Imperforate Hymen with Hematocolpometra

Home , Clitoromegaly, Cryptomenorrhea, Hydrocolpos, Hymenotomy, Imperforate hymen, Uterine malformation

T h e new england journal o f medicine

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15-year-old premenarchal girl presented with a 4-day history Ludger Wilhelm Poll, M.D. of increasing abdominal pain. Her medical history was unremarkable, and she Peter Flake, M.D. reported that she had never been sexually active. Physical examination revealed A Berufsgenossenschaftliche Unfallklinik a palpable, nontender, nonpulsatile midline mass extending from the pelvis to the Duisburg umbilicus. The patient declined a vaginal examination. Abdominal ultrasonography Duisburg, Germany revealed a large pelvic mass of uncertain origin. The uterus could not be identified [email protected] ultrasonographically. Computed tomographic images of the pelvis showed a hyperdense, nonenhancing, fluid-filled pelvic mass measuring 25 cm by 11 cm by 12 cm (Panel A, axial image, white arrows; Panel B, sagittal image, white arrows) that was causing substantial compression of the bladder (Panels A and B, black arrows). A tubular structure at the superior pole of the mass was identified as the uterus (Panel B, arrowhead). The patient was given a presumptive diagnosis of hematocolpometra (accumulation of menstrual blood in the vagina and uterus). Subsequent vaginal examination revealed a thickened imperforate hymen. After hymenotomy, two liters of coagulated blood drained from the vagina and uterus. The patient was discharged after 3 days without pain or vaginal bleeding. Copyright © 2011 Massachusetts Medical Society.

n engl j med 365;2 nejm.org july 14, 2011 157 The New England Journal of Medicine Downloaded from nejm.org on June 18, 2015. For personal use only. No other uses without permission. Copyright © 2011 Massachusetts Medical Society. All rights reserved. Hindawi Publishing Corporation Case Reports in Urology Volume 2013, Article ID 731019, 3 pages http://dx.doi.org/10.1155/2013/731019

Case Report A Rare Presentation of Imperforate Hymen: A Case Report

Beena Salhan, Olufunmilayo Theresa Omisore, Priyadarshi Kumar, and John Potter

Northampton General Hospital, Cliftonville, Northampton NN15BD, UK

Correspondence should be addressed to Olufunmilayo Theresa Omisore; [email protected]

Received 3 July 2013; Accepted 18 August 2013

Academic Editors: S.-S. Chen, N. Eke, and S. K. Hong

Copyright © 2013 Beena Salhan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Introduction. Acute urinary retention in a child is rare. Haematocolpos can cause a mechanical obstruction, resulting in acute urinary retention. Case Report. A 12-year-old girl presented to the surgical department with a one-day history of acute urinary retention and suprapubic tenderness. She had not started menses but had described period-like pains every month for the past six months. On examination, she had a palpable bladder with over 500 mls of residual urine and a bluish-grey bulge posterior to her urethral meatus. An US scan showed a large mass posterior to her bladder resembling a haematocolpos, and this was confirmed with an MRI scan. She was catheterised and eventually underwent a hymenectomy using a cruciate incision. She made a good recovery postoperatively. Conclusion. In the case of a peripubertal female presenting with acute urinary retention, haematocolpos should be considered as a diagnosis.

1. Introduction palpable and she was noted to have a nontender bluish-grey bulge posterior to the urethra on examination of her external Thisisacasereportofacuteurinaryretentionasa genitalia. Neurological examination was normal. result of an imperforate hymen causing haematocolpos. Urine dipstick was normal, and a urinary pregnancy test The incidence of imperforate hymen is 1 in 2000 girls, was negative. A bladder scan revealed over 500 mls of residual and approximately half of these will present with urinary urine; therefore, a 10 Ch urinary catheter was inserted, which retention [1]. Haematocolpos is a rare condition, where the relieved her suprapubic pain. On repeat examination, the vaginaisfilledwithmenstrualblood,causedbyuterovaginal bladder was no longer palpable and a PR examination was pathologies such as an imperforate hymen [2]. Most cases normal with no palpable masses. Initial blood tests showed a of imperforate hymen are sporadic in nature; however there mildly raised WCC at 11.7 and raised neutrophils at 10.15; all have been reports of familial cases, where both recessive other blood results were unremarkable. inheritance and dominant inheritance have been shown An ultrasound scan of the kidneys showed an 11 × [3]. 7.8 × 8 cm fluid-filled mass lying posterior to the bladder, inseparable from and lying immediately inferior to the uterus 2. Case (Figure 1). The mass had a fluid level, and findings were consistent with a hydrometra. The right kidney showed mild In September 2012, a 12-year-old girl presented to the acci- hydronephrosis. No other abnormal findings were detected. dent and emergency department with a one-day history Following the ultrasound findings, she was referred to of acute urinary retention associated with suprapubic pain the gynaecology department and underwent an MRI scan. anddysuria.Therewasnohistoryofvomitingorachange This showed an 11 × 7.8 × 8 cm mass lying within the in bowel habit. She reported cyclical abdominal cramping midline of the pelvis, which had several fluid layers indicating pains in the preceding six months but denied having started that it consisted of blood products (Figures 2(a) and 2(b)). menses. Her birth history and developmental history were Superiorly, the fluid was in continuation with a single uterine unremarkable. cavity, and inferiorly, it extended down to the perineum. On examination, her abdomen was soft with mild tender- Appearances were consistent with a hugely distended uterus ness suprapubically and in the left iliac fossa. Her bladder was filled with menstrual products. 2 Case Reports in Urology

Figure 1: Ultrasound scan showing fluid-filled uterus.

(a) (b)

Figure 2: MRI scans showing the differential layers of fluid indicative of blood products.

Subsequently, she underwent a hymenotomy (using a retention can subsequently occur due to the pressure effect cruciate incision) with drainage of her hydrocolpos. Postop- imposed on the bladder and urethra [5]. eratively, she made a good recovery with a successful removal This case serves to illustrate that in peripubertal females of the urinary catheter. Since returning home, she has started with amenorrhoea and acute urinary retention, even though experiencing normal menses and has had no further urinary uncommon, a diagnosis of haematocolpos should be consid- problems. ered and excluded.

Conflict of interests 3. Discussion The authors declare that they have no conflict of interests. Acuteurinaryretentionisnotacommonpresentationinchil- dren and is more common in males [4]. When young females present, the causes can include mechanical obstructions References (urinary tract stones, urethral strictures, trauma to external [1] M. J. Lausten-Thomsen and H. Mogensen, “Hymen imperfora- genitalia, and imperforate hymen), neurological disorders, tus with atypical symptom presentation,” Ugeskrift for Laeger, and urinary tract infection [4]. vol. 169, no. 6, pp. 523–524, 2007. Imperforate hymen is a rare genital tract anomaly which [2] E. Deligeoroglou, C. Iavazzo, C. Sofoudis, T. Kalampokas, and has an incidence of about 1 in 2000 [1]. Acute urinary G. Creatsas, “Management of hematocolpos in adolescents Case Reports in Urology 3

with transverse vaginal septum,” Archives of Gynecology and Obstetrics,vol.285,no.4,pp.1083–1087,2012. [3] R. Sakalkale and U. Samarakkody, “Familial occurrence of imperforate hymen,” Journal of Pediatric and Adolescent Gyne- cology,vol.18,no.6,pp.427–429,2005. [4] S. A. Asgari, M. Mansour Ghanaie, N. Simforoosh, A. Kajbafzadeh, and A. Zare’, “Acute urinary retention on children,” Urology Journal,vol.2,no.1,pp.23–27,2005. [5] J. Burgis, “Obstructive Mullerian¨ anomalies: case report, diagnosis, and management,” American Journal of Obstetrics and Gynecology,vol.185,no.2,pp.338–344,2001. Human Reproduction, Vol.27, No.6 pp. 1637–1639, 2012 Advanced Access publication on March 20, 2012 doi:10.1093/humrep/des084

CASE REPORT Gynaecology A uterovaginal septum and imperforate hymen with a double pyocolpos

Luigi Fedele*, Giada Frontino, Francesca Motta, and Elisa Restelli Department of Obstetrics and Gynecology, Fondazione IRCCS Ca` Granda, Clinica Mangiagalli, University of Milano, Via della Commenda 12, 20122 Milan, Italy

*Correspondence address. Fax: +39-0250320252; E-mail: [email protected]

Submitted on January 11, 2012; resubmitted on February 1, 2012; accepted on February 16, 2012 Downloaded from

abstract: The presence of both a uterovaginal septum and imperforate hymen is described in a young patient presenting with ongoing chronic pelvic pain and a double pyocolpos. Ultrasound and magnetic resonance imaging scans were performed. The patient underwent laparoscopic adesiolysis, hymenotomy with drainage of 200 mL of pus, and excision of a complete longitudinal vaginal septum. Over the past 5 years of regular follow-up examinations, the patient has always reported regular menstrual cycles and an absence of pelvic pain. http://humrep.oxfordjournals.org/

Key words: Mu¨llerian / uterovaginal septum / septate uterus / vaginal septum / imperforated hymen

Introduction The patient was brought to the operating room, where she underwent an open laparoscopy followed by a vaginal procedure. Laparos- A septate uterus is the most common uterine malformation and it ori- copy showed severe adhesions involving the omentum, anterior ginates from the incomplete resorption of the adjacent walls of the abdominal wall, bowel, uterus and adnexae. The uterus was laterode- two fused Mu¨llerian ducts. This septum may extend to the cervical viated to the right, had a mid-sagittal indentation of the external by guest on June 18, 2015 canal and can be associated with a vaginal septum which can be fundus and a large isthmic bulge. The pelvic adhesions were freed and partial or can reach the introitus. An imperforate hymen is a congenital no endometriosis nodules were seen. The tubes and ovaries appeared resorptive defect which, on the other hand, does not apparently to be normal. The hymen showed fibrotic scarring resulting from the derive from the Mu¨llerian ducts. The rare finding of both a uterovaginal prior left hymeneal incision and drainage. We performed hymenotomy septum and coexisting imperforate hymen (Fig. 1) is described in this on both left and right hemimembranes, and drained 200 ml of pus case of a young patient presenting with ongoing chronic pelvic pain and from both hemivaginas. The longitudinal vaginal septum was identified a double pyocolpos. and septoplasty was performed with straight Haney clamps placed along the anterior and posterior aspects of the vaginal septum. The Case Report 6-cm septum was excised along its length and the edges of the vaginal mucosa were reapproximated, both anteriorly and posteriorly, with a A premenarchal 13-year-old girl presented with severe chronic pelvic series of interrupted 3-0 Vicryl stitches. One month after surgery, the pain which had started 1 year earlier. The patient reported that her gy- patient had an adequate vaginal diameter without evidence of mucosal naecologist had justified her symptoms as being caused by cystitis, al- scarring or narrowing. The patient has undergone regular annual check- though she had not undergone a uterine culture or other uterine tract ups since then, and after 5 years of follow-up, reports regular menstrual examinations. She was ultimately seen by a gynaecologist who per- cycles and no recurrence of symptoms since surgery. formed a hymeneal incision for a pyocolpos due to an imperforate hymen. Despite this, symptoms persisted, and the patient was sent 1 Discussion year later to our tertiary referral centre for female genital malformations. At gynaecologic examination, the vaginal dimple ended blindly with a The presence of a uterovaginal septum and concomitant imperforate slight bulge. A pelvic ultrasound showed a septate uterus and an endo- hymen is likely to be coincidental, since these portions of the female vaginal hypoechoic cystic mass of 21 cm. Magnetic resonance imaging genitals derive from two distinct embryological structures, and al- (MRI) scans noted a uterovaginal septum with a right hematocolpos of though both are resorptive defects, they occur at different stages of 20 × 6 cm (Fig. 2). Both kidneys were documented to be normal. embryological development. Although the septate uterus is the most

& The Author 2012. Published by Oxford University Press on behalf of the European Society of Human Reproduction and Embryology. All rights reserved. For Permissions, please email: [email protected] 1638 Fedele et al.

resulting mass effect in the vagina and uterus are referred to as hematocolpos and hematometrocolpos, respectively. A pyocolpos may result from an infection that is ascending through microperforations in the membrane, which may also explain why our patient did not firstly present with a muco/hydrometra. However, the pyocolpos with which the patient presented was most likely iatrogenic due to the previous hymeneal incision. The combination of a uterovaginal septum with a imperforate hymen does not seem to fit into the existing classification systems and is inconsistent with the theory of linear caudal to cephalad Mu¨llerian fusion as described by Crosby and Hill (1962). According to this theory, uterine development results from the fusion of the Mu¨llerian ducts, which starts at the caudal-most aspect known as the Mu¨llerian tubercle, and proceeding in a cranial direction. Septal resorption is thought to follow shortly thereafter, beginning at any point of fusion, and moving in either or both directions. The case reported here is not supported by this theory because a dual vagina/cervix complex

suggests failure of caudal fusion, whereas a septate uterus indicates Downloaded from normal cephalad fusion with failure of septal resorption. The present case does in fact ﬁt better into the alternative hypothesis sug- Figure 1 Schematic drawing showing a complete uterovaginal gested by Musset et al. (1967), which proposed a three-stage process septum and imperforate hymen. in which the medial aspects of the Mu¨llerian ducts begin to fuse in the

middle and proceed in both the cephalad and caudad directions sim- http://humrep.oxfordjournals.org/ ultaneously. This is then followed by rapid cellular proliferation between the ducts which result in the development of the uterine body and cervix, and septal resorption, all of which occur simultan- eously in both directions. The dual vagina/cervix complex in this case could therefore be due to failed fusion of the Mu¨llerian ducts starting at the uterine isthmus towards the caudal direction. The septate uterus and vagina could then be explained by completely failed septal resorption after normal fusion.

The hymen is formed from the endoderm of the urogenital sinus by guest on June 18, 2015 epithelium and represents the junction of the sinovaginal bulbs with the urogenital sinus. An imperforate hymen is the result of failure of canalization of the vaginal plate. Since the lower third of the vagina is thought to derive from the urogenital sinus, this described association of a uterovaginal septum and imperforate hymen might shed new light onto the possibly intertwined embryological derivation of these caudal structures. An alternative view is presented in a recent study (Kimberley et al., 2012) which described 7 patients out of 31 Figure 2 Coronal section of the pelvis showing right laterodeviated septate uterus (shown with a white ‘u’) and pyocolpos (shown with a with Rokitansky syndrome also presenting with hymenal variations. black asterisk). The authors suggest that those women without a hymen are also more likely to have renal tract anomalies, and postulate that in these patients the primary problem is with the underlying Wolffian frequently encountered Mu¨llerian anomaly, this specific anomaly of a duct defect, rather than being primarily a Mullerian duct problem. coexisting longitudinal vaginal septum and imperforate hymen has Cases like these and the one described in this report and the one been previously described only in one case (Oakes et al., 2010)in described by Oakes et al. give some insight on how there is still the scientific literature. However, a case of a concurrent imperforate much to be learned regarding the complex embryological networks hymen with a transverse vaginal septum in a unicornuate uterus has that are engaged in organ formation. been described by Creatsas et al.(Deligeoroglou et al., 2007), while The importance of understanding the embryological background of both a longitudinal and a transverse vaginal septum were encountered rare malformations allows the clinician to adequately treat each case by (Moawad et al. (2009). All authors indeed conclude that an ad- despite the absence of a classification system for following the equate anatomical evaluation of each patient is paramount in tailoring current treatment guidelines. The authors of the only other case of the appropriate conservative and surgical treatment. uterovaginal septum and imperforate hymen reported in the literature While a septate uterus is commonly diagnosed during a work-up for (Oakes et al., 2010) performed hymenotomy with a subsequent infertility, the diagnosis of imperforate hymen is made in adolescence, vaginal septoplasty and did not perform laparoscopy. Our decision when the retained secretions consist of menstrual products, and the to perform laparoscopy in this patient was based on the possibility Uterovaginal septum and imperforate hymen 1639 of treating possible pelvic adhesions and the presence of endometri- References osis that may occur secondarily to chronic menstrual reflux in obstructive malformations such as in the present case. Crosby WM, Hill EC. Embryology of the Mu¨llerian duct system. Obstet Gynecol 1962;20:507–515. Deligeoroglou E, Deliveliotou A, Makrakis E, Creatsas G. Concurrent Authors’ roles imperforate hymen, transverse vaginal septum, and unicornuate L.F.: author and main surgeon, G.F.: co-author and assistant surgeon, uterus: a case report. J Pediatr Surg 2007;42:14. Kimberley N, Hutson JM, Southwell BR, Grover SR. Vaginal agenesis, the F.M.: assistant surgeon, E.R.: assistant. hymen, and associated anomalies. J Pediatr Adolesc Gynecol 2012; 25:54–58. Funding Moawad NS, Mahajan ST, Moawad SA, Greenfield M. Uterus didelphys and longitudinal vaginal septum coincident with an obstructive The material contained in the manuscript has not been published, has transverse vaginal septum. J Pediatr Adolesc Gynecol 2009;22: not been submitted, nor is being submitted elsewhere for publication. e163–e165. This study has not been funded and none of the authors has any con- Musset R, Muller P, Netter A, Solal R, Vinourd JC, Gillet JY. Etat du haut flict of interest related to the material of the manuscript submitted. appareil urinaire chez les porteuses de malformations uterines, Etude de 133 observations. La Presse Medicale 1967;75:1331–1336. Conflict of interest Oakes GB, Hussain HK, Smith YR, Quint EH. Concomitant resorptive defects of the reproductive tract: a uterocervicovaginal septum and None declared. imperforate hymen. Fertil Steril 2010;93:268.e3–268.e5. Downloaded from http://humrep.oxfordjournals.org/ by guest on June 18, 2015 Hindawi Publishing Corporation Case Reports in Obstetrics and Gynecology Volume 2015, Article ID 429740, 3 pages http://dx.doi.org/10.1155/2015/429740

Case Report Abdominal Tumor in a 14-Year-Old Adolescent: Imperforate Hymen, Resulting in Hematocolpos—A Case Report and Review of the Literature

George Marios Makris,1,2 Doris Macchiella,1 Dennis Vaidakis,2 Charalampos Chrelias,2 Marco Johannes Battista,1 and Charalampos Siristatidis2

1Department of Obstetrics & Gynecology, Medical School, Johannes Gutenberg University of Mainz, Langenbeck Straße 1, 55131Mainz, Germany 23rd Department of Obstetrics and Gynecology, Medical School, University of Athens, “Attikon” Hospital, Rimini Street 1, Chaidari, 12462 Athens, Greece

Correspondence should be addressed to George Marios Makris; [email protected]

Received 21 October 2014; Revised 17 January 2015; Accepted 19 January 2015

Academic Editor: Olivier Picone

Copyright © 2015 George Marios Makris et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Background. Abdominal masses in female adolescents are uncommon. A rare cause of this condition is hematocolpos due to imperforate hymen. Case.Wepresentacaseofanunusuallymassiveasymptomaticabdominalbulkina14-year-oldfemalepatient, who sought for medical advice after unusual abdominal pain lasting for few weeks. The patient was otherwise asymptomatic, apart from an unusual dramatic expansion of her abdominal wall during the last month. We describe the surgical management and the follow-up of the patient. Summary and Conclusion. Clinicians should keep in mind that an imperforate hymen can cause abdominal growth due to hematocolpos and include it in the differential diagnosis of such a clinical entity in female adolescents. 2D ultrasound is usually efficient for the confirmation of the diagnosis of hematocolpos, but 3D ultrasound is more accurate. Wide excision should be undertaken, as an initial approach, to avoid recurrence.

1. Introduction and can be manifested as fetal ascites or renal failure [2, 3], sometimes leading to variable degrees of hydroureter and/or An emerging abdominal tumor in young females is a rare hydronephrosis [4]. For its diagnosis, 2D sonography is the situation and requires a specific clinical and ultrasonographic usually indicated imaging method of choice. 3D sonography approach. Common causes of a newly diagnosed abdominal and MRI are rarely suggested and used, although they both mass in young females include cysts and solid tumors of provide a better visualization and differentiation of the tissues variousorigins.Inthiscontext,hematocolposisarare and a safer distinction among other causes of hematocolpos, entity that can cause such symptoms: it comprises the such as vaginal septum or partial agenesis. In addition, an blood collection in the distal closed vagina and is usually endocrine profile of the patient is usually necessary [5, 6]. diagnosed in young adolescents with no menstruation and Surgical management is the treatment of choice, through cyclic abdominal pain. Its incidence is approximately one incision or excision of the hymen, using cold knife, scissors, every 2000 young adolescents [1]andin90%ofcasesis electrocoagulation, or laser. The recurrence rate remains low, caused by an imperforate hymen. occurring more often during minor surgical approaches, such Usual clinical signs include cyclical low abdominal pain, asafteracruciateincision.Notably,aspontaneousruptureof urinary retention, back pain, primary amenorrhea, and/or a an imperforate hymen is likely to precede any decision for quickly enlarging pelvic tumor. It may also affect neonatal age surgical management [7]. Finally, further issues have to be 2 CaseReportsinObstetricsandGynecology

Figure 1: Clinical presentation of megahematocolpos.

Figure 2: 2D ultrasound imaging of megahematocolpos. weighted, such as the bleeding and the subsequent emotional stress of the young female after the procedure, along with the completion and keeping of legal documentation.

