Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.
Department of Gynaecology, Beaumont Hospital, Dublin, Ireland.
J Med Case Rep. 2024 Aug 3;18(1):361. doi: 10.1186/s13256-024-04695-w.
Herlyn-Werner-Wunderlich syndrome , a rare Müllerian ducts congenital disease, is characterized by a diphtheritic uterus, blind hemivagina, and ipsilateral renal agenesis. Diagnosis is at young age by ultrasound and magnetic resonance imaging, and the prognosis is good. Usually, complications evolve endometriosis and secondary pelvic inflammation.
A 40-year-old female patient, Brazilian, white, primigravida, diagnosed at 30 years with a didelphic uterus on ultrasound, and 4 years later, with a left ovarian endometrioma, multiple ovarian cysts, and left renal agenesis on magnetic resonance imaging. Subsequently, due to dyspareunia and a feeling of swelling, the patient underwent transvaginal ultrasound with bowel preparation, and a hematocolpos was found and Herlyn-Werner-Wunderlich syndrome was suspected; 10 years after the diagnosis she had a planned pregnancy. She presented frequent contractions following the 15th week of pregnancy and fortunately there were no complications or premature labor. Labor was inducted at 40 weeks and 6 days without progress and a cesarean section was indicated and performed without complications. Herlyn-Werner-Wunderlich syndrome often goes unnoticed, leading to inadequate treatment. Individuals with Herlyn-Werner-Wunderlich syndrome commonly face fertility issues, such as high miscarriage rate (21-33%), and obstetric complications, such as spontaneous abortions (40% risk), intrauterine growth restriction, postpartum hemorrhage, increased fetal mortality, preterm delivery (21-29%), and elevated rates of cesarean sections. In addition, there is higher susceptibility of developing endometriosis, especially with hemivaginal obstruction, and pelvic adhesions.
Early diagnosis enables timely treatment and, consequently, fewer complications. Still, when these factors are absent, vaginal birth may still be possible. The true prevalence and incidence of complications related to Herlyn-Werner-Wunderlich syndrome are still unknown.
Herlyn-Werner-Wunderlich 综合征是一种罕见的苗勒管先天性疾病,其特征为隔膜子宫、盲阴道和同侧肾发育不全。该病通常在年轻时期通过超声和磁共振成像进行诊断,预后良好。但通常会出现子宫内膜异位症和继发性盆腔炎等并发症。
一名 40 岁的巴西白人初产妇,30 岁时超声检查发现双子宫,4 年后磁共振成像显示左侧卵巢子宫内膜异位囊肿、多个卵巢囊肿和左侧肾发育不全。此后,由于性交困难和肿胀感,患者进行了经阴道超声检查并进行了肠道准备,发现了血性阴道积水,疑似 Herlyn-Werner-Wunderlich 综合征;诊断 10 年后她计划怀孕。妊娠 15 周后出现频繁宫缩,但幸运的是没有并发症或早产。妊娠 40 周+6 天无进展,行剖宫产术,无并发症。Herlyn-Werner-Wunderlich 综合征常常未被发现,导致治疗不足。患有 Herlyn-Werner-Wunderlich 综合征的个体通常面临生育问题,如流产率高(21-33%)和产科并发症,如自然流产(40%的风险)、宫内生长受限、产后出血、胎儿死亡率增加、早产(21-29%)和剖宫产率升高。此外,更容易发生子宫内膜异位症,尤其是在阴道半阻塞和盆腔粘连的情况下。
早期诊断可以及时治疗,从而减少并发症。尽管如此,如果这些因素不存在,阴道分娩仍可能发生。与 Herlyn-Werner-Wunderlich 综合征相关的并发症的真实患病率和发病率仍不清楚。