MR Imaging Evaluation of Obstructing Vaginal

The Egyptian Journal of Radiology and Nuclear Medicine xxx (2017) xxx–xxx

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The Egyptian Journal of Radiology and Nuclear Medicine

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Original Article MR imaging evaluation of obstructing vaginal malformations with hematocolpos or hematometra in adolescent girls: A cross sectional study ⇑ Deb Kumar Boruah a, , Rajanikant R. Yadav b, Kangkana Mahanta a, Antony Augustine a, Manoj Gogoi c, Lithingo Lotha d a Department of Radio-diagnosis, Assam Medical College and Hospital, Dibrugarh, Assam, India b Department of Radio-diagnosis, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India c Department of Pediatric Surgery, Assam Medical College and Hospital, Dibrugarh, Assam, India d Department of Obstetrics & Gynecology, Assam Medical College and Hospital, Dibrugarh, Assam, India article info abstract

Article history: Objective: Vaginal or uterine outlet obstruction leads to hematocolpos or hematometra. Detection of the Received 2 December 2016 etiology of this entity is important to guide adequate surgical management and thereby avoid complica- Accepted 29 April 2017 tions and to preserve fertility. The aim of this study was to evaluate obstructing vaginal malformations in Available online xxxx adolescent girls presenting with hematocolpos or hematometra with MR imaging. Materials and methods: A hospital based prospective study was conducted in a tertiary care centre from Keywords: September 2015 to October 2016. The study included 17 adolescent females who were evaluated with Herlyn Werner Wunderlich syndrome MRI. (HWWS) Result: Of 17 adolescent female with vaginal or uterine outflow obstructive anomalies with hematocol- Mullerian duct anomaly Magnetic Resonance Imaging (MRI) pos or hematometra, where 6 patients (35.3%) had HWWS, 6 patients (35.3%) had imperforate hymen, 2 Hymenectomy patients (11.8%) had transverse vaginal septum, 1 patient each (5.9%) had cervico-vaginal atresia, unicor- Vaginal septum nuate uterus and communicating rudimentary Uterine horn. MRI revealed hematocolpos in 15 patients (88.2%), hematometra in 13 patients (76.5%), endometriotic ovarian cysts in 6 patients (35.3%) and hematosalpnix in 3 patients (17.6%). Conclusion: Early radiological diagnosis of the cause of vaginal or uterine outflow obstruction is important to guide adequate surgical management which if undertaken promptly helps to avoid complications due to reflux from vaginal or uterine outflow obstruction. Ó 2017 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/ 4.0/).

1. Introduction atresia, abnormal vaginal opening, cloacal malformation and acquired vaginal stenosis. Even though hematocolpos and Congenital vaginal or uterine outflow obstruction may occur at hydrometrocolpos are rare anomalies of female genital tract, the different levels with different clinical presentations [1]. In hemato- incidence of these anomalies is reported between 3.8 and 0.1% [2]. colpos there is accumulation of menstrual blood in the vaginal cav- Embryologically, the vagina develops from different sources. ity. Its occur due to obstruction in vaginal outflow tract. Vaginal The upper 2/3rd of the vagina develops from the fused parameso- outflow obstruction is commonly associated with imperforate nephric ducts and the lower 1/3rd from the urogenital sinus [3]. hymen, transverse vaginal septum, vaginal atresia, hemi-vaginal Due to different origins of vaginal portions, vaginal outflow atresia (Herlyn Werner Wunderlich syndrome), cervico-vaginal obstruction clinically manifests as hydrometrocolpos in the neonatal period and hematometrocolpos at puberty. Majority of patients with vaginal outflow obstruction may