2. Case A 14-year-old girl was admitted to the Pediatric Emergency Department of the Department of Obstetrics and Gynecol- ogy, Johannes Gutenberg University of Mainz, Mainz, Ger- many, with primary amenorrhea, an expanding abdominal mass, and mild abdominal pain. There was no history of severe abdominal pain during the last year and the patient complained of polyuria during the last month; there were no signs of defecation. Her parents sought for medical Figure 3: Postoperative 3D ultrasound imaging. assistance because of a growing tumor in her abdomen. At clinical examination, secondary sexual characteristics were present and within normal ranges. The clinical presentation was quite impressive: a thin girl with a BMI of 22 with a As the diagnosis was clear, surgical management was painless, nontender, soft, and homogenous mass, distorting decided after providing the written informed consent of both her abdominal wall and expanding up to 5 cm over the parents and scheduled for the following day. A hymenotomy umbilicus (Figure 1). was performed under general anesthesia: at first, laser was The patient’s vital signs were normal; laboratory tests used, followed by electrocoagulation, and an oval shaped revealed a hemoglobin concentration of 13 g/dL and white piece of hymen was excised. A total sum of 2400 mL dark blood cell count of 11/nL, while CRP and tumor markers’ red, tarry blood was drained from the vagina. Of note, the concentrations were within normal ranges. In addition, her maximum quantity reported in the literature is 3000 mL endocrine hormonal profile was indicating a girl with a [8, 9]; spontaneous drainage was continued the following mature hypothalamic-pituitary axis. Urinalysis was normal. day too. No suturing of the remnant hymen was performed. Clinical examination of the abdomen did not reveal any pain Antibiotics were given prophylactically for the next 4 days. or signs of peritonism. Clinical gynecological examination 3D imaging during the first postoperative day revealed after retracting the labia minor revealed an imperforate a waveform vagina, with a length of approximately 21 cm, hymen, which was bulging forwards. Rectal digital exami- whereas the size of uterus regressed for 10 cm under the nation revealed a large bulky mass positioned anteriorly. A umbilicus but did not enter the minor pelvis. The patient structure of 34 cm length, 11 cm width, and 11 cm height was was discharged from the hospital after two days and a weekly evidenced at 2D transabdominal ultrasound (Figure 2). follow-up with 3D ultrasound was scheduled. Menstrua- On the cranial, frontal end of the structure, cranial from tion occurred 20 days postoperatively and vaginal length the umbilicus and adapted to the front abdominal wall, was normalized 3 days after. During a scheduled follow- we observed a uterus of normal size (no hematometra) up appointment, 2 months postoperatively, a small amount (Figure 3), while both ovaries were present with a normal of blood was detected in the vagina through 3D imaging; appearance. Both kidneys were present, with no anomalies or recurrence of the hematocolpos was confirmed after genital dilatation of the ureters. inspection. Reoperation was booked immediately, leading to 3D ultrasound displayed the clarity of the wall of this a wider triangular tissue excision. structure: it appeared straight, with no adherence to the The patient is not sexually active yet and during the last neighboring organs, homogenous, with a fluid-like content 12 months she has normal menstrual cycle and vaginal length, in it. measured at ultrasound. Case Reports in Obstetrics and Gynecology 3

3. Summary and Conclusion [9] J. C. Doyle, “Imperforate hymen with and without hematocolpos,” California and Western Medicine,vol.56,no.4,pp.242– The approach of a young patient presenting with a newly 247, 1974. diagnosed abdominal tumor is always a demanding process. It causes fear to the child, emotions of guilt to the parents, and additional responsibility to the clinician. Apart from the pediatrician, other medical specialties can assist towards diagnosis and management, such as general surgeon, gynecologist, endocrinologist, and radiologist. Although hematocolpos consists of a rare clinical feature, it should be always considered a possible diagnosis in young females with primary amenorrhea and abdominal mass. Both diagnosis and treatment of hematocolpos are relative easy, but due to the sensitive nature of the disease, the approach of the patients presenting with that disease is demanding. Since the most serious complication is recurrence, in our experience, werecommendwidetissueexcisionasaninitialapproach, through a triangular or oval shape, instead of a cross or “X” shape incision. 2D ultrasound is the diagnostic tool of choice, but 3D ultrasound can reveal more details, such as the exact relationship of the feature with the neighboring organs and structures, since it provides better tissue differentiation and can assist in the vaginal length surveillance.

Conflict of Interests The authors declare no potential conflict of interests.

References

[1] R. Sakalkale and U. Samarakkody, “Familial occurrence of imperforate hymen,” Journal of Pediatric and Adolescent Gyne- cology,vol.18,no.6,pp.427–429,2005. [2] Y. Jacquemyn, L. de Catte, and M. Vaerenberg, “Fetal ascites associated with an imperforate hymen: sonographic observation,” Ultrasound in Obstetrics and Gynecology,vol.12,no.1,pp. 67–69, 1998. [3] C. Aygun, O. Ozkaya,¨ S. Ayyyld´ yz,´ O. Gung¨ or,B.Mutlu,and¨ S¸. Kuc¸¨ uk¨ od¨ uk,¨ “An unusual cause of acute renal failure in a newborn: hydrometrocolpos,” Pediatric Nephrology,vol.21,no. 4, pp. 572–573, 2006. [4] M.-C. Shen and L.-Y. Yang, “Imperforate hymen complicated withpyocolposandlobarnephronia,”Journal of the Chinese Medical Association, vol. 69, no. 5, pp. 224–227, 2006. [5] D. Bursac,Z.Duic,J.Z.Partl,J.Valetic,andS.Stasenko,“Hema-´ tocolpos resulting from an imperforated hymen diagnosed by ultrasound in a patient with recurrent urinary tract infections,” Journal of Pediatric and Adolescent Gynecology,vol.25,no.5,pp. 340–341, 2012. [6] J.M.Froehlich,T.Metens,B.Chilla,N.Hauser,M.K.Hohl,and R. A. Kubik-Huch, “MRI of the female pelvis: a possible pitfall in the differentiation of haemorrhagic vs. fatty lesions using fat saturated sequences with inversion recovery,” European Journal of Radiology,vol.81,no.3,pp.598–602,2012. [7] Z. Kurdoglu, M. Kurdoglu, and Z. Kucukaydin, “Spontaneous rupture of the imperforate hymen in an adolescent girl with hematocolpometra,” ISRN Obstetrics and Gynecology, vol. 2011, ArticleID520304,2pages,2011. [8] A. Cecutti, “Hematocolpos with imperforate hymen,” Canadian Medical Association journal, vol. 90, pp. 1420–1421, 1964. JIMSA October-December 2012 Vol. 25 No. 4 Pictorial CME 255

Hematometrocolpos Secondary to Imperforate Hymen B. Mallikarjunappa, Ashish S.R. Department of Radio-Diagnosis, Adichunchanagiri Hospital & Research Centre, Teaching Hospital of A.I.M.S, Balagangadharanatha Nagara, Karnataka, India

14 year old girl referred to our department with lower abdominal pain with abdominal distension.on examination large tender midline cystic mass noted extending from pelvis to epigatrium.

Ultrasound showing Huge midline oval hypoechoiec mass with scattered internal echoes.uterus on top of the mass,bladder not made out seperately rest normal.

Ultrasound showing over distended bladder catheterisation.Catheter is anterior to the lesion,little urine drained. No vaginal opening bulging membrane with bluish hue seen at the introitus.secondary sexual characters well developed.patient not attained menarche.

C.T and MRI confirmed the diagnostic findings of ultrasound.

DIAGNOSIS: Hematometrocolpos secondary to imperforate hymen. Hydrocolpos is characterised by an expanded fluid filled vaginal cavity , when associated with distention of the uterine cavity as well ,the term hydrometrocolpos is used . When the fluid ismixed with menstrual products, it is termed as hematocolpos and hematometrocolpos . Causes: Imperforate hymen (most common); Complete vaginal stenosis; Segmental vaginal atresia; Transverse vaginal septum Associations: Imperforate anus; uterus didelphys; Renal agenesis. IMPERFORATE HYMEN Imperforate hymen is the most common and most distal form of vaginal outflow obstruction. Persistance of the intact hymenal membrane results in the condition of imperforate hymen.The imperforate hymen is a solid membrane interposed between the proximal uterovaginal tract and introitus. PATHOPHYSIOLOGY: Any obstruction of the vaginal tract during the prenatal,perinatal,or adolescent period results in the entrapment of vaginal and uterine secretions . In patients with imperfoarate hymen,this obstruction is at the level of interoitus and becomes evident when the distensible membrane bulges between the labia. Various terms such as mucocolpos,hematocolpos,and pyocolpos, are used to describe this condition depending on the retained contents . When the diagnosis is made in adolescence, the retained secretions consist of menstrual products, and the resulting mass effect in the vagina and uterus are referred to as hematocolpos and hematometrocolpos, respectively. MANAGEMENT: This patient underwent cruciate excision of the hymen under GA. More than 2 litres of blood coloured fluid drained.after two days of hospital stay, the girl is relieved not only from the hospital-even from her pain, distress, girl went home with ooh and ALL IZZ WELL.

Correspondence: Dr. B. Mallikarjunappa, Associate Professor,Department of Radio-Diagnosis, Adichunchanagiri Hospital & Research Centre, Teaching Hospital of A.I.M.S, Balagangadharanatha Nagara-571488, Nagamangala Taluk, Maandya District, Karnataka, India

Open Access

Case report Imperforate Hymen - a rare cause of acute abdominal pain and tenesmus: case report and review of the literature

Aruyaru Stanley Mwenda1,&

1Transmara District Hospital-Kilgoris, Kenya

&Corresponding author: Aruyaru Stanley Mwenda, Transmara District Hospital-Kilgoris, Kenya

Key words: Imperforate hymen, amenorrhea, pubertal girls, urine retention

Received: 05/12/2012 - Accepted: 16/05/2013 - Published: 21/05/2013

Abstract Imperforate hymen is a rare condition that presents with amenorrhea, cyclical abdominal pains and urine retention among pubertal girls. A 14 year old girl with imperforate hymen underwent hymenotomy for hematocolpometra, having presented with abdominal pains and tenesmus.

Pan African Medical Journal. 2013;15:28. doi:10.11604/pamj.2013.15.28.2251

This article is available online at: http://www.panafrican-med-journal.com/content/article/15/28/full/

© Aruyaru Stanley Mwenda et al. The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Pan African Medical Journal – ISSN: 1937- 8688 (www.panafrican-med-journal.com) Published in partnership with the African Field Epidemiology Network (AFENET). (www.afenet.net)

Page number not for citation purposes 1 Introduction malformations. It occurs sporadically but few familial cases have been reported [8]. Imperforate hymen, despite being the commonest female genital tract malformation [1], is a rare occurrence with a prevalence of Imperforate hymen can present during three main stages in life; 0.014-0.1% [1-3]. It mostly presents during puberty [1, 4] although diagnoses in utero [3, 5, 6] and during the new born period and 1. In utero: This is the rarest and occurs due to maternal childhood [3, 7] are also documented. estrogenic stimulation that leads to uterovaginal secretions filling up the blind vagina and presenting as hydrocolpos There are few cases of Imperforate hymen reported in Africa. A diagnosed through obstetric ultrasound [6]. The diagnosis case of unique presentation with tenesmus besides other should be confirmed post natally. documented symptoms was managed at a rural Kenyan hospital. There is no recorded case of imperforate hymen presenting with 2. New-born-infanthood-childhood: In new-born period this may tenesmus according to literature search. In this article, a review of occur due to maternal estrogenic stimulation that leads to the literature concerning the symptomatology of imperforate hymen uterovaginal secretions filling up the blind vagina and among pubertal girls is also presented. presenting with hydrocolpos [1, 3, 7, 8, 10].

3. At puberty: This is the commonest. It occurs when a girl starts Patient and observation menstruating and the menstrual blood accumulates in the vagina [3, 10]. The age of presentation (mean, range) is 13.2 14 year old Kenyan girl of African descent presented to hospital with and 11-16 years respectively according to Liang et al [5] or 12 a weeklong complaint of lower abdominal pains associated with and 10-15 years respectively according to Lui et al [9]. Liang tenesmus. She had reduced appetite and poor intake of food due to and colleagues did a ten year retrospective analysis of 15 the colicky pains. There was no abdominal distension but she had women treated for imperforate hymen through telephone observed some suprapubic fullness. She did not have constipation, based researcher administered questionnaire and a subsequent diarrhoea, vomiting or fevers. Her urinary habits were normal. She physical and sonographic examination. In their study, Lui et al had never had her menstrual periods but she had developed did a ten year retrospective analysis of the data of 15 patients secondary sexual characteristics. treated for imperforate hymen but did not do any follow up patient interview or examination. Kurgodlu and colleagues On examination, she was in severe pain, walking stooped over and argue that the age of presentation is 2.5-4 years after had moderately tender suprapubic mass corresponding to a uterus thelarche [12]. at 16 weeks. Rectal examination revealed an anterior mass. Perineal examination revealed a bulging imperforate hymen exaggerated on Among the pubertal girls, imperforate hymen will present in the valsava manoeuvre. Pelvic ultrasound done revealed distended following ways. uterus and vagina all filled up with homogenous thick fluid (Figure 1, Figure 2). A diagnosis of hematocolpometra was made. Amenorrhea

In theatre, an X-shaped incision of the hymen was made under I. Primary amenorrhea anaesthesia and approximately 600mls of thick chocolate coloured blood evacuated. The edges of the hymen were everted and a. This is because the girl has started menstruating but does not anchored by Vicryl 2/0 sutures. Analgesic cream and prophylactic experience any menstrual flow as the blood accumulates in the oral antibiotics were prescribed. She made uneventful recovery and vagina, then in the uterus and occasionally, eventually into the was doing well at 1 month. She was however lost to follow-up after fallopian tubes [3, 4, 7]. that. II. Secondary amenorrhea

Discussion a. This can occur following spontaneous closure of previously perforate hymen [8]. This can happen with a micro perforate

or stenosed hymen. In such initial light periods will be Imperforate hymen is a layer of connective tissue that forms a experienced but continuous stenosis leads to complete transverse septum and obstructs the vaginal opening at the level of obstruction and amenorrhea [8]. the introitus [5]. Usually, the hymen is a membrane that b. It can also occur as a result of stenosis of the hymenal opening embryologically develops through the fusion of the caudal end of following surgical or sexual trauma [8]. the paramesonephric ducts and the urogenital sinus [4, 5,7,8]. The c. Lastly, it can occur as failure of hymenotomy [10]. In the central portion of this membrane perforates through the months following hymenotomy the patient experiences her degeneration of its epithelial cells [5]. Failure of the degeneration of menstrual flow but the margins of the hymenotomy incision the epithelial cells and subsequent perforation leads to a hymen that adhere and eventually occlude the vaginal outflow leading to is termed imperforate [5]. amenorrhea.

The function of the hymen is not clear but is thought to include III. Cryptomenorrhea innate immunity as it provides a physical barrier to infections during the pre-pubertal period when the vaginal immunity is not fully Pain developed [3].

Recurrent cyclical lower abdominal/pelvic pains (up to 60%) [2, 4, Imperforate hymen is rarely associated with other female genital 8, 9, 11, 12]. This is due to continued distension of the vagina and tract malformations [1, 4] although some authors [2, 9] have uterus by accumulating menstrual blood. emphasized the need to rule out associated mullerian

Page number not for citation purposes 2 Low back pain (38-40%) [4, 13, 14]. Occurs as referred pain following irritation of the sacral plexus and nerve roots by the distended vagina and uterus. Conclusion

Obstruction Imperforate hymen is a rare condition but should be easy to diagnose when it presents. It should be suspected in pubertal girls I. Urinary outflow obstruction and its complications (58%) who presented with acute abdominal pain. [9] a. Acute urine retention (3-60%) [7, 9, 10, 13, 15].This occurs by a Competing interests number of mechanisms i. Pressure on the bladder by the distended uterus causing The author declares no competing interest. angulation at the bladder neck and kinking of the urethra [10] ii. ii. Direct pressure on the urethra causing urethral Tables and figures tamponade [10]

iii. iii. The bulging hymen distends the vagina and may cause Figure 1: Distended uterus cephalad angulation at the urethral meatus further

stretching the urethra and worsening tamponade [15]. Figure 2: Bladder compression by the distended uterus

b. Complications of prolonged or recurrent urine retention

/obstruction References i. Hydroureters [2]

ii. Hydronephrosis [2] 1. Nagai N et al. Life threatening acute renal failure due to iii. Renal failure [1] imperforate hymen in an infant. Paediatrics International. 2012 iv. Acute bacterial nephritis [16] Apr;54(2):280-2. PubMed | Google Scholar II. Vaginal outflow obstruction- Cryptomenorrhea III. Intestinal obstruction 2. Eksioglu AS et al. Imperforate hymen causing bilateral hydroureteronephrosis in an infant with bicornuate uterus. a. Constipation (20-27%) [9, 13] Urology Case Reports. 2012;2012:102683. doi: b. Tenesmus 10.1155/2012/102683. Epub 2012 Jun 7. PubMed | Google IV. Lymphovenous obstruction Scholar Compression of the pelvic veins and lymphatics can impair lymphovenous return from the lower limbs leading to oedema [1]. 3. Basaran M et al. Hymen sparing surgery for imperforate hymen; case reports and review of the literature. J pediatr Mass Adolesc Gynecol. 2009; 22(4): e61-e64. PubMed | Google Scholar I. Distended uterus felt as pelvic mass on abdominal examination (20%) [9] 4. Dane C et al. Imperforate hymen-a rare cause of abdominal II. The distended vagina is felt as a pelvic mass on digital rectal examination pain: two cases and review of the literature. J pediatr Adolesc Gynecol. 2007; 20(4):245-247. PubMed | Google Scholar III. A bluish bulging hymen is observed beneath the labia (60%) [9] 5. Liang CC et al. Long-term follow-up of women who underwent IV. A cystic retropubic mass is revealed on ultra sonography or MRI [9] surgical correction for imperforate hymen. Arch Gynecol Obstet. 2003; 269(1): 5-8. PubMed | Google Scholar With above in mind and a high index of suspicion, it is easy to make a diagnosis of imperforate hymen. Late presentation may be 6. Ayaz UM et al. Ultrasonographic diagnosis of congenital accompanied with complications such as ruptured hematosalpinx [9, hydrometrocolpos in prenatal and newborn period: a case 11], endometriosis [4, 15] and infection (pyocolpos and nephritis) report. Medical ultrasonography. 2011; 13(3): 234-236. [5, 16]. A clinical diagnosis negates the need for extensive PubMed | Google Scholar laboratory and radiological investigations [10] and reduces the delay of intervention and length of hospital stay [9]. 7. Ercan CM et al. Imperforate hymen causing hematocolpos and acute urinary retention in an adolescent girl. Taiwanese Journal The management is aimed at re-establishing vaginal outflow and of Obstetrics and Gynecology. 2011; 50(1):118-120. PubMed mainly consists of surgical hymenotomy under local or general | Google Scholar anaesthesia [7]. Simple vertical, T-shaped, cruciform, X-shaped and cyclical incisions may be used [4, 7, 8]. X-shaped incision has the 8. Khan Z.A et al. Imperforate hymen: a rare case of secondary advantage of reduced risk of injury to the urethra-which should be amenorrhea. J Obstet Gynaecol. 2011;31(1): 91-92. PubMed | stented during the procedure [7]. Pressure on the uterus in order to Google Scholar expel more blood is discouraged as it can lead to retrograde flow through the tubes causing endometriosis and tubal adhesions [15]. 9. Lui CT et al. A retrospective study on imperforate hymen and Hymenectomy and hymenotomy with a two week indwelling hematocolpos in a regional hospital. Hong Kong J emerg med. catheter have also been reported [8]. The outcome is good and the 2010; 17(5): 435-440. PubMed | Google Scholar recurrences are rare [5]. 10. Abu-Ghanem S et al .Recurrent urinary retention due to imperforate hymen after hymenotomy failure: a rare case

Page number not for citation purposes 3 report and review of the literature. Urology. 2010; 78(1): 180- 182. PubMed | Google Scholar 14. Drakonaki EE et al. Hematocolpometra due to an imperforate hymen presenting with low back pain. J Ultrasound Med. 2010; 11. Kloss BT et al. Hematocolpos secondary to imperforate hymen. 29(2): 321-322. PubMed | Google Scholar Int J Emerg Med. 2010; 3(4): 481-482. PubMed | Google Scholar 15. Anselm OO, Ezegwui UH. Imperforate hymen presenting as acute urinary retention in a 14-year old Nigerian girl. J Surg 12. Kurgodlu Z et al. Spontaneous rupture of the imperforate Tech case Rep. 2010; 2(2): 84-86. PubMed | Google hymen in an adolescent girl with hematocoplometra. ISRN Scholar obstetrics and Gynecology. 2011;2011:520304. doi: 10.5402/2011/520304. Epub 2010 Sep 29. PubMed | Google 16. Shen MC, Yang LY. Imperforate hymen complicated with Scholar pyocolpos and lobar nephronia. J Chin Med Assoc. 2006; 69(5): 224-227. PubMed | Google Scholar 13. Mou JWC et al. Imperforate hymen: cause of lower abdominal pain in teenage girls. Singapore Med J. 2009; 50(11): e378- e379. PubMed | Google Scholar

Figure 1: Distended uterus

Figure 2: Bladder compression by the distended uterus

Page number not for citation purposes 4 International Journal of Reproduction, Contraception, Obstetrics and Gynecology Rathod S et al. Int J Reprod Contracept Obstet Gynecol. 2014 Sep;3(3):839-842 www.ijrcog.org pISSN 2320-1770 | eISSN 2320-1789

DOI: 10.5455/2320-1770.ijrcog20140965 Case Report Imperforate hymen and its complications: report of two cases and review of literature

Setu Rathod, Sunil Kumar Samal*, Anandraj Rajsekaran, P. Reddi Rani, Seetesh Ghose

Department of Obstetrics & Gynecology, Mahatma Gandhi Medical College & Research Institute, Pillaiyarkuppam- 607402, Pondicherry, India

Received: 28 July 2014 Accepted: 8 August 2014

*Correspondence: Dr. Sunil Kumar Samal, E-mail: [email protected]

© 2014 Rathod S et al. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT Imperforate hymen is a rare female genital tract malformation, arises as a result of complete failure of the inferior end of the vaginal plate to canalize. We report two cases of imperforate hymen with different presentation. Our first case was a 14 year old girl presented with lower abdominal pain and acute retention of urine with history of cyclical lower abdominal pain for last 6 months. Examination revealed mass of 14 weeks gravid uterus with bulged imperforate hymen. The second case was a 16 year old girl presents with primary amenorrhoea with mass per abdomen. There was history of difficulty in micturition and constipation for last 4 months. On examination, a mass of size corresponding to 22 weeks gravid uterus with bulged bluish colour imperforate hymen was found. Both the cases were managed with incision and passive drainage of collected menstrual blood. On follow up both the cases resumed menstruation and doing well.

