New Consideration of Herlyn‐Werner‐Wunderlich Syndrome Diagnosed by Ultrasound

Abstract

Objectives-As a rare malformation of the female reproductive system, Herlyn-Werner-Wunderlich syndrome (HWWS) was categorized into 3 classifications. It was recommended recently that, on the basis of the past classification, cervicovaginal atresia without communicating uteri should be newly added as classification 4. The surgical intervention will differ by type. To better optimize patient counseling as well as the preoperative evaluation and planning, our objective was to describe the ultrasound characteristics of each type of HWWS, including the new type.Methods-From January 1995 to November 2015, 37 cases of HWWS in with complete ultrasound information confirmed by surgery in the Peking Union Medical College Hospital were reviewed. We analyzed their ultrasound features, including hematometra, hematocervix, hematocolpos, and an ovarian chocolate cyst.Results-All of the ultrasound images of the 37 patients showed uterus didelphys with ipsilateral renal agenesis. Compared with the other 3 types, classification 4 showed distinctive ultrasound characteristics. Most cases of classification 4 showed hematometra (5 of 7 [71.4%]) and an ipsilateral ovarian chocolate cyst (6 of 7 [85.7%]), which was significantly higher than in the other 3. A rudimentary uterine horn was also a distinctive characteristic in this type. Meanwhile none of the classification 4 cases showed hematocervix or hydrocolpos, which were common signs of the other 3.Conclusions-According to this new classification criteria for HWWS, ultrasound characteristics of the new classification 4 differ from the others. As classification 4 was suggested to have a different surgical option, we should pay attention to its ultrasound characteristics, which might help in providing more information about the treatment and prognosis to the gynecologist.

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

“…Posteriormente, nueve estudios fueron excluidos por no tener acceso al texto completo; finalmente, se incluyeron 74 publicaciones que cumplieron con los criterios de inclusión (Tabla 1). 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,…”

“…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…”

“…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)(78)…”

“…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,<...>…”

“…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)67,68,(70)(71)(72)74,(77)(78)(79)(80)(81)(82), complementado con resonancia magnética nuclear (RMN) en el 74,6 % (10,…”