European Journal of Cardiovascular Medicine

Herlyn-Werner-Wunderlich (HWW) syndrome is rare congenital anomaly of Mullerian and Mesonephric ducts characterised by triad of uterinedidelphys, obstructed hemivagina and ipsilateral renal agenesis.1,5,6 It is also known as obstructed  hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Etiology is unknown but may be caused by developmental abnormality of Mullerian and wolfian ducts.2,3 Incidence is 0.1%–3.8%.2It is classified into complete and incompletely obstructed hemivagina. Symptoms may be apparent soon after menarche or later depending upon the type of malformation. Completely obstructed hemivagina may present shortly after menarche with acute pelvic pain. Where as in cases of incompletely obstructed hemivagina symptoms are nonspecific & variable resulting in delay in diagnosis

A 38 years old, Nulliparous women came to our obstetrics and Gynaecology outpatient department with a chief complaint of primary infertility. She attained menarche at   the age of 14years and had, regular menstrual cycles since then, with dysmenorrhea throughout the cycle. Her marital life was of 8years, non consanguineous marriage. Patient also has history of dyspareunia & no other complaints. She was known case of Diabetes mellitus since 6 years and on maintenance medication with GLUCORIS MP2 and was compliant. Family history was not significant.

On local examination, external genitalia was normal. On per speculum examination small cervix deviated to left side flushed with vagina, with partial longitudinal vaginal septum noted on the right side of the cervix, with blind

vaginal ending on right side. On per vaginal examination small cervix felt on left side and on right side per vaginal examination was difficult to invaginate fingers because of septum and blind ending of vagina.

Initial investigations for primary infertility were done, Human semen analysis of the partner was done and showed within normal parameters. Patient ultrasound picture showed non visualization /absent Right kidney. Uterus showing ? bicornuate, anteverted, normal in size with ET – 5.7mm. Both ovaries are normal in size and echotexture. MRI showing uterine didelphys with, right renal agenesis.

Figure 1

Figure 2

CORONAL SECTIONS:

FIGURE 1. Showing FUNDUS OF UTERUS showing didelphys

FIGURE 2. Showing cervix and vagina

Figure 3- AXIAL VIEW OF UTERUS showing didelphys

Diagnostic hysterolaparoscopy was done showing uterus with broad fundus noted with mild dimpling in the centre. Bilateral ovaries normal. Left side fallopian tube was normal. Right fallopian tube is long and torturous, fimbrial end couldn’t found, end could not be traced, burrying in to retroperitoneum.. Chromopertubation (CPT) done through the visible left sided cervical OS shown spillage at left fallopian tube, no spillage on right fallopian tube.

Mullerian duct anomalies, classified by Buttram and Gibbons, encompass conditions like uterine didelphys, resulting from inadequate fusion or developmental issues of the Mullerian ducts during fetal development. These anomalies often coincide with abnormalities in the mesonephric duct. When Wolffian ducts are absent on one side, the corresponding kidney and ureter fail to form. In uterine didelphys, this absence can displace the Mullerian ducts laterally, leading to incomplete fusion with the urogenital sinus and potentially causing obstructed hemivagina. Lan Zhu et al. proposed a classification based on the degree of vaginal septum obstruction. Clinical manifestations differ between type 1 (completely obstructed hemivagina) and type 2 (incompletely obstructed hemivagina).

This is a very rare syndrome having difficulty in diagnosis, though battery of investigations applied for the case that suggest Diagnostic Conundrum. Early detection and intervention may help such case to have successful obstetric carrier.

1. Herlyn-Werner-Wunderlich Syndrome with Ureteric Remnant Abscess Managed Laparoscopically: A Case Report.Meneses AD, Filho WMNE, Raulino DMR, Martins EBL, Vieira SC. Oman Med J. 2017 Mar;32(2):157-160.
2. Tridenti G, Armanetti M, Flisi M, Benassi L. Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis in teenagers: Report of three cases. Am J Obstet Gynecol. 1988; 159:882–3.
3. Burgis J. Obstructive Mullerian anomalies: Case report, diagnosis, and management. Am J Obstet Gynecol. 2001; 185:338–44.
4. Lan Zhu, Na Chen, Jia- Li Tong, Wei Wang, Lei Zhang, Jing-He Lang, Classification of Herlyn-Werner-Wunderlich Syndrome.Chin Med J (Engl) 2015 Jan 20; 128(2): 222–225.
5. Chandler TM, Machan LS, Cooperberg PL, Harris AC, Chang SD. Mullerian duct anomalies: from diagnosis to intervention. Br J Radiol 2009. Dec;82(984):1034-1042.
6. Herlyn U, Werner H. Simultaneous occurrence of an open Gartner-duct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities. Geburtshilfe Frauenheilkd. 1971; 31:340–