Keywords: Imperforate hymen, Primary amenorrhea, Acute urinary retention

INTRODUCTION which she took medication from local hospital. For last one month there was increased frequency and difficulty Imperforate hymen is the commonest female genital tract in micturition. Although she had reduced appetite and malformation and is noted in approximately 1 in 2000 poor intake of food due to colicky pain, there was no females.1 Although the most common age of presentation history of constipation, diarrhoea, vomiting or fevers. is around puberty2,3 diagnosis in utero4-6 and during the new born period and childhood4,7 are also reported. We She was catheterised with an indwelling Foleys catheter report two cases of imperforated hymen with different and one litre of straw coloured urine was drained. symptoms of presentation. Abdominal examination revealed, pain and tenderness in lower abdomen with a suprapubic mass corresponding to CASE REPORT a uterus at 14 weeks. Examination of external genitalia revealed a bulging bluish colour imperforate hymen Case 1 (Figure 1) which was exaggerated on valsalva manoeuvres. The mass was found to be anterior to rectum A 14 year old girl presented with colicky lower in per rectal examination. Pelvic ultrasonography abdominal pain and acute retention of urine for one day. revealed distended uterus and vagina all filled up with She had not attended her menarche but had developed homogenous thick fluid with internal echos (Figure 2). secondary sexual characters. There was history of She was diagnosed as a case of hematometra and cyclical colicky lower abdominal pain with backache for hematocolpos with imperforate hymen and planned for

http://dx.doi.org/10.5455/2320-1770.ijrcog20140965 Volume 3 · Issue 3 Page 839 Rathod S et al. Int J Reprod Contracept Obstet Gynecol. 2014 Sep;3(3):839-842 incision and drainage. In operation theatre, a cruciate Examination of genitalia revealed bulged blush colour incision was given and 800 ml of thick tarry blood was imperforated hymen (Figure 3) and per rectal drained. The quadrants of the hymens are excised; the examination revealed that the mass was anterior to mucosal margins were everted and anchored by fine rectum. Abdominal and pelvic ultrasonography revealed delayed absorbable suture (Vicryl 2/0). Local analgesic large fluid collection in dilated and enlarged vagina, cream and prophylactic oral antibiotics were prescribed. uterus and tubes with fine internal echo’s suggestive of Postoperative period was uneventful and she was hematocolpos, hematometra with hematosalpinx (Figure discharged on 2nd postoperative day after removal of 4 & 5). Bilateral mild hydroureteronephrosis due to mass catheter. Follow up after one month revealed patent effect of the lesion with no free fluid in peritoneal cavity outflow tract and consummation of normal menstrual was reported in ultrasound. CT scan revealed no cycle. associated Mullerian duct and skeletal anomalies. She was planned for incision and drainage. In theatre, X shaped or cruciate incisions was made through the hymenal membrane at the 2-, 4-, 8-, and 10-o’clock positions after putting an indwelling Foleys catheter and 1000 ml of collected chocolate coloured blood was drained passively (Figure 6). The individual quadrants were excised along the lateral wall of the vagina, avoiding excision of the vagina. Margins of vaginal mucosa were approximated with fine delayed-absorbable suture (Figure 7). Local analgesic cream and oral antibiotic were given. She had an uneventful postoperative period and was discharged on 2nd postoperative day after removal of the catheter. She had

menstruation after one month and is doing well in follow Figure 1: 14 year old girl with bluish bulged up examination. imperforate hymen with associated vulvar distension.

Figure 2: Ultrasound scan showing hematometra and Figure 3: 16 year old girl with imperforate hymen hematocolpos. presented as a case of primary amenorrhoea and mass abdomen. Case 2

We report a case of 16 year old girl who presented with primary amenorrhoea and mass per abdomen. She had history of colicky cyclical lower abdominal pain 4 months back for which she was treated in a local peripheral hospital after which pain subsided. She gave history of difficulty in micturition, constipation and occasional pain in back for last 4 months.

Examination revealed well developed secondary sexual characters with tanner stage IV breast, pubic and axillary hair. On per abdominal examination, the size of mass corresponds to 22 weeks gravid uterus and it was well defined, mobile, tender with non-palpable lower border. Figure 4: Ultrasound scans showing hematometra and hematocolpos.

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Imperforate hymen is rarely associated with other female genital tract malformations2,3 although some authors9,10 have emphasized the need to rule out associated Mullerian malformations. If the hymen is imperforate, mucus and blood from endometrial sloughing accumulate in the vagina which can present during three main stages in life. As congenital hydrometrocolpos in intrauterine period which is the rarest and occurs due to maternal estrogenic stimulation that leads to uterovaginal secretions filling up the blind vagina and diagnosed 6 through obstetric ultrasound. The diagnosis should be Figure 5: Ultrasound scans showing hematometra and confirmed postnatally. In new-born and childhood period right hematosalpinx with normal ovary. this may occur due to maternal estrogenic stimulation that leads to uterovaginal secretions filling up the blind vagina and presenting with hydrocolpos.2,4,7 However, more commonly adolescent girls present after menarche when menstrual blood trapped in the vagina behind the imperforate hymen which is known as hematocolpos creating a bluish bulge at the introitus. With cyclic menstruation, the vaginal canal becomes greatly distended, and the cervix may begin to dilate and allow formation of a hematometra and hematosalpinx.

The age of presentation (mean, range) is 12 and 10-15 years respectively according to Lui et al.10 and 13.2 and 11-16 years respectively according to Liang et al.5 The common mode of presentation of imperforate hymen includes- Figure 6: Passive drainage of chocolate coloured altered blood after cruciate incision. I. Amenorrhea, which may be primary due to accumulation of blood behind the imperforate hymen3,4,7 or secondary which can occur following spontaneous closure of previously perforate hymen. The later mainly occurs in micro perforate or stenosed hymen following surgical or sexual trauma where initial light periods will be experienced but continuous stenosis leads to complete obstruction and amenorrhea.11

II. Recurrent cyclical lower abdominal/pelvic pains (up to 60%)3,7,9 due to continued distension of the vagina and uterus by accumulating menstrual blood and low back 3 pain (38-40%) which is a referred pain following irritation of the sacral plexus and nerve roots by the Figure 7: Mucosal margins are approximated with distended vagina and uterus. fine delayed-absorbable suture. III. Obstruction DISCUSSION 1. Urinary outflow obstruction and its complications 10 The hymen is the membranous vestige of the junction (58%). between the sinovaginal bulbs and the urogenital sinus. It 7,10,12 generally becomes perforate or patent during fetal life to A. Acute retention of urine which is due to pressure establish a connection between the vaginal lumen and the on the bladder by the distended uterus causing perineum. Imperforate hymen is due to complete failure angulation at the bladder neck and kinking of the 12 of the inferior end of the vaginal plate to canalize.1 urethra and direct pressure on the urethra causing 13 Although most cases occur sporadically, cases of urethral tamponade. imperforate hymen involving multiple family members B. Chronic or prolonged urinary retention leading to 9 14 have been reported.8 The function of the hymen is not hydroureteronephrosis, acute bacterial nephritis 2 clear but is thought to include innate immunity as it and renal failure provides a physical barrier to infections during the prepubertal period when the vaginal immunity is not fully 2. Vaginal outflow obstruction which is seen as a bluish 13 developed.4 bulge at the introitus.

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3. In chronic cases intestinal obstruction leading to renal failure due to imperforate hymen in an infant. constipation (20-27%)10 and tenesmus13 also seen. Pediatr Int. 2012;54(2):280-2. 3. Dane C, Dane B, Erginbas M, Cetin A. Imperforate 4. Lymphovenous obstruction due to compression of the hymen-a rare cause of abdominal pain: two cases and 2 pelvic veins and lymphatics can lead to edema of limbs. review of the literature. J Pediatr Adolesc Gynecol. 2007;20(4):245-7. IV. Mass per abdomen due to distended uterus and vagina 10 4. Basaran M, Usal D, Aydemir C. Hymen sparing with accumulated menstrual blood. surgery for imperforate hymen: case reports and review of the literature. J Pediatr Adolesc Gynecol. V. Retrograde menstruation may lead to the development 2009;22(4):61-4. of endometriosis and laparoscopy can be performed at the 1 5. Liang CC, Chang SD, Soong YK. Long-term follow- time of excision of an imperforate hymen to detect this. up of women who underwent surgical correction for imperforate hymen. Arch Gynecol Obstet. Differential diagnosis of imperforate hymen includes 2003;269:5-8. other obstructive reproductive tract anomalies like lower 6. Ayaz UM. Ultrasonographic diagnosis of congenital transverse vaginal septum. The associated vulvar hydrometrocolpos in prenatal and newborn period: a distension, however, uniquely suggests imperforate 1 case report. Med Ultrasonography. 2011;13(3):234- hymen. Imperforate hymen is usually a clinical diagnosis 6. which can be confirmed by ultrasonography. 7. Ercan CM, Karasahin KE, Alanbay I, Ulubay M, The treatment includes surgical hymenotomy under Baser I. Imperforate hymen causing hematocolpos anaesthesia following catheterisation with an indwelling and acute urinary retention in an adolescent girl. Foleys catheter to re-establish vaginal outflow. An X- Taiwan J Obstet Gynecol. 2011;50(1):118-20. shaped incision at 2-, 4-, 8-, and 10-o’clock positions is 8. Lim YH, Ng SP, Jamil MA. Imperforate hymen: used which has the advantage of decrease risk of injury to report of an unusual familial occurrence. J Obstet the urethra. The quadrants of the hymen are then excised, Gynecol Res. 2003;29:399. and the mucosal margins are approximated with fine 9. Eksioglu AS, Maden HA, Cinar G, Yildiz YT. delayed-absorbable suture.15 Pressure on the uterus in Imperforate hymen causing bilateral order to expel more blood is discouraged as it can lead to hydroureteronephrosis in an infant with bicornuate retrograde flow through the tubes causing endometriosis uterus. Case Rep Urol. 2012;2012:102683. and tubal adhesions.13 Needle aspiration of mucocolpos or 10. Lui CT, Chan TWT, Fung HT, Tang SYH. A hematocolpos should be avoided as in can lead to retrospective study on imperforate hymen and infection and pyocolpos formation.1 The outcome of hematocolpos in a regional hospital. Hong Kong J surgical hymenotomy is good and the recurrences are Emerg Med. 2010;17(5):435-40. rare.5 11. Khan ZA. Imperforate hymen: a rare case of secondary amenorrhea. J Obstet Gynecol. ACKNOWLEDGEMENTS 2011;31(1):91-2. 12. Abu-Ghanem S, Novoa R, Kaneti J, Rosenberg E. We would like to thank the dept. of obstetrics & Recurrent urinary retention due to imperforate gynecology, SCB medical college, Cuttack and Mahatma hymen after hymenotomy failure: a rare case report Gandhi medical college and research institute, and review of the literature. Urology. Puducherry for their valuable support and co-operation of 2010;78(1):180-2. patients and their families admitted to these hospitals. 13. Anselm OO, Ezegwui UH. Imperforate hymen presenting as acute urinary retention in a 14-year old Funding: No funding sources Nigerian girl. J Surg Tech Case Rep. 2010;2(2):84-6. Conflict of interest: None declared 14. Shen MC, Yang LY. Imperforate hymen complicated Ethical approval: Not required with pyocolpos and lobar nephronia. J Chin Med Assoc. 2006;69(5):224-7. REFERENCES 15. Dominguez CE, Rock JA, Horowitz IR. Surgical conditions of the vagina and urethra. In: Rock JA, 1. Schorge JO, Schaffer JI, Halvorson LM, Hoffman B, eds. TeLinde’s Operative Gynaecology. 10th ed. Bradshaw K. Anatomic disorders. In: Schorge JO, New Delhi: Wolters Kluwer Health/Lippincott eds. Williams Gynecology. 1st ed. New York: Williams & Wilkins; 2008: 508-511. McGraw Hill Medical; 2008: 412-413. DOI: 10.5455/2320-1770.ijrcog20140965 2. Nagai K, Murakami Y, Nagatani K, Nakahashi N, Cite this article as: Rathod S, Samal SK, Rajsekaran Hayashi M, Higaki T, et al. Life threatening acute A, Reddi Rani P, Ghose S. Imperforate hymen and its

complications: report of two cases and review of literature. Int J Reprod Contracept Obstet Gynecol 2014;3:839-42.

International Journal of Reproduction, Contraception, Obstetrics and Gynecology Volume 3 · Issue 3 Page 842 Research Paper Medical Science Volume : 5 | Issue : 2 | Feb 2015 | ISSN - 2249-555X

Imperforate hymen- Can it be treated by a simple vertical incision –A case report.

Keywords imperforate hymen, hymenotomy, cyclic pelvic pain Nigar Asma Assistant professor, department of obstetrics and gynecology ,integral institute of medical sciences and research , dasuli, kursi road , lucknow

ABSTRACT Imperforate hymen is a rare congenital anomaly ,with an incidence of 0.05%-0.1% , although is the most common obstructive congenital anomaly of the female genital tract.It is thought that cause of this anomaly may be congenital defect in the degeneration of central cells of hymen. Generally it presents as an isolated anomaly , but some times genitourinary anomalies may be seen with it. Patients are generally asymptomatic until menarche . Patients may present with primary amenorrhea ,cyclic abdominal pain, urinary retention ,suprapubic lump and tenesmus. Diagnosis is made with history and pelvic examination . Surgical treatment for this anomaly is hymenectomy after a cruciate or x shaped hymenotomy incision. Here we are reporting a case of imperforate hymen treated with a more conservative surgery which involved a simple vertical incision(hymenotomy) .Post operative follow up was uneventful and patient attained normal menses. Hence It could be an alternative option as it is a hymen sparing procedure which involves preservation of hymen and so maintains integrity of female genitalia . This procedure is more acceptable by the patient and their families.

Introduction Case report Imperforate hymen is a rare congenital anomaly of female A 16 year old female came to our surgery department genital tract and is observed in 0.01-0.5 % of new born. [ with the complaints of severe abdominal pain and reten- 1 ,2]. It is a developmental defect in which there is defec- tion of urine for 2 days . she suffered twice with the tive degeneration of central cells of hymen. It is generally same episode of acute retention of urine with abdominal regarded as an isolated congenital anomaly , sometimes pain for the past three months . For this she was cathe- genitourinary anomalies may be seen with it. These pa- terised in some other hospital . she also had not attained tients remain asymptomatic until puberty ,when they pre- menarche. No other significant history was found in per- sent with the complaints of primary amenorrhea ,cyclic sonal and family history. On her physical examination sec- abdominal pain, suprapubic bulge, urinary retention, tenes- ondary sexual characters were normally present. On local mus and constipation.[ 3] pelvic examination it was noted that hymenal opening was not there, instead a bluish bulge was there. An USG Diagnosis is made easily by simple genital examination in scan showed a large mass posterior to her bladder sug- which bluish bulging hymenal membrane is found. It usu- gesting haematocolpos. Bilateral ovaries were normal. She ally manifests as a sporadic case , however familial inherit- was catheterised and underwent hymenotomy by simple ance has also been reported. [2] vertical incision under general anaesthesia because of patient’s preference. .Approx 600 ml dark colored tarry Approximately 58% of patients may complain of pain blood was suctioned . foleys catheter no 16 f was insert- while urinating and retention of urine because of collec- ed for one week.Post operative period was uneventful. tion of menstrual blood in the vagina (hematocolpos).Un- She was discharged and followed up after one and a half treated or late treatment may result in complications like months. She started having normal menses and was doing infertility, endometriosis and adhesions.[4] well. There was no stenosis or infection.

Standard treatment of this condition is hymenectomy after Discussion- a cruciate incision or × shaped hymenotomy incision .An The incidence of imperforate hymen in newborn has been intact hymen is important in some cultures and religions. found to be a rare congenital anomaly, with the reported Patients and families have fears about loosing virginity af- incidence of 0.05%-0.1% .It is thought to be a develop- ter surgical interventions. mental defect in which due to defective degeneration of central cells of hymen , it remains imperforated.This con- We report a cases admitted to our emergency wwith re- dition causes collection of menstrual blood behind im- tention of urine, was diagnosed as a case of imperfo- perforated hymen at menarche. Patient are usually not rate hymen ,and was treated with a conservative hymen asymptomatic until puberty. At puberty they typically pre- sparing surgery with single vertical hymenotomy incision sent with cyclic abdominal pain and primary amenorrhea. . The procedure is less invasive than other methods de- Other complaints are urinary retention, back pain and scribed in the literature and more comfortable for the constipation. patients. These patients are easily missed when they present with The aim of this case report is to show simple virginity pre- retention of urine in emergency department. Proper history serving socially acceptable procedure to provide an intact and pelvic examination is not done like it was seen in our hymen. patient in which patient had two previous episodes of urinary retention in which catheterisation was done but con-

698 X INDIAN JOURNAL OF APPLIED RESEARCH Research Paper Volume : 5 | Issue : 2 | Feb 2015 | ISSN - 2249-555X dition remain undiagnosed. Pic 1. showing bulging bluish membrane of hematocolpos. It is interesting that patient was catheterised twice before coming to us but the nurse who catheterised did not no- tice the abnormal bluish bulge that was enough for diagnosis , neither the doctor suspected the possibility of imperforate hymen.

Acar et al reviewed 65 cases and found average age of 14 years at the time of diagnosis and hematocolpos was inconsistently present.[ 5]

Standard surgical procedure for this condition is hymenectomy using cruciate incision. but in our case we performed hymen sparing surgery in which single vertical incision was used and hymen was not cut.

Hymen sparing surgery was done primarily by M Basaran who treated cases with simple vertical incision .They used few oblique suture to prevent refusion.[6] In our case we did not use sutures instead Foleys catheter was inserted for one week.

Temizkan et al also did virginity sparing surgery for imperforate hymen in two patients .They did simple central excision of the hymen leaving an intact annular hymen ,but they did not use Foleys catheter. No restenosis was seen in their patients.[ 7]

Acar a et al did central oval incision with insertion of 16 f Foleys catheter for two weeks on 65 patients and found equally good results.[ 5] . Cheli et al conducted a study in which radial incision technique was applied on 3 out of 5 cases .In rest of two cases hymenotomy procedure with simple excision was performed and foleys catheter was inserted[8] , as was done in our case.

Ali A et al did simple central excision of the hymen making an annular intact hymen using a Foleys catheter for two weeks in order to prevent restenosis. [9]

In our case we gave simple vertical incision .after drainage of blood from the vagina , Foleys catheter 16F was put for one week and then she was discharged .There was no stenosis and infection in the follow up and patient resumed normal menses.

