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Hysterosalpingography in the Assessment of Congenital Cervical Anomalies

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Hysterosalpingography in the Assessment of Congenital Cervical Anomalies Pictorial Review Hysterosalpingography in The Assessment of Congenital Cervical Anomalies Fatemeh Zafarani, M.Sc., Firoozeh Ahmadi, M.D.*, Gholam Shahrzad, M.D. 'HSDUWPHQWRI5HSURGXFWLYH,PDJLQJ5HSURGXFWLYH%LRPHGLFLQH5HVHDUFK&HQWHU5R\DQ,QVWLWXWHIRU5HSURGXFWLYH %LRPHGLFLQH7HKUDQ,UDQ Abstract Cervical abnormalities may be congenital or acquired. Congenital anomalies of the cer- vix are rarely isolated, and more commonly accompany other uterine anomalies. Various imaging tools have been used in the assessment of Müllerian duct anomalies (MDAs). &XUUHQWO\PDJQHWLFUHVRQDQFHLPDJLQJ 05, LVWKHPRGDOLW\RIFKRLFHIRUGH¿QLWLYH GLDJQRVLVDQGFODVVL¿FDWLRQRIWKHVH0'$V+\VWHURVDOSLQJRJUDSK\LVDEDVLFWRROIRU evaluation of infertility and allows us to detect a spectrum of anatomical malformations of the utero-cervix in the setting of MDAs. It provides good outlines of the uterine cavity and fallopian tubes, as well as the cervical canal and isthmus. However, hysterosalpingo- grams (HSG) cannot be performed in patients with isolated congenital maldevelopment (agenesis/disgenesis) of the cervix. This part of pictorial review illustrates the various radiographic appearances of congenital malformations of the utero-cervix with a brief overview of the embryologic features. Accurate diagnosis of such cases is considered essential for optimal treatment and categorization of each anomaly. Keywords: Hysterosalpingography, Congenital, Cervix, Uterine, Anomalies Citation: Zafarani F, Ahmadi F, Shahrzad Gh. Hysterosalpingography in the assessment of congenital cervical anomalies. Int J Fertil Steril. 2017; 11(2): 71-78. doi:10.22074/ijfs.2017.4716. Introduction of the uterine cervix, a woman’s reproductive po- tential may be adversely affected. Female genital malformations include various forms of developmental and morphological mal- Several imaging tools have been used in the as- formations of the vagina, cervix, uterus, adnexa sessment of Müllerian duct anomalies (MDAs). