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Herlyn-Werner-Wunderlich syndrome (HWHS) is a rare de- velopmental anomaly of Mullerian ducts, characterized by the presence of a uterus didelphys with longitudinal vaginal septum obstructing the hemivagina and the presence of ip- silateral renal agenesis. It is a very rare syndrome with only a few cases that have been reported. We describe, In this report, the clinical presentation and the successful surgical management of an 11-year-old girl who presented with acute lower abdominal pain and palpable tender mass in the lower abdomen. The diagno- sis of Herlyn-Werner-Wunderlich syndrome was suspect- ed by the ultrasonography and confirmed by the Magnetic resonance imaging (MRI). Successful management was done by resection of the vaginal septum using the Lig- ature device through the hymen opening. Awareness is necessary in order to diagnose and treat this disorder properly before the occurrence of complications.

Uterus didelphys, Vaginal septum, Renal agenesis, Her- lyn-Werner-Wunderlich syndrome

The incidence of Mullerian duct anomalies in the literature is estimated to be from 0.5 to 5.0% in the general population [1 ,2]. Crosby and Hill in 1962 first- ly described the theory of the development of Mul- lerian ducts [3 ]. During the embryonic period, The ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410156 AlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156 • Page 2 of 5 • inal pain. There was no associated symptom of vomit- ing, fever or diarrhea. History revealed that the menarche had started 5 months back, the menses were regular every 30 days and the menstrual bleeding usually lasted 4-5 days. The last cycle was associated with mild lower abdominal pain. On physical examination: Patient general conditions were stable, the pulse: 84 /m.,.BP: 110/80 mg and RR 20/min. Abdominal examination revealed tenderness on palpation of the lower abdomen with a vague tender mass occupying mainly the hypogastrium and the left lower quadrant of the abdomen. The mass was tense, cystic, regular arising from the pelvis and reaching to the umbilicus. Rectal examination revealed a tense, cys- tic mass was felt anteriorly and to the left side. Vagi - nal examination showed that the vulva are apparently normals and that the posterior vaginal wall is bulged through the left side by the swelling. Routine hemato- logical and biochemical profile were normal. Abdominal ultrasonography showed 5.7 × 8.0 cm mass posterior to the bladder connected to the uter- us, filled with turbid fluid... Another uterine corn like a mass was seen along the right side of the pelvic mass. Features are suggestive of hematometra/hematocolpos with bicornuate uterus, two uterine bodies, and ab- sence of the left kidney. Pelvic MRI showed uterus Didelphis with two hemi- uteri and two hemi-vaginas, the left hemi-vagina was blind, (Figure 1), left hematometro-colpos, and hema- after menarche [10]. MOSTYN P. and EMBREY B. in 1950 described a case of obstructed Hemi-vagina and a uter- us didelphys as well as an ipsilateral renal anomaly [11]. While Herlyn and Werner in 1971 initially described the syndrome as blind hemi-vagina with ipsilateral renal agenesis [12], finally, Wunderlich in 1976 added the bi - cornuate uterus as a feature of the syndrome [13]. Nowadays, Herlyn-Werner-Wunderlich syndrome (HWW), represents a complex female genital malfor- mation with uterus didelphys, unilateral low vaginal obstruction, and ipsilateral renal agenesis. Case Presentation An 11-year-old girl, admitted to the pediatric surgery department complaining of an increasing lower abdom- Figure 1: MRI image transverse cut. LU: Left uterus horne, Dilated; LV: Left vagina, Dilated; RU: Right uterus horne; RV: Right patent hemivagina. Figure 2: MRI image coronal view. LU: Left uterus horne, Dilated (metrocolpos) >; LV: Left va- gina, Dilated (Hematocolpos); LS: Left salpinx (hematosal- pinx); RU: Right uterus horne; RV: Right vagina. Figure 3: MRI image sagital view. Showing the hemato metro-colpo-salpinx. B: Bladder; R: Rectum. ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410156 AlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156 • Page 3 of 5 • With the patient in the lithotomy position, with speculum examination, the right hem-ivagina was com- pressed but patent, while there was a bulging from the left side. A puncture was made in the lower extremity of the bulging to identify the obstructed vaginal sep- tum and aspiration was done with drainage of 200 ml of altered brown colored blood. Then the septum was incised with electrocautery, finally, The vaginal septum was widely excised until the cervix using the ligasure device. Postoperative was smooth, the patient was dis - charged on the 3 rd postoperative day. On OPD follow up, she was menstruating regularly, ul- trasonography revealed that the size of the right uterus was reduced with the disappearance of the vaginal mass.

