Preoperative MRI assessment of hematometrocolpos with vaginal septum: added value of vaginal gel usage, reporting tips with surgical correlation.
Preoperative MRI assessment of hematometrocolpos with vaginal septum: added value of vaginal gel usage, reporting tips with surgical correlation.
Phoebe Hiu Wai Lo, Siu Chun Wong, Hoi Ming Kwok, Ting Wong, Ka Fai Johnny Ma, Siu Fan Rebecca Wan, Tze Man Mimi Fung, Chee Hang Cheryl Lung
The objectives of our study are 1) to demonstrate the benefit of vaginal gel instillation in pelvic MRI assessment of vaginal septum through illustrations of companion cases with surgical and pathological correlation, 2) to highlight the essential imaging features of vaginal septum that should be reported to facilitate the surgical planning and 3) present associated pelvic complications and urogenital anomalies for comprehensive assessment.
Introduction: Hematometrocolpos is the accumulation of blood products within the uterine cavity and vagina as a consequence of vagina outflow obstruction to menstrual blood passage. Patients may present in early adolescence with primary amenorrhea and/ or cyclical pelvic pain, owing to progressive accumulation of retained menstrual blood causing vagina distension during puberty. If left untreated, complications of hematometrocolpos can arise and result in adverse long term sexual and reproductive outcome, such as hematosalpinx or endometriosis due to retrograde menstruation, or superimposed pelvic infection and these predispose to pelvic adhesion formation and subfertility. Vaginal septum is an important aetiology of vaginal obstruction amenable to surgical correction, among other important differentials including imperforate hymen and vaginal atresia. Prompt diagnosis and comprehensive evaluation of the full spectrum of vaginal and mullerian anomalies facilitates patient counselling, optimal planning for corrective surgery with drainage of hematometrocolpos for pain relief, septum excision and vaginoplasty to restore vaginal patency for menstrual flow and future sexual wellbeing, and treatment of co-existing urological anomalies. Embryology of Vaginal Development: During development of the uterovaginal canal, the Müllerian ducts fuse and develop into the uterus, cervix, and upper vagina. Distally, Müllerian ducts reach the urogenital sinus, forming a sinovaginal bulb which develops and canalises into the lower vagina. Incompletion of any part of the development or canalisation process will result in congenital outflow tract obstruction. Vaginal septum arises as a result of either lateral fusion defect of the Müllerian duct which gives rise to longitudinal vagina septum, or a vertical fusion defect which results in transverse vaginal septum formation. Vaginal septum can also exist as part of the other complex Müllerian malformation, such as obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome (also known as Herlyn Wener Wunderlick syndrome). Aetiology and Prevalence of Vaginal Septum: A vaginal septum can develop at any level of the vagina but most commonly in the upper vagina followed by the mid vagina and lower vagina. Septa can be complete or incomplete and generally are less than 1 cm thick. This septum is a membrane of fibrous connective tissue with vascular and muscular components. The prevalence of vaginal septum is reported to be ranging from 1 in 2,100 to 1 in 72,000 females. In particular, transverse vaginal septum has an incidence of one in 20,000 females. However, detection and visualisation of vaginal anomalies, such as transverse or longitudinal vaginal septum and partial vaginal agenesis, by ultrasonography or conventional MRI could be challenging due to lack of tissue contrast between the collapsed vaginal walls. Both vaginal septum and the vaginal wall can appear hypointense on T2 weighted imaging due to their fibrous and muscular contents respectively. Use of vaginal gel instillation for optimal vaginal distension has been advocated for better delineation of vaginal anomalies in international guidelines. Intraluminal vaginal lesions can also be better visualised with improved vaginal distension. Administration of vaginal gel before pelvic MRI is inexpensive and well tolerated by patients with improved diagnostic usefulness of MRI for vaginal anomalies. The useful information regarding vaginal septum characteristics obtained from MRI examination with vaginal gel opacification is conducive to subsequent vaginal correction surgical planning. Patient preparation and Procedure for Vaginal Gel instillation: In our centre, written informed consent is obtained from the adolescent patient and her guardian before MRI examination with vaginal gel instillation, with understanding of the indications and improved diagnostic benefit of vaginal gel instillation. Small risks of hymenal tear and