{"paper_id":"12e2d7d1-fcf5-46ff-9087-f0af8f6ea663","body_text":"Obstetrics and \nGynaecology Cases - Reviews\nAlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156\nVolume 6 | Issue 6\nDOI: 10.23937/2377-9004/1410156\nISSN: 2377-9004\nOpen Access\nAlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156\n• Page 1 of 5 •\nCitation: AlZaiem M, Alatili AA, Alawi A, Zaiem F (2019) Herlyn-Werner-Wunderlich Syndrome: A Rare \nCause of Acute Abdomen in Adolescent Girls. Obstet Gynecol Cases Rev 6:156. doi.org/10.23937/2377-\n9004/1410156\nAccepted: December 28, 2019: Published: December 30, 2019\nCopyright: © 2019 AlZaiem M, et al. This is an open-access article distributed under the terms of the \nCreative Commons Attribution License, which permits unrestricted use, distribution, and reproduction \nin any medium, provided the original author and source are credited.\nHerlyn-Werner-Wunderlich Syndrome: A Rare Cause of Acute \nAbdomen in Adolescent Girls\nMaher AlZaiem, MD, CU, ABPS1*, Abdulnaser Ali Alatili, MD2, Ahmed Alawi, SBPS3 and Fadi Zaiem, \nMBBS4\nMullerian ducts (paramesonephric) develop from \nthe coelomic epithelium and grow caudally along the \nWolffian ducts (mesonephric) toward the urogenital \nsinus forming the two uterovaginal canals. At the 11 \nweeks of gestation, the Mullerian ducts fused later-\nally to form a single canal which becomes the uterus \nand the upper tow third of the vagina [4 ]. Meanwhile, \nthe sino-vaginal bulbs invaginate from the urogenital \nsinus and meet the caudal end of the fused Mullerian \nducts to form the vaginal plate. Finally, the vaginal \nplate is reabsorbed and being canalized to form the \nlower part of the vagina. This process of resorption \nis completed by the 24 weeks of gestation. Defective \nof fusion, or failure of resorption of the inferior por-\ntions of the Müllerian ducts during early embryolog-\nical life results in uterus didelphys (double uterus) \nor septated uterus. The vagina may be septated as \nwell [5 ,6]. Uterus Didelphys occurred in about 0.16% \nof fertile women [7 ]. The coincidence of Renal agen-\nesis on the same side of the obstructed vagina can \nbe explained by an embryologic arrest at 8 weeks of \npregnancy, simultaneously affecting the two neigh-\nbors: The Müllerian (parameso nephric) and Wolffian \n(metanephric) ducts [8 ,9].\nPurslow CE in 1922, firstly described a case of a \nyoung woman with regular menstruation had gradually \nincreasing pelvic pain and appearance of a pelvic mass \n*Corresponding author: Dr. Maher AlZaiem, Consultant Pediatric Surgeon, Maternity and Children Hospital, Mecca, PO \nBOX: 12955, Saudi Arabia, Tel: +966598759063\n1Consultant Pediatric Surgeon, Maternity and Children Hospital, Mecca, Saudi Arabia \n2Pediatric Surgeon, Maternity and Children Hospital, Mecca, Saudi Arabia\n3Consultant Pediatric Surgeon, KFAFH, Jeddah, Saudi Arabia\n4Maternity and Children Hospital, Mecca, Saudi Arabia\nCASe RePoRt\nCheck for\nupdates\nAbstract\nHerlyn-Werner-Wunderlich syndrome (HWHS) is a rare de-\nvelopmental anomaly of Mullerian ducts, characterized by \nthe presence of a uterus didelphys with longitudinal vaginal \nseptum obstructing the hemivagina and the presence of ip-\nsilateral renal agenesis. It is a very rare syndrome with only \na few cases that have been reported.\nWe describe, In this report, the clinical presentation and \nthe successful surgical management of an 11-year-old \ngirl who presented with acute lower abdominal pain and \npalpable tender mass in the lower abdomen. The diagno-\nsis of Herlyn-Werner-Wunderlich syndrome was suspect-\ned by the ultrasonography and confirmed by the Magnetic \nresonance imaging (MRI). Successful management was \ndone by resection of the vaginal septum using the Lig-\nature device through the hymen opening. Awareness is \nnecessary in order to diagnose and treat this disorder \nproperly before the occurrence of complications.