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Case Reports
. 2024 Aug 3;18(1):361.
doi: 10.1186/s13256-024-04695-w.

Patient with Herlyn-Werner-Wunderlich syndrome and endometriosis achieves successful full-term pregnancy (40 weeks and 6 days): a case report

Juliana Vieira Queiroz Almeida Oliveira  1 , Chris Elizabeth Philip  2 , Thayná Andreza Ribeiro Pereira  3 , Gabriela Martins Perez Garcia  4 , Quésia Tamara Mirante Ferreira Villamil  4
Affiliations
Case Reports

Patient with Herlyn-Werner-Wunderlich syndrome and endometriosis achieves successful full-term pregnancy (40 weeks and 6 days): a case report

Juliana Vieira Queiroz Almeida Oliveira et al. J Med Case Rep. .

Abstract

Introduction: 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.

Case report: 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.

Conclusion: 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.

Keywords: Case reports; Herlyn–Werner–Wunderlich syndrome; Müllerian ducts; Obstructed hemivagina, ipsilateral renal anomaly; Pregnancy.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Ultrasound showing the didelphys uterus at diagnosis. In the image on the left, the right (“Corno dir”) and left horns (“Corno esq”) can be visualized, as well as the two cervical canals (“colo”) of the patient, marked in the lower region of the image. On the right, we can see the didelphic uterus, with arrows pointing to the right (“DIR”) and left (“ESQ”) uteri
Fig. 2
Fig. 2
Ultrasound showing presence of fetal heartbeat in one uterus
See this image and copyright information in PMC

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