Clinical characteristics of 70 patients with Herlyn-Werner-Wunderlich syndrome

Int J Gynaecol Obstet. 2013 May;121(2):173-5. doi: 10.1016/j.ijgo.2012.11.023. Epub 2013 Mar 1.

Abstract

Objective: To analyze the clinical characteristics of patients with Herlyn-Werner-Wunderlich (HWW) syndrome.

Methods: Seventy patients with a confirmed diagnosis of HWW syndrome admitted to the Peking Union Medical College Hospital between January 1995 and December 2010 were retrospectively reviewed, and long-term follow-up data were analyzed.

Results: The lateral distribution of hemivaginal obstruction and renal agenesis favored the right side in 42 patients (60.0%). Age at onset of symptoms was significantly different between patients with complete and those with incomplete hemivaginal obstruction (12.86 ± 1.84 vs 20.68 ± 7.43 years; P<0.05). The primary gynecologic complaint was dysmenorrhea, which occurred in 45 (64.3%) patients. Endometriosis was observed in 12 (17.1%) patients, and 14 (20.0%) patients with hemivaginal obstruction had acute pelvic inflammation history. All patients underwent vaginal septectomy. There were 52 pregnancies among 28 (84.8%) of the 33 women who wished to conceive.

Conclusion: The clinical presentation of patients with HWW syndrome was significantly different between those with complete and those with incomplete hemivaginal obstruction. Full resection of the vaginal septum resulted in good outcomes.

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Child
  • Congenital Abnormalities / pathology*
  • Dysmenorrhea / epidemiology
  • Dysmenorrhea / etiology
  • Endometriosis / epidemiology
  • Endometriosis / etiology
  • Female
  • Follow-Up Studies
  • Humans
  • Kidney / abnormalities
  • Kidney / pathology
  • Kidney Diseases / congenital*
  • Kidney Diseases / pathology
  • Middle Aged
  • Pregnancy
  • Retrospective Studies
  • Syndrome
  • Treatment Outcome
  • Uterus / abnormalities*
  • Vagina / abnormalities*
  • Vagina / surgery
  • Young Adult

Supplementary concepts

  • Hereditary renal agenesis