Peer review under responsibility of The Egyptian Society of Radiology and Nuclear experience sexual difﬁculty, menstrual irregularities and infertility. Medicine. Hematocolpos commonly associated with hematometra, hematos- ⇑ Corresponding author at: RCC-4, M-Lane, Assam Medical College Campus, alpnix and haemorrhagic (endometriotic) cysts in ovaries [4] Dibrugarh, Assam 786002, India. which occurs commonly in untreated patients of vaginal outﬂow E-mail address: [email protected] (D.K. Boruah). https://doi.org/10.1016/j.ejrnm.2017.04.010 0378-603X/Ó 2017 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Please cite this article in press as: Boruah DK et al. . Egypt J Radiol Nucl Med (2017), https://doi.org/10.1016/j.ejrnm.2017.04.010 2 D.K. Boruah et al. / The Egyptian Journal of Radiology and Nuclear Medicine xxx (2017) xxx–xxx obstruction. Recurrent endometriosis occurs as a long standing Medical Systems Corporation, made in Tokyo, Japan). MRI scan complication of vaginal or uterine outflow obstruction, hence early were done in Siemens Avanto 1.5 Tesla B15 machine (Siemens diagnosis and management of these conditions is of atmost impor- Medical Systems, Erlangen, Germany). The initial USG and MRI tance to avoid complications [4]. findings were correlated with Colposcopic examination and or sur- Imperforate hymen accounts for about 0.1% of hematocolpos gical findings (see Table 2). cases at puberty [5,6]. Imperforate hymen may sometimes be diagnosed soon after birth but more commonly it is diagnosed in adolescence [7]. 2.2. MRI protocols HWWS occurs due to defective development in lateral fusion or reabsorption failure of paired para-mesonephric ducts. It com- All patients were subjected to MRI scan of lower abdomen with prised of uterine didelphys, obstructed hemi-vagina and ipsilateral phased- array surface coil. Initial fast spin echo sagittal T1WI and renal agenesis. The estimated incidence of HWWS is varies from fat suppressed T2WI images of uterus was obtained to ascertain 0.1% to 10% [8–10] the uterine lie followed by fast spin echo T2WI and fat suppressed The prevalence of transverse vaginal septum had been reported T2WI imaging planes to obtained true coronal images of uterus. as 1 in 30,000 to 1 in 84,000 females [11,12]. Sagittal T1W images were obtained with TE:10–12 ms, TR: 500– Uncommonly isolated vaginal atresia can cause of hematocol- 600 ms, slice thickness 4 mm, flip angle 150° and FOV of 190– pos or hematometra with primary amenorrhea [13]. 200. Sagittal T2WI images were obtained with TE: 90–105 ms, T The aim of this study was to evaluate obstructing vaginal mal- R:4800–6000 ms, slice thickness 4 mm, flip angle 1500 and FOV formations in adolescent girls presenting with hematocolpos or of 190–200. True coronal T2W images for uterus were obtained hematometra with MR imaging. with TE: 90–105 ms, TR: 5000–6000 ms, 256 Â 256 matrix, echo train length 13–14, slice thickness 4 mm with 1 mm interslice gap, flip angle of 150° and FOV of 240–260. Fat suppressed axial 2. Materials and methods T2W images were obtained with TE:90–100 ms, TR: 4500–5500 ms, flip angle 150°, slice thickness of 1–2 mm with no interslice After approval from the institutional ethics review committee, a gap and FOV of 200–240. GRE (gradient recalled echo) images were hospital based prospective study was conducted. The study group obtained in axial planes with TE: 18–24 ms, TR: 600–660 ms, inter- comprised of 17 adolescent girls presenting to the departments slice gap of 1–2 mm, section thickness 3–4 mm, flip angle 28 and of Obstetrics & Gynaecology, Pediatric Surgery and Radio- FOV of 200–240. diagnosis of a tertiary care centre from September 2015 to October Diffusion weighted images (DWI) were obtained in axial plane 2016 (see Table 1). using a multi-slice spin echo planer imaging sequence. Imaging parameters were TE: 80–90 ms, TR: 2800–3200 ms, interslice gap 2.1. Patient selection of 1–2 mm, section thickness 3–4 mm, flip angle 90°, FOV of 190–200. RF band width of 1390–1420 and matrix of 128 Â 128. We included adolescent girls with cyclical lower abdominal Diffusion probing gradients were applied in the three orthogonal pain, lower abdominal lump and clinical or preliminary ultrasono- directions with same strength. Diffusion weighted MR images were graphic suspicion of vaginal/uterine anomalies. Informed consent acquired with a diffusion weighted factor of 1000 s/mm2. was obtained from patients/parents/guardian before undergoing Fast(Turbo) spin echo 3D imaging technique SPACE imaging MRI scan. Medical records of all patients were evaluated. Follow were done in coronal plane. The imaging parameters were TE: up were done in all 17 patients with Ultrasonography. Initial Ultra- 200–250 ms, TR: 1200–1400 ms, section thickness 0.8–1 mm, flip sonography (USG) were done using Aplio-500 Machine (Toshiba angle 150°, FOV of 200–240 and acquisition matrix of 300 Â 300. Finally fat suppressed coronal T2WI images of abdomen including the Kidneys were obtained using parameters; TE: 90–100 ms, Table 1 TR: 4000–6000 ms, echo train length 27–29, slice thickness Summarizes the clinical findings in 17 adolescent girls with hematocolpos or 0 hematometra. 4–6 mm, flip angle of 150 and FOV of 280–380.

Serial Menarche/ Presentation Clinical presentation no age (y) age (y) 2.3. Evaluation 1 No 13 Cyclical lower abdominal pain 2 No 14 Palpable hypogastric mass 3 No 11 Lower abdominal pain and dysuria Seventeen patients of hematocolpos and hematometra were 4 Yes/13 15 Urinary retention with abdominal examined to look for detail morphological anatomy of uterus, cer- pain vix and vagina and their anatomical variations, status of signal 5 No 12 Palpable hypogastric lump, low intensity of collection either in vaginal canal, endo-cervical canal, backache fallopian tubes or ovaries. Associated extra-uterine anomalies like 6 No 12 Palpable hypogastric lump 7 No 12 Cyclical lower abdominal pain renal anomaly or syndromic causes were also sought. Sagittal 8 No 15 Cyclical lower abdominal pain images provide uterine position or axis, any abnormal collections 9 No 13 Cyclical lower abdominal pain in endometrial, cervical or vaginal canal. Also helps in assessment 10 Yes/14 16 Cyclical lower abdominal pain of status of transverse vaginal septum or imperforate hymen in 11 No 13 Cyclical lower abdominal pain, dysuria vaginal canal. 12 No 14 Urinary retention with abdominal True coronal images provides of status of uterine cavity, cervical pain canal, vaginal canal and presence of any T2WI hypointense septa 13 No 15 Cyclical lower abdominal pain either in uterine cavity, cervix or vagina. 14 No 14 Urinary retention with abdominal DWI helps in assessment of endometrial cavity, cervical canal, pain 15 Yes/14 15 Cyclical lower abdominal pain status of fallopian tubes and endometriotic ovarian cysts. 16 No 15 Cyclical lower abdominal pain Fast(Turbo) spin echo 3D SPACE imaging with reconstruction in 17 No 16 Urinary retention with lower all three planes helps in better delineation of endometrial, cervical abdominal lump and vaginal canals and status of vaginal septum.

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Table 2 Summarizes the MRI ﬁndings in 17 adolescent girls with hematocolpos or hematometra.

Serial MRI ﬁndings with signal intensity in hematocolpos/hematometra no Uterine morphology T1WI T2WI DWI 1 Uterine didelphys, left hemi-hematocolpos, hemi-hematometra, bilateral ovarian Central hypo and Hyperintense Restricted in hematocolpos and endometriotic cysts, longitudinal vaginal septum, absent left Kidney and peripheral right ovarian hemorrhagic cyst hypertrophied right Kidney hyperintense 2 Uterine didelphys, left hemi-hematometra, left hemi-hematocolpos, absent left Hyperintense Hyperintense Restricted in left hemi-vaginal Kidney hematocolpos and hemi- hematometra 3 Uterine didelphys. Longitudinal vaginal septum with proximal defect, Right renal Hyperintense Hyperintense Restricted in bilateral hemi- agenesis vaginal canals 4 Uterine didelphys, left hematometra, left hemi-hematocolpos, longitudinal Hyperintense Hyperintense Restricted in left hemi-vaginal vaginal septum, left renal agenesis canal 5 Normal uterine morphology, hematometra, hematocolpos, bilateral Hyperintense Hyperintense Restricted in vaginal and hematosalpnix and hemorrhagic cysts in both ovaries, thicker imperforate hymen endometrial cavity collections and ovarian cysts 6 Normal uterine morphology, hematometra, hematocolpos, T2 hypointense Hyperintense Hyperintense Restricted diffusion in transverse vaginal septum with small central hole in lower 1/3rd of vaginal canal hematometra and hematocolpos 7 Normal uterine morphology, hematometra, hematocolpos, bilateral Isointense Hyperintense No diffusion restriction hematosalpnix, bilateral hemorrhagic ovarian cysts, T2 hypointense transverse vaginal septum in lower 1/3rd 8 Normal uterine body morphology, poorly develop cervix and atretic vaginal Hyperintense Hyperintense Restricted diffusion in canal, hematometra, hemorrhagic right ovarian cyst hematometra and right ovarian cyst 9 Unicornuate uterus, cervico-vaginal hypoplasia, hematometra in developed right Hyperintense Hyperintense Restricted diffusion uterine horn, ectopic pelvic left kidney 10 Rudimentary left uterine horn with an endometrial cavity that communicates Hyperintense Hyperintense Restricted diffusion with the uterus, longitudinal vaginal septum, defect in superior portion of vaginal septum, left hemi-vaginal hematocolpos, hematometra in left rudimentary horn, left ovarian hemorrhagic cysts 11 Normal uterine morphology, hematometra, hematocolpos Hyperintense Hyperintense Restricted 12 Normal uterine morphology, hematocolpos Isointense Hyperintense Restricted 13 Uterine didelphys, left hemi-hematometra, left hemi-hematocolpos, absent left Restricted Kidney 14 Normal uterine morphology, hematometra, hematocolpos Hyperintense Hyperintense Restricted 15 Uterine didelphys, longitudinal vaginal septum, right hemi-vaginal Hyperintense Hyperintense Restricted hematocolpos, right renal agenesis 16 Normal uterine morphology, hematocolpos Hyperintense Hyperintense Restricted 17 Normal uterine morphology, hematocolpos Hyperintense Hyperintense Restricted

