Anatomical variations, treatment and outcomes of Herlyn-Werner-Wunderlich syndrome: a literature review of 1673 cases

Arch Gynecol Obstet. 2023 Nov;308(5):1409-1417. doi: 10.1007/s00404-022-06856-y. Epub 2023 Feb 24.

Abstract

Purpose: This study aimed to perform a systematic review of patients with Herlyn-Werner-Wunderlich syndrome (HWWS) and present the prevalence of symptoms, anatomical variants, endometriosis, surgical interventions, and short- and long-term outcomes.

Methods: A structured search was performed in PubMed, Scopus, Embase, and China National Knowledge Infrastructure, and studies published between 1 January, 2000 and 19 April, 2022 were included. The following data on HWWS were recorded: symptoms, anatomical variations, surgical interventions and short- and long-term outcomes.

Results: A total of 1673 patients were included in our analysis. The main symptoms were dysmenorrhea (53.8%), abnormal uterine bleeding (28.9%), and vaginal discharge (26.6%). The principal anomalies were right-obstructed hemivagina (57.3%), haematocolpos (81.7%), uterus didelphys (88.8%), and ipsilateral renal agenesis (93.1%). A majority of patients belonged to classification 1.1 (46.7%), with a blind hemivagina, and classification 2.1 (39.2%), with a small communication between two vaginas. The mainstay of treatment was vaginal septum excision (91.8%). Minimally invasive surgery (48.5%) was performed only after vaginal surgery (61.9%), and only a few patients required a second surgery (2.2%). Endometriosis was found in 9.6% of the patients. Fifty-two percent of them had ipsilateral ovarian endometriosis cysts. Pregnancy rate of these patients was 72.1%. The rate of adverse pregnancy outcomes was 22.4%. The caesarean section rate was 61.2%.

Conclusions: Patients with HWWS presented with nonspecific symptoms and demonstrated various combinations of anomalies. The most common anatomical variants are classifications 1.1 and 2.1. Vaginal septum excision is effective in relieving symptoms and preventing complications, with hysteroscopic surgery as an option where there is concern about protecting the hymen from minor injury. The pregnancy rate for these patients after surgery was satisfactory, and the rate of adverse pregnancy outcomes after surgery was acceptable. We advise females with urological anomalies to be screened for Müllerian anomalies because of the close association between these two types of anomalies. Thus, HWWS contributes to the occurrence of endometriosis; however, more research is required to investigate the relationship between pelvic endometriosis and HWWS.

Keywords: Anatomical variations; Herlyn–Werner–Wunderlich syndrome; Obstructed hemivagina and ipsilateral renal agenesis syndrome; Pelvic endometriosis; Pregnancy.

Publication types

  • Systematic Review
  • Review

MeSH terms

  • Abnormalities, Multiple* / epidemiology
  • Abnormalities, Multiple* / surgery
  • Cesarean Section / adverse effects
  • Endometriosis* / complications
  • Endometriosis* / surgery
  • Female
  • Humans
  • Kidney / abnormalities
  • Pregnancy
  • Treatment Outcome
  • Uterus / abnormalities
  • Uterus / surgery
  • Vagina / abnormalities
  • Vagina / surgery

Supplementary concepts

  • Uterine Anomalies