Herlyn-Werner-Wunderlich syndrome – a rare genitourinary anomaly in females: a series of four cases

Abstract

We present case series of four patients with an important syndrome known as Herlyn-Werner-Wunderlich syndrome. Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain during menses secondary to haematocolpos. Awareness is necessary to diagnose and treat this disorder properly before complications occur. Magnetic resonance imaging is the preferred modality for the delineation of uterine malformation. When renal anomalies are encountered, a screening should also be made for congenital abnormalities of the reproductive tract and vice versa.

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

“…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…”

“…A continuación, se exponen las características clínicas presentadas por las pacientes adultas con SHWW. El síntoma más frecuente es dismenorrea, con en el 64 % de los casos (12)(13)(14)(15)(17)(18)(19)(20)24,28,29,36,39,(41)(42)(43)49,51,53,60,(63)(64)(65), seguida de dolor pélvico con el 35,2 % de los casos (9,11,13,16,20,27,28,32,34,40,44,46,47,52,58,(60)(61)(62)66,(70)(71)(72)(73)(74)(76)(77)…”

“…Respecto a los signos clínicos 12 estudios presentan masa palpable en abdomen o pelvis (14,20,21,32,37,41,45,50,58,61,66,72). El diagnóstico imagenológico fue realizado mediante ecografía pélvica transabdominal o transvaginal en el 92,1 % de los casos (12,(14)(15)(16)(17)(18)20,21,24,(26)(27)(28)(29)(30)(31)(32)(35)(36)(37)(38)(39)(40)(41)(42)(44)(45)(46)(48)(49)(50)53,56,58,(61)(62)(63)(64)67,<...>…”

Chronic pelvic pain caused by Herlyn-Werner-Wunderlich Syndrome: Case report and discussion

“…It is associated to renal agenesis ipsilateral to the obstructed vagina. (4) This syndrome was described for the first time in 1971 by Herlyn and Werner. Then in 1976 renal aplasia with bicornuate uterus and simple vagina in the presence of an isolated hematocervix was described by Wunderlich.…”

A Rare Case of Herlyn-Werner-Wünderlich Syndrome with an Ectopic Ureter and A Communication Between a Hydrocolpos and The Bladder Neck in A Uterus Didelphys

“…Cases of uterus didelphys with an obstructed hemivagina and kidney agenesia can present alongside other urinary tract malformations of the same side (OHVIRA) [6,7], an embryological alteration occurring in the 8th week of gestation [8].…”