ATYPICAL PRESENTATION AND DELAYED DIAGNOSIS OF HERLYN-WERNER-WUNDERLICH SYNDROME: A CASE REPORT AND LITERATURE REVIEW

Aruna Rangasamy, Marvinash Rao, Aruku Naidu Apana

Abstract


Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare and complex Mullerian duct abnormality that is diagnostically challenging. It is also known as OHVIRA syndrome. The triad of uterine didelphys, obstructed hemivagina and ipsilateral renal anomaly characterises the syndrome. A 25-year-old lady was referred with prolonged foul-smelling vaginal discharge. Her periods were regular with normal flow. A vaginal examination revealed a normal cervix and fullness of left adnexa. A pelvic ultrasound showed a left adnexal mass. Computed tomography imaging of the abdomen and pelvis revealed two uterine cavities with a large cystic lesion at the level of the cervix. This lesion was communicating with the uterine cavity, raising the possibility of obstruction. Only the right kidney was present. A diagnosis of HWWS was made. The patient underwent diagnostic laparoscopy, examination under anaesthesia and excision of the vaginal septum. At follow-up, she remained well at 6 weeks and 3 months. The normal mesonephric duct plays an important role in the formation of the uterus, vagina and the upper urinary tract. HWWS occurs when one of the mesonephric ducts is absent or injured during embryogenesis. Women with HWWS usually present after menarche with varied symptoms, causing delays in diagnosis and treatment. Therefore, having a high suspicion index and awareness of Mullerian duct abnormalities is crucial. The gold standard for the evaluation of HWWS is magnetic resonance imaging. Surgical excision of the septum is the advocated treatment of choice. Prompt intervention lowers complications that impair fertility.

Keywords


Herlyn-Werner-Wunderlich syndrome; Mullerian duct abnormality; OHVIRA; Renal agenesis; Uterus didelphys

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References


Burgis J. Obstructive Müllerian anomalies’: Case report, diagnosis, and management. Am J Obstet Gynecol. 2001;185(2):338–44.

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 Apr;31(4):340–7.

Wunderlich M. [Unusual form of genital malformation with aplasia of the right kidney]. Zentralbl Gynakol. 1976;98(9):559–62.

Acien P, Acien M. Malformations of the Female Genital Tract and Embryological Bases. Curr Womens Health Rev. 2013;3(4):248–88.

Rock JA, Jones HW. The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Obstet Gynecol. 1980;138(3):339–42.

Zhu L, Chen N, Tong JL, Wang W, Zhang L, Lang JH. New classification of herlyn-werner-wunderlich syndrome. Chin Med J (Engl). 2015;128(2):222–5.

Khaladkar SM, Kamal V, Kamal A, Kondapavuluri SK. The Herlyn-Werner-Wunderlich syndrome – A case report with radiological review. Pol J Radiol. 2016;81:395–400.

Ahmad Z, Goyal A, Das C, Deka D, Sharma R. Herlyn-Werner-Wunderlich syndrome presenting with infertility: Role of MRI in diagnosis. Indian J Radiol Imaging. 2013;23(3):243–6.

Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril. 2007;87(4):918–22.

Cheng C, Subedi J, Zhang A, et al. Vaginoscopic Incision of Oblique Vaginal Septum in Adolescents with OHVIRA Syndrome. Sci Rep. 2019 Dec 1;9(1):20042.

Altchek A, Paciuc J. Successful Pregnancy Following Surgery in the Obstructed Uterus in a Uterus Didelphys with Unilateral Distal Vaginal Agenesis and Ipsilateral Renal Agenesis: Case Report and Literature Review. J Pediatr Adolesc Gynecol. 2009;22(5):e159-62.

Vo Nhu Q, Le Trong B, Nguyen Thanh T. Herlyn-Werner-Wunderlich syndrome: A report of three cases in adolescents and adult woman. Radiol Case Rep. 2021;16(9):2792–8.

Venetis CA, Papadopoulos SP, Campo R, Gordts S, Tarlatzis BC, Grimbizis GF. Clinical implications of congenital uterine anomalies: a meta-analysis of comparative studies. Reprod Biomed Online. 2014 Dec;29(6):665–83.

Heinonen PK. Pregnancies in women with uterine malformation, treated obstruction of hemivagina and ipsilateral renal agenesis. Arch Gynecol Obstet. 2013;287(5):975–8.

Heinonen PK. Clinical implications of the didelphic uterus: Long-term follow-up of 49 cases. Eur J Obstet Gynecol Reprod Biol. 2000;91(2):183–90.




DOI: http://dx.doi.org/10.17576/JSA.2024.1402.05

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