A Variant of Herlyn-Werner-Wunderlich Syndrome Presenting With Acute Abdomen: A Case Report and Review of Literature

Gupta, Nishant; Gandhi, Darshan; Gupta, Sonali; Goyal, Pradeep; Li, Shuo; Kumar, Yogesh

doi:10.1177/2333794x18769817

Cited by 10 publications

(19 citation statements)

References 14 publications

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“…Usually, HWW syndrome remains undiagnosed and asymptomatic during early childhood with normal external genitalia [10]. Classically, a patient with HWW syndrome can present with severe dysmenorrhea a few months to 1 year after attaining menarche.…”

Section: Discussionmentioning

confidence: 99%

Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report

et al. 2019

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BackgroundHerlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hematocolpos. We present the first case report on Herlyn-Werner-Wunderlich syndrome from Bangladesh.Case presentationA 15-year-old Asian girl presented with lower abdominal pain of 3 months’ duration. She had had menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. Abdominal examination found a tender mass on the right iliac fossa. Further evaluation with ultrasound revealed distended endometrial cavity filled with complex fluid and nonvisualization of the right kidney. Pelvic magnetic resonance imaging showed absent right kidney and two separate endometrial stripes surrounded by endometrium and a muscular layer. The right endometrial cavity and cervix were distended with blood. This magnetic resonance imaging finding is consistent with Herlyn-Werner-Wunderlich syndrome with uterine didelphyis, right-sided hematometra resulting from obstructed hemivagina, and ipsilateral agenesis of the right kidney. The vaginal septum was resected for vaginoplasty. She was discharged 5 days after surgery and came for follow-up after 7 days. Vaginal examination revealed a healthy wound with no adhesion of the vaginal wall. She also informed us that she had started regular menstruation without any pain 30 days after the operation.ConclusionAn unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to complications such as endometriosis and infertility. Awareness is required; otherwise, misdiagnosis clearly can occur.

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Section: Discussionmentioning

confidence: 99%

Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report

et al. 2019

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“…En las 74 publicaciones incluidas se encontraron 6 estudios observacionales retrospectivos (4, 13-17), 10 series de casos (10)(11)(18)(19)(20)(21)(22)(23)(24)(25) y 58 reportes de caso (12,, que incluyeron un total de 663 pacientes. La mayoría de las publicaciones fueron en India con 11 estudios (17,29,39,(44)(45)(46)(47)52,60,62,63), seguido de USA con 9 estudios (30,32,53,56,69,70,72,75,78), China (10,11,13,15,24,25,33,67) e It alia (4,20,23,34,37,51,66,71) con 8 estudios cada uno. La serie de casos con mayor población fue publicada en China…”

Section: Resultsunclassified

“…La población incluida presenta una edad media al diagnóstico de 16,8 años (0 a 53 años). Un reporte de caso de diagnóstico ecográfico antenatal (54), 4 de diagnóstico neonatal (22,26,38,50), 12 entre 1 y 12 años (28,37,48,51 ,52,59,61,66,73,78,80,82), 26 entre 13 y 19 años (27,29,30,32,39,41,42,(44)(45)(46)(47)49,55,56,58,60,63,(68)(69)(70)(71)(72)74,76,77,79) y 15 reportes de mayores de 20 años (31,(33)(34)(35)(36)40,43,53,57,62,64,65,67,75,81).…”

Section: Resultsunclassified

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Síndrome de Herlyn-Werner-Wunderlich: reporte de caso y revisión de la literatura

Gutiérrez-Montufar

Zambrano-Moncayo

Otálora-Gallego

et al. 2021

Rev. Colomb. Obstet. Ginecol.

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Objetivo: presentar un reporte de caso de síndrome de Herlyn Werner Wunderlich (SHWW) y hacer una revisión de la literatura para determinar los hallazgos clínicos e imagenológicos en estas pacientes. Materiales y métodos: se presenta el caso de una mujer de 16 años que consultó, en un hospital de las fuerzas militares en Bogotá, por dolor pélvico recurrente, su diagnostico final fue SHWW. Se realizó una búsqueda sistemática de la literatura en las diferentes bases de datos, revisiones sistemáticas, cohortes, series de casos y reportes de casos en pacientes con diagnóstico de SHWW en cualquier edad, se obtuvo información sobre las características de presentación clínica, y las tecnologías diagnósticas más frecuentemente utilizadas. Se hace resumen narrativo de los hallazgos. Resultados: se incluyeron 77 publicaciones, un total de 676 pacientes. Los síntomas más frecuentes fueron dismenorrea (63,9 %), seguido de dolor pélvico (35,2%). Las tecnologías diagnósticas más utilizadas fueron el ultrasonido pélvico en un 92,1% y la resonancia magnética nuclear en un 74,6% de los casos. La histeroscopia y laparoscopia son poco utilizados en el diagnóstico. Conclusión: el SHWW es una entidad poco frecuente, debe hacer parte del estudio complementario de la agenesia renal del paciente pediátrico y del diagnóstico diferencial de la dismenorrea primaria en pacientes en la adolescencia. Se requiere evaluar con estudios de cohorte más grandes la utilidad de la histeroscopia en estas pacientes.

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“…Due to the normal appearance of the external genitalia, this syndrome often remains undiagnosed and asymptomatic in early childhood [ 20 ]. Diagnosis is determined on the basis of radiological examinations.…”

Section: Discussionmentioning

confidence: 99%

Herlyn-Werner-Wunderlich Syndrome: Comparison of Two Cases

Kozłowski

Nowak

Boboryko

et al. 2020

IJERPH

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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.

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A Variant of Herlyn-Werner-Wunderlich Syndrome Presenting With Acute Abdomen: A Case Report and Review of Literature

Cited by 10 publications

References 14 publications

Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report

Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report

Síndrome de Herlyn-Werner-Wunderlich: reporte de caso y revisión de la literatura

Herlyn-Werner-Wunderlich Syndrome: Comparison of Two Cases

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