infection are also explained. We place a 50 to 60 ml of sterile aqueous gel in a syringe, which is placed in an upright position to help expel as many air bubbles from the gel mixture as possible. The syringe is then connected to a 8 Fr Foley catheter and extension tubing with further priming of the tubings to expel air from the catheter dead space. Patient is in supine position with frog-leg posture for optimal exposure of the perineum. Cleansing with aqueous Hibitane and draping of the perineum would be performed. The Foley catheter would be inserted into vagina by the attending radiologist in the MRI suite before scan commencement. Our gynaecologists colleagues would also provide assistance should there be any difficulty in identification of the hymenal opening. Gel was then instilled into the vagina and catheter was removed before the MRI procedure. MRI Protocol: MRI examinations are performed on a 1.5 Tesla or 3 Tesla scanner with a pelvic phased array coil. Our pelvic MRI protocol for assessing vaginal anomalies consists of T2-weighted turbo spin-echo (TSE) sequences in axial, sagittal and coronal planes for anatomical assessment, ensuring the field of view covers the entire perineum. Volumetric 3D T2-weighted MR images are also acquired, allowing curved or multiplanar reformats. Dedicated coronal and axial planes along the uterine body axis are obtained to evaluate the coexisting mullerian anomalies. T1-weighted TSE imaging in axial planes with and without fat saturations of the pelvis are acquired for identification of blood contents in hematocolpos and endometriotic lesions. Diffuse weighted imaging with apparent diffusion coefficient (ADC) maps are obtained, with B-value choice of 50, 400 and 800. Further T2-weighted MRI of the abdomen (axial / coronal planes) or with additional MR urography are done for assessment of associated urinary tract anomalies. Case illustration: Two companion cases of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome with vaginal septum diagnosed by pelvic MRI were presented with illustration of characteristic MRI findings. One of them was performed with vaginal gel instillation which enabled optimal visualisation of vaginal septum compared with the other case without gel usage. Case 1: A 14-year-old female with regular follow-up in the paediatric unit for known congenital single right kidney, was noted to have primary amenorrhea, and found to have hydrocolpos on routine follow up ultrasound urinary system. Gynaecological examination revealed a palpable mass at 2cm from the introitus. Abdominal and pelvic ultrasonography revealed a 7.1cm x 4.3cm cystic structure caudal to the uterine cervix suggestive of hydrocolpos (Fig. 1a) and absent left kidney (not shown). MRI demonstrated a triad of two uterine bodies with preserved normal zonal anatomy (Fig. 1b, c), two separate cervices, and a hydrocolpos with hypointense T1, hyperintense T2 signal that is connected to the left cervix (Fig. 1b-f). The caudal end of the hydrocolpos ends above the level of the perineum (green arrows in Fig. 1d, e). A thin collapsed structure with internal hyperintense T2 signal is located to the right side of and being compressed by the hydrocolpos (Fig. 1g), suspected to be a right hemivagina. Without use of vaginal gel to achieve optimal distension, the nature of the collapsed structure, which was subsequently confirmed to be the collapsed right hemivagina, its relation with the right uterus and cervix, and the cause of the hydrocolpos were difficult to be delineated. Bilateral polycystic ovaries are present (Fig. 1b, c) without other adnexal mass detected. Absent left kidney was evident on abdominal survey (Fig. 1h). No hydronephrosis or ectopic ureter. The constellation of uterine anomalies, obstructed left hemivagina and left renal agenesis would suggest an underlying OHVIRA syndrome. Examination under anaesthesia and vaginoscopy subsequently performed confirmed obstructed left hemivagina by vaginal septum with patient right hemivagina. Vaginal septotomy and drainage of hematometrocolpos were done. She made an uneventful recovery with restoration of normal menstrual flow thereafter. Case 2: Case of a 16 year-old with OHIVRA Syndrome with pelvic MRI assessment with vaginal gel. She has a known history of single left kidney with primary complaint of dysmenorrhoea and pelvic pain since menarche at age 12. Pelvic ultrasonography showed two widely divergent uterine horns and a large 10cm x 8.4cm cystic mass with hypoechoic contents, appearing to communicate with the right uterine cavity that could represent hematometrocolpos. Right kidney was not visualised. MRI was performed with vaginal gel instillation for vaginal distension. MRI showed two widely divergent uterine corpi (Fig. 2a) with two cervices, which are connected to two vaginal canals separated