\nKeywords\nUterus didelphys, Vaginal septum, Renal agenesis, Her-\nlyn-Werner-Wunderlich syndrome\nIntroduction\nThe incidence of Mullerian duct anomalies in the \nliterature is estimated to be from 0.5 to 5.0% in the \ngeneral population [1 ,2]. Crosby and Hill in 1962 first-\nly described the theory of the development of Mul-\nlerian ducts [3 ]. During the embryonic period, The \n\nISSN: 2377-9004\nDOI: 10.23937/2377-9004/1410156\nAlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156\n• Page 2 of 5 •\ninal pain. There was no associated symptom of vomit-\ning, fever or diarrhea.\nHistory revealed that the menarche had started 5 \nmonths back, the menses were regular every 30 days \nand the menstrual bleeding usually lasted 4-5 days. The \nlast cycle was associated with mild lower abdominal \npain.\nOn physical examination: Patient general conditions \nwere stable, the pulse: 84 /m.,.BP: 110/80 mg and RR \n20/min. Abdominal examination revealed tenderness \non palpation of the lower abdomen with a vague tender \nmass occupying mainly the hypogastrium and the left  \nlower quadrant of the abdomen. The mass was tense, \ncystic, regular arising from the pelvis and reaching to \nthe umbilicus. Rectal examination revealed a tense, cys-\ntic mass was felt anteriorly and to the left side. Vagi -\nnal examination showed that the vulva are apparently \nnormals and that the posterior vaginal wall is bulged \nthrough the left side by the swelling. Routine hemato-\nlogical and biochemical profile were normal.\nAbdominal ultrasonography showed 5.7 × 8.0 cm \nmass posterior to the bladder connected to the uter-\nus, filled with turbid fluid... Another uterine corn like a \nmass was seen along the right side of the pelvic mass. \nFeatures are suggestive of hematometra/hematocolpos \nwith bicornuate uterus, two uterine bodies, and ab-\nsence of the left kidney.\nPelvic MRI showed uterus Didelphis with two hemi-\nuteri and two hemi-vaginas, the left hemi-vagina was \nblind, (Figure 1), left hematometro-colpos, and hema-\nafter menarche [10]. MOSTYN P. and EMBREY B. in 1950 \ndescribed a case of obstructed Hemi-vagina and a uter-\nus didelphys as well as an ipsilateral renal anomaly [11]. \nWhile Herlyn and Werner in 1971 initially described the \nsyndrome as blind hemi-vagina with ipsilateral renal \nagenesis [12], finally, Wunderlich in 1976 added the bi -\ncornuate uterus as a feature of the syndrome [13].\nNowadays, Herlyn-Werner-Wunderlich syndrome \n(HWW), represents a complex female genital malfor-\nmation with uterus didelphys, unilateral low vaginal \nobstruction, and ipsilateral renal agenesis.\nCase Presentation\nAn 11-year-old girl, admitted to the pediatric surgery \ndepartment complaining of an increasing lower abdom-\n \nFigure 1: MRI image transverse cut. \nLU: Left uterus horne, Dilated; LV: Left vagina, Dilated; RU: \nRight uterus horne; RV: Right patent hemivagina.\n \nFigure 2: MRI image coronal view. \nLU: Left uterus horne, Dilated (metrocolpos) >; LV: Left va-\ngina, Dilated (Hematocolpos); LS: Left salpinx (hematosal-\npinx); RU: Right uterus horne; RV: Right vagina.\n \nFigure 3: MRI image sagital view.\nShowing the hemato metro-colpo-salpinx.\nB: Bladder; R: Rectum.