A Herlyn-Werner-Wunderlich Syndrome Variant with Ipsilateral Renal Absence and a Contralateral Duplex Collecting System in a 26-Year-Old Female

Zhou, Yong; Fu, Xiujuan; Qian, Honglang; Lin, Kejian; Wang, Jinhua; Zhou, Shuyang; Hu, Xian; Jin, Hang-mei

doi:10.1159/000357446

Cited by 10 publications

(7 citation statements)

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“…The association of uterus didelphys with an obstructed hemivagina is explained by a defect in embryogenesis at the eighth week of gestation affecting the mesonephric and Müllerian ducts, in which there is non-fusion of Müllerian ducts or failed resorption of the uterine/vaginal septum 1 . Other defects which can be found include renal dysplasia, duplication of the kidneys and ureters 11,12 , ectopic ureter, as well as high-riding aortic bifurcation, IVC duplication, intestinal malrotation 13 and ovarian malposition. Renal agenesis predicts an ipsilateral obstructive Müllerian duct abnormality 50% of the time and is ipsilateral to the dilated uterine cavity.…”

Section: Discussionmentioning

confidence: 99%

Herlyn-Werner-Wunderlich syndrome: a case report

Piccinini¹,

Doski²

2015

Rev. Bras. Ginecol. Obstet.

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Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital disorder of the Müllerian ducts in which there is uterus didelphys, obstructed hemivagina and unilateral renal agenesis. The most common presentation is an abdominal mass secondary to hematocolpos, pain and dysmenorrhea. However, in some cases, such as the one we present here, menses are normal due to an obstructed hemivagina, and diagnosis can be delayed. We describe evaluation and surgical management of a 13-year-old girl with this condition who was diagnosed by computed tomography (CT) scan and confirmed by pelvic ultrasound and surgical exploration, as well as a review of the literature.

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Section: Discussionmentioning

confidence: 99%

Herlyn-Werner-Wunderlich syndrome: a case report

Piccinini¹,

Doski²

2015

Rev. Bras. Ginecol. Obstet.

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“… 5 MRI and ultrasound are the most widely used diagnostic tools, with even 100% accuracy being reported for MRI because of its high accuracy and detailed elaboration of uterovaginal anatomy. 4 , 14 Several studies have reported the renal malformations in HWWS, including renal agenesis, multicystic dysplastic kidney, and absent kidney. However, the scoliosis among HWWS has not been reported.…”

Section: Discussionmentioning

confidence: 99%

“… 16 , 17 Renal agenesis accompanies Müllerian duct abnormalities include the kidneys, fallopian tube, ovary, cervix, and upper vagina, all that generate from the same ureteric bud that fails to form correctly. 4 , 5 …”

Section: Discussionmentioning

confidence: 99%

“… 1 , 2 Although the true incidence of this syndrome remains unknown, the overall incidence of obstructive Müllerian duct anomalies can range from 0.1% to 3.8%. 3 , 4 HWWS is usually discovered at puberty with nonspecific symptoms, such as increasing pelvic pain, dysmenorrheal, and palpable mass due to the associated hematocolpos or hematometra, which result from retained, longstanding menstrual flow in the obstructed vagina. 3 – 7 The exact cause, pathogenesis, and embryologic origin of HWWS remain a subject of discussion.…”

Section: Introductionmentioning

confidence: 99%

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Scoliosis in Herlyn–Werner–Wunderlich Syndrome

Shen

et al. 2014

Medicine

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Herlyn–Werner–Wunderlich syndrome (HWWS) is a congenital Müllerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Little is reported about spinal deformity associated with this syndrome.This study presents a case of scoliosis occurring in the setting of HWWS and explores the possible association between the 2 diseases.A previously unreported scoliosis in HWWS is described. The patient is a 12-year-old Chinese female with scoliosis that underwent a posterior correction at thoracic 5–thoracic 12 (T5–T12) levels, using the Moss-SI (Johnson & Johnson, American) spinal system. At 24-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. Six months after scoliosis correction surgery, the patient went to our clinics for the treatment of HWWS. She was performed a vaginal septum resection and detected with pyocolpos. Her follow-up was symptom free at the fourth postoperative month. The prevalence of scoliosis among patients with HWWS was 8.57% that is much higher than the incidence of congential scoliosis among general population (1/1000).To the best of our knowledge, this is the first report of HWWS with thoracic scoliosis. During surgery, surgeons and anesthesiologists must pay particular attention to the Müllerian duct anomaly and renal agenesis associated with HWWS. There is a potential association between congenital scoliosis and HWWS.

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“…The three characteristics of this syndrome include uterine didelphys (double uterus), hemivaginal obstruction, and ipsilateral kidney agenesis [ 6 , 7 ]. HWWs’ etiology and pathogenesis are unknown [ 8 - 10 ]. The incidence of HWWS is 0.6–10% of all Müllerian abnormalities (23%), 0.1 to 3.8% in the female population; up until 2011, only about 200 cases of HWWS have been documented worldwide [ 3 , 11 - 15 ].…”

Section: Introductionmentioning

confidence: 99%

Herlyn-Werner-Wunderlich syndrome: Challenges in diagnosis and management

Roziana,

Nora,

Maharani

et al. 2023

Narra J

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Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) is a complex congenital malformation characterized by a triad including uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. In this case report, we present a case of HWWS along with the challenges in diagnosis and multi-step treatment processes. A 25-year-old woman presented to Dr. Zainoel Abidin Hospital in Banda Aceh, Indonesia with a chief complaint of lower back pain for the past six months (two months after the marriage). The patient also complained of late menstruation, followed by thick yellow vaginal discharge resembling malodorous pus occurring after menstruation. Additionally, a history of fever and pain during intercourse was reported. Vaginal examination revealed purulent fluid emerging from a pinpoint hole on the right vaginal wall. Vaginal palpation revealed a two-branched uterus, along with a palpable cystic mass on the right vaginal wall. Ultrasound examination indicated the presence of the right and left hemi-uteri, measuring 4.7x1.35 cm and 5.7x1.26 cm in size, respectively with both ovaries appearing normal. Fluid accumulation was observed in the right hemivagina, while the left kidney exhibited normal features, and the right kidney showed signs of hypoplasia. The patient was diagnosed with uterus didelphys, fluid accumulation in the hemivagina, and right kidney hypoplasia. Magnetic resonance imaging (MRI) confirmed uterus didelphys and revealed narrowing of the right hemivagina, suggestive of HWWS. Hysteroscopy was performed to resect the vaginal septum with laparoscopic guidance, along with drainage of hematocolpos, pyocolpos and placement of an intracervical mold. Despite HWWs having conservative gradual management, it was opted to perform vaginal septum resection, hematocolpos and pyocolpos drainage and placement of an intracervical mould in this case, in order to relieve symptoms and restore the reproductive and sexual functions.

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A Herlyn-Werner-Wunderlich Syndrome Variant with Ipsilateral Renal Absence and a Contralateral Duplex Collecting System in a 26-Year-Old Female

Cited by 10 publications

References 10 publications

Herlyn-Werner-Wunderlich syndrome: a case report

Herlyn-Werner-Wunderlich syndrome: a case report

Scoliosis in Herlyn–Werner–Wunderlich Syndrome

Herlyn-Werner-Wunderlich syndrome: Challenges in diagnosis and management

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