Herlyn–Werner–Wunderlich syndrome: diverse presentations and diagnosis on MRI

Zhang, H.; Ning, Gang; Fu, Chuan; Li, Bao; Guo, Yingkun

doi:10.1016/j.crad.2020.01.016

Cited by 27 publications

(33 citation statements)

References 17 publications

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“…Ultrasound examination is an inexpensive and convenient method of screening for HWWS; however, its diagnostic accuracy depends on the experience of the examiner. MRI is the imaging modality of choice [ 6 , 7 ] for the diagnosis and classification of HWWS, as it provides details about uterine morphology, including outline and intrauterine cavity shape and continuity with each vaginal lumen, and the character of the fluids in these cavities. It can also ascertain associated pathologies such as endometriosis, vaginitis, pelvic inflammation, and adhesion, as well as renal agenesis.…”

Section: Discussionmentioning

confidence: 99%

“…Symptoms typically present after menarche, with regular advanced pelvic pain [4] . Hematocolpos is caused by the retention of menstrual blood in the obstructed hemivagina and is usually detected as a cystic pelvic mass [6] . Surgery is the main treatment, and it includes removal of the vaginal septum and drainage of the effusion [7] ; however, complete removal of the vaginal septum in a single procedure may be difficult in case of local inflammation in the vagina and cervix.…”

Section: Introductionmentioning

confidence: 99%

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Herlyn–Werner–Wunderlich syndrome and its complications: A report of two cases and literature review

Li¹,

Liu²,

Li³

2021

Radiology Case Reports

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Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital malformation characterized by uterus didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. The obstructed vagina affects menstrual flow, leading to related clinical symptoms after menarche. However, the age of onset, initial symptoms, and clinical complications differ among patients owing to the different types of vaginal septum. Herein, we report 2 cases. The first case is of a 20-year-old woman who presented with fever; she was diagnosed with vaginitis and pelvic inflammation due to the vaginal septum with ostiole. The second case is of a 12-year-old girl who complained of abdominal pain; she was diagnosed as having pelvic inflammation, omentitis, and suppurative appendicitis due to the atretic vaginal septum.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Herlyn–Werner–Wunderlich syndrome and its complications: A report of two cases and literature review

Li¹,

Liu²,

Li³

2021

Radiology Case Reports

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“…Among the uterine malformations, bicorporeal uterus and double cervix occur in 62.5% of patients, septate uterus in 22.5% and bicornuate uterus in 15%. Ipsilateral vaginal septum was found in 75% of patients and ipsilateral cervical obstruction in 25% ( Zhang et al, 2020 ). Ipsilateral renal agenesis, as well as ipsilateral paravaginal cysts and ureter remnants invading the obstructed vagina/cervix have also been reported ( Zhang et al, 2020 ; Aveiro et al, 2011 ).…”

Section: Disruption Of Müllerian Developmental and Differentiation Processesmentioning

confidence: 99%

“…Ipsilateral vaginal septum was found in 75% of patients and ipsilateral cervical obstruction in 25% ( Zhang et al, 2020 ). Ipsilateral renal agenesis, as well as ipsilateral paravaginal cysts and ureter remnants invading the obstructed vagina/cervix have also been reported ( Zhang et al, 2020 ; Aveiro et al, 2011 ). The incidence of HWWS in the population is unknown, although a study reported 0.1-3.8% ( Aveiro et al, 2011 ).…”

Section: Disruption Of Müllerian Developmental and Differentiation Processesmentioning

confidence: 99%

Studying Müllerian duct anomalies – from cataloguing phenotypes to discovering causation

González

Artibani

Ahmed

2021

Disease Models &Amp; Mechanisms

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Müllerian duct anomalies (MDAs) are developmental disorders of the Müllerian duct, the embryonic anlage of most of the female reproductive tract. The prevalence of MDAs is 6.7% in the general female population and 16.7% in women who exhibit recurrent miscarriages. Individuals affected by these anomalies suffer from high rates of infertility, first-trimester pregnancy losses, premature labour, placental retention, foetal growth retardation and foetal malpresentations. The aetiology of MDAs is complex and heterogeneous, displaying a range of clinical pictures that generally lack a direct genotype-phenotype correlation. De novo and familial cases sharing the same genomic lesions have been reported. The familial cases follow an autosomal-dominant inheritance, with reduced penetrance and variable expressivity. Furthermore, few genetic factors and molecular pathways underpinning Müllerian development and dysregulations causing MDAs have been identified. The current knowledge in this field predominantly derives from loss-of-function experiments in mouse and chicken models, as well as from human genetic association studies using traditional approaches, such as microarrays and Sanger sequencing, limiting the discovery of causal factors to few genetic entities from the coding genome. In this Review, we summarise the current state of the field, discuss limitations in the number of studies and patient samples that have stalled progress, and review how the development of new technologies provides a unique opportunity to overcome these limitations. Furthermore, we discuss how these new technologies can improve functional validation of potential causative alterations in MDAs.

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“…[17] предложили субклассификацию синдрома OHVIRA, обозначенную как 3О: obstruction (обструкция), ureteric orifice (мочеточниковое отверстие) и outcome (исход). Было описано 6 типов сочетания аномальной формы матки с разными уровнями обструкции влагалища, с паравагинальными кистами, эктопией мочеточника в замкнутую гемивагину на разных уровнях [18,19]. Авторы надеялись, что такой подход позволит учесть разнообразие анатомических вариантов, а самое главноеупростит планирование предстоящего хирургического вмешательства.…”

Section: история изученияunclassified

Herlyn–Werner–Wunderlich syndrome in the prepubescent period (literature review and clinical observations)

Алиева

Кохреидзе

Сухоцкая

et al. 2021

Androl. genit. hir.

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Herlyn–Werner–Wunderlich syndrome (OHVIRA syndrome) is a combined malformation of the genitourinary systeme, characterized by various combinations of uterus dydelphys with unilateral obstructed (or blind) hemivagina and ipsilateral renal agenesis. The causes of mistakes in diagnosis and treatment are common because of relative rarity of anomaly, insufficient awareness of practitioners about the syndrome and the lack of multidisciplinary approach. Untimely and tactically chaotic diagnosis of Herlyn–Werner–Wunderlich syndrome leads to a misconception about the clinical situation, wrong choice in treatment, and, as a consequence, to complications such as strictures, widespread adhesions and inflammation, as well as irreversible changes in the topography of organs of the small pelvis with a subsequent deterioration in reproductive status of patient. This article provides a review of the literature on the problem, considers the clinical cases of diagnosing this defect in prepubertal patients.

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Herlyn–Werner–Wunderlich syndrome: diverse presentations and diagnosis on MRI

Cited by 27 publications

References 17 publications

Herlyn–Werner–Wunderlich syndrome and its complications: A report of two cases and literature review

Herlyn–Werner–Wunderlich syndrome and its complications: A report of two cases and literature review

Studying Müllerian duct anomalies – from cataloguing phenotypes to discovering causation

Herlyn–Werner–Wunderlich syndrome in the prepubescent period (literature review and clinical observations)

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