\n\nISSN: 2377-9004\nDOI: 10.23937/2377-9004/1410156\nAlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156\n• Page 3 of 5 •\nWith the patient in the lithotomy position, with \nspeculum examination, the right hem-ivagina was com-\npressed but patent, while there was a bulging from the \nleft side. A puncture was made in the lower extremity \nof the bulging to identify the obstructed vaginal sep-\ntum and aspiration was done with drainage of 200 ml \nof altered brown colored blood. Then the septum was \nincised with electrocautery, finally, The vaginal septum \nwas widely excised until the cervix using the ligasure \ndevice. Postoperative was smooth, the patient was dis -\ncharged on the 3\nrd postoperative day.\nOn OPD follow up, she was menstruating regularly, ul-\ntrasonography revealed that the size of the right uterus \nwas reduced with the disappearance of the vaginal mass.\nDiscussion\nPatient with (HWWS) usually becomes symptom-\natic after menarche. Having two normal uterus and \nobstructed hemivagina, the patient will have regu-\nlar menses through the non-obstructed vaginal side \nwhich coincides with a cyclic pelvic pain (dysmenor-\nrhea) from the encumbered blood in the obstructed \nvaginal side, that leads to hematometra, hematocol-\npous and retrograde menstrual flow, which explains \nthe increased prevalence of endometriosis, pelvic ad-\nhesions and retention of urine in these patients [14]. \nA ten years review of this anomaly showed that (73%) \nof patients presented with dysmenorrhea, (71%) with \npelvic or paravaginal mass [15]. While In our case the \npresenting symptom was an acute abdominal pain \nresembling to acute abdomen, this type of presen-\ntation was also reported by Aydin R who stated that \nthe acute abdomen presentation is uncommon [16].\nLan Zhu, et al. proposed New Classification of \nHerlyn-Werner-Wunderlich Syndrome based on a \ncomplete or incomplete obstructed hemivagina [17] \naccording to this classification, the hemivagina in our \ncase was completely obstructed.\nDiagnosis of HWWS is usually confirmed by so-\nnography and magnetic resonance imaging (MRI). So-\nnography can detect the pelvic cystic mass and may \ndetect the uterine malformations, however Some au-\nthors now consider MRI as the gold standard for the \ndiagnosis, as it provides more detailed information \nregarding the uterine contour, the shape of the in-\ntrauterine cavity and its continuity with each vaginal \nlumen, the character of the septum, and the nature \nof the fluid content, as well as the presence of the \nassociated aspects such as endometriosis, or renal \nanomalies [18, 19]. In our case, the ultrasonography \nwas done in an emergency base, and then the MRI \nimages clarified the exact uterine abnormalities and \nmade the diagnosis of the case clear.\nMany associated Urological malformations have \nbeen described with HWWS including, renal duplica-\ntion, multicystic dysplastic kidney and renal agene -\ntosalpinx overlying the dome of the urinary bladder \n(Figure 2 and Figure 3), Also, it showed left renal agen-\nesis (Figure 4).\nThe presence of associated renal agenesis conformed \nthe diagnosis of Herlyn-Werner-Wunderlich (HWW) \nsyndrome. The condition was explained to the family \nand the decision was made to perform a laparoscopic \nexploration followed by a vaginal exam under anesthe-\nsia followed by resection of the obstructed vaginal sep-\ntum. Laparoscopic exploration showed the uterus Di -\ndelphis, the left-sided uterus and the left tube were sig-\nnificantly dilated, the right-sided uterus, tube, and both \novaries were normals (Figure 5) laparoscopic Peritoneal \ntoilet with aspiration removal of the accumulated blood \nfrom the pelvis.\n \nFigure 4: MRI image coronal view.\nDemonstrating the absence of left kidney.\n \nFigure 5: Laparoscopic view. \nRU: Right uterus horne; LU: Left uterus horne; V: Vagina; \nB: Blood in the peritoneal cavity.