Fat suppressed coronal T2WI images of whole abdomen used to (76.5%) and T1WI hypo to isointense signal intensities in 4 patients assessed any associated renal anomalies like renal agenesis, ecto- (23.5%). Diffusion weighted images (DWI) showed diffusion pic location or fusion anomalies. restriction with low ADC value in 15 patients (88.2%). The largest dimension of obstructed collection either hematocolpos or 2.4. Statistical analysis hematometra had mean long axis diameter of 5.83 cm ± 4 cm [SD] and mean short axis diameter of 3.2 cm ± 2.3 cm [SD]. Fifteen Data were presented in terms of percentage, mean and standard patients (88.2%) showed hematocolpos, 13 patients (76.5%) deviation. Calculations were done using SPSS programs (Statistical showed hematometra, 6 patients (35.3) showed ovarian Package for the Social Science version 16. SPSS Inc., Chicago, USA). endometriotic cysts and 3 patients (17.6%) showed hematosalpnix. Out of 17 adolescent girls, the uterine morphology was normal 2.5. Results in 9 patients (52.9%), didelphic in 6 patients (35.3%), unicornuate in 1 patient (5.9%) and another 1 patient (5.9%) had communicating We evaluated 17 adolescent females of vaginal/uterine outﬂow rudimentary uterine horn . obstructive anomalies with hematocolpos or hematometra. The All 6 patients (35.3%) of HWWS had uterine didelphys, mean age of presentation was 13.8 years ± 1.5[SD]. The patients obstructed hemi-vagina, longitudinal vaginal septum and ipsilat- were presented with cyclical lower abdominal pain in 9 patients eral renal agenesis. Left sided obstructed hemi-vagina was noted (52.9%) followed by palpable hypogastric mass in 4 patients in 4 patients (23.5%) and right sided obstructed hemi-vagina in 2 (23.4%), lower abdominal pain with urinary retention in 3 patients patients (11.8%). (Figs. 1–4). Longitudinal vaginal septum was (17.6%). Six patients (35.3%) had Herlyn Werner Wunderlich syn- demonstrated on MRI in all 6 patients of HWWS, where 5 patients drome (HWWS), 6 patients (35.3%) had Imperforate Hymen, 2 had communication of obstructed hemi-vagina with the contralat- patients (11.8%) had transverse vaginal septum, 1 patient each eral hemi-vagina via the proximal longitudinal septal defect. One (5.9%) had cervico-vaginal atresia, unicornuate uterus and commu- patient showed complete T2 hypointense longitudinal septum nicating rudimentary Uterine horn. In all 17 patients bilateral ovar- without septal defect (Fig. 5). All 6 patients of HWWS underwent ian follicular activity were well demonstrated on MRI. surgical excision and marsupialization of longitudinal vaginal sep- Variable distension of obstructed vagina, hemi-vagina and tum via transvaginal approach. cervico-uterine canals were demonstrated, where hematocolpos, Six patients (35.3%) had Imperforate Hymen with inferiorly hematometra or ovarian endometriotic cyst showed variable signal bulged imperforate hymen on colposcopy and MRI. No uterine intensities on MRI. Hematocolpos, hematometra or ovarian anomaly was demonstrated in these patients (Fig. 6). All these 6 endometriotic cyst showed T1WI bright signals in 13 patients patients underwent Hymenectomy. Follow up USG showed

