In-office Hysteroscopic Treatment of Herlyn-Werner-Wunderlich Syndrome: A Case Series

Fascilla, Fabiana Divina; Olivieri, Claudiana; Cannone, Rossella; Palma, Domenico De; Manosperta, Francesco; Costantino, A.; Carugno, Josè; Vicino, Mario; Cicinelli, Ettore; Bettocchi, Stefano

doi:10.1016/j.jmig.2020.04.013

Cited by 23 publications

(26 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In the described cases, the combination of ultrasound and diagnostic laparoscopy allowed the determination of a definite diagnosis of the anomaly. It should be noted, however, that the diagnosis can be made without laparoscopy, and it is used in selected cases during treatment [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…According to Aveiro et al, resection of the vaginal septum is the treatment of choice for obstructed hemivagina [ 1 ], but Fascilla et al prefer a hysteroscopic incision [ 5 ]. The advantages of the last method include, among others, avoiding the risk of the use of general anesthesia in an operating room setting and the possibility of performing it in the office [ 5 ]. Regular gynecological control is important in postoperative management, aimed at assessing the patency of the residual vagina.…”

Section: Discussionmentioning

confidence: 99%

“…Our case study showed that the treatment of HWWS is a multi-stage process that requires cooperation between the patient and a specialized treatment center. However, according to the latest information, HWWS treatment can be a one-step treatment and take place in the office [ 5 ]. Table 1 compares the diagnostic and therapeutic procedures performed in the described patients with selected cases from the literature ( Table 1 ).…”

Section: Discussionmentioning

confidence: 99%

“…OHVIRA stands for obstructed hemivagina and ipsilateral renal anomaly [ 3 ]. The prevalence of obstructive Müllerian malformations is unknown, but is reported to be 0.1% to 3.8% in women [ 4 , 5 ]. The prevalence of Herlyn-Werner-Wunderlich Syndrome is unknown.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Herlyn-Werner-Wunderlich Syndrome: Comparison of Two Cases

Kozłowski

Nowak

Boboryko

et al. 2020

IJERPH

View full text Add to dashboard Cite

Background: Herlyn-Werner-Wunderlich Syndrome is a rare malformation syndrome characterized by uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. Symptoms appear most often after menarche and are secondary to hematocolpos. We compare clinical symptoms, diagnosis and treatment of two patients, a 13-year-old and a 17-year-old. Case report: Despite the non-uniform clinical symptoms, it should be noted that in both patients, the 13-year-old and the 17-year-old, hematocolpos, which was probably the cause of lower abdominal pain, was diagnosed with ultrasound. The diagnosis was complemented by laparoscopy, which determined the diagnosis of malformation of uterus didelphys with obstructed hemivagina. The patients had a history of kidney agenesis, which, after gynecological diagnosis, turned out to be ipsilateral. In the 13-year-old, agenesis was diagnosed by uroscintigraphy, while in the 17-year-old it was diagnosed by urography. Incision and drainage of the residual vagina was performed in the course of therapeutic management. In both cases, the clinical situation required a repeated widening of the orifice. Conclusions: Lower abdominal pain accompanying hematocolpos suggested Herlyn-Werner-Wunderlich Syndrome (HWWS) as the cause of symptoms. 3D transvaginal ultrasound enabled the determination of a congenital uterine defect with high probability, although inconclusive cases required confirmation by laparoscopy. Incision of the blocked vagina and drainage of hematocolpos were the key components of treatment. The treatment of HWWS is a multi-step process.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Herlyn-Werner-Wunderlich Syndrome: Comparison of Two Cases

Kozłowski

Nowak

Boboryko

et al. 2020

IJERPH

View full text Add to dashboard Cite

show abstract

“…Объем операции варьирует от классического или эндоскопического иссечения влагалищной перегородки, что обеспечивает дренирование частично аплазированной гемивагины, до лапароскопической экстирпации гемиматки при очень высоком уровне атрезии. Некоторые специалисты в качестве хирургического лечения предлагают проводить одноэтапное амбулаторное гистероскопическое иссечение влагалищной перегородки без применения общей анестезии с использованием биполярных электродов размером 5 Fr [27]. Таким образом, в ряде случаев эндоскопия может осуществляться без общей анестезии.…”

Section: клинические проявления и диагностикаunclassified

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

Алиева

Кохреидзе

Сухоцкая

et al. 2021

Androl. genit. hir.

View full text Add to dashboard Cite

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.

show abstract

Hemihysterectomy in a patient with uterus didelphys, vaginal septum and ipsilateral renal agenesis: A case report and literature review

Peixoto Silva,

Souza Neves,

Tannure Saraiva

et al. 2023

Intl J Gynecology & Obste

View full text Add to dashboard Cite

Uterus didelphys is a rare Müllerian anomaly, often diagnosed during menarche or in women with a personal history of infertility and/or recurrent pregnancy loss. Its association with other genitourinary anomalies is frequent and may determine the existence of established syndromes. This case report refers to a 13‐year‐old female patient diagnosed with OHVIRA syndrome (Obstructed Hemivagina with Ipsilateral Renal Agenesis), a condition wherein the presence of a didelphic uterus is associated with hemivagina obstruction and ipsilateral renal agenesis. The patient presented with cyclic pelvic pain, related to the presence of hematocolpos and hematometra, which persisted despite several surgical approaches, including vaginal septum excision and correction of cervical stenosis. The recurrence of the condition indicated exploratory laparotomy, revealing two hemi‐uteri and two uterine cervixes, with hematometra on the right. A subtotal hemihysterectomy was performed on the right. Post‐procedure, the patient developed with regular menstrual cycles and improvement of pelvic pain complaints. Given the limited prevalence and low index of suspicion, the potential requirement for surgical intervention and its potential impact on reproductive future, diagnosing and treating OHVIRA syndrome and other Müllerian anomalies poses notable challenges in clinical practice. Hence, sharing different therapeutic approaches of a rare diagnosis with the scientific community is of paramount importance to aid in early diagnosis and effective management of similar clinical cases.

show abstract

In-office Hysteroscopic Treatment of Herlyn-Werner-Wunderlich Syndrome: A Case Series

Cited by 23 publications

References 23 publications

Herlyn-Werner-Wunderlich Syndrome: Comparison of Two Cases

Herlyn-Werner-Wunderlich Syndrome: Comparison of Two Cases

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

Hemihysterectomy in a patient with uterus didelphys, vaginal septum and ipsilateral renal agenesis: A case report and literature review

Contact Info

Product

Resources

About