\n\nISSN: 2377-9004\nDOI: 10.23937/2377-9004/1410156\nAlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156\n• Page 4 of 5 •\nhysteroscopic treatment results. Human Reproduction Up-\ndate 7: 161-174.\n3. Crosby WM, Hill EC (1962) Embryology of the mullerian \nduct system. Review of present-day theory. Obstet Gyne-\ncol.\n4. Chung DH, Townsend CM, Beauchamp RD, Evers BM, \nMattox KL (2012) Sabiston. Textbook of Surgery. (19\nth edn), \nElsevier Saunders, Philadelphia 1849.\n5. Moawad NS, Mahajan ST, Moawad SA, Greenfield M \n(2009) Uterus didelphys and longitudinal vaginal septum \ncoincident with an obstructive transverse vaginal septum. J \nPediatr Adolesc Gynecol.\n6. De Silve PHDH (1976) Rudimentary horn of a bicornuate \nuterus: Discussion of 16 cases with a review of literature. J \nNational SciCoun Sri Lanka 4: 55-73. \n7. Gray SW, Skandalakis JE, Broecker BH (1994) Female re-\nproductive system. In: Skandalakis JE, Gray SW, Embryol-\nogy for surgeons. (2\nnd edn), Lippincott Williams & Wilkins, \nBaltimore 816-847.\n8. Cox D, Ching BH (2012) Herlyn-Werner-Wunderlich syn-\ndrome: A rare presentation with pyocolpos. J Radiol Case \nRep 6: 9-15. \n9. Smith NA, Laufer MR (2007) Obstructed hemivagina and \nipsilateral renal anomaly (OHVIRA) syndrome: Manage -\nment and follow-up. Fertility and Sterility 87: 918-922. \n10. Purslow CE (1922) A case of unilateral haematocolpos, \nhaematometra and haematosalpinx. J Obstet Gyaecol Br \nEmp 29: 64.\n11. Mostyn P, Embrey B (1950) A case of uterus didelphys with \nunilateral gynatresia. Br Med J.\n12. Herlyn U, Werner H (1971) Simultaneous occurrence of an \nopen Gartnerduct cyst, a homolateral aplasia of the kidney \nand a double uterus as a typical syndrome of abnormalities. \nGeburtshife Frauenheikd 31: 340-347.\n13. Wunderlich M (1976) Unusual form of genital malformation \nwith aplasia of the right kidney. Zentralbl Gynakol 98: 559- \n562.\n14. PJ Woodward, R Sohaey, TP Mezzetti Jr (2001) Endome -\ntriosis: Radiologic-pathologic correlation. Radio-Graphics \n21: 193-216.\n15. Phupong V, Pruksananonda K, Taneepanichskul S, Tresu-\nkosol D, Virutamasen P (2000) Double uterus with unilater-\nally obstructed hemivagina and ipsilateral renal agenesis: A \nvariety presentation and a 10-year review of the literature. J \nMed Assoc Thai 83: 569-574.\n16. Aydin R, Ozdemir AZ, Ozturk B, Bilgici MC, Tosun M (2014) \nA rare cause of acute abdominal pain: Herlyn-Werner-Wun-\nderlich syndrome. Pediatr Emerg Care 30: 40-42.\n17. Lan Zhu, Na Chen, Jia-Li Tong, Wei Wang, Lei Zhang, et al. \n(2015) New classification of herlyn-wernerwunderlich syn-\ndrome. Chin Med J (Engl) 128: 222-225. \n18. Epelman M, Dinan D, Gee MS, Servaes S, Lee EY, et al. \n(2013) Mullerian duct and related anomalies in children and \nadolescents. Magn Reson Imaging Clin N Am 21: 773-789. \n19. Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazza-\nto RL, et al. (2012) Herlyn-Werner-Wunderlich syndrome: \nMRI findings, radiological guide (two cases and literature \nreview), and differential diagnosis. BMC Med Imaging 12: 4.\n20. Li S, Qayyum A, Coakley FV, Hricak H (2000) Association \nof renal agenesis and mullerian duct anomalies. J Comput \nAssist Tomogr 24: 829-834.\nsis which is the most commonly reported associated \nurologic anomaly [20, 21]. In our case the absence of \nthe right kidney in association with the other abnor-\nmalities have confirmed the diagnosis of HWWS.\nThe optimal treatment in such case is full excision \nand marsupialization of the obstructing vaginal sep-\ntum allowing both uteri to drain through the patent \nvagina [22]. In our case we have performed a full ex-\ncision of the vaginal septum using a ligature devise \nwhich could be introduced through the hymen open-\ning without disturbing its integrity, which is an im-\nportant factor for the community.