Delayed diagnosis of Herlyn-Werner-Wunderlich syndrome with diffuse adenomyosis in bilateral horns

Abstract

The Herlyn-Werner-Wunderlich syndrome is a rare congenital disorder with uterus didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis, more common on right side. HWW syndrome usually presents at puberty with symptoms like pelvic pain, dysmenorrhea and palpable mass due to the associated hematocolpos or hematometra. Delayed presentation in adulthood as infertility can occur in case of incomplete vaginal septum. Short term complications like pyohematocolpos, pyosalpinx, or pelviperitonitis, and long-term complications, such as endometriosis, increased risk of abortion and infertility can occur. Ultrasound is the initial investigation which demonstrates uterine anomaly, renal agenesis and hematocolpos.Magnetic resonance imaging (MRI) is the imaging modality of choice which helps in confirmation of diagnosis, delineation of vaginal septum, communication between 2 cavities and associated pathologies like adenomyosis better.Vaginal septum resection is the treatment of choice for obstructed hemivagina with hematocolpos.We present you a case of 43 years female with cyclical dysmenorrhea starting few years after menarche with uterine didelphys, left sided vaginal septum with hematocolpos and left renal agenesis. Along with this, diffuse bilateral adenomyosis was present which is uncommonly associated with this anomaly as obstruction is at the level of vagina. Patient was diagnosed late due to misdiagnosis and later treated with septal resection for hematocolpos and with hormonal medications for adenomyosis.

Keywords

• Herlyn-Werner-Wunderlich syndrome
• Adenomyosis, Hemivagina
• Septoplasty