Conclusion- As standard surgical procedure for imperforate hymen is cruciate incision over the bulging hymenal membrane .Another method could be single vertical incision , as was done in this case .This hymen sparing procedure seems to be more acceptible by most of the patient as it involves preservation of hymenal tissue . The result of one cases is not sufficient to provide a conclusion so studies with large number of cases are required to show the efficacy of procedure ,so that it will have world wide acceptance

REFERENCE 1.Walsh B, Shih R .An unusual case of urinary retention in a competitive gymnast .J Emerg Med.2006;31:279-81 | 2 .Lim Y H, Ng SP, Jamil MA. Imperforate hymen :Report of an unusual familial occurence .J Obstet Gynecol . Res 2003 ;29 no.6 :399-401 | 3 -Wang W, Chen MH ,Yang W, Hwang DL. Imperforate hymen presenting with chronic constipation and lumbago. Report of one case –Acta Paediatric Taiwan.2004;45:340-2 | .4-Chircop R –A case of retention of urine and hematocolpometra . Eur J Med .2003; 10:244-5 | 5- Acar A, Balci O, Karatayli R, Capar M, Colakoglu MC. The treatment | of 65 women with imperforate hymen by a central incision | and application of Foley catheter. BJOG.2007;114(11):1376-9. | 6-Basaran M, Usal D,Aydemir C –Hymen sparing for imperforate hymen :case report and review literature .J Paediatric Adolescent Gynecol .2009 AUG ;22(4):e 61-4. DOI : 10.1016/j.jpag.2008.03.009 | 7.O Temizkan, S K Kucur, S Agar. Virginity sparing surgery for imperforte hymen. Report of two cases and review of literature .J Turkish Ger Gynecol Association. 2012;13(4):278-80 | 8. Cheli D, Kehila M , Sfar E, Zououi B .Imperforate hymen : Can it be treated without damaging the hymenal structure ? Sante 2008 apr-jun 18 (2):83-7 | 9.Ali A, Cetin C, Nedim C ,Kazim G.Treatment of imperforate hymen by application of foley catheter .Eur J Obstet Gynecol Reprod Biol 2003 jan 10;106 (1):72-5 | INDIAN JOURNAL OF APPLIED RESEARCH X 699 Case Report Singapore Med J 2009; 50(11) : e378

Imperforate hymen: cause of lower abdominal pain in teenage girls Mou J W C, Tang P M Y, Chan K W, Tam Y H, Lee K H

ABSTRACT Imperforate hymen is a relatively rare congenital anomaly. However, it is not an uncommon cause of lower abdominal pain presenting in teenage girls. Without careful history taking and thorough examination, the condition can be missed easily. We report an imperforate hymen presenting as abdominal pain in three teenage girls aged 12, 12 and 13 years, respectively, within a six-month period. The presentation was reviewed and the various types of hymenotomy Fig. 1 Photograph shows the bulged hymen with a collection were discussed. of menstrual blood.

Keywords: adolescents, abdominal pain, haematocolpos, hymenotomy, imperforate hymen Singapore Med J 2009; 50(11): e378-e379

INTRODUCTION Imperforate hymen is a relatively rare congenital Department of anomaly, in which the hymenal membrane occludes Surgery, the vaginal orifice, resulting in haematocolpos, which Division of Paediatric Surgery and often leads to abdominal pain in adolescent girls. It was Paediatric Urology, Chinese University of reported that imperforate hymen occurs in one in 1,000 Fig. 2 Operative photograph shows the appearance of the Hong Kong, to one in 10,000.(1) We report three cases of imperforate Prince of Wales introitus after the hymenotomy. Hospital, hymen, presented over a period of six months, that were Shatin NT, Hong Kong SAR initially missed. The importance of detailed history Mou JWC, MBChB, taking and thorough examination is highlighted. Case 2 MRCS In July 2006, a 12-year-old premenarchal girl presented Resident CASE REPORTS with a five-month history of lower abdominal pain Tang PMY, MBBS, MRCS Case 1 associated with tenesmus and dysuria. On the day of Resident In April 2006, a 12-year-old premenarchal girl was admission, she could not pass urine for more than 12 Chan KW, MBChB, admitted with a two-month history of cyclic lower hours. Despite the drainage of 300 ml of urine through a FRCSE Associatte Consultant abdominal pain. Before admission, she had been treated Foley catheter, there was still the presence of a large lower Tam YH, MBChB, by several general practitioners with analgesics. On abdominal mass. Further detailed examination of the FRCSE admission, there was an obvious huge lower abdominal perineum revealed a bluish bulging imperforate hymen. Associatte Consultant mass, and on careful perineal examination, a bluish The diagnosis was made clinically, and a hymenotomy Lee KH, MBChB, FRCSE bulging imperforate hymen was identified (Fig. 1). A and drainage of 500 ml of blood clot were performed. Consultant hymenotomy and drainage of around 600 ml of old blood She could pass urine after the removal of the Foley Correspondence to: products were performed (Fig. 2). She was discharged catheter on postoperative Day 1 and was discharged Dr Jennifer Wai Cheung Mou on postoperative Day 2 with an uneventful recovery. on Day 2 with no more tenesmus or urinary symptoms. Tel: (852) 2632 2953 Fax: (852) 2637 7974 On follow-up, she had remained well and had normal During follow-up, she had had normal menses since the Email: jennifermou@ menses. operation. surgery.cuhk.edu.hk Singapore Med J 2009; 50(11) : e379

Case 3 imaging studies would be needed for better surgical In September 2006, a 13-year-old premenarchal girl planning. was admitted with a ten-month history of on-and-off The treatment for imperforate hymen is hymenotomy. lower abdominal pain. She had attended the accident Before the procedure, the urethra has to be stented to and emergency department on several occasions for the avoid possible damage during the procedure. Two same symptom. On physical examination, the abdomen techniques of hymenotomy are commonly advocated: a was soft with no palpable mass. Perrectal examination simple incision or a small excision of the membrane. In revealed a large pelvic mass, but due to labial adhesion, Case 3, a simple cruciate incision of the hymen, without the hymen was not seen. Ultrasonography showed a big the excision of any part of the membrane, was made in heterogeneous collection, measuring 11 cm × 6.6 cm × the first operation in an attempt to preserve the traditional 7.8 cm, in the vagina. Differential diagnoses included Chinese concept of the importance of first-coitus bleed. a simple imperforate hymen with haematocolpos Acar et al advocated the use of “mini-hymenotomy” (0.5- or transverse vaginal septum. Examination under cm incision) together with keeping a Foley catheter in anaesthesia revealed an imperforate hymen above the situ for two weeks.(5) However, it is not the universally- labial adhesion. A mini-hymenotomy was performed accepted method due to the high recurrence rate. It is and 500 ml of old menstral blood was drained. She was generally advocated that at least part of the membrane be then discharged on postoperative Day 3. At the six- excised. In addition, the incidence of recurrence might month follow-up, she was found to have no menses after further be reduced by plicating the edge of the incised surgery and examination revealed a completely intact membrane. hymen with no opening. She then underwent a re-do Though the outcome after adequate hymenotomy hymenotomy and had regular menses afterwards. for imperforate hymen is usually excellent, follow-up is still necessary to ensure that there is no recurrence DISCUSSION of the problem. With adequate surgery, symptoms Imperforate hymen is an anomaly, which when presenting of dyspareunia, abnormal menstruation or persistent during the adolescent period, can usually be diagnosed problems of micturition/defaecation seldom recur.(4) In by thorough history taking and a physical examination. conclusion, imperforate hymen is not an uncommon Adolescents typically present with primary amenorrhoea, cause of abdominal pain and abnormal menstruation a cyclic pattern of lower abdominal/pelvic pain, with or in adolescent girls. Without a careful history taking without associated symptoms like back pain (38%–40%), and physical examination, the diagnosis can be missed urine retention (37%–60%) or constipation (27%).(2-4) On easily, resulting in a delay in diagnosis and treatment. physical examination, a lower abdominal mass may be An adequately-performed hymenotomy usually leads to palpable, or a pelvic mass may be detected on bimanual an excellent outcome. rectal examination. The diagnosis of imperforate hymen REFERENCES can often be established readily during the perineal 1. Heger AH, Ticson L, Guerra L, et al. Appearance of the genitalia examination when a bluish bulging imperforate hymen in girls selected for nonabuse: review of hymenal morphology and is found at the introitus. However, the condition can be nonspecific findings. J Pediatr Adolesc Gynecol 2002; 15:27-35. easily missed if a careful history taking and detailed 2. Nazir Z, Rizvi RM, Qureshi RN, Khan ZS, Khan Z. Congenital vaginal obstructions: varied presentation and outcome. Pediatr examination are not carried out, as illustrated by our Surg Int 2006; 22:749-53. cases. This highlights the importance of pursuing the 3. Wang W, Chen MH, Yang W, Hwang DL. Imperforate hymen basic principles in medicine, viz. thorough history presenting with chronic constipation and lumbago: report of one case. Acta Paediatr Taiwan 2004; 45:340-2. taking and physical examination. In girls presenting with 4. Liang CC, Chang SD, Soong YK. Long-term follow-up of women abdominal pain, a careful examination of the introitus, who underwent surgical correction for imperforate hymen. Arch apart from perrectal examination, is mandatory. Imaging Gynecol Obstet 2003; 269:5-8. 5. Acar A, Baki O, Karatayli R, Capar M, Colakoglu MC. The or laboratory studies are usually not indicated for a treatment of 65 women with imperforate hymen by a central classical presentation of imperforate hymen. However, if incision and application of Foley catheter. BJOG 2007; the diagnosis is uncertain, as illustrated in the third case, 114:1376-9. Hindawi Publishing Corporation Case Reports in Urology Volume 2012, Article ID 102683, 4 pages doi:10.1155/2012/102683

Case Report Imperforate Hymen Causing Bilateral Hydroureteronephrosis in an Infant with Bicornuate Uterus

Ayse Secil Eksioglu,1 Hasim Ata Maden,2 Gokce Cinar,1 and Yasemin Tasci Yildiz1

1 Department of Pediatric Radiology, Dr. Sami Ulus Women and Children’s Hospital, Babur¨ Caddesi, No. 44, Altındag, 06080 Ankara, Turkey 2 Department of Pediatric Surgery, Dr. Sami Ulus Women and Children’s Hospital, Babur¨ Caddesi, No. 44, Altındag, 06080 Ankara, Turkey

Correspondence should be addressed to Ayse Secil Eksioglu, [email protected]

Received 22 March 2012; Accepted 24 April 2012

Academic Editors: G. L. Gravina, C. Liao, and S. Takahashi

Copyright © 2012 Ayse Secil Eksioglu et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

A rare case of imperforate hymen associated with bicornuate uterus in an infant is presented as a cause of bilateral hydroureteronephrosis and pelvic mass in infancy. The importance of postoperative radiologic evaluation for diagnosis of accompanying uterine abnormalities is introduced. A 8-month-old girl with restlessness and intermittent fever was brought to the daily outpatient clinic by her parents. Ultrasound exam showed bilateral grade 4 hydroureteronephrosis and a large cystic pelvic mass. Magnetic resonance scan of the pelvis revealed marked hematocolpos. A cruciate incision was made over the hymen under general anesthesia. During a 6-month followup gradual resolution of bilateral hydroureteronephrosis was documented. Although the details of the uterine anomaly were obscured in preoperative imaging, postoperative US and MR demonstrated bicornuate uterus. Postoperative pelvic radiologic examination is highly recommended to verify the resolution of hematocolpos and to screen for any concomitant anomalies that can have long-term clinical signiﬁcance.

1. Introduction hydroureteronephrosis and pelvic mass in infancy and to introduce postoperative radiologic evaluation for diagnosis Imperforate hymen which has an incidence of 0.014%–0.1% of accompanying uterine abnormalities. Informed consent is usually asymptomatic until menstruation starts [1–3]. was taken from the patient’s parents, and the case was pre- However, under endogenous maternal estrogen stimulation, sented as a poster in 31st National Radiology Congress, secretions produced by the fetal uterovaginal mucosa can Antalya, Turkey in November 2010. accumulate in the vagina and uterus resulting in hydrocolpos before puberty. This may cause a mechanical eﬀect on the urethra and bladder and lead to obstructive urinary symp- 2. Case toms. When so, manifestation as a pelvic mass which severely compresses the bladder, and the ureters causing hydroureter- A 8-month-old girl presenting with restlessness and inter- onephrosis is rare in infancy [4–6]. mittent fever of unknown etiology was brought to the daily Since imperforate hymen is generally considered not to clinic by her parents. The parents did not complain about any be associated with other Mullerian¨ abnormalities, further problems related to urination, and there was some amount of investigation of these patients for concomitant urogenital daily urine output. abnormalities has been thought to be unnecessary until today She had been born term after an uneventful pregnancy [7]. via normal vaginal delivery. Far to the parents’ knowledge the The aim of this report is to increase the awareness newborn examination was normal. On physical examination, about the possibility of this condition as a cause of bilateral she had normal vital signs. She was found to have a midline 2 Case Reports in Urology

(a) (b)

Figure 1: Pelvic US. (a) Transverse view shows a large well-defined cystic mass with internal echoes, which could easily be misinterpreted as overly distended urinary bladder. Note the bilateral ureteral dilatation. (b) Insertion of a Foley catheter makes it clear that the cystic mass is separate from the urinary bladder which is severely compressed and therefore hard to detect on ultrasound. abdominal mass. The rest of the examination was normal. Initial laboratory values were unremarkable, except for a mild leukocytosis and plenty of erythrocytes in the urine. The urine culture did not reveal any pathological findings. Blood urea levels and creatinine levels were normal. Abdominopelvic sonography showed bilateral grade 4 hydronephrosis and a large well-circumscribed midline cystic mass including internal echoes. The cyst reached up to the umbilical level (Figure 1(a)). No bladder could be identified on control pelvic ultrasonography (US) exams until the exam was repeated after the insertion of a Foley urethral catheter (Figure 1(b)). Magnetic resonance (MR) scan of the abdomen and pelvis was obtained; it revealed hematocolpos that was causing marked distention of the uterus and cervix. The urinary bladder was significantly compressed (Figure 2). The presumptive diagnosis of hydrometrocolpos secondary to an obstructing lesion was made. Figure 2: Sagittal-T2-weighted MR image. Marked distention of The patient underwent voiding cystourethrogram the uterus and cervix is demonstrated. Note the compressed urinary (VCUG) which demonstrated no reflux but a compressed bladder with little urine in it. urinary bladder with a diminished urine volume of approximately 25cc (Figure 3). The diagnosis of imperforate hymen was made under sedation during the instrumentation for the procedure by the inspection of a protuberant mass on retraction of the labia. The family had not noted any perineal abnormalities prior to presentation to the clinic. The patient was taken into the operating room, and a simple cruciate incision was made over the hymen under general anesthesia which resulted in drainage of approximately 500 mL cloudy, yellowish, nonbloody mucosal secretions from the vagina. No acute or subacute complications occurred. During a period of 6-month followup, repeated ultrasound exams documented the gradual resolution of bilateral hydroureteronephrosis. The suspicion of bicornuate uterus raised by pelvic control ultrasound was verified by a postoperative MR exam (Figures 4(a), 4(b),and4(c)).

3. Discussion

Imperforate hymen is an uncommon congenital disorder Figure 3: Preoperative VCUG. Image reveals no reﬂux but a urinary of the female genital tract [1, 2]. The hymen is an embry- bladder which could not receive appropriate amount of contrast ological remnant of mesodermal tissue which is supposed to material due to severe compression secondary to hematocolpos. Case Reports in Urology 3

(a) (b) (c)

Figure 4: T1-weighed postoperative MR images. (a) and (b): two consecutive pelvic coronal images clearly show the two cavities (arrows) of the uterus separated by an incomplete longitudinal septum which was diﬃcult to depict earlier. (c) axial view through corpus shows bicornuate uterus.

perforate during the later stages of embryonic development Whereas imperforate hymen is a problem that could be [8]. The usual clinical presentation of imperforate hymen is easily solved by a minor operation without sequela [9], even as an expanding abdominal mass and cyclic lower abdominal though rare [7], any accompanying uterine anomaly like or back pain in an adolescent girl with primary amenorrhea. bicornuate uterus as in this case could potentially have a long It is rarely diagnosed in the neonatal period or infancy. lasting impact on fertility [10]. Hydrocolpos or mucocolpos triggered by endogenous mater- Early diagnosis of accompanying genital anomalies nal estrogen stimulation rarely presents as bilateral severe would not affect the immediate management but would save hydroureteronephrosis in infancy [4–6]. time and money on the long range. Postoperative imaging is This particular case did not present as acute urinary also recommended for the followup of resolution of findings retention which would be far more alarming. Less obvious in response to surgery. changes in urination can be missed by the family in a child Although preoperative US and MR examination both of this age. Imperforate hymen can be hidden under a very revealed a very distended uterus in the form of a large cystic ff nonspecific set of complaints with a broad di erential diag- mass and the details of the uterine anomaly were obscured, nosis, like fever of unknown etiology and restlessness. postoperative radiologic imaging was diagnostic. Not every In this case ultrasound examination revealed bilateral case receives pre- and/or postoperative MR exams. The diag- grade 4 hydronephrosis but was unable to demonstrate the nosis is usually based on clinical examination and preopera- normal pelvic anatomy. It revealed a giant pelvic cystic mass tive ultrasound. No further information regarding the pelvic without any change in appearance on more than one ultra- anatomy may be obtained. sound and which could be easily misinterpreted as a dis- We suggest postoperative radiologic examination, prefer- tended urinary bladder since the bladder could not be visu- ably by pelvic ultrasound since it is more accessible and alized on either exam. Insertion of a Foley catheter might be cheaper, both to verify the resolution of hematocolpos and helpful to distinguish between a real pelvic mass or urinary to screen for any concomitant anomalies that can have long- bladder overdistension in such cases. term clinical significance. Incorporation of the external genitalia into the newborn nursery exam and well baby examination is highly recommended so that genital anomalies can be diagnosed early. When the diagnosis of imperforate hymen is made in a new- References born or an infant, assuming that there are no urinary signs [1]I.M.Usta,J.T.Awwad,J.A.Usta,M.M.Makarem,andK. or obstruction, observation throughout childhood and a S. Karam, “Imperforate hymen: report of an unusual familial planned hymenotomy after the onset of puberty and before occurrence,” Obstetrics and Gynecology, vol. 82, no. 4, pp. 655– menarche is optimal. Surgery in the presence of adequate 656, 1993. estrogenization avoids scarring and needs to repeat surgery. [2] L. M. Winderl and R. K. Silverman, “Prenatal diagnosis of When there are signs of urinary obstruction or an abdominal congenital imperforate hymen,” Obstetrics and Gynecology, mass as in this case, immediate surgery is needed. vol. 85, no. 5, pp. 857–860, 1995. 4 Case Reports in Urology

[3] T. Soyer, “Labial Synechia, Imperforated hymen, vaginal agenesis, atresia and stenosis,” Turkiye Klinikleri Journal of Pediatric Surgery, vol. 2, no. 1, pp. 57–64, 2009. [4] N. S. Johal, S. Bogris, and I. Mushtaq, “Neonatal imperforate hymen causing obstruction of the urinary tract,” Urology, vol. 73, no. 4, pp. 750–751, 2009. [5]A.Gyimadu,B.Sayal,S.Guven,andG.S.Gunalp,“Hema- tocolpos causing severe urinary retention in an adolescent girl with imperforate hymen: an uncommon presentation,” Archives of Gynecology and Obstetrics, vol. 280, no. 3, pp. 461– 463, 2009. [6] F. Shariﬁaghdas, H. Abdi, H. Pakmanesh, and N. Eslami, “Imperforate hymen and urinary retention in a newborn girl,” Journal of Pediatric and Adolescent Gynecology, vol. 22, no. 1, pp. 49–51, 2009. [7] C. Dane, B. Dane, M. Erginbas, and A. Cetin, “Imperforate hymen-A rare cause of abdominal pain: two cases and review of the literature,” Journal of Pediatric and Adolescent Gynecol- ogy, vol. 20, no. 4, pp. 245–247, 2007. [8]A.Derbent,S.Simavli,N.Kos¨¸us¨¸, and H. Kafali, “Sponta- neous re-closure of hymen during pregnancy, seventeen years after hymenotomy: case report,” Turkiye Klinikleri Jinekoloji Obstetrik, vol. 20, no. 6, pp. 392–394, 2010. [9] C.-C. Liang, S.-D. Chang, and Y.-K. Soong, “Long-term follow-up of women who underwent surgical correction for imperforate hymen,” Archives of Gynecology and Obstetrics, vol. 269, no. 1, pp. 5–8, 2003. [10] J. M. Levsky and R. T. Mondshine, “Hematometrocolpos due to imperforate hymen in a patient with bicornuate uterus,” American Journal of Roentgenology, vol. 186, no. 5, pp. 1469– 1470, 2006. M EDIATORSof INFLAMMATION

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CASE REPORT

Imperforate hymen causing congenital hydrometrocolpos

V. Vitale • B. Cigliano • G. Vallone

Received: 18 October 2012 / Accepted: 8 December 2012 / Published online: 2 March 2013 Ó Societa` Italiana di Ultrasonologia in Medicina e Biologia (SIUMB) 2013

Abstract A 3-day-old girl in good health was referred to Non era visibile l’utero; i reni erano normali senza our department for the evaluation of an abdominal mass evidenza di idronefrosi. Ovaie normali. L’esame fisico detected at birth. Prenatal ultrasound (US) examinations della neonata, confermava il sospetto ecografico mettendo had shown no anomaly. US examination revealed the in evidenza la presenza di una membrana bombata che presence of a hypoechoic and corpusculated cystic forma- copriva l’introito vaginale sulla base del quale e` stata fatta tion of about 8 9 5 9 4 cm located in the mid region of diagnosi di idrometrocolpo. Si e` proceduto con un’imen- the abdomen. The uterus was not visible and the kidneys ectomia e successiva aspirazione di circa 100 ml di liquido were normal with no sign of hydronephrosis. The ovaries lattescente. Non vi erano altre anomalie congenite presenti were normal. Physical examination confirmed US findings e il controllo ecografico successivo ha mostrato regolare revealing the presence of a curved membrane which cov- struttura dell’utero. ered the vaginal opening. Based on these findings, the patient was diagnosed to have hydrometrocolpos. Hymen- Introduction ectomy was performed and about 100 ml of milky fluid was subsequently removed by aspiration. The patient pre- Hydrometrocolpos is an accumulation of uterine and vag- sented no other congenital anomalies and US follow-up inal secretions as well as menstrual blood in the uterus and showed a normal structure of the uterus. vagina. Usually this condition manifests at puberty caused by an obstruction of the female genital tract. Keywords Imperforate hymen Á Congenital The most frequent cause of hydrometrocolpos is the hydrometrocolpos Á Pelvic mass Á Ultrasound presence of imperforate hymen due to failure of partial resorption of this membrane during the embryonic devel- Riassunto Una neonata di 3 giorni, in buona salute, e` opment; the incidence is 0.0014–00.1 % in full-term giunta alla nostra osservazione per la valutazione di una newborns [1, 2]. Congenital hydrometrocolpos is a rare massa addominale riscontrata alla nascita. I controlli event with an incidence of about 0.006 % [3]. We present ecografici prenatali erano negativi. L’esame ecografico ha the US features of hydrometrocolpos in a newborn girl mostrato la presenza di una formazione cistica a sede before and after surgical treatment. mediana ipoecogena corpuscolata di circa 8 9 5 9 4 cm.