Patient with (HWWS) usually becomes symptom- atic after menarche. Having two normal uterus and obstructed hemivagina, the patient will have regu- lar menses through the non-obstructed vaginal side which coincides with a cyclic pelvic pain (dysmenor- rhea) from the encumbered blood in the obstructed vaginal side, that leads to hematometra, hematocol- pous and retrograde menstrual flow, which explains the increased prevalence of endometriosis, pelvic ad- hesions and retention of urine in these patients [14]. A ten years review of this anomaly showed that (73%) of patients presented with dysmenorrhea, (71%) with pelvic or paravaginal mass [15]. While In our case the presenting symptom was an acute abdominal pain resembling to acute abdomen, this type of presen- tation was also reported by Aydin R who stated that the acute abdomen presentation is uncommon [16]. Lan Zhu, et al. proposed New Classification of Herlyn-Werner-Wunderlich Syndrome based on a complete or incomplete obstructed hemivagina [17] according to this classification, the hemivagina in our case was completely obstructed. Diagnosis of HWWS is usually confirmed by so- nography and magnetic resonance imaging (MRI). So- nography can detect the pelvic cystic mass and may detect the uterine malformations, however Some au- thors now consider MRI as the gold standard for the diagnosis, as it provides more detailed information regarding the uterine contour, the shape of the in- trauterine cavity and its continuity with each vaginal lumen, the character of the septum, and the nature of the fluid content, as well as the presence of the associated aspects such as endometriosis, or renal anomalies [18, 19]. In our case, the ultrasonography was done in an emergency base, and then the MRI images clarified the exact uterine abnormalities and made the diagnosis of the case clear. Many associated Urological malformations have been described with HWWS including, renal duplica- tion, multicystic dysplastic kidney and renal agene - tosalpinx overlying the dome of the urinary bladder (Figure 2 and Figure 3), Also, it showed left renal agen- esis (Figure 4). The presence of associated renal agenesis conformed the diagnosis of Herlyn-Werner-Wunderlich (HWW) syndrome. The condition was explained to the family and the decision was made to perform a laparoscopic exploration followed by a vaginal exam under anesthe- sia followed by resection of the obstructed vaginal sep- tum. Laparoscopic exploration showed the uterus Di - delphis, the left-sided uterus and the left tube were sig- nificantly dilated, the right-sided uterus, tube, and both ovaries were normals (Figure 5) laparoscopic Peritoneal toilet with aspiration removal of the accumulated blood from the pelvis. Figure 4: MRI image coronal view. Demonstrating the absence of left kidney. Figure 5: Laparoscopic view. RU: Right uterus horne; LU: Left uterus horne; V: Vagina; B: Blood in the peritoneal cavity. ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410156 AlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156 • Page 4 of 5 • hysteroscopic treatment results. Human Reproduction Up- date 7: 161-174. 3. Crosby WM, Hill EC (1962) Embryology of the mullerian duct system. Review of present-day theory. Obstet Gyne- col. 4. Chung DH, Townsend CM, Beauchamp RD, Evers BM, Mattox KL (2012) Sabiston. Textbook of Surgery. (19 th edn), Elsevier Saunders, Philadelphia 1849. 5. Moawad NS, Mahajan ST, Moawad SA, Greenfield M (2009) Uterus didelphys and longitudinal vaginal septum coincident with an obstructive transverse vaginal septum. J Pediatr Adolesc Gynecol. 6. De Silve PHDH (1976) Rudimentary horn of a bicornuate uterus: Discussion of 16 cases with a review of literature. J National SciCoun Sri Lanka 4: 55-73. 7. Gray SW, Skandalakis JE, Broecker BH (1994) Female re- productive system. In: Skandalakis JE, Gray SW, Embryol- ogy for surgeons. (2 nd edn), Lippincott Williams & Wilkins, Baltimore 816-847. 8. Cox D, Ching BH (2012) Herlyn-Werner-Wunderlich syn- drome: A rare presentation with pyocolpos. J Radiol Case Rep 6: 9-15. 9. Smith NA, Laufer MR (2007) Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: Manage - ment and follow-up. Fertility and Sterility 87: 918-922. 10. Purslow CE (1922) A case of unilateral haematocolpos, haematometra and haematosalpinx. J Obstet Gyaecol Br Emp 29: 64. 11. Mostyn P, Embrey B (1950) A case of uterus didelphys with unilateral gynatresia. Br Med J. 12. Herlyn U, Werner H (1971) Simultaneous occurrence of an open Gartnerduct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities. Geburtshife Frauenheikd 31: 340-347. 13. Wunderlich M (1976) Unusual form of genital malformation with aplasia of the right kidney. Zentralbl Gynakol 98: 559- 562. 14. PJ Woodward, R Sohaey, TP Mezzetti Jr (2001) Endome - triosis: Radiologic-pathologic correlation. Radio-Graphics 21: 193-216. 15. Phupong V, Pruksananonda K, Taneepanichskul S, Tresu- kosol D, Virutamasen P (2000) Double uterus with unilater- ally obstructed hemivagina and ipsilateral renal agenesis: A variety presentation and a 10-year review of the literature. J Med Assoc Thai 83: 569-574. 16. Aydin R, Ozdemir AZ, Ozturk B, Bilgici MC, Tosun M (2014) A rare cause of acute abdominal pain: Herlyn-Werner-Wun- derlich syndrome. Pediatr Emerg Care 30: 40-42. 17. Lan Zhu, Na Chen, Jia-Li Tong, Wei Wang, Lei Zhang, et al. (2015) New classification of herlyn-wernerwunderlich syn- drome. Chin Med J (Engl) 128: 222-225. 18. Epelman M, Dinan D, Gee MS, Servaes S, Lee EY, et al. (2013) Mullerian duct and related anomalies in children and adolescents. Magn Reson Imaging Clin N Am 21: 773-789. 19. Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazza- to RL, et al. (2012) Herlyn-Werner-Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging 12: 4. 20. Li S, Qayyum A, Coakley FV, Hricak H (2000) Association of renal agenesis and mullerian duct anomalies. J Comput Assist Tomogr 24: 829-834. sis which is the most commonly reported associated urologic anomaly [20, 21]. In our case the absence of the right kidney in association with the other abnor- malities have confirmed the diagnosis of HWWS. The optimal treatment in such case is full excision and marsupialization of the obstructing vaginal sep- tum allowing both uteri to drain through the patent vagina [22]. In our case we have performed a full ex- cision of the vaginal septum using a ligature devise which could be introduced through the hymen open- ing without disturbing its integrity, which is an im- portant factor for the community. Fedele L, et al. in a report of 87 cases of HWWS revealed that 72.4% of cases were of classic variety and 27.6% of cases were variants of uterine septum or cervical agenesis [23]. In this type of variety of the syndrome, where the unilateral obstruction is at the level of cervix, Himadri Bal, performed Unilateral sub- total hysterectomy with salpingectomy [24]. Laparoscopy may not be mandatory in the treat- ment of HWWS, but it could be helpful in confirming the diagnosis when radiological imaging is inconclu- sive. Also, laparoscopy is useful in the diagnosis and treatment of endometriotic lesions and to release pelvic adhesions resulting from retrograde menstru- ation from the obstructed hemivagina [25], in our case the laparoscopy was useful to aspirate the in- traperitoneal retrograde menstruation which was ac- cumulated in the peritoneal cavity, and cleaning the abdominal cavity aiming to prevent future formation of endometriosis. The potential complications of this syndrome are pyohematocolpos or pyosalpinx, which may lead to pel- vic peritonitis. While long-term complications are en- dometriosis, pelvic adhesions leading to chronic pelvic pain, and infertility. Which require early diagnosis and treatment of this syndrome in order to avoid theses complications and to preserve the fertility [26]. Where- fore patient affected by (HWWS) needs further follow up to predict these possible complications.