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Fig. 1. 13 years old female patient presented with cyclical lower abdominal pain n for 5 months. USG images (a, b & c) revealed uterine didelphys. Hypoechoic collection was noted in left para-median location of vaginal canal (white arrow) with collection and thicker internal echo debris in caudally directed transverse image (white * in image c).

Fig. 2. Same patient in Fig. 1: Sagittal T1WI (a) and fat suppressed T2WI (b) MRI revealed T1WI central hypo and peripheral hyperintense and T2WI hyperintense collection in vaginal canal (red *). Fat suppressed coronal T2W image (c) showed atretic lower 1/3rd of left hemivaginal canal with T2WI hyperintense collection in left hemivaginal canal which bulged inferiorly (red arrow) and causes right lateral displacement of longitudinal T2WI hypointense vaginal septum (yellow arrow). Right hemi-vaginal canal was normal (yellow arrow). Axial T1W and fat suppressed axial T2W images (d & e) showed slight T1 hyperintense and T2 hyperintense right ovarian endometriotic cyst (white arrow). Fat suppressed coronal T2W image (f) showed left renal agenesis which consistent with part of HWWS. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) adequate drainage of hematocolpos and hematometra in these Kidney. Hematometra was demonstrated in right sided unicornu- patients. ate uterus. Colposcopy showed smaller atretic vaginal opening. Two patients (11.8%) had transverse vaginal septum in lower Conservative treatment was provided to this patient with oral con- 1/3rd of vaginal canal. Central hole or defect was well demon- traceptive pills (OCP). strated in 1 patient (Fig. 7). No uterine anomaly was demonstrated Another 1 patient (5.9%) had hematometra in left sided cavitary in these patients with transverse vaginal septum. These 2 patients communicating rudimentary uterine horn with longitudinal vagi- underwent surgical excision of transverse septum with marsupial- nal septum, left hemi-vaginal hematocolpos and hematometra in ization of margins of septum with normal vaginal mucosa/ wall. No left rudimentary uterine horn. Conservative treatment was pro- post operated complication was noted in follow up imaging. vided to this patient with OCP. One patient (5.9%) of cervico-vaginal atresia showed hemato- The MR imagings findings were correlated with Colposcopic colpos and ovarian endometriotic cyst (Fig. 8). Colposcopy showed examination (see Table 3). The MR imaging had sensitivity of smaller introitus with smaller vaginal canal and underdeveloped 91.7%, specificity of 40%, positive predictive value 78.6%, negative cervix. This patient underwent repeated cervical canal dilatation predictive value 66.7% and accuracy of 76.5% for detection of vaginal with vaginoplasty. or uterine outflow obstructive anomalies presented with hematocol- One patient (5.9%) had right sided unicornuate Uterus with pos or hematometra. MR imaging fails to detect slight oblique to ver- hypoplastic cervix and vagina with ectopic pelvic location of left tically oriented vaginal septum in a patient of HWWS and which was

Fig. 3. 14 years female was presented with palpable hypogastric mass. USG images (a, b & c) showed bicornuate uterus with hypoechoic collections in left uterine cavity (white arrow) and left hemi-vaginal canal (white *). Sagittal T1WI and T2W (d, e & f) MR images showed T1WI and T2WI hyperintense collections in left hemi-vaginal cavity and left sided uterine horn (white arrow).

Fig. 4. Same patient in Fig. 3. Fat suppressed T2W images (a, b & c) showed uterine didelphys with T2WI hyperintense left hemi-vaginal hematocolpos and hemi- hematometra. T2WI hyperintense right ovarian endometriotic cyst was also noted. Left renal agenesis was noted in image c. Fat suppressed axial T2WI images (d & e) showed T2WI hyperintense collection in left hemi-vaginal canal (red arrow) and left sided uterine horn (white arrow). Compressed right hemi-vagina was noted in image e (yellow arrow). Axial diffusion weighted image (f) shows diffusion restriction in left hemi- hematocolpos and hemi-hematometra. These findings consistent with HWWS. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) detected on Colposcopic examination. However colposcopy failed to in 3 patients (50%) of HWWS which were confirmed with surgical detect longitudinal vaginal septum in 1 patient of HWWS. Col- findings. Colposcopy also not able to detect any changes in patients poscopy as well as MRI fails to detect longitudinal vaginal septum with unicornuate uterus and rudimentary uterine horn.

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Fig. 5. 15 years female patient was presented with lower abdominal pain and urinary retention. Sagittal T1WI and T2WI images (a & b) showed T1WI hyperintense and T2WI iso to hypointense collection noted in vaginal cavity (white block arrow). Coronal T2W (c) image showed uterine didelphys (white arrow) and normal bilateral ovaries with follicles. Fat suppressed axial T2WI (d & e) images shows bilateral hemi-vaginal canals (yellow & red arrow) with T2 iso to hypointense collection in upper portion of left hemi-vagina (red arrow). T2WI hypointense longitudinal vaginal septum was noted (yellow *). Coronal T2WI image (f) showed left renal agenesis. (For interpretation of the references to colour in this ﬁgure legend, the reader is referred to the web version of this article.)

Fig. 6. 12 years female patient presented with lower abdominal pain and hypogastric lump. Sagittal T1WI (a & b) and coronal T2WI (c, d & e) images showed T1WI and T2WI hyperintense collections in distended vaginal cavity (red *) and endometrial cavity. T2WI hyperintense endometriotic cysts were noted in bilateral ovaries with bilateral hematosalpnix (white block arrow). T1WI isointense and T2WI hypointense thicker inferiorly bulged imperforate hymen was noted (red block arrow). Axial T2WI (f) showed T2WI hypointense dependent debris in the distended vaginal cavity collection. (For interpretation of the references to colour in this ﬁgure legend, the reader is referred to the web version of this article.)

Fig. 7. 12 years patient presented with hypogastric lump. USG image (a) showed hypoechoic collection in distended vaginal canal with internal echo debris. Minimal hypoechoic collection was noted in endometrial cavity. Sagittal T1WI (b & c) and T2W images in sagittal, coronal and axial (d, e & f) plane showed T1WI and T2WI hyperintense collections in distended vaginal and endometrial cavities. T2WI hypointense complete transverse vaginal septum was noted in lower 1/3 rd of vaginal canal (white arrow). Central hole was noted in the T2WI hypointense septum (red block arrow). (For interpretation of the references to colour in this ﬁgure legend, the reader is referred to the web version of this article.)

Fig. 8. 15 years female patient with cyclical lower abdominal pain. Sagittal T1WI, T2WI, coronal T2WI and T1WI (a, b, c & d) images showed T1WI and T2WI hyperintense collection in endometrial cavity (thin arrow) with poorly developed cervix and vagina (block arrow). Axial T1WI and T2WI images (e & f) showed T1WI and T2WI hyperintense hematometra and right ovarian endometriotic cyst (thin arrow).

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Table 3 Summarizes the Colposcopic ﬁndings, management and associated anomalies in 17 adolescent girls with hematocolpos or hematometra.

Serial no Colposcopy ﬁndings Diagnosis Management Associated anomalies 1 Bulge left hemi-vagina, Foul smelling vaginal HWWS Excision of vaginal septum Left renal agenesis discharge, incomplete longitudinal vaginal septum 2 Single vaginal ostium, incomplete longitudinal HWWS Excision of vaginal septum Left renal agenesis vaginal septum with bulging of vaginal septum towards right side 3 Two vaginal ostium with incomplete HWWS Excision of vaginal septum Right renal agenesis longitudinal vertical vaginal septum and intercommunication between the hemi-vaginal canals via the proximal septal defect 4 Single vaginal ostium, longitudinal vaginal HWWS Excision of vaginal septum Left renal agenesis, Mild septum scoliosis of lumbar spine 5 Inferiorly bulged imperforate hymen IH Hymenectomy No 6 Inferiorly bulged transverse vaginal septum with TVS Excision and No small central hole, foul smelling discharge marsupulization of septum 7 Inferiorly bulged transverse vaginal septum TVS Excision and No marsupulization of septum 8 Atretic single vaginal opening, foul smelling Cervico-vaginal atresia Conservative cervico- No discharge vaginal canal dilatation 9 Small single vaginal opening with hypoplastic Unicornuate uterus with Conservative with OCP Ectopic pelvic left kidney vaginal and cervical canals cervico-vaginal hypoplasia 10 Longitudinal vaginal septum with defect in it’s Rudimentary left uterine Excision of vaginal septum No superior portion, foul smelling discharge horn communicates with the uterus, longitudinal vaginal septum 11 Inferiorly bulged imperforate hymen IH Hymenectomy No 12 Inferiorly bulged imperforate hymen IH Hymenectomy No 13 Bulged left hemi-vagina, Foul smelling vaginal HWWS Excision of vaginal septum Left renal agenesis discharge, incomplete longitudinal vaginal septum 14 Inferiorly bulged imperforate hymen IH Hymenectomy No 15 Bulged right hemi-vagina, incomplete HWWS Excision of longitudinal Right renal agenesis longitudinal vaginal septum vaginal septum 16 Inferiorly bulged bluished imperforate hymen IH Hymenectomy No 17 Inferiorly bulged imperforate hymen IH Hymenectomy No

Abbreviation: T1WI-T1 weighted image, DWI-diffusion weighted image, HWWS-Herlyn Werner Wunderlich syndrome, TVS-Transverse vaginal septum, IH-Imperforate Hymen.

The One sample T test showed p value of 0.0005 and 95% confi- showed transverse vaginal septum in lower 1/3rd of vagina with dence interval of MR imaging in evaluation of utero-vaginal out- small central aperture hole in 1 patient. Dennie et al. detected flow obstruction was 0.62–1.03. transverse vaginal septum in lower 3rd of vagina in three patients [18]. 2.6. Discussion Vaginal atresia either isolated [19] or part of complex syndromic anomalies like Mayer-Rokitansky Kuster Hauser (MRKH) In our study we have encountered various congenital anomalies syndrome, Bardet-Biedl syndrome, Kaufman-Mc Kusick syndrome, of female genital tract causing vaginal or uterine outflow obstruc- Fraser syndrome [20] and Winter syndrome causes vaginal outflow tion subsequently leading to hematocolpos and hematometra in tract obstruction which may presented with hematocolpos and or adolescent girls. hematometra. In our study sample, only one patient had isolated Congenital anomalies of female genital tract especially Mulle- cervico-vaginal atresia with hematocolpos. rian duct anomalies are result from non-development, defective Rarely chronic and recurrent vaginitis or vaginal biopsy causing vertical or lateral fusions or reabsorption failure of the Mullerian vaginal scaring and stricture formation which subsequently lead- (para-mesonephric) ducts. Various Mullerian duct anomalies had ing to hematocolpos [21], but in our study no such case has been been classified by the American Society of Reproductive Medicine encountered. according to anatomic findings into VII classes [14]. Ultrasonography is a preliminary imaging tool in the diagnosis Clinical, USG and MRI scan helps in establishing the different of mullerian duct anomalies. MRI is a suitable imaging modality pattern of vaginal or uterine outflow tract obstructions like imper- which provides contour of uterus, shape of uterine cavity, continu- forate hymen, transverse vaginal septum, longitudinal vaginal sep- ity of vagina, septal anatomy, fluid content nature as well as asso- tum and cervico-vaginal atresia [15]. ciated endometriosis and renal or vertebral anomalies. 3D SPACE The congenital vaginal septum are either transverse or longitu- MRI sequence provides more details of endometrial, cervical or dinal. Transverse vaginal septum is associated with urogenital or vaginal canal status. gastrointestinal anomalies [16]. The transverse vaginal septum Sometime uncommon presentation of congenital female genital varies in their location and thickness, where the thicker septum tract anomalies can lead to delayed diagnosis and erroneous man- usually located closed to cervix. agement. Hence high index suspicion and pelvic MRI can be helpful Deligeoroglou et al. found location of transverse vaginal septum in early diagnosing these various vaginal or uterine outlet obstruc- in upper third in 46% patients, middle third in 40% patients and tive entities [22,23]. Surgical management is imperative in most of lower third 14% in patients [17]. In our study sample 2 patients these patients to preserve the reproductive functions .

Adolescent female with cyclical abdominal pain, lower abdominal lump or urinary retention

Preliminary USG showed hematocolpos or Preliminary USG showed hematocolpos or hematometra without sonographically hematometra with imperforate hymen or detected any obstructing lesion transverse vaginal septum

Pelvic MRI for detecting longitudinal Colposcopy vaginal septum, obstructed hemi- Confirmed on colposcopy then vagina or cervico-vaginal atresia patient can be treated surgically without pelvic MRI or Pelvic MRI can be done as a luxury imaging

Surgical correlation Surgical correlation

Fig. 9. Imaging algorithm for approaching vaginal or uterine outﬂow obstruction.

Preliminary USG and Colposcopy can diagnosed some outlet Oral contraceptive pills may be needed for menstrual suppres- obstructions like transverse vaginal septum and imperforate sion to prevent further accumulation of hematocolpos or hymen, where MRI act as luxury imaging. However MRI provide hematometra if surgical intervention has been deferred [5]. more anatomical details like position, thickness of septum with presence or absence of hole/aperture in the septum. In our study 2.7. Imaging algorithm for approaching vaginal or uterine outflow sample MRI fails to detect oblique to longitudinally oriented vagi- obstruction nal septum in 50% of patients of HWWS. Some time MRI also fails to detect communication or defect within the longitudinal vaginal The imaging algorithm flow chart has been described in Fig. 9. septum in non-obstructed form. But MRI should be performed in preliminary Sonographic suspi- cious cases of HWWS, vaginal atresia, cervical atresia, endo- 2.8. Limitation cervical canal atresia with hematocolpos and associated vertebral or renal anomalies which helps to avoid unnecessary laparoscopy Our descriptive study including MRI assessment of only 17 or laparotomy. patients of vaginal or uterine outflow obstruction with hematocol- Occasionally Tumour markers like CA125 and CA19-9 raised in pos or hematometra in one year duration which preclude assess- patients with Hematocolpos and hematometra [24,15] and in such ment of actual specificity and accuracy of the imaging modality. situations pelvic MRI plays a crucial role in diagnosing these We look forward to do a study in future including a larger sample entities. size in a longer duration. Deligeoroglou et al. found concomitant association of Imperfo- rate hymen, proximally located transverse vaginal septum and uni- 2.9. Conclusion cornuate uterus in a patient [16], but in our study sample no such case of concomitant association was noted. Early radiological diagnosis of the cause of vaginal or uterine Early recognition of these vaginal/uterine outlet obstructive outflow obstruction is important to guide adequate surgical man- causes is important in order to preserve fertility and avoid compli- agement which if undertaken promptly helps to avoid complica- cations from retrograde tubal reflux and subsequent endometriosis tions due to reflux from vaginal or uterine outflow obstruction. [25]. Although prognosis is good in most of the causes of vaginal outlet obstruction with the major concern being preservation of Conflict of interest fertility. Patient with didelphys uterus have a high likelihood of becoming pregnant, where about 80% able to conceive [26] with We have no conflicts of interest to declare. high risk of premature delivery (22%) and abortion (74%). Watanabe et al. reported adenocarcinoma in obstructed side of cervix and clear cell carcinoma in obstructed hemi-vagina in References HWWS due to delayed recognition of this entity [27]. [1] Johal NS, Bogris S, Mushtaq I. Neonatal imperforate hymen causing obstruction The primary objective for management of imperforate hymen, of the urinary tract. Urology 2009;73:750–1. transverse vaginal septum and HWWS is to relieve the outflow [2] Laufer MR, Goldstein DP, Hendren WH. Structural anomalies of the female obstruction. Vaginoscopic excision of the imperforate hymen, reproductive tract. In: Emans SJH, Laufer MR, Goldstein DP, editors. Pediatric transverse vaginal septum is considered less invasive and has a and adolescent gynecology, Lippincott; Philadelphia; 2005. p. 334–416. [3] Drews U, Sulak O, Schenck PA. Androgens and the development of the vagina. lower risk of recurrence. Biol Reprod 2002;67:1353–9.

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