by a longitudinal septum, extending to the distal right vaginal wall (Fig. 2e-f). Vaginal gel distension of the left hemivagina is demonstrated, which is patent without atretic segment. Gross right haemtometrocolpos (Fig. 2b-d) shows hyperintense T1 signal after fat suppression and T2 shading appearance compatible with subacute blood products, with no gel content present within. The entire course of the oblique vaginal septum from cervix level down to the distal right vagina at about 2 cm above the hymenal opening was well depicted, measuring about 15 cm in length with the inferior portion being thicker (5mm) compared with the superior aspect (1mm) (Fig. 2f). Homogeneous hypointense T2 signal along the septum would favour underlying fibrous contents. No communication tract across the septum between the right hematocolpos and normal left hemivagina was seen. Both ovaries are normal. Single left kidney and absent right kidney were also demonstrated (not included in images). Surgical and Pathological correlation Examination under anaesthesia confirmed MRI findings of blind-ended left hemivagina and normal left cervix, without opening in the septum seen. The longitudinal vaginal septum was excised (Fig. 3a) and drainage of hematocolpos was performed, yielding about 450ml of old blood product. Further examination revealed a healthy looking right cervix. Findings are consistent with OHVIRA syndrome (Rock and Jones classification type one.) She made a smooth post operative recovery and her dysmenorrhoea resolved on subsequent follow up. Microscopic examination of the vaginal septum revealed fibrovascular tissue with smooth muscle bundles, with focal coverage by squamous epithelium. (see microscopy photos). No evidence of dysplasia or malignancy was shown. The fibrous nature and smooth muscle contents with relatively reduced water contents account for hypointense signals of the septum on T2 weighted images. Salient reporting points Comprehensive evaluation of the spectrum of Mullerian malformation and urological anomalies is crucial in personalised treatment planning. Specific to the vaginal septum evaluation, reporting of the following features are recommended to facilitate surgical planning, enabling gynaecologists to identify the thinnest portion of vaginal septum for easier incision and achieve complete septal trimming to restore patency of the vaginal canal with reduced operative time. 1) starting point 2) description of its course (eg longitudinal/ horizontal/ oblique) 3) end point in which level of the vagina and distance from perineum 4) estimation of the septum length and anteroposterior dimension. 5) septal thickness (the thinner part can be selected as target for easier incision) 6) septal signal (suggesting fibrous/ muscular nature), presence of openings as communicating channels between bilateral hemivagina 7) associated abnormal communication between uterine cavities/ cervices. 8) Dimensions of the hematocolpos for estimation in the amount of accumulated blood products. Detailed assessment of vaginal septum configuration and fistulation tracts in the cervix enables preoperative classifications of the patients with OHVIRA syndrome into 3 subtypes as proposed by the Rock and Jones classifications [5, 6]. Type one OHVIRA syndrome has a complete vaginal obstruction with complete septum, as shown in our 2 companion cases. Type two describes incomplete vaginal obstructions with openings along the septum allowing communication between hemivagina. The opening is usually pinhole sized, enabling draining of a small amount of blood product. Type three describes a complete vaginal obstruction with laterally communicating double uterus through cervical fistulation. Early diagnosis and surgical treatment can prevent complications and preserve future fertility. In contrast, without early and proper treatment, hemivaginal obstruction can lead to complications such as tubal infection, adhesion, pelvic endometriosis or even infertility attributed to prolonged retrograde menstrual flow from the obstructed side. MRI pelvis of a 14-year-old girl showed left hematocolpos complicated with haematosalpinx (Fig. 4), which can predispose to tubal adhesion, endometriosis or even subfertility. Concomitant urogenital anomalies are commonly associated with OHVIRA syndrome. Therefore, it is important to examine the urogenital system at cross-sectional imaging (USG and MRI). While typical presentation of OHVIRA syndrome includes ipsilateral renal agenesis, a range of urological anomalies have been encountered, such as multicystic dysplastic kidneys, crossed fused renal ectopia, ectopic ureters and duplication of kidneys and ureters. Renal anomalies are usually located on the ipsilateral side as the obstructed hemivagina. The right side being affected is twice as often as the left side.
USG pelvis shows hydrocolpos (H in Fig. 1a). MRI T2W coronal (Fig. 1b), T2W axial (Fig. 1c) and T1W axial (Fi. 1d) images show two uterine bodies with preserved normal zonal anatomy (orange asterisks in Fig. 1b, c), a hydrocolpos (H) with low T1 and high T2 signals. The polycystic ovaries are shown by red arrows in Fig. 1b-c. The hydrocolpos (H) is connected to the left cervix (blue arrowheads in Fig. 1e, f). The caudal end of the hydrocolpos ends above the level of the perineum (green arrows in Fig. 1e, f). Left distended hydrocolpos (H) compressed the right hemivagina to slit-like (yellow arrow in Fig. 1g). Left renal agenesis is evident with an empty left renal fossa (blue star in Fig. 1h).
MRI T2W axial (Fig. 2a) and coronal (Fig. 2e, f) images show uterine didelphys (orange asterisks). The right uterine horn with a separate cervix (blue arrowhead in Fig. 2e) connects to a right hemivagina (H in Fig. 2e). The right hemivagina (H) is distended with subacute blood products evident by T2 shading sign (Fig. 2b) and high T1 signal after fat-suppression (Fig. 2c, d). A longitudinal vaginal septum (blue arrows in Fig. 2f) separates two vaginal canals. The lower end of the vaginal septum ends at the distal right vaginal wall (blue arrows in Fig. 2g, h) above the perineum. Left hemivagina is visualised (V in Fig. 2g, h) after vaginal gel instillation. Right hematocolpos (H) exerts mass effect on the urinary bladder (B in Fig. 2i) and left hemivagina (V in Fig. 2i). Distal vagina shows normal-looking H-shaped configuration with urethra situated anteriorly (U in Fig. 2j) and rectum posteriorly (R in Fig. 2j).
Fig. 3a shows a gross sample of the excised vaginal septum. Microscopy photos of the vaginal septum show smooth muscle cells on original and high magnification (Fig.3b, c respectively), and squamous epithelium on original and high magnification (green arrows in Fig. 3d, e respectively).
A left adnexal serpiginous cystic lesion (blue arrows in Fig. 4a, b) with high T1-weighted signal after fat-suppression are suggestive of subacute blood products, consistent with left hematosalpinx.
In this educational poster, we described the embryology and imaging features of vaginal septum. We demonstrated the benefits of vaginal gel instillation in pelvic MRI assessment of vaginal septum through illustrations of companion cases in enabling optimised visualisation of vaginal septum for detailed evaluation, exclusion of other vaginal congenital abnormalities and classification of the OHVIRA subtypes to facilitate accurate surgical planning. Early correct diagnosis and treatment reduce the risks of adverse long term sexual and reproductive outcomes. Radiologists should collaborate with gynaecologists through optimised imaging techniques and appropriate choice of treatment in achieving successful surgical outcomes, alleviating patient’s suffering and improving future sexual and reproductive well-being. Reference: 1. Tanitame K, Tanitame N, Urayama S, Ohtsu K. Congenital anomalies causing hemato/hydrocolpos: imaging findings, treatments, and outcomes. Jpn J Radiol. 2021 Aug;39(8):733-740. Epub 2021 Apr 11. 2. Ferreira DM, Bezerra RO, Ortega CD, Blasbalg R, Viana PC, de Menezes MR, Rocha Mde S. Magnetic resonance imaging of the vagina: an overview for radiologists with emphasis on clinical decision making. Radiol Bras. 2015 Jul-Aug;48(4):249-59. 3. Maciel C, Bharwani N, Kubik-Huch RA, Manganaro L, Otero-Garcia M, Nougaret S, Alt CD, Cunha TM, Forstner R. MRI of female genital tract congenital anomalies: European Society of Urogenital Radiology (ESUR) guidelines. Eur Radiol. 2020 Aug;30(8):4272-4283. Epub 2020 Mar 27. 4. Papaioannou G, Koussidis G, Michala L. Magnetic resonance imaging visualization of a vaginal septum. Fertil Steril. 2011 Nov;96(5):1193-4. Epub 2011 Sep 29. 5. Cheng C, Subedi J, Zhang A, Johnson G, Zhao X, Xu D, Guan X. Vaginoscopic Incision of Oblique Vaginal Septum in Adolescents with OHVIRA Syndrome. Sci Rep. 2019 Dec 27;9(1):20042. 6. Rock JA, Jones HW Jr. The double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis. Am J Obstet Gynecol. 1980 Oct 1;138(3):339-42.