Description of the case V. Vitale Á G. Vallone (&) Divisione di Diagnostica Pediatrica, Dipartimento di Scienze Biomorfologiche e Funzionali, Universita` Federico II di Napoli, A 3-day-old girl born by Cesarean section at 38 weeks of Naples, Italy gestation was referred to our department for the evaluation e-mail: [email protected] of an abdominal mass detected at US examination performed on the day of birth. The patient was otherwise B. Cigliano Dipartimento di Pediatria, in good condition and weighed 3.330 kg. Prenatal US Universita` Federico II di Napoli, Naples, Italy examinations showed no anomaly. 123 38 J Ultrasound (2013) 16:37–39

Fig. 3 Longitudinal pelvic US scan obtained after drainage Fig. 1 Longitudinal scan reveals a large hypoechoic mass in the mid region of the abdomen extending from the upper middle to the lowest normal appearance of the uterus and vagina with no signs region of other congenital anomalies (Fig. 3).

Discussion

Hydrometrocolpos is an unusual finding in newborn infants. It occurs when a genital tract obstruction is associated with accumulation of cervical and endometrial gland secretions. This condition may be caused by congenital malformations of the genital tract such as vaginal atresia, transverse vaginal septum and imperforate hymen. It may also be associated with the McKusick–Kaufman syndrome, an autosomal recessive disorder characterized by vaginal atresia with hydrometrocolpos, polydactyly, congenital heart defects and non-immune mediated hydrops fetalis [4]. In the present case, imperforate hymen was the cause of Fig. 2 Axial US scan shows the mass and the right ovary which is hydrometrocolpos. intact Imperforate hymen is a result of the hymen failing to rupture during the eighth week of gestation; it may be an US examination was carried out on GE Logiq 9 using a isolated abnormality or associated with other malforma- micro-convex 5–9 MHz probe. The image showed the tions, such as imperforate anus, bifid clitoris, polycystic presence of an inhomogeneous hypoechoic and corpuscu- kidney. lated cystic formation of about 8 9 5 9 4 cm with well- As described in other cases [5], hydrometrocolpos is demarcated margins; it was located in the mid region of the usually diagnosed prenatally as the cause of abdominal abdomen extending from the upper middle to the lowest cystic mass. However, in the present case prenatal exam- region (Figs. 1, 2). The bladder was empty and com- inations were all negative. pressed, the kidneys and ovaries were normal. The uterus Differential diagnosis of a perinatally identified was not visible. abdominal mass should include ovarian cysts, intra- Hydrometrocolpos was suspected and subsequently abdominal sacrococcygeal teratoma (type IV), neuroblas- confirmed at physical examination of the external genitalia, toma, mesoblastic nephroma, bowel duplication, genital- which revealed the presence of a soft oval mass with urinary anomalies and anterior sacral meningocele. an imperforate hymen at the vaginal opening. After inci- Hydrometrocolpos may cause urinary stasis and acute sion of the hymenal membrane, approximately 100 ml of renal failure due to obstructive uropathy [6, 7]. In the milky fluid was aspirated. Subsequent US imaging showed present case, physical and US examinations showed that 123 J Ultrasound (2013) 16:37–39 39 both kidneys were normal with no sign of hydronephrosis congenital hydrometrocolpos. J Matern Fetal Neonatal Med and that there were no other congenital anomalies. 15:135–137 2. Winderl LM, Silverman RK (1995) Prenatal diagnosis of congen- In hydrometrocolpos caused by imperforate hymen, ital imperforate hymen. Obstet Gynecol 82:655–656 hymenectomy has proved to be an adequate, conservative 3. Bhargava P, Dighe M (2009) Prenatal US diagnosis of congenital treatment [8]. A peculiar phenomenon was in the present imperforate hymen. Pediatr Radiol 39:1014 case the amount of fluid removed (about 100 ml). 4. Tseng JJ, Ho JY, Chen WH, Chou MM (2008) Prenatal diagnosis of isolated fetal hydrocolpos secondary to congenital imperforate In conclusion, although hydrometrocolpos is a rare hymen. J Chin Med Assoc 71(6):325–328 event, this disorder should be kept in mind if pre- and/or 5. El-Messidi A, Fleming NA (2006) Congenital imperforate hymen postnatal examinations reveal the presence of a pelvic and its life-threatening consequences in the neonatal period. mass. J Pediatr Adolesc Gynecol 19:99–103 6. Sharifiaghdas F, Abdi H, Pakmanesh H, Eslami N (2009) Imperforate hymen and urinary retention in a newborn girl. Conflict of interest The authors have no conflict of interest to J Pediatr Adolesc Gynecol 22:49–51 declare. 7. Aygun C, Ozkaya O, Ayyildiz S, Gu¨ngo¨r O, Mutlu B, Ku¨çu¨ko¨du¨k S (2006) An unusual cause of acute renal failure in a newborn: hydrometrocolpos. Pediatr Nephrol 21:572–573 References 8. Gu¨rates¸ B, Kazez A, Sapmaz E, Bulgan E, Yayla M (2000) Prenatal diagnosis of congenital imperforate hymen. Perinatoloji 1. Messina M, Severi FM, Bocchi C, Ferrucci E, Di Maggio G, Dergisi 8:117–119 Petraglia F (2004) Voluminous perinatal pelvic mass: a case of

123 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology

Imperforate Hymen

Author: Paula J Adams Hillard, MD; Chief Editor: Richard Scott Lucidi, MD more...

Updated: Jun 12, 2013

Background

Imperforate hymen is at the extreme of a spectrum of variations in hymenal configuration. Variations in the embryologic development of the hymen are common and result in fenestrations, septa, bands, microperforations, anterior displacement, and differences in rigidity and/or elasticity of the hymenal tissue. Inspection of the external genitalia and anus are important components of the physical examination of the female neonate and child.[1]

While this examination can and should be accomplished by the pediatrician, the observant delivering obstetrician can learn much about the normal variations in genital configuration by examining the female neonate in the delivery room, keeping in mind the influence and structural changes induced by maternal estrogens. Under this influence, the labia majora are plump, the hymen is elastic and often fimbriated, and the mucosal surfaces (ie, introitus, fossa navicularis, vaginal vestibule) are pale pink.

Problem

Imperforate hymen has been diagnosed with prenatal ultrasound documentation of bladder outlet obstruction due to hydrocolpos or mucocolpos. However, in spite of the recommendations for inspection of the external genitalia during the neonatal and early childhood period, variations in hymenal anatomy commonly escape diagnosis until the time of menarche. See the image below.

Imperforate hymen, classic appearance of bulging, blue-domed, translucent membrane.

Different normal variants in hymenal configuration are described, varying from the common annular, to crescentic, to navicular ("boatlike" with an anteriorly displaced hymenal orifice). Hymenal variations are rarely clinically significant before menarche. In the case of a navicular configuration, urinary complaints (eg, dribbling, retention, urinary tract infections) may occasionally result. Sometimes, a cribriform (fenestrated), septate, or navicular configuration to the hymen can be associated with retention of vaginal secretions and prolongation of the common condition of a mixed bacterial vulvovaginitis.

Occasionally, a hymenal tag will protrude from the vaginal vestibule, leading to concerns about a tumor or other significant pathology. These hymenal tags are of no clinical significance, and they do not require therapy if hymenal origin can be excluded based on findings from a careful examination.

Imperforate hymen in infancy or childhood

On occasion, an infant or young child may be thought to have an imperforate hymen. However, after the neonatal period, when maternal estrogen levels have declined, examination of the area may be challenging owing to the small area involved. Careful examination with pressure applied to the fourchette may reveal microperforations, sometimes with an anteriorly displaced opening just beneath the urethra. Capraro described a surgical technique similar to a perineotomy to correct such a defect; however, in asymptomatic patients, waiting until puberty is generally recommended before deciding whether such a technique is necessary.

The hymenal changes that result from estrogenization (increased elasticity and fimbriation) may reveal the hymen to be open and obviate the need for surgery. With estrogen stimulation, the hymen could be described as having the appearance of an annular "scrunchie" (ie, a fabric-covered elastic hair tie). In addition, surgical procedures to http://emedicine.medscape.com/article/269050-overview 1/10 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology the vagina and hymen during childhood, when endogenous estrogen levels are low, may result in scarring and the need for subsequent surgical revision. Thus, surgery during this time should generally be avoided if possible. If the hymen is suspected to be imperforate during childhood, re-examination should be performed after the onset of estrogen production, as signaled by breast development. If required, surgery can be performed at this time when healing is optimal and prior to the accumulation of a hematocolpos.

In a review of 23 cases of imperforate hymen, Posner et al emphasizes the ease of making a diagnosis of imperforate hymen by routine genital examinations in childhood.[2] The authors compared the significant delays and difficulties in making the diagnosis after the onset of puberty, primarily because the diagnosis was not considered, with the simplicity of making the diagnosis in asymptomatic prepubertal children by a simple genital examination.

Sexual abuse

Accurate description of the morphology and integrity of the hymen is critical in the diagnosis of female sexual abuse. Imperforate hymen has been described as occurring as a result of scarring from penetration and abuse, thus emphasizing the importance of an early examination to document the congenital, rather than acquired, etiology.[3] Concerns about hymenal disruption and lacerations associated with sexual abuse with digital or penile penetration have led to discussions of the normal hymenal diameter. However, this concept has now largely been abandoned.[4]

Experts in sexual abuse assessment have used unaided visual examination and colposcopy to examine the integrity of the hymenal ring. A normal examination or nonspecific findings are commonly found in cases of alleged sexual abuse unless the abuse is quite recent.[5]

Lacerations through the hymen into the fossa navicularis and introitus suggest a penetrating injury. Frequently, sexual abuse evaluations are conducted at some time remote from the immediate injury; thus, normal findings or healed or healing lacerations may be noted.

Muram concluded that the use of the colposcope by an experienced examiner adds little to an evaluation by an experienced examiner with expertise in abuse.[6] In addition, Muram proposed a scale that the examiner can use to evaluate physical findings as normal, abnormal and nonspecific, abnormal and suggestive of abuse, and definitive for abuse.[6] That last category includes only the situation in which sperm are found during the examination. Additional aids to the examination of the hymen have been described, including the procedure of inserting a Foley catheter into the vagina and inflating the balloon behind the hymen to stretch the hymenal margin and allow for a better examination.[7]

Anatomic anomalies

The classic image of an imperforate hymen is noted at the time of typical diagnosis: after the onset of menses, when a hematometrocolpos is present (see the image below).

Imperforate hymen, classic appearance of bulging, blue-domed, translucent membrane.

Consider anatomic anomalies that can be confused with imperforate hymen in the differential diagnosis. These anomalies include the following:

Acquired labial adhesions (see image below)

http://emedicine.medscape.com/article/269050-overview 2/10 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology

Extensive labial adhesion. Not to be confused w ith imperforate hymen.

Obstructing or partially obstructing vaginal septa (longitudinal or transverse) Vaginal cyst Vaginal agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome) with or without the presence of a uterus or functional endometrium (see image below)

Vaginal agenesis. Not to be confused w ith imperforate hymen.

Complete androgen insensitivity syndrome (previously termed testicular feminization)

Epidemiology

Frequency

Imperforate hymen is likely the most frequent obstructive anomaly of the female genital tract, but estimates of its frequency vary from 1 case per 1000 population to 1 case per 10,000 population. A population-based study estimated the frequency at 0.5 case per 1000 women (95% confidence interval, 0.3-0.7).[8]

Heger et al examined 147 premenarchal girls with a mean age of 63 months to collect normative data on genital anatomy; an imperforate hymen was found in only one patient (< 1%) and hymenal septa were found in 3 (2%).[9]

Imperforate hymen usually occurs sporadically, but a handful of cases have been reported to be familial.[10, 11] Examination of first-degree relatives/female siblings of affected individuals has been recommended.

Etiology

Imperforate hymen and related genital tract anomalies result from abnormal or incomplete embryologic development. Pathophysiology

The genital tract develops during embryogenesis, from 3 weeks' gestation to the second trimester. The initial development of both the male and female genital tracts is identical and is referred to as the indifferent stage of http://emedicine.medscape.com/article/269050-overview 3/10 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology development. Note the following:

Paired wolffian (mesonephric) ducts connect the mesonephric kidney to the cloaca. The metanephric or true kidney derives from the ureteric bud (arising from the mesonephric duct) at about the fifth embryonic week. The paramesonephric or müllerian ducts can be identified during the sixth week of embryologic development and lie lateral to the wolffian ducts until they reach the caudal end of the mesonephros, where they come toward the midline. During the seventh week, the urorectal septum forms to separate the rectum from the urogenital sinus. By the ninth week, the müllerian ducts move caudally to reach the urogenital sinus, forming the uterovaginal canal and inserting into the urogenital sinus.

By the 12th week, the paired müllerian ducts have fused into a single tube (ie, primitive uterovaginal canal). Two solid evaginations from the distal aspects of the müllerian tubercle form the sinovaginal bulbs (of urogenital sinus origin) or vaginal plate. The initial or cephalad portion of the müllerian ducts forms the fimbria and fallopian tubes; the more distal segment forms the uterus and upper vagina. The canalization of the paramesonephric ducts and/or upper vagina joins with the vaginal plate, which canalizes beginning caudally and creates the lower vagina. By the fifth month of gestation, the canalization of the vagina is complete. The hymen itself is formed from the proliferation of the sinovaginal bulbs, becoming perforate before or shortly after birth. An imperforate hymen results when this "sheet" of tissue fails to completely canalize. Varying degrees of perforation result in findings such as a cribriform or septate hymen.

Gonadal development

The development of the gonads occurs from the migration of primordial germ cells to the genital ridge, while the genital tract itself develops from the müllerian ducts (paramesonephric ducts), urogenital sinus, and vaginal plate. Thus, anomalies of the vagina, hymen, and uterus are not accompanied by abnormalities of ovarian development.

In girls with hymenal anomalies, hormonal and endocrinologic function is normal, leading to expected pubertal breast and pubic hair development. In cases of uterovaginal agenesis, imaging may fail to detect ovaries in the normal location (they may be located high and/or lateral in the pelvis), leading to unnecessary concern that the ovaries may be absent. Patients and families can be easily reassured that given both embryologic development and normal hormonal function (evidenced by the presence of normal breast development), the ovaries are present and functioning appropriately.

Because the mesodermal layer contributes to the development of the kidneys, gonads, and ductal structures, defects or insults in embryologic development may result in congenital defects of the kidneys or ureters that accompany abnormalities of the vagina and uterus. These anomalies should be considered with vaginal and uterine anomalies. However, given the embryologic origins of hymenal anomalies, urologic abnormalities are not associated.

The lining of the urethra and urinary bladder derives from endoderm, and the urogenital sinus forms the urethra and vestibule in females. The ectoderm fuses with the endoderm to contribute to the patency and canalization of the genital tract. Defects in this process lead to fusion failures and imperforate and obstruction defects.

Familial occurrence

Familial occurrence, although rare, is reported and screening by history or examination of family members is warranted.[10, 11] Dominant transmission (either sex-linked or autosomal) and sibships suggesting a recessive mode of inheritance are described.[12] The inheritance of müllerian defects likely is polygenic or multifactorial, although some syndromes of heritable disorders are described with associated genital and nongenital anomalies.

Anomalies of the female reproductive tract

Anomalies of the female reproductive tract can result from agenesis or hypoplasia, vertical fusion and/or canalization defects, lateral fusion and/or duplication abnormalities, or failure of resorption, resulting in septa. Recent reports have noted the concurrent presence of lateral fusion defects with imperforate hymen.[13]

Presentation

Prenatal diagnosis

Rarely, diagnosis of imperforate hymen in the fetus has been made with obstetric ultrasonography. In such cases, the anomaly is visible on the imaging study because of hydrocolpos, hydrometrocolpos, or mucocolpos.[14, 15]

Diagnosis in infancy or childhood

The diagnosis is infrequently made during infancy in the neonatal nursery. The infant may have a bulging, yellow- gray mass at or beyond the introitus. Several case reports describe the presence of an abdominal mass in association with urinary obstruction.

Ultrasonography is an essential first step in diagnosis, precluding unwise and unplanned surgical intervention with resultant injury to the urethra or other pelvic structures, and excluding other more complicated anomalies.

Routine examination of the female genitalia by primary care clinicians during childhood is strongly recommended so that genital abnormalities can be diagnosed early.[1] Observation throughout childhood, with a planned hymenotomy after the onset of puberty is a reasonable course of action in most cases diagnosed in infancy or childhood, assuming no urinary symptoms or obstruction is present. Surgery in the presence of adequate estrogenization avoids scarring and the potential need for a repeat surgery that can occur to correct scarring when surgery is performed on the unestrogenized hymen and vagina. http://emedicine.medscape.com/article/269050-overview 4/10 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology If the diagnosis is equivocal (ie, imperforate hymen vs labial adhesions vs late-onset congenital adrenal hyperplasia), referral to a pediatric gynecologist may be warranted. Typically, a mucocele is not present even if the condition is noted at birth. If a patient is diagnosed with an asymptomatic imperforate hymen in infancy or childhood beyond the neonatal period, the optimal time for surgical repair is after the onset of puberty and prior to menarche.

Diagnosis and surgical repair in adolescence

Diagnosis of imperforate hymen depends on an awareness of the condition as a possible anomaly and surveillance with well-child care. The typical presenting complaint is primary amenorrhea, but this is a late presentation of a condition that should have been diagnosed at an earlier time. Textbooks frequently state that amenorrhea is not pathologic until age 16 years. Statistically, this statement is not evidence-based, as age 15 years represents the 98th percentile for girls in the United States and other developed countries.[16]

Additionally, failure to menstruate beyond 2-3 years from the onset of breast development, thelarche, is also statistically uncommon, and should be investigated to determine a cause. Imperforate hymen is one uncommon, but important, anatomic cause of primary amenorrhea.

When the condition presents as abdominal pain or an abdominal mass (see image below), diagnostic testing is often extensive because the condition is not considered.[1] An abdominal mass may prompt the consideration of an ovarian tumor and tumor markers may be obtained. While a false-positive elevation of CA-125 in premenopausal women has numerous causes, and testing has thus been discouraged, elevated CA-125 and 19.9 have been described with imperforate hymen, and may delay the diagnosis.[17, 18]

Abdominal mass w ith imperforate hymen.

Surgical repair after the onset of puberty but before menarche is optimal. The most common scenario is that in which a young woman presents with increasingly severe intermittent abdominal and pelvic pain due to a large hematocolpos and hematometra. This situation is preventable, as routine examinations of the genitalia can detect this obstruction and allow correction before menarche.

Walsh and Shih present a case of a 14-year-old elite athlete who presented to the emergency department and her pediatrician on multiple occasions over the course of several months with symptoms of cyclic abdominal pain, urinary retention, and constipation due to hematocolpos and hematometra.[19] This is an all too common presentation. In this reported case, even after placement of a Foley catheter for urinary retention on 2 separate occasions, the diagnosis of imperforate hymen was missed.

While these young adolescents typically present to an emergency department with relatively acute pain, this condition should generally not be managed emergently until the definitive diagnosis is made. Defining the anatomy with appropriate imaging techniques and arranging for the most skilled and experienced gynecologist to perform surgery on a scheduled rather than emergent basis is essential. If necessary, menstrual suppression with gonadotropin-releasing hormone (GnRH) analogs can minimize pain pending appropriate imaging and clarification of anatomy. This is more likely to be necessary with complex genital anomalies than with a straightforward imperforate hymen.

Urinary pressure and even retention, with hydroureter and/or hydronephrosis, may occur due to the mass effect and resultant obstruction. Vaginal and rectal pressure is typically present. Severe constipation and low-back pain are described as presenting symptoms. The laborlike menstrual cramps may be severe and cyclic, although the cyclic nature of the symptoms may not be easily or immediately appreciated by the young woman or her family.

