHVIRA is crucial to prevent further complications like infertility and to maintain normal reproductive life, thus preventing marital disharmony and improving socioeconomic status. [fertilityscienceresearch.org]
Batt RE, Mitwally MF (2003) Endometriosis from thelarche to midteens: pathogenesis and prognosis, prevention and pedagogy. J Pediatr Adolesc Gynecol 16:337–347 PubMed CrossRef 38. [springermedizin.de]
Expectant mothers are often manages with cervical sutures and C-sections to prevent fetal distress during labour. References [ edit ] ^ Mandava, Anitha; Prabhakar, R. R.; Smitha, S. (2012-04-01). [en.wikipedia.org]
MRI is the most effective method and helps to prevent unnecessary surgery (8). [mejfm.com]
Conclusions It could be desirable to achieve to an early diagnosis and treatment of HWW syndrome, in order to relieve acute symptoms, preserve normal fertility and prevent several medical complications. [link.springer.com]