The Herlyn-Werner-Wunderlich Syndrome - A Case Report with Radiological Review

Abstract

Background: HWW syndrome is a very rare congenital anomaly of urogenital tract involving Mullerian ducts and mesonephric ducts. It is characterised by a triad of symptoms - uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. It can be classified based on a completely or incompletely obstructed hemivagina. It presents soon after menarche or shows delayed presentation depending on the type. It can exhibit acute pelvic pain shortly after menarche and may show non-specific and variable symptoms with resultant delay in diagnosis. The most common presentation is pain and dysmenorrhea, and pain and abdominal mass in the lower abdomen secondary to haematocolpos and/or haematometra.

Case report: Presentation of a clinical case of a 13-year-old patient with HWW syndrome presenting with regular menses, dysmenorrhea and painful lump in hypogastric region on the left side of midline. We described the role of imaging modalities in diagnosis of the Herlyn-Werner-Wunderlich syndrome with a review of literature. USG and MRI showed left renal agenesis with compensatory hypertrophy of the right kidney, uterus didelphys with haematometra and haematocervix in the left uterus with evidence of blood in a dilated retort-shaped left fallopian tube and a normal right uterus. The unique feature of our case is haematometra and haematocervix with cervical and vaginal atresia found on the left side (classification 1.2) with associated left renal agenesis.

Conclusions: HWW syndrome can present early or late, depending on the type. In patients with uterine and vaginal abnormalities, a work-up for associated renal anomalies should be performed. Early intervention is needed to reduce the risk of endometriosis and infertility.

Keywords: Didelphis; Hematometra; Mullerian Ducts.

Figure 1

(USG of the abdomen and pelvis) – showing an absent left kidney, uterus didelphys, haematometra in the left uterine horn.

Figure 2

(Coronal STIR abdomen and pelvis) – showing absent left kidney with mild compensatory hypertrophy of the right kidney.

Figure 3

(Sagittal T2WI pelvis) – showing normal right uterine horn, haematometra and haematocervix in the left uterine horn and haematosalpinx overlying the urinary bladder dome.

Figure 4

(T1WI and T2WI coronal pelvis) – showing uterus didelphys, haematometra in the left uterine horn, left haematosalpinx. (A, B) T1W-coronal; (C, D) T2W-coronal.

Figure 5

(Axial T1FS pelvis [T1 FATSAT]) – showing left haematosalpinx overlying the urinary bladder dome, uterus didelphys, haematometra and haematocervix in the left uterine horn, normal right uterine horn.

Figure 6

Classification 1 – Completely obstructed hemivagina. Classification 1.1 – with blind hemivagina (A). There is no communication between the duplicated uterus and vagina. Haematocolpos occurs a few months after menarche. Haemoperitoneum due to bleeding from the fallopian tube can be found at surgery [10]. If neglected, the condition can progress to secondary endometriosis, pelvic adhesions, pyosalpinx and pyocolpos [10,11]. Classification 1.2 – Cervicovaginal atresia without communicating uteri (B). Cervix behind the septum is atretic or maldeveloped. Menses from the uterus behind the septum cannot flow through atretic cervix. Hence, clinical features are similar to as in classification 1.1. Classification 2 – Incompletely obstructed hemivagina. Classification 2.1 – Partial reabsorption of the vaginal septum (C). A small communication exists between two vaginas with resultant incomplete obstruction. Patients have a late presentation. Classification 2.2, with communicating uteri (D). A small communication exists between two duplicated cervices with a completely obstructed hemivagina. Menses from the uterus behind the septum can flow through communication to the contralateral cervix. Drainage is often impeded due to small communication. Courtesy: Zhu et al. New classification of Herlyn-Werner-Wunderlich syndrome. Chin Med J, 2015; 128: 222–25.