Herlyn Werner Wünderlich syndrome. Case report
Keywords:
Herlyn-Werner-Wünderlich syndrome, renal agenesis, uterus didelphys, hemivagina, uterine septumAbstract
Herlyn-Werner-Wünderlich is a syndrome characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. It is an uncommon müllerian ducts congenital anomaly, affecting the urogenital tract because of the close embryonic origin of the urogenital system. Its diagnosis is of late onset because symptoms appear after the menarche and the intensity of the symptoms varies among patients. Treatment is based on a proper and timely diagnosis, as well as a surgical correction of the defects. A resection of the uterine septum generally improves the symptoms and creates a favorable future prognosis, making an intrauterine pregnancy possible. We present a case of a 22-year old female patient with delayed diagnosis of Herlyn-Werner-Wünderlich syndrome, with no hemivaginal septum, whose chief complaints were dysmenorrhea and recurring purulent vaginal discharge despite unsuccessful surgical interventions. A mini laparotomy Strassman metroplasty was performed, resolving her symptomatology.
Downloads
References
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):222-225.
Osornio-Sánchez V, Santana-Ríos Z, Fulda-Graue S, Pérez Becerra R, Urdiales-Ortiz A, Martínez A, et al. Síndrome de Herlyn Werner Wünderlich. Revisión de la literatura y reporte de caso. Rev Mex Urol 2012;72(1):31-34.
Angotti R, Molinaro F, Bulotta AL, Bindi E, Cerchia E, Sica M, et al. Herlyn-Werner-Wunderlich syndrome: An "early" onset case report and review of Literature. Int J Surg Case Rep. 2015;11:59-63.
Cox D, Ching BH. Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos. J Radiol Case Rep. 2012;6(3):9-15.
Mehra S, Chamaria K, Garga UC, Kataria A, Ahuja A. Imaging Diagnosis of Herlyn-Werner-Wunderlich Syndrome- An Extremely Rare Urogenital Anomaly. J Clin Diagn Res 2015;9(5):TD06-8.
Herlyn U, Werner H. Simultaneous occurrence of an open Gartnerduct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities. Geburtshilfe Frauenheilkd 1971;31(4):340-347.
Wunderlich M. Unusual form of genital malformation with aplasia of the right kidney. Zentralbl Gynakol. 1976;98(9):559-562.
Piccinini PS, Doski J. Herlyn-Werner-Wunderlich syndrome: a case report. Rev Bras Ginecol Obstet 2015;37(4):192-196.
Aydin R, Ozdemir AZ, Ozturk B, Bilgici MC, Tosun M. A Rare Cause of Acute Abdominal Pain. Pediatric Emergency Care 2014;30(1):40-42.
Moore K, Persaud T. Embriología Clínica. 7th edition ed. Spain: Elsevier; 2004.
The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions. Fertil Steril 1988;49(6):944-945.
Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato RL, Sansoni I, et al. Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging 2012;12:4.
Bravo A, Correa M, San Martín N. Síndrome de Herlyn-Werner- Wunderlich. Reporte de un caso y revisión de la literatura. REMS. 2010;6(1):24-28.
Schorge J, Schaffer J, Halvorson L, Hoffman B, Bradshaw K, Cunningham F. Williams Ginecología, 1st edition. McGraw-Hill. Mexico. 2009.
Smith N, Laufer M. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007;87(4):918-922.
Tong J, Zhu L, Lang J. Clinical characteristics of 70 patients with Herlyn-Werner-Wunderlich syndrome. Int J Gynaecol Obstet 2013;121(2):173-175.
Sharma D, Janu MK, Gaikwad R, Usha MG. Herlyn-Werner- Wunderlich Syndrome Complicated with Pyocolpos: An Unusual Cause of Postabortal Sepsis. J Gynecol Endosc Surg 2011;2(2):105- 108.
Nabeshima H, Nishimoto M, Shiga N, Utsunomiya H, Yaegashi N. Laparoscopic Strassman metroplasty in a postmenarcheal adolescent girl with Herlyn-Werner-Wunderlich mullerian anomaly variant, obstructed noncommunicating didelphic uterus without gartner duct pseudocyst. J Minim Invasive Gynecol 2013;20(2):255-2558.
Sheih C-P, Li Y-W, Huang T-S, Liao Y-J, Chen W-J. Spontaneous Rupture of the Uterovaginal Septum in 2 Girls with Unilateral Hematocolpos and Ipsilateral Vaginal Ectopic Ureter. The Journal of Urology 2000;163(6):1947-1948.
Holloway J, Jehle J, Hudson H, Wilson C. Uterus didelphys with unilateral imperforate vagina: spontaneous rupture of the vaginal septum. Obstet Gynecol 1980;55(3):50S-1S.
Dorais J, Milroy C, Hammoud A, Chaudhari A, Gurtcheff S, Peterson CM. Conservative treatment of a Herlyn-Werner- Wunderlich mullerian anomaly variant, noncommunicating hemiuterus with Gartner duct pseudocyst. J Minim Invasive Gynecol 2011;18(2):262-266.
Downloads
Published
Issue
Section
License
Copyright (c) 2016 Jaime Acosta, Jorge García, Fabricio Macías, Ivan Miranda, Marco Tapia, Danilo Acosta
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