Unfortunately, the typical findings at diagnosis may include a large collection of blood within the uterus (hematometra) and an even larger collection of blood within the more distensible vagina (hematocolpos). Additional findings may include blood-filled fallopian tubes (hematosalpinges) and signs of retrograde menses, occasionally to the point of the development of intra-abdominal endometriosis and severe pelvic adhesions. The classic teaching is that endometriosis associated with obstructive anomalies resolves spontaneously and does not cause problems with subsequent pain and infertility compared with endometriosis arising spontaneously; however, this assertion is anecdotal rather than evidence-based.

Clinically, families are often concerned about whether the ovaries are normal when vaginal or hymenal anomalies are present; the course of separate embryologic development allows assurance of normal hormonal function without any need for hormonal testing or ovarian imaging. The exception to this is the diagnosis of androgen insensitivity syndrome with XY chromosomal complement in which the gonads require removal to prevent malignant transformation.

Differential diagnosis http://emedicine.medscape.com/article/269050-overview 5/10 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology The differential diagnosis of an imperforate hymen includes many conditions, some rare and others relatively common. Absolute confirmation of the diagnosis of an imperforate hymen is imperative prior to any attempted surgical repair in order to prevent vaginal scarring that can occur if a thick vaginal septum is inadvertently confused with a thin imperforate hymen.

Labial adhesions

See the image below.

Extensive labial adhesion. Not to be confused w ith imperforate hymen.

The presence of acquired labial adhesions in a prepubertal girl is a common situation that is often confused with absence of the vagina. Labial adhesions, sometimes incorrectly termed vaginal adhesions, are not congenital and result from acquired labial agglutination most commonly due to inflammation. Small areas of labial adhesions can be managed expectantly. Extensive labial adhesions or those associated with such symptoms as recurrent urinary tract infections, urinary dribbling, or recurrent vulvovaginitis can be managed easily using the topical application of estrogen cream for 2-6 weeks. Such treatment results in marked thinning of the adhesions, often with spontaneous resolution.

Separation of thick adhesions is possible in an office setting with a child who can be restrained; however, this procedure ultimately is counterproductive because the examination frequently is difficult and traumatic, resulting in the subsequent inability to adequately examine the genital area due to the child's refusal because of memories of pain. Such traumatic lysis should be avoided. General anesthesia in an operative setting may thus be required.

Management of labial adhesions can be problematic as recurrence is common. Parents or caretakers must be instructed on how to ensure the child maintains excellent perineal hygiene and avoids vulvovaginitis. Families are often incorrectly encouraged to avoid baths in favor of showers; while bubble-baths may occasionally contribute to vulvar inflammation, a plain water bath with soaking and cleansing of the interlabial folds using a washcloth without soap is preferable to a shower, which makes interlabial cleansing more difficult. The daily application of a topical emollient (such as A&D ointment) helps reduce the risk of recurrence until endogenous pubertal estrogen stimulation alleviates the risk. Thus, the application of a topical emollient should be continued until the child shows signs of estrogen-stimulated breast development.

Rarely, an adopted child will be found to have what appears to be labial adhesions, and these may be suggestive of female genital mutilation that occurred at a young age. The thick adhesions that result from this trauma may require surgical separation and management by a gynecologist with experience in managing female genital mutilation.

Labial adhesions may be confused with posterior labial fusion encountered in persons with congenital adrenal hyperplasia and may be differentiated by careful physical examination with attention to the presence or absence of clitoromegaly. This abnormality is noted at birth, rather than acquired.

The differential diagnosis for a cystic mass at the hymen includes ectopic ureter, hymenal cyst, hymenal skin tag, periurethral cyst, and vaginal cyst.[20]

Incomplete hymenal obstruction

In the case of incomplete hymenal obstruction due to a cribiform hymen or hymenal band, the typical presenting symptom is difficulty inserting a tampon or even the inability to achieve vaginal intercourse in an adolescent. Anatomic variations must be distinguished from involuntary vaginismus or contraction of the perineal and pelvic musculature or levator ani muscles, which can be associated with the learning process of tampon insertion, becoming a vicious cycle when persistent insertion is attempted without success and causes pain.

Hymenotomy occasionally may be indicated in the case of a rigid inelastic hymen, particularly for young female athletes (eg, swimmers, divers, gymnasts, cheerleaders) who may be hypoestrogenic, leading to the rigid hymenal configuration. As athletes, these girls are often eager to use tampons. A reasonable alternative to surgical correction involves the use of progressive dilation in a motivated young woman, along with topical estrogen. In these athletes with a rigid hymen, an evaluation for hypoestrogenism associated with overly vigorous physical activity should be considered; if present, estrogen replacement improves the hymenal characteristics and increases hymenal elasticity. http://emedicine.medscape.com/article/269050-overview 6/10 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology Hymenal bands

This condition is typically amenable to division using a local anesthetic in the office; however, the young woman's age and tolerance of such an office procedure must be predicted and judged. Her degree of motivation for tampon use or intercourse impacts the timing at which she requests such a procedure. A typical presenting history of an individual with a hymenal band is the ability to insert a tampon but extreme difficulty removing it. The author has encountered a patient in whom the tampon string became wrapped around the hymenal band, leading to marked edema and pain when removal was attempted.

Obstructing longitudinal or transverse septa

These conditions require careful preoperative evaluation to define the anatomy prior to any attempted surgical reconstruction. The repair of such complicated anomalies should usually be referred to a gynecologist at a tertiary care center where these cases are not a rarity. MRI is the optimal imaging modality for defining complicated female reproductive anatomy.[21]

Vaginal agenesis or androgen insensitivity

The evaluation and management of vaginal agenesis or androgen insensitivity syndrome is beyond the scope of this article, but these conditions should be considered in the differential diagnosis. Like imperforate hymen, primary amenorrhea is typically the presenting complaint

Androgen insensitivity is diagnosed based on findings of a blind vaginal pouch, with an XY chromosomal complement. Mayer-Rokitansky-Kuster-Hauser syndrome (uterovaginal agenesis) may include uterine remnants, some containing endometrium as well as myometrium. These patients should be referred to a gynecologist who specializes in adolescents and who has experience in managing these conditions.

Others

The presentation of an abdominal mass must be differentiated from urinary obstruction or tumors such as sacrococcygeal teratoma with abdominal extension, ovarian tumor, or other masses like mesenteric cysts or anterior meningoceles.[20]

Neovagina options

The options for creation of a neovagina are nonoperative (preferred approach) or operative, such as a McIndoe, Davydov, Vecchietti, or Williams procedures.[22]

Nonoperative management, using progressively larger Lucite dilators, is generally thought to be the first-line approach to management. Nonsurgical management at a time when the young woman is motivated to use vaginal dilators minimizes the potential for scarring and has high rates of success.[22]

Coital dilation has also been described as a successful management strategy.

Indications

An imperforate hymen at the time of puberty must be corrected surgically. The surgical decision-making process should focus on appropriate diagnosis and timing of surgical repair. While the patient may present with acute pain, the repair should not be performed emergently before carefully defining the anatomy. The surgery should be performed by a gynecologist who is skilled and experienced in the care of adolescents with genital anomalies.

Relevant Anatomy

An imperforate hymen presenting after the onset of menstrual shedding is visible upon examination as a translucent thin membrane just inferior to the urethral meatus that bulges with the Valsalva maneuver. This bluish discoloration is due to the presence of a hematocolpos visible behind the translucent hymenal membrane. Vaginal septa do not typically appear translucent (see the image below).

http://emedicine.medscape.com/article/269050-overview 7/10 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology Imperforate hymen, classic appearance of bulging, blue-domed, translucent membrane.

Depending on the size and volume of the hematometra, hematocolpos, or hematosalpinges, a pelvic or abdominal mass may be palpable during abdominal or rectal examination. See the image below.

Abdominal mass w ith imperforate hymen.

Radiographic documentation must demonstrate that the true diagnosis is not an obstructing transverse vaginal septum or other anomaly. Pelvic ultrasonography via the transabdominal, transperineal, or transrectal route is indicated as the initial diagnostic test, followed by MRI if any questions remain about the anatomy. Transperineal ultrasonography can be helpful in measuring the thickness of the septum. Because renal and urologic abnormalities are associated with müllerian abnormalities, imaging of the upper urinary tract can help diagnose ipsilateral renal agenesis, duplex collecting systems, and other complex renal anomalies if there are uterovaginal anomalies.

The prevalence of renal agenesis is estimated at 1 case per 600-1200 persons in patients with müllerian anomalies on the basis of autopsy studies. As many as 25-90% of women with renal anomalies are suggested to have concurrent genital anomalies; thus, abdominal and pelvic imaging of these patients is also warranted for these patients.

Contraindications

The contraindications for a surgical repair of an imperforate hymen relate to the surgeon's inexperience with this condition, failure to adequately consider the alternative diagnoses, or failure to carefully define the anatomy.

Contributor Information and Disclosures Author Paula J Adams Hillard, MD Professor, Department of Obstetrics/Gynecology, Stanford University Medical Center

Paula J Adams Hillard, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Obstetricians and Gynecologists, American Medical Association, Association of Professors of Gynecology and Obstetrics, Association of Reproductive Health Professionals, North American Society for Pediatric and Adolescent Gynecology, Phi Beta Kappa, Society for Adolescent Health and Medicine

Disclosure: Received consulting fee from Bayer-Schering for scientific advisory board; Received honoraria from Merck for speaking and teaching; Partner received grant/research funds from Teva Pharmaceuticals for site pi--; Received grant/research funds from The Canary Foundation for site pi.

Specialty Editor Board Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment.

Chief Editor Richard Scott Lucidi, MD FACOG, Associate Professor of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, Virginia Commonwealth University School of Medicine

Richard Scott Lucidi, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Society for Reproductive Medicine

Disclosure: Nothing to disclose.

Additional Contributors Robert K Zurawin, MD Associate Professor, Chief, Section of Minimally Invasive Gynecologic Surgery, Department of Obstetrics and Gynecology, Baylor College of Medicine

Robert K Zurawin, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Society for Reproductive Medicine, Association of Professors of Gynecology and Obstetrics, Central Association of Obstetricians and Gynecologists, Society of Laparoendoscopic Surgeons, Texas Medical Association, AAGL, Harris County Medical Society, North American Society for Pediatric and Adolescent Gynecology

Disclosure: Received consulting fee from Ethicon for consulting; Received consulting fee from Bayer for consulting; Received consulting fee from Hologic for consulting. http://emedicine.medscape.com/article/269050-overview 8/10 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology Acknowledgements The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Judith A Lacy, MD to the development and writing of this article.

References

1. Braverman PK, Breech L,. American Academy of Pediatrics. Clinical report--gynecologic examination for adolescents in the pediatric office setting. Pediatrics. 2010 Sep. 126(3):583-90. [Medline].

2. Posner JC, Spandorfer PR. Early detection of imperforate hymen prevents morbidity from delays in diagnosis. Pediatrics. 2005 Apr. 115(4):1008-12. [Medline].

3. Botash AS, Jean-Louis F. Imperforate hymen: congenital or acquired from sexual abuse?. Pediatrics. 2001 Sep. 108(3):E53. [Medline].

4. Stewart ST. Hymenal characteristics in girls with and without a history of sexual abuse. J Child Sex Abus. 2011 Sep. 20(5):521-36. [Medline].

5. Adams JA, Harper K, Knudson S, Revilla J. Examination findings in legally confirmed child sexual abuse: it's normal to be normal. Pediatrics. 1994 Sep. 94(3):310-7. [Medline].

6. Muram D. The medical evaluation of sexually abused children. J Pediatr Adolesc Gynecol. 2003 Feb. 16(1):5-14. [Medline].

7. Persaud DI, Squires JE, Rubin-Remer D. Use of Foley catheter to examine estrogenized hymens for evidence of sexual abuse. J Pediatr Adolesc Gynecol. 1997 May. 10(2):83-5. [Medline].

8. Parazzini F, Cecchetti G. The frequency of imperforate hymen in northern Italy. Int J Epidemiol. 1990 Sep. 19(3):763-4. [Medline].

9. Heger AH, Ticson L, Guerra L, et al. Appearance of the genitalia in girls selected for nonabuse: review of hymenal morphology and nonspecific findings. J Pediatr Adolesc Gynecol. 2002 Feb. 15(1):27-35. [Medline].

10. Sakalkale R, Samarakkody U. Familial occurrence of imperforate hymen. J Pediatr Adolesc Gynecol. 2005 Dec. 18(6):427-9. [Medline].

11. Lim YH, Ng SP, Jamil MA. Imperforate hymen: report of an unusual familial occurrence. J Obstet Gynaecol Res. 2003 Dec. 29(6):399-401. [Medline].

12. Stelling JR, Gray MR, Davis AJ, Cowan JM, Reindollar RH. Dominant transmission of imperforate hymen. Fertil Steril. 2000 Dec. 74(6):1241-4. [Medline].

13. Oakes MB, Hussain HK, Smith YR, Quint EH. Concomitant resorptive defects of the reproductive tract: a uterocervicovaginal septum and imperforate hymen. Fertil Steril. 2010 Jan. 93(1):268.e3-5. [Medline].

14. Yildirim G, Gungorduk K, Aslan H, Sudolmus S, Ark C, Saygin S. Prenatal diagnosis of imperforate hymen with hydrometrocolpos. Arch Gynecol Obstet. 2008 Nov. 278(5):483-5. [Medline].

15. Bhargava P, Dighe M. Prenatal US diagnosis of congenital imperforate hymen. Pediatr Radiol. 2009 Sep. 39(9):1014. [Medline].

16. ACOG Committee on Adolescent Health Care. ACOG Committee Opinion No. 349, November 2006: Menstruation in girls and adolescents: using the menstrual cycle as a vital sign. Obstet Gynecol. 2006 Nov. 108(5):1323-8. [Medline].

17. Buyukbayrak EE, Ozyapi AG, Karsidag YK, Pirimoglu ZM, Unal O, Turan C. Imperforate hymen: a new benign reason for highly elevated serum CA 19.9 and CA 125 levels. Arch Gynecol Obstet. 2008 May. 277(5):475-7. [Medline].

18. Kalmantis K, Koumpis C, Daskalakis G, Papantoniou N, Mesogitis S, Antsaklis A. Imperforate hymen with hematocolpometra combined with elevated Ca125. Bratisl Lek Listy. 2009. 110(2):120-2. [Medline].

19. Walsh B, Shih R. An unusual case of urinary retention in a competitive gymnast. J Emerg Med. 2006 Oct. 31(3):279-81. [Medline].

20. Bajaj M, et al. Imperforate hymen: a not so benign condition. J Paediatr Child Health. 2006. 42(11):745-6.

21. Church DG, Vancil JM, Vasanawala SS. Magnetic resonance imaging for uterine and vaginal anomalies. Curr Opin Obstet Gynecol. 2009 Oct. 21(5):379-89. [Medline].

22. American College of Obstetrics and Gynecology. Committee opinion: no. 562: müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 2013 May. 121(5):1134-7. [Medline].

23. Blask AR, Sanders RC, Rock JA. Obstructed uterovaginal anomalies: demonstration with sonography. Part II. Teenagers. Radiology. 1991 Apr. 179(1):84-8. [Medline].

24. Shaw LM, Jones WA, Brereton RJ. Imperforate hymen and vaginal atresia and their associated anomalies. J R Soc Med. 1983 Jul. 76(7):560-6. [Medline]. [Full Text].

25. Olive DL, Henderson DY. Endometriosis and mullerian anomalies. Obstet Gynecol. 1987 Mar. 69(3 Pt 1):412-5. [Medline].

26. Joki-Erkkilä MM, Heinonen PK. Presenting and long-term clinical implications and fecundity in females with obstructing vaginal malformations. J Pediatr Adolesc Gynecol. 2003 Oct. 16(5):307-12. [Medline].

27. Basaran M, Usal D, Aydemir C. Hymen sparing surgery for imperforate hymen: case reports and review of http://emedicine.medscape.com/article/269050-overview 9/10 6/18/2015 Imperforate Hymen: Background, Problem, Epidemiology literature. J Pediatr Adolesc Gynecol. 2009 Aug. 22(4):e61-4. [Medline].

28. Dane C, Dane B, Erginbas M, Cetin A. Imperforate hymen-a rare cause of abdominal pain: two cases and review of the literature. J Pediatr Adolesc Gynecol. 2007 Aug. 20(4):245-7. [Medline].

29. Acar A, Balci O, Karatayli R, Capar M, Colakoglu MC. The treatment of 65 women with imperforate hymen by a central incision and application of Foley catheter. BJOG. 2007 Nov. 114(11):1376-9. [Medline].

30. Rock JA, Zacur HA, Dlugi AM, Jones HW Jr, TeLinde RW. Pregnancy success following surgical correction of imperforate hymen and complete transverse vaginal septum. Obstet Gynecol. 1982 Apr. 59(4):448-51. [Medline].

http://emedicine.medscape.com/article/269050-overview 10/10 Mercado-Alvarado et al., Trop Med Surg 2013, 1:5 http://dx.doi.org/10.4172/2329-9088.1000144 Tropical Medicine & Surgery

CaseResearch Report Article OpenOpen Access Access

Imperforated Hymen: An Unexpected Cause of Pediatric Abdominal Pain, Case Report and Review of Literature Joanna Mercado-Alvarado*, Jorge L Falcon and Juan Zequeira University of Puerto Rico, USA

Abstract Introduction: Imperforate Hymen is the most common female genital tract malformation with prevalence of up to 0.1% and can present with wide variety of symptoms, ranging from abdominal pain to urinary retention. Case: Case of 11 y/o female with cyclical abdominal pain and palpable mass up to umbilicus. Patient diagnosed with imperforate hymen and taken to operating room (OR) by Gynecology team for hymenotomy; 2,500 mL of blood was evacuated. Discussion: Imperforate Hymen is an uncommon cause of abdominal pain. Presentation varies from abdominal pain and difficulty urinating, to urinary retention and tenesmus. Ultrasound is the study of choice for further evaluation and definite treatment is via hymenotomy. Conclusion: Imperforate hymen is an easily missed diagnosis in the Emergency Department (ED). It has to be included in the differential diagnosis for abdominal pain in pre-menarchal females.

Introduction Trans-Abdominal Ultrasound (US) evaluation showed a cystic mass of 24 cm×12 cm×16 cm. Further evaluation with computed Imperforate hymen, as its name implies, is a condition where the tomography of the abdomen and pelvis showed a hyperdense, non- hymen, a thin membrane in the shape of a half moon, covers the entire enhancing, fluid-filled pelvic mass confirming US findings (Figure 2). opening of the Vagina [1]. This condition, although rare, is the most She was taken for genital examination were bulging imperforate hymen common female genital tract malformation with a prevalence of up to occluding the vagina was seen (Figure 3). 0.1% [2,3]. Diagnosis can go undetected until a patient starts experience one, or many, of a variety of symptoms. Obstetrics and Gynecology service was consulted and patient was taken to the OR. Vertical hymenotomy was performed and 2,500 ml Symptoms can range from mild abdominal pain and tenesmus chocolate-colored menstrual bloody fluid was drained. Patient was to urinary retention and hematocolpos, a mass that forms due to the discharged on post-op day number two without any complications. accumulation of menstrual blood that cannot leave the vaginal cavity. In this case, we present a young female with cyclical abdominal pain and a palpable pelvic mass. Setting this case apart is the size of the mass; over twice the size of any hematocolpos previously published on case reports [4]. The Case Case of an 11 year-old pre-menarchal female with lower abdominal and pelvic pain for four days after moving some boxes in her bedroom.

Associated to amenorrhea, increased urinary frequency and palpable abdominal non-pulsatile mass that extended above the umbilicus (Figure 1). She denied any poor oral intake, nausea, vomiting or problem with bowel movements. She had never been evaluated by a gynecologist. Upon further history, her sister had menarche at 10 years Figure 2: Sagital view from abdomino-pelvic CT scan old and mother also had menarche at 10 years old. showing huge abdomino-pelvic mass

*Corresponding author: Joanna Mercado-Alvarado, U.P.R. School of Medicine, Department of Emergency Medicine, University of Puerto Rico, USA, E-mail: [email protected]

Received September 16, 2013; Accepted September 26, 2013; Published September 30, 2013

Citation: Mercado-Alvarado J, Falcon JL, Zequeira J (2013) Imperforated Hymen: An Unexpected Cause of Pediatric Abdominal Pain, Case Report and Review of Literature. Trop Med Surg 1: 144. doi:10.4172/2329-9088.1000144

Copyright: © 2013 Mercado-Alvarado J. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the Figure 1: Protuberant abdomen due to enlarged mass. original author and source are credited.