\nFedele L, et al. in a report of 87 cases of HWWS \nrevealed that 72.4% of cases were of classic variety \nand 27.6% of cases were variants of uterine septum \nor cervical agenesis [23]. In this type of variety of the \nsyndrome, where the unilateral obstruction is at the \nlevel of cervix, Himadri Bal, performed Unilateral sub-\ntotal hysterectomy with salpingectomy [24].\nLaparoscopy may not be mandatory in the treat-\nment of HWWS, but it could be helpful in confirming \nthe diagnosis when radiological imaging is inconclu-\nsive. Also, laparoscopy is useful in the diagnosis and \ntreatment of endometriotic lesions and to release \npelvic adhesions resulting from retrograde menstru-\nation from the obstructed hemivagina [25], in our \ncase the laparoscopy was useful to aspirate the in-\ntraperitoneal retrograde menstruation which was ac-\ncumulated in the peritoneal cavity, and cleaning the \nabdominal cavity aiming to prevent future formation \nof endometriosis.\nThe potential complications of this syndrome are \npyohematocolpos or pyosalpinx, which may lead to pel-\nvic peritonitis. While long-term complications are en-\ndometriosis, pelvic adhesions leading to chronic pelvic \npain, and infertility. Which require early diagnosis and \ntreatment of this syndrome in order to avoid theses \ncomplications and to preserve the fertility [26]. Where-\nfore patient affected by (HWWS) needs further follow \nup to predict these possible complications.\nConclusion\nHerlyn-Werner-Wunderlich syndrome is a rare \nurogenital congenital anomaly. The aim of reporting \nthis case is to highlight the entity and the importance \nof early management to relieve the symptoms and to \nprevent the complications, since timely diagnosis re-\nquires a high index of clinical suspicion and radiolog-\nical confirmation.\nReferences\n1. Heinonen PK (1984) Uterus didelphys: A report of 26 cas-\nes. European Journal of Obstetrics & Gynecology and Re-\nproductive Biology 17: 345-350.\n2. Grimbizis GF, Camus M, Tarlatzis BC, Bontis JN, Devroey \nP (2001) Clinical implications of uterine malformations and \n\nISSN: 2377-9004\nDOI: 10.23937/2377-9004/1410156\nAlZaiem et al. Obstet Gynecol Cases Rev 2019, 6:156\n• Page 5 of 5 •\n24. Himadri Bal, BS Duggal, Nikita Gonnade, Sanjay Khalad -\nkar (2017) Herlyn-Werner-Wunderlich syndrome. Medical \nJournal of Dr. D.Y. Patil University.\n25. Moon Hyeong Cho, Da Hyun Kim, Jung Mi Byun, Young \nNam Eun Jung Jung, Kim DaeHoonJeong, et al. (2017) \nCase Report Herlyn-Werner-Wunderlich syndrome: An un-\nusual presentation with pyocolpos. Obstet Gynecol Sci 60: \n374-377. \n26. Kabiri D, Arzy Y, Hants Y (2013) Herlyn-Werner-Wunder -\nlich syndrome: Uterus didelphys and obstructed hemivagi -\nna with unilateral renal agenesis. Isr Med Assoc J 15: 66.\n21. Vercellini P, Daguati R, Somigliana E, Vigano P, Lanzani \nA, et al. (2007) Asymmetric lateral distribution of obstructed \nhemivagina and renalagenesis in women with uterus didel-\nphys: Institutional case series and a systematic literature \nreview. Fertility Sterility 87: 719-724.\n22. Jindal G, Kachhawa S, Meena GL, Dhakar G (2009) Uterus \ndidelphys with unilateral obstructed hemivagina with he-\nmatometrocolpos and hematosalpinx with ipsilateral renal \nagenesis. J Hum Reprod Sci 2.\n23. Fedele L, Motta F, Frontino G, Restelli E, Bianchi S (2013) \nDouble uterus with obstructed hemivagina and ipsilateral \nrenal agenesis: Pelvic anatomic variants in 87 cases. Hum \nReprod 28: 1580-1583.","source_license":"CC0","license_restricted":false}