(double uterus-septate vagina-renal agenesis syndrome, OHVIRA syndrome)
Prevalence: < 1.106. Complex congenital malformation of the female internal genitalia that combines a didelphic uterus, an imperforate septate vagina (cause of unilateral hematocolpos) and ipsilateral renal agenesis due to an anomaly of the mullerian remnants.
Clinical picture: usually, dysmenorrhea, pelvic pain, vaginal discharge and pelvic mass. The diagnosis is suggested on intravaginal and abdominal echography. and confirmed on laparoscopy that specifies the type of uterine malformation and evaluate the potential complications of the vaginal obstruction: endometriosis, adhesions caused by the hematocolpos or hematosalpinx, ascending inflammatory process.
Treatment: resection of the intravaginal septum (colpotomy), marsupialisation of the blind hemivagina, and hemihysterectomy to keep the controlateral hemiuterus intact and preserve the reproductive potential of the patient.
Anesthetic implications:
single kidney; check renal function
References :
- Sanghvi Y, Shastri P, Mane SB, Dhende NP.
Prepubertal presentation of Herlyn-Werner-Wunderlich syndrome : a case report.
J Pediatr Surg 2011; 46: 1277-80.
- Aveiro AC, Miranda V, Cabral AJ, Nunes S, Paulo F, Freitas C.
Herlyn-Werner-Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls.
BMJ Case reports 2011; doi 10.1136/bcr04.2011.4147
Updated: September 2017