Trop Med Surg ISSN: 2329-9088 TPMS, an open access journal Volume 1 • Issue 5 • 1000144 Citation: Mercado-Alvarado J, Falcon JL, Zequeira J (2013) Imperforated Hymen: An Unexpected Cause of Pediatric Abdominal Pain, Case Report and Review of Literature. Trop Med Surg 1: 144. doi:10.4172/2329-9088.1000144

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cm×8 cm [3,5,9,11,12,16] and containing up to 1L of blood [2,5-8,10- 14] had been reported prior to our study. Our patient had double the volume and a 50% larger mass than any reported to our best knowledge. These masses place pressure on surrounding structures leading to the symptoms the patient experiences. In some cases ultrasound might not be enough for evaluation of other complications, and other tools like computed tomography scans or magnetic resonance imaging must be used [10]. Urinary retention should always be treated via catheterization until definitive treatment via hymenotomy can be achieved. Variations of hymenotomy exist to comply with cultural beliefs [3,8,10]. Because the hymen is a symbol of virginity in some communities, its destruction Figure 3: Bulging imperforate hymen through vaginal introitus. can be a source of social problems for some girls [10]. Hymental tissue in case of imperforate hymen tends to form a tougher border, making simple incision and sutures more than enough [3]. Multiple Discussion types of incisions have proven effective: cruciate incision, longitudinal Imperforate hymen is a rare congenital anomaly reported at incision or excision of part of membrane [10,12]. Standard treatment an approximate rate of 0.1% and occurs due to the incomplete is surgical hymenectomy with T,X, plus, or cruciform incisions and canalization of the Mullerian system and the urogenital system removal of excess hymenal tissue [3]. The patient described in this [1]. In the embryological period, the lateral portion of the hymen case repor received a vertical incision as a part of a newer practice originates from a fold of urogenital sinus at the union of the Mullerian for hymen preservation. It involves a midline vertical incision, where ducts, whereas in its posterior part, it originates from the cells of the the hymenal oriﬁce was kept patent by 4 or 5 absorbable sutures that urogenital sinus externally and from Mullerian ducts internally. Usually were formed by oblique location of the inner and outer needle sites in the eighth week of gestation, it partially ruptures in the inferior part to prevent realignment of the edges as described in Basaran et al. [3]. of the Mullerian ducts, remaining as a fold of mucous membrane Hymenotomy is a minor procedure that does not cause significant around the entrance of the vagina. Failure to partially rupture results morbidity and provides complete relief of all the symptoms. Follow up in a persistence of the septum, which can be diagnosed as imperforate is always necessary to make sure there is no refusion of the hymen. hymen clinically [5]. Conclusion Imperforate hymen is an isolated abnormality, where diagnosis should ideally be done at birth by careful examination of the external Imperforate hymen, although being the most common female genitalia of all newborn females [2-17]. During the neonatal period, it genital tract malformation, remains an uncommon cause for abdominal may present with fetal ascites or acute renal failure. The hematocolpos pain in the pediatric population. It is a diagnosis that can easily be or hydrocolpos may lead to variable degrees of hydroureter, and overlooked in the fast paced setting of the Emergency Department. It is hydronephrosis. If the diagnosis is not made in the newborn period and of utmost importance to perform a complete physical examination, to the hymen remains imperforate, the mucus will be reabsorbed and the obtain a meticulous menstrual history of other female family members, child usually remains asymptomatic until menarche [10]. At menarche, and to have a high clinical suspicion in order to facilitate early detection. usually between 9 to 13 years of age, the child starts getting cyclic It must be included, particularly, in the differential diagnosis of every abdominal pains associated with primary amenorrhea. Retained blood pre-pubertal and pre-menarchal young female with abdominal pain. in the vagina, uterus, and fallopian tubes can result in hematocolpos, References hematometra and hematosalpinx. Hematocolpos gets worse with each 1. Children's Hospital Boston (2013) Imperforate hymen. Retrieved From. menstrual period [17]. 2. Mwenda AS (2013) Imperforate Hymen - a rare cause of acute abdominal pain Over thirty cases of hematocolpometra due to imperforate hymen and tenesmus: case report and review of the literature. Pan Afr Med J 15: 28. have been reported [2-17]. Most of these cases present with cyclic 3. Basaran M, Usal D, Aydemir C (2009) Hymen sparing surgery for imperforate abdominal pain, but presentation can vary widely, from low back pain hymen: case reports and review of literature. J Pediatr Adolesc Gynecol 22: [15] to acute urinary retention [3,5-12] and tenesmus [2,12]. Of the e61-64. 20 cases being reviewed [2-17], 55% experienced urinary retention as 4. Domany E, Gilad O, Shwarz M, Vulfsons S, Garty BZ (2013) Imperforate hymen a result of mass effect. Diagnosis is done through a thorough history presenting as chronic low back pain. Pediatrics 132: e768-770. and physical exam, which needs to include genitalia; something that 5. Ercan CM, Karasahin KE, Alanbay I, Ulubay M, Baser I (2011) Imperforate is not always performed. Most of these reported cases presented in hymen causing hematocolpos and acute urinary retention in an adolescent girl. adolescence upon menarche. The youngest patient was a 3-month-old Taiwan J Obstet Gynecol 50: 118-120. girl who had suffered from repeated urinary tract infections because of 6. Rabani SM (2013) A rare non urologic cause for urinary retention; report of 2 urinary retention related to pyocolpos [10]. Menarche typically occurs cases. Nephrourol Mon 5: 766-768. within 2 to 3 years after the larche (breast budding), at Tanner stage IV 7. Anselm OO, Ezegwui UH (2010) Imperforate hymen presenting as acute breast development, and is rare before Tanner stage III development urinary retention in a 14-year-old nigerian girl. J Surg Tech Case Rep 2: 84-86. [18-20]. Also, upon evaluation of the vagina, a bluish membrane will be 8. Buick RG, Chowdhary SK (1999) Backache: a rare diagnosis and unusual seen protruding the vaginal introitus. complication. Pediatr Surg Int 15: 586-587. If patient or parents refuse genital exam evaluation, imaging studies 9. Hsu KP, Chen CP, Chien SC, Hsu CY (2008) Hematocolpometra associated with an imperforate hymen and acute urinary retention mimicking a pelvic can greatly help with diagnosis. Ultrasound will show an echogenic fluid mass. Taiwan J Obstet Gynecol 47: 222-223. accumulation in the vagina that can extend to uterus. Masses of up to 18

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10. Abu-Ghanem S, Novoa R, Kaneti J, Rosenberg E (2011) Recurrent urinary 16. Kurdoglu Z, Kurdoglu M, Kucukaydin Z (2011) Spontaneous rupture of the retention due to imperforate hymen after hymenotomy failure: a rare case imperforate hymen in an adolescent girl with hematocolpometra. ISRN Obstet report and review of the literature. Urology 78: 180-182. Gynecol 2011: 520304.

11. Dane C, Dane B, Erginbas M, Cetin A (2007) Imperforate hymen-a rare cause 17. Kumar K, Waseem M (2008) An uncommon cause of abdominal pain in an of abdominal pain: two cases and review of the literature. J Pediatr Adolesc adolescent. South Med J 101: 1065-1066. Gynecol 20: 245-247. 18. Tintinalli JE, Stapczynski JS, Cline DM, Ma OJ, Cydulka RK, et al. (2010) 12. Mou JW, Tang PM, Chan KW, Tam YH, Lee KH (2009) Imperforate hymen: Tintinalli's emergency medicine: A comprehensive study guide. (7thedn. ed., p. cause of lower abdominal pain in teenage girls. Singapore Med J 50: e378-379. 866). China: McGraw-Hill.

13. Lardenoije C, Aardenburg R, Mertens H (2009) Imperforate hymen: a cause of 19. Zitelli B, Davis (2007) Atlas of pediatric physical diagnosis. (5thedn., pp. 685- abdominal pain in female adolescents. BMJ Case Rep 2009. 687). China: Mosby Elsevier.

14. Stone SM, Alexander JL (2004) Images in clinical medicine. Imperforate hymen 20. American Academy of Pediatrics Committee on Adolescence; American College with hematocolpometra. N Engl J Med 351: e6. of Obstetricians and Gynecologists Committee on Adolescent Health Care, Diaz A, Laufer MR, Breech LL (2006) Menstruation in girls and adolescents: 15. Wall EM, Stone B, Klein BL (2003) Imperforate hymen: a not-so-hidden using the menstrual cycle as a vital sign. Pediatrics 118: 2245-2250. diagnosis. Am J Emerg Med 21: 249-250.

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Trop Med Surg ISSN: 2329-9088 TPMS, an open access journal Volume 1 • Issue 5 • 1000144 6/18/2015 Reminder of important clinical lesson: Imperforate hymen: a cause of abdominal pain in female adolescents

BMJ Case Rep. 2009; 2009: bcr08.2008.0722. PMCID: PMC3029536 Published online 2009 May 26. doi: 10.1136/bcr.08.2008.0722 Reminder of important clinical lesson

Imperforate hymen: a cause of abdominal pain in female adolescents

Céline Lardenoije, Robert Aardenburg, and Helen Mertens

Orbis Medical Centre, Obstetrics and Gynaecology, PO Box 5500, Sittard, 6130 MB, The Netherlands Céline Lardenoije, Email: [email protected]

Abstract A 16-year-old girl presented with primary amenorrhea and had had cyclical abdominal pain for almost a year. At examination we observed a painful mass in the lower abdomen and normal secondary sex characteristics. Perineal examination showed a bluish bulging hymen. Transabdominal ultrasonography revealed a dense mass in the pelvis measuring about 12×11 cm. We diagnosed an imperforate hymen with haematocolpos and haematometra. The hymen was opened surgically and a large quantity of menstrual blood was drained from the vagina and uterus. Postoperative recovery was normal without any pain. The patient now menstruates regularly. An imperforate hymen occurs in 0.05% of women. It is important to be aware of this while examining a female adolescent presenting with cyclical abdominal pain and primary amenorrhea. Late discovery of an imperforate hymen may lead to pain, infections, hydronephrosis and endometriosis with subfertility as a possible consequence.

BACKGROUND Imperforate hymen is a rare cause of abdominal pain in female adolescents. It is seen in approximately 1 in 2000 females, although information on the true incidence is difficult to obtain.1,2 The lack of menses in an adolescent girl of (post-)menarcheal age with cyclical abdominal pain, urinary retention, constipation and/or a (symptomatic) lower abdominal mass suggests this condition should be considered.3 This case illustrates that there is quite often a considerable delay before a proper diagnosis is reached. The aim of this communication is to increase awareness of imperforate hymen among clinicians examining adolescent girls with lower abdominal pain.

CASE PRESENTATION A 16-year-old girl was admitted with a history of lower abdominal pain. She was asymptomatic until a year previously. She then started developing cyclical crampy pain in the lower abdomen, which lasted for 7 days every month. The pain had become more severe during the previous 2 months and the size of her abdomen had increased over the past few months. There was no history of nausea, vomiting, fever, altered bowel habits or problems with urinating. She was 13 years old and had not yet had a menstrual period, but did have pubic hair and breast buds, confirming the onset of puberty. The patient denied any vaginal discharge and there was no history of sexual activity. No other members of her family had similar or other physical complaints. The medical history was unremarkable.

INVESTIGATIONS On physical examination, the secondary sex characteristics, such as pubic hair and breast buds, were well developed. A mobile, non-tender mass, arising from the pelvis to the belly button, was felt in the abdomen (fig 1, http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3029536/?report=printable 1/6 6/18/2015 Reminder of important clinical lesson: Imperforate hymen: a cause of abdominal pain in female adolescents upper image). Gynaecologic examination revealed a bulging bluish hymen (fig 2). No other gross external abnormalities of the external genitalia were observed. A pelvic ultrasound showed a homogenous, hypoechoic mass in the vagina and uterus measuring about 12×11 cm. Both the ovaries were normal.

DIFFERENTIAL DIAGNOSIS We diagnosed an imperforate hymen with consequent accumulation of blood in the vagina and uterus (haematocolpos and haematometra, respectively).

TREATMENT Two days later the patient underwent a hymenectomy with a cruciate incision. Approximately 500 ml of viscous, chocolate coloured blood and clots were drained (fig 3).

OUTCOME AND FOLLOW-UP Immediately after the operation the size of the abdomen had regained normal proportions (fig 1, lower image).

During a 2-month follow-up, the patient was asymptomatic and started to have regular menstrual cycles.

DISCUSSION Imperforate hymen is a rare though serious cause of abdominal pain in female adolescents. It is seen in approximately 1 in 2000 females.1,2,4 The hymen is an embryological remnant of mesodermal tissue that normally perforates during the later stages of embryo development. The hymen is called imperforate if there is no perforation of this membrane.

The reason for non-perforation of this membrane is unknown. Imperforate hymen occurs mostly in a sporadic manner, although some familial occurrences have been reported. Both the recessive and dominant modes of transmission have been suggested, but no genetic markers or mutations have been proven as aetiological factors.5,6 An imperforate hymen in younger children (<10 years) is discovered by chance in 90% of cases, while 100% of affected adolescents first present with symptoms.2

The most common symptoms of an imperforate hymen are cyclical abdominal pain and urinary retention, usually presenting between the ages of 13 and 15 years (when menarche occurs).7,8 There is primary amenorrhea but secondary sex characteristics are well developed. Because the vaginal outflow is obstructed by the non-perforated hymen, menstrual blood accumulates in the vagina (haematocolpos) and the uterus (haematometra). This may lead to mechanical effects on the urethra, bladder, intestines or pelvic blood vessels which can result in urinary retention, obstipation or oedema of the legs.7,9–14 Irritation of the sacral plexus or nerve roots can cause lower back pain.8 Problems with intercourse are rarely mentioned, probably because most of the patients are still sexually inactive.15

Some symptoms of appendicitis are similar to those of an imperforate hymen, and there are cases where groundless appendectomies have been performed.16 In addition, there is often an initial diagnosis of infection of the bladder, nephrolithiasis or abdominal tumour, which leads to unnecessary examinations and treatment. The history and physical examination are frequently incomplete.2 One should always consider an imperforate hymen if there is a discrepancy between the Tanner stage and menarcheal status.2

Gynaecological examination reveals a bluish bulging hymen and generally an abdominal mass. The diagnosis can be established with an abdominal ultrasound showing the pelvic cystic mass. Imperforate hymen should be differentiated from a low transverse vaginal septum using the Valsalva manoeuvre: an imperforate hymen should bulge and a transverse vaginal septum should not.4 Imperforate hymen is usually not associated with any other Müllerian abnormalities and extensive investigation for urogenital anomalies is unnecessary.4 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3029536/?report=printable 2/6 6/18/2015 Reminder of important clinical lesson: Imperforate hymen: a cause of abdominal pain in female adolescents Early diagnosis of an imperforate hymen is important, since it can lead to serious complications such as infections, hydronephrosis, kidney failure, endometriosis and subfertility.17–19 A retrospective study showed that eight of nine patients with haematocolpos or haematometra due to an outflow obstruction had endometriosis at the time of intra-abdominal operation.20

Treatment of haematocolpos or haematometra due to an imperforate hymen is to make cruciate incision in the hymen which allows the accumulated blood to drain away. This has to be done aseptically as a closed vagina lacks protective Doederlein’s bacilli and the pH is alkaline or weakly acidic; there is a poor natural resistance to bacteria entering from the lower genital tract and the blood and debris provide a good culture medium.4 The complications of a hymenectomy are bleeding, scarring and stenosis of the vaginal opening.21 Less invasive treatments for an 22,23 imperforate hymen include the use of CO2 lasers or a Foley catheter. In a retrospective study of the long term results of surgical correction of imperforate hymen, nine of 15 patients had an irregular menstrual cycle and six of 15 patients had dysmenorrhea (over a follow-up of 8.5 years). Most patients had no sexual dysfunction. Pre-operative complaints of cryptomenorrhea (n=15), abdominal pain (n=11), palpable mass in the lower abdomen (n=9), urinary retention (n=6), dysuria (n=3) and problems defecating (n=4) almost all disappeared after surgery.15 Eight patients worried about their future fertility; two of them were attempting pregnancy and were successful. Another study showed that 86% of patients who attempted pregnancy succeeded after surgical correction of imperforate hymen.18

LEARNING POINTS In adolescent girls with cyclical pain, it is very important to take a complete gynaecological history and perform a gynaecological examination as serious complications may follow an unnecessary delay in diagnosis. Consider an imperforate hymen when there is a lack of menses in an adolescent girl with cyclical abdominal pain. Consider an imperforate hymen if there is a discrepancy between the Tanner stage and menarcheal status.

Footnotes

Competing interests: none. Patient consent: Patient/guardian consent was obtained for publication.

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15. Liang CC, Chang SD, Soong YK. Long-term follow-up of women who underwent surgical correction for imperforate hymen. Arch Gynecol Obstet 2003; 269: 5–8 [PubMed: 14605815]

16. Nazir Z, Rizvi RM, Qureshi RN, et al. Congenital vaginal obstructions: varied presentation and outcome. Pediatr Surg Int 2006; 22: 749–53 [PubMed: 16871398]

17. Loscalzo IL, Catapano M, Loscalzo J, et al. Imperforate hymen with bilateral hydronephrosis: an unusual emergency department diagnosis. J Emerg Med 1995; 13: 337–9 [PubMed: 7673625]

18. Rock JA, Zacur HA, Dlugi AM, et al. Pregnancy success following surgical correction of imperforate hymen and complete transverse vaginal septum. Obstet Gynecol 1982; 59: 448–51 [PubMed: 7078896]

19. Hijazeen R. Imperforate hymen with bilateral hydronephrosis in a neonate. Saudi J Kidney Dis Transpl 1995; 9: 33–5 [PubMed: 18408280]

20. Olive DL, Henderson DY. Endometriosis and mullerian anomalies. Obstet Gynecol 1987; 69: 412–15 [PubMed: 3822289]

21. Chang JW, Yang LY, Wang HH, et al. Acute urinary retention as the presentation of imperforate hymen. J Chin Med Assoc 2007; 70: 559–61 [PubMed: 18194899]

22. Friedman M, Gac D, Peretz B. Management of imperforate hymen with the carbon dioxide laser. Obstet Gynecol 1989; 74: 270–2 [PubMed: 2748065]

23. Ali A, Cetin C, Nedim C, et al. Treatment of imperforate hymen by application of Foley catheter. Eur J Obstet Gynecol Reprod Biol 2003; 106: 3–4 [PubMed: 12475572]

Figures and Tables

Figure 1 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3029536/?report=printable 4/6 6/18/2015 Reminder of important clinical lesson: Imperforate hymen: a cause of abdominal pain in female adolescents

Swollen abdomen with haematometra due to an imperforate hymen and the abdomen after hymenectomy.

Figure 2

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3029536/?report=printable 5/6 6/18/2015 Reminder of important clinical lesson: Imperforate hymen: a cause of abdominal pain in female adolescents

Imperforate hymen.

Figure 3

Surgical treatment of an imperforate hymen.

Articles from BMJ Case Reports are provided here courtesy of BMJ Group

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3029536/?report=printable 6/6 DOI: 10.1111/j.1471-0528.2007.01446.x General gynaecology www.blackwellpublishing.com/bjog

The treatment of 65 women with imperforate hymen by a central incision and application of Foley catheter

A Acar, O Balci, R Karatayli, M Capar, MC Colakoglu Department of Obstetrics and Gynecology, Meram Medicine Faculty, Selcuk University, Konya, Turkey Correspondence: Dr O Balci, Department of Obstetrics and Gynecology, Meram Medicine Faculty, Selcuk University, Akyokus, 42080, Konya, Turkey. Email [email protected], [email protected]

Accepted 16 May 2007.

Objective To determine the surgical outcome of 65 women with Results After the procedure, hymenal orifice created remained imperforate hymen treated with a central surgical incision and open and intact in all women except two women. Closure of insertion of a Foley catheter. artificially created hymenal orifice in these two women was believed to be related to inappropriate administration of estrogen Design A prospective study. cream. Subsequent treatment with local estrogen treatment Setting The study was carried out at Department of Obstetrics results in the hymenal orifice remaining opened in these and Gynecology, Faculty of Meram Medicine, Selcuk University, two women. between 1 January 1996 and 30 June 2006. Conclusions We have previously reported the technique in 2002, Population A total of 65 women diagnosed as imperforate hymen. but now we are able to demonstrate results of our technique in an expanded number of women. This technique is less invasive Methods A central oval incision was performed to imperforate than other methods and prevents many social problems related hymenal membrane, then 16F Foley catheter was protruded and to virginity by preventing destruction of the integrity of the balloon was insufflated. Catheter was removed after 2 weeks the hymenal structure and providing an annular-intact duration. Estrogen cream was prescribed to all women for hymenal ring. application onto hymenal structure for 2 weeks. Keywords Foley catheter, imperforate hymen, intact hymenal Main outcome measures Efficacy of procedure in treatment of ring. imperforate hymen, preserving hymenal structural integrity that is accepted as important for virginity in some societies.

Please cite this paper as: Acar A, Balci O, Karatayli R, Capar M, Colakoglu M. The treatment of 65 women with imperforate hymen by a central incision and application of Foley catheter. BJOG 2007;114:1376–1379.