Herlyn-Werner-Wunderlich syndrome is a rare urogenital congenital anomaly. The aim of reporting this case is to highlight the entity and the importance of early management to relieve the symptoms and to prevent the complications, since timely diagnosis re- quires a high index of clinical suspicion and radiolog- ical confirmation.

1. Heinonen PK (1984) Uterus didelphys: A report of 26 cas- es. European Journal of Obstetrics & Gynecology and Re- productive Biology 17: 345-350. 2. Grimbizis GF, Camus M, Tarlatzis BC, Bontis JN, Devroey P (2001) Clinical implications of uterine malformations and ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410156 AlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156 • Page 5 of 5 • 24. Himadri Bal, BS Duggal, Nikita Gonnade, Sanjay Khalad - kar (2017) Herlyn-Werner-Wunderlich syndrome. Medical Journal of Dr. D.Y. Patil University. 25. Moon Hyeong Cho, Da Hyun Kim, Jung Mi Byun, Young Nam Eun Jung Jung, Kim DaeHoonJeong, et al. (2017) Case Report Herlyn-Werner-Wunderlich syndrome: An un- usual presentation with pyocolpos. Obstet Gynecol Sci 60: 374-377. 26. Kabiri D, Arzy Y, Hants Y (2013) Herlyn-Werner-Wunder - lich syndrome: Uterus didelphys and obstructed hemivagi - na with unilateral renal agenesis. Isr Med Assoc J 15: 66. 21. Vercellini P, Daguati R, Somigliana E, Vigano P, Lanzani A, et al. (2007) Asymmetric lateral distribution of obstructed hemivagina and renalagenesis in women with uterus didel- phys: Institutional case series and a systematic literature review. Fertility Sterility 87: 719-724. 22. Jindal G, Kachhawa S, Meena GL, Dhakar G (2009) Uterus didelphys with unilateral obstructed hemivagina with he- matometrocolpos and hematosalpinx with ipsilateral renal agenesis. J Hum Reprod Sci 2. 23. Fedele L, Motta F, Frontino G, Restelli E, Bianchi S (2013) Double uterus with obstructed hemivagina and ipsilateral renal agenesis: Pelvic anatomic variants in 87 cases. Hum Reprod 28: 1580-1583.