Introduction treated by simple star-shaped incision or by other surgical interventions protecting the bartholin gland oriﬁces to sepa- The imperforate hymen is a common genital disorder.1 The rate the hymen and the meatus.3 incidence of hymen imperforatus is approximately 1 among The aim of this study was to determine the outcome in 2000 girls. Some cases are recognised because of mucocolpos 65 women with imperforate hymen whereby the integrity of at birth, but the diagnosis is usually not detected before hymenal structure is preserved with a central oval incision puberty. The symptoms that appear after the onset of puberty and the insertion of a Foley catheter and local treatment with are due to accumulation of menstrual blood. Menstrual blood estrogen cream for 2 weeks. usually accumulates in the uterus and upper vagina resulting in abdominal pain, distension of the lower abdomen and Methods often acute urinary retention.1 The management is easy, but many women visit several doctors before the exact diagnosis Sixty-ﬁve women with cyclical pelvic pain and primary ame- is achieved.2 Haematocolpos due to imperforate hymen is norrhoea were included into the study at the Department

1376 ª 2007 The Authors Journal compilation ª RCOG 2007 BJOG An International Journal of Obstetrics and Gynaecology Surgical outcome of 65 women with imperforate hymen of Obstetrics and Gynecology, Faculty of Meram Medicine, Selcuk University, between 1 January 1996 and 30 June 2006. All these women were diagnosed with imperforate hymen by visual inspection of totally closed hymenal structure, bulging of hymen out of the vagina and ultrasonographic detection of haematocolpos. First of all, women were informed about classical surgical methods, but they usually opted for an alternative procedure to attempt to preserve their hymenal ring as much as possible. Women were positioned in the lithotomy (Figure 1). The hymen was perforated by a sterile injection from the middle of the distended and imperforate hymenal membrane, and then the perforation site was enlarged to 0.5 cm in diameter to allow for the insertion of a Foley catheter (Figure 2). After drainage of the blood in the vagina using irrigation with a saline solution, a 16F Foley catheter was then inserted Figure 2. Perforation of imperforate hymen by sterile injector needle. and the balloon of the catheter is insufflated by 10 ml of water (Figure 3). The distal end of the Foley catheter is fixed to medial part of the patient’s thigh by using a plaster to Results prevent any traction on it, and the presence of the Foley catheter allowed further drainage of blood in the uterus The mean age of women was 13.9 ± 2.1 years. The main and vagina. Estrogen cream (Premarin vaginal cream; presenting complaints of all these women were primary Wyeth, Istanbul, Turkey) was applied to the hymenal open- amenorrhoea and pelvic pain. Some of these women also ing for the next 14 days to augment vaginal epithelisation. A complained of low back pain. Haematocolpos was detected single dose intravenous prophylactic antibiotic was admin- in all the 65 women (100%). istered to all women (1 g Ceftriaxon, Rocephin; Roche, Istan- After the procedure, the hymenal orifices created remained bul, Turkey). open and hymenal structures were found to be annular and Women were observed in hospital for 6 hours after the intact except in two women. In two women, we observed procedure and were warned about the risks of infection before reclosure of hymenal orifice that we created. Failure in these they were discharged home. We advised women to keep vulva two women was believed to be related to the inappropriate clean with iodine solution. We reviewed women for pelvic administration of the estrogen cream to the hymen. Sub- infection 1 week after the procedure. The Foley catheter was sequent repeat surgical incision and drainage followed by removed after 2 weeks of operation (Figure 4), and women a sufficient amount of local estrogen treatment resulted in were followed up monthly for the initial 6 months and then a successful outcome for these two women. Complications every 6 months thereafter. such as bleeding and infection were not detected in any

Figure 1. Patient with imperforate hymen in lithotomic position. Figure 3. Insertion of Foley catheter.

ª 2007 The Authors Journal compilation ª RCOG 2007 BJOG An International Journal of Obstetrics and Gynaecology 1377 Acar et al.

cases, which we believe were related to the inappropriate usage of local topical estrogen cream. In the literature, there are reports on labial adhesions being successfully treated with topical estrogen application.8 The closure of the central hymenal incisional defect in our series is also prevented by the administration of topical estrogen cream for 2 weeks.7 In a study performed by Liang et al., women who underwent surgical correction for imperforate hymen was found to have irregular menstrual cycles in the majority of cases. Most of these women were also worried about their future fertility and six women suffered from dysmenorrhoea. Out of the 11 women who began having intercourse, 2 women fell pregnant with none of them complained of sexual dysfunction. After hymenectomy, it was also reported that the women with Figure 4. Hymenal structure after removal of Foley catheter after 2 weeks imperforate hymen were markedly relieved of crypto- duration. menorrhoea, and there was improvements in micturition and defecation.9 There is no known complication of this method, and no women. The mean follow-up period for these women was study similar to this was reported in the literature. This tech- 56.5 ± 16.2 months. nique is less invasive than other methods and can potentially The mean age at marriage among these women was 19 ± 4 preserve the integrity of the hymenal ring. j years. Twenty-eight women got married in the follow-up period, and they were all found to bleed following the first coitus. None of the women complained of severe dyspareunia at the first coitus. Nineteen of the married women fell preg- References nant in the follow-up period and 15 of them delivered vagi- 1 Bakos O, Berglund L. Imperforate hymen and ruptured hematosalpinx: nally, whereas the remaining 4 women underwent caesarean a case report with a review of the literature. J Adolesc Health 1999; section. 24:226–8. 2 Johansen JK, Larsen UR. Imperforate hymen. A simple, but overlooked diagnosis. Ugeskr Laeger 1998;160:5948–9. Discussion and conclusion 3 Salvat J, Slamani L. Hematocolpos. J Gynecol Obstet Biol Reprod (Paris) 1998;27:396–402. In the classical surgical treatment of imperforate hymen, stel- 4 John AR. Surgical conditions of the vagina and the urethra. In: late or cruciate incisions are made through the hymenal Thompson JD, Rock JA, editors. Te Linde’s Operative Gynecology, 7th edn. Philadelphia, PA: Lippincott Company; 1992. pp. 1125–40. membrane. The individual quadrants are excised along the 5 Letts M, Haasbeck J. Hematocolpos as a cause of back pain in preme- lateral wall of the vagina. Inset margins of vaginal mucosa narchal adolescents. J Pediatr Orthop 1990;10:731–2. are approximated using with fine delayed-absorbable sutures. 6 Muram D. Pediatric and adolescent gynecology. In: Pernoll ML, editor. These procedures produce good surgical outcome, but the Current Obstetric and Gynecologic Diagnosis and Treatment, 7th edn. hymenal structure is usually damaged.4–6 Because the hymen East Norwalk, CT: Appleton and Lange; 1991. pp. 629–56. 7 Ali A, Cetin C, Nedim C, Kazim G, Cemalettin A. Treatment of imper- is accepted as a sign of virginity, damage to the hymen may be forate hymen by application of Foley catheter. Eur J Obstet Gynecol undesirable in some families and societies. The technique Reprod Biol 2003;106:72–5. described in this manuscript was originally used on a girl 8 Motta T, Clerici C, Dize O, Casazza S, Alberton A. Adhesion of labial who insisted on preservation of her hymen as an indication minora in children: topical treatment with an estriol cream. vol. 4. In: of her virginity.7 We have already reported the technique in Proceedings of the European Congress on Pediatric and Adolescent Gynecology, Rhodas, Greece. 1988. pp. 170–7. 2002 in a limited number of women, but we are now able to 9 Liang CC, Chang SD, Soong YK. Long-term follow-up of women who evaluate our results with an expanded number of women to underwent surgical correction for imperforate hymen. Arch Gynecol confirm our previous results and also to report two failed Obstet 2003;269:5–8.

1378 ª 2007 The Authors Journal compilation ª RCOG 2007 BJOG An International Journal of Obstetrics and Gynaecology Surgical outcome of 65 women with imperforate hymen

Commentary on ‘The treatment of 65 women with imperforate hymen by Foley catheter insertion’

Acar et al.1 have described a novel technique for the treatment of imperforate hymen. The authors state that the operation is performed to maintain the hymenal structures as a sign of virginity in some cultures. I submit that until a woman has intercourse, she remains a virgin, regardless of whether or not she has had a corrective surgical procedure performed upon her hymen. In my opinion, the idea that a woman’s virginity is solely determined by her anatomy is detrimental to the social status of women worldwide. As a physician, it is my duty to serve my patients with the best care medicine can provide and to act as an advocate on their behalf to advance their standing in society regardless of race, religion or sexual orientation. The idea that the ‘deﬂoration’ of a woman’s hymen upon marriage to a man perpetuates the idea that women are no more than prizes to be vied over and eventually won, possessed and dominated. In this case, as all potential women for the treatment would be minors, the patient herself will be unlikely to make decisions regarding the treatment plan; rather, her parents or guardians will do so. As such, it calls into question the role of the physician treating a minor patient. Are we to be the enforcers of religious beliefs upon a patient, simply because she has not yet reached an age suited for consent? Truly, this dilemma plagued my thoughts from the moment I ﬁrst read the article and shall continue to do so. Optimally, a patient should be able to choose a treatment plan which suits her understanding of her own body, the society in which she lives and her role in that society. This decision-making process should be a dialogue between the patient and her physician, free of coercion and bias. Given the intimate nature of the clinical situations we face as obstetrician-gynecologists, this mind-set should be ever present during our encounters with patients. Academic debate of opposing viewpoints in controversial matters such as this is an essential component of the advance- ment of knowledge: medical, social and ethical. We must be sure that our practices and values are in line with one another. In our global society, will we act to ensure the equality of those among us who have been subjugated in the past? or will we continue to perpetuate the submission of one individual to another? I submit these questions to challenge the minds of BJOGs readership with the hopes that they will analyse and identify where their biases lay and how they may reconcile them with their duties as a healthcare provider. j

CM Estes

Reference

1 Acar A, Balci O, Karatayli R, Capar M, Colakoglu M. The treatment of 65 patients with imperforate hymen by a central incision and application of Foley catheter. BJOG 2007;114:1376–79.

ª 2007 The Authors Journal compilation ª RCOG 2007 BJOG An International Journal of Obstetrics and Gynaecology 1379 Hindawi Publishing Corporation Case Reports in Obstetrics and Gynecology Volume 2014, Article ID 142039, 3 pages http://dx.doi.org/10.1155/2014/142039

Case Report Tuboovarian Abscess as Primary Presentation for Imperforate Hymen

Jeh Wen Ho, D. Angstetra, R. Loong, and T. Fleming

Department of Minimally Invasive Gynaecology, The Gold Coast University Hospital, Level 1B Block North, 1Hospital Boulevard, Southport, QLD 4215, Australia Correspondence should be addressed to Jeh Wen Ho; [email protected]

Received 29 January 2014; Revised 17 March 2014; Accepted 24 March 2014; Published 16 April 2014

Academic Editor: Ching-Chung Liang

Copyright © 2014 Jeh Wen Ho et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Objective. Imperforate hymen represents the extreme in the spectrum of hymenal embryological variations. The archetypal presentation in the adolescent patient is that of cyclical abdominopelvic pain in the presence of amenorrhoea. We reported a rare event of imperforate hymen presenting as a cause of tuboovarian abscess (TOA). Case Study. A 14-year-old girl presented to the emergency department complaining of severe left iliac fossa pain. It was her first episode of heavy bleeding pervagina, and she had a history of cyclical pelvic pain. She was clinically unwell, and an external genital examination demonstrated a partially perforated hymen. A transabdominal ultrasound showed grossly dilated serpiginous fallopian tubes. The upper part of the vagina was filled with homogeneous echogenic substance. Magnetic resonance imaging (MRI) demonstrated complex right adnexa mass with bilateral pyo-haemato-salpinges, haematometra, and haematocolpos. In theatre, the imperforate hymen was opened via cruciate incision and blood was drained from the vagina. At laparoscopy, dense purulent material was evacuated prior to an incision anddrainageofthepersistentrightTOA.Conclusion. Ideally identification of imperforate hymen should occur during neonatal examination to prevent symptomatic presentation. Our case highlights the risks of late recognition resulting in the development of sepsis and TOA.

1. Introduction 2. Case Study

Imperforate hymen represents the extreme in the spectrum A 14-year-old girl presented to the emergency department of of embryological variations in hymenal configuration, with the Gold Coast University Hospital complaining of severe left reported incidence ranging from 0.014% to 0.1% [1]. Ante- iliac fossa pain with her first episode of heavy bleeding per natal diagnosis of imperforate hymen is challenging; thus, vagina since the morning of presentation. Her background neonatal diagnosis is optimal to prevent symptoms and history was unremarkable with no significant medical or complicationsseenwhenprimarypresentationoccursin surgical history. She had no previous sexual encounters (virgo adolescence. intacta). Further questioning elucidated that she had not The archetypal presentation in the adolescent patient gone through menarche and had cyclical pelvic pain for the is that of cyclical abdominopelvic pain in the presence of previous three linebreak months. amenorrhoea [2]. The accumulated blood behind an intact Examination revealed a clinically unwell patient. She hymen may compress the adjacent pelvic organs or vessels, presented as febrile to 39 degrees Celsius, with tachycardia of resulting in some of the less common presentations such 118 bpm. Her blood pressure was 120/60, showing a postural as urinary retention, back pain, or constipation. We report drop to 105/40. Her abdomen was soft; however there was a rare case of spontaneous partial rupture of an imper- marked lower abdominal tenderness on palpation, rebound forate hymen resulting in tuboovarian abscess (TOA) and tenderness and abdominal guarding. External genital exami- sepsis. nation was limited by copious amounts of both old and fresh 2 CaseReportsinObstetricsandGynecology

Echogenic substance within endometrial cavity Right tuboovarian abscess

Haematometra Dilated vagina filled Free ﬂuid with echogenic substance Bladder

Haematocolpos

Figure 1: Transabdominal ultrasound of uterus and vagina. Figure 3: Sagittal view T1 weighted MRI abdomen/pelvis.

Right adnexa Bilateral pyo- complex mass haemato-salpinges

Figure 4: Axial view T1 weighted MRI pelvis. Figure 2: Transabdominal ultrasound of right adnexa-complex mass noted 70 mm × 45 mm × 42 mm. in the abdominal cavity was drained, and division of omen- tal adhesions to uterus and fallopian tubes, caecum, and appendix was necessary to adequately view pelvic organs. A blood.However,itdidrevealapartiallyperforatedhymen right TOA was identified, incised, and drainedFigure ( 5). with the rest of the hymen still intact. Intravenous antibiotics A thorough washout was performed and a pelvic drain was were empirically commenced. inserted. Biochemical workup indicated signs of infection, with a c-reactive protein of 315, elevated white cell count of 12, She had an uncomplicated recovery and was discharged and a neutrophil count of 8.9. Blood cultures were obtained, six days following the procedure with a course of oral and ultimately a coagulase negative staphylococcus (probable antibiotics. At outpatient review six weeks following surgery, skin contaminant) was grown. The urine specimen was she reported a resolution of her dysmenorrhoea with the grossly contaminated with blood and grew no organism. The menstrual cycle experienced postoperatively. beta-human chorionic gonadotropin was negative. The transabdominal ultrasound images showed grossly 3. Discussion dilated, serpiginous fallopian tubes with low level internal echoes and peripheral vascularity. There was echogenic sub- A literature search was conducted using Medline, Pubmed, stance within the endometrial cavity; the upper part of vagina and Cochrane with the terms [mullerian anomaly] OR [mul- was dilated and filled with homogenous echogenic substance lerian tract] OR [imperforate hymen] OR [vaginal septum] (Figure 1). A right complex mass was noted on ultrasound AND [pelvic abscess] OR [pelvic infection]. This yielded (Figure 2). The lower vagina was not well visualized. The several case reports of common and unusual presentations for renal tract was assessed as normal. Magnetic resonance imperforate hymen. imaging (MRI) further elucidated the complex right adnexa On occasion, antenatal ultrasound can detect an imper- mass with bilateral pyo-haemato-salpinges, haematometra, forate hymen due to the presence of hydrocolpos in the fetus and haematocolpos (Figures 3 and 4). The pictures were in in response to maternal oestrogens [11]. Imperforate hymen keeping with a partial imperforate hymen rather than vaginal can also be identified on examination of the newborn, and septum. if it is asymptomatic at this time, the recommendation is to An examination under anaesthesia with diagnostic delay surgical management until puberty, when the Oestro- laparoscopy was undertaken. The imperforate hymen was genization improves elasticity and healing. The diagnosis is opened via a cruciate incision and blood drained from infrequently made in infancy, if the patient presents with a distended and oedematous upper vagina. Purulent material mucocele, in which case hymenectomy may be indicated. Case Reports in Obstetrics and Gynecology 3

in symptomatic presentations, with the potential for long- term menstrual and reproductive ramifications. Our case Right tubo- highlights the risks of late recognition of imperforate hymen, ovarian abscess through the development of sepsis and TOA.

Conflict of Interests The authors declare that there is no conflict of interests Figure5:Imagefromdiagnosticlaparoscopyprocedure. regarding the publication of this paper.

Table 1: Unusual presentations of imperforate hymen. References

Presentation Authors Year [1] A. S. Eksioglu, H. A. Maden, G. Cinar, and Y. T. Yildiz, Bapat and Bergsman [3]2008 “Imperforate hymen causing bilateral hydroureteronephrosis in Back pain/sciatica Drakonaki et al. [4]2010an infant with bicornuate uterus,” Case Reports in Urology,vol. 2012,ArticleID102683,4pages,2012. Recurrent UTI Bursac et al. [5]2012 [2] J.C.PosnerandP.R.Spandorfer,“Earlydetectionofimperforate Iatrogenic pyocolpos Lok and Yip [6]2001 hymen prevents morbidity from delays in diagnosis,” Pediatrics, Pelvic abscess Sanfilippo and Mansuria [7]2006 vol. 115, no. 4, pp. 1008–1012, 2005. Dane et al. [8]2007[3] R. Bapat and C. Bergsman, “Hematometrocolpos presenting as Urinary retention Ercan et al. [9]2011sciatica, constipation, and urinary retention,” Clinical Pediatrics, Gyimadu et al. [10]2009vol. 47, no. 1, pp. 71–73, 2008. [4]E.E.Drakonaki,I.Tritou,G.Pitsoulis,K.Psaras,andE. Sfakianaki, “Hematocolpometra due to an imperforate hymen A retrospective cohort study demonstrated a bimodal presenting with back pain: sonographic diagnosis,” Journal of distribution of age at diagnosis. 43% of patients were diag- Ultrasound in Medicine,vol.29,no.2,pp.321–322,2010. nosedatlessthan4yearsofage,andtheremaining57% [5] D. Bursac, Z. Duic, J. Z. Partl, J. Valetic, and S. Stasenko, “Hema- were diagnosed over 10 years of age. In the group of patients tocolpos resulting from an imperforated hymen diagnosed by ultrasound in a patient with recurrent urinary tract infections,” diagnosedover10years,100%ofpatientswereasymptomatic, Journal of Pediatric & Adolescent Gynecology,vol.25,no.5,pp. compared with just 10% of girls diagnosed under the age of 4 340–341, 2012. years [2]. [6] I. H. Lok and S.-K. Yip, “Iatrogenic pyocolpos in a young girl In spite of recommendations for early inspection of the with imperforate hymen,” Australian and New Zealand Journal external genitalia, variations in hymenal anatomy frequently of Obstetrics and Gynaecology,vol.41,no.1,pp.104–105,2001. escape diagnosis until menarche. Although the most com- [7] A. M. Sanfilippo and S. M. Mansuria, “Microperforate hymen mon adolescent presentation of an imperforate hymen is resulting in pelvic abscess,” Journal of Pediatric and Adolescent haematocolpos manifesting as abdominal pain [2], there are Gynecology,vol.19,no.2,pp.95–98,2006. several case reports of more unusual or severe presentation of [8] C. Dane, B. Dane, M. Erginbas, and A. Cetin, “Imperforate imperforate hymen, and these are presented in Table 1. hymen—a rare cause of abdominal pain: two cases and review Of interest are the two case reports of pelvic infection of the literature,” JournalofPediatricandAdolescentGynecology, arising from an imperforate hymen, which demonstrates the vol. 20, no. 4, pp. 245–247, 2007. breakdown of the barrier from a sterile haematocolpos, either [9] C. M. Ercan, K. E. Karasahin, I. Alanbay, M. Ulubay, and I. iatrogenically [6], or embryologically [7]. This paralleled our Baser, “Imperforate hymen causing hematocolpos and acute case presentation in that our patient did not become septic urinary retention in an adolescent girl,” Taiwanese Journal of until the spontaneous rupture of the hymen which thus Obstetrics and Gynecology, vol. 50, no. 1, pp. 118–120, 2011. facilitated ascending infection of her reproductive tract. [10] A. Gyimadu, B. Sayal, S. Guven, and G. S. Gunalp, “Hemato- Only one paper reviewed the long term outcome of late colpos causing severe urinary retention in an adolescent girl diagnoses of imperforate hymen. It reported the persistence with imperforate hymen: an uncommon presentation,” Archives of menstrual dysfunction even after hymenectomy, with of Gynecology and Obstetrics,vol.280,no.3,pp.461–463,2009. 60% demonstrating abnormal menstruation and 40% with [11] C.-C. Liang, S.-D. Chang, and Y.-K. Soong, “Long-term follow- ongoing dysmenorrhea [11]. Furthermore, the data assessing up of women who underwent surgical correction for imperfo- fertility in these patients is sparse but encouraging. 86% of rate hymen,” Archives of Gynecology and Obstetrics,vol.269,no. womenwhoattemptedpregnancyconceivedaftersurgical 1,pp.5–8,2003. correction of their imperforate hymen [12]. [12]J.A.Rock,H.A.Zacur,A.M.Dlugi,H.W.JonesJr.,andR. W. TeLinde, “Pregnancy success following surgical correction of imperforate hymen and complete transverse vaginal septum,” 4. Conclusion Obstetrics and Gynecology,vol.59,no.4,pp.448–451,1982. Ideally identification of the imperforate hymen should occur during the neonatal examination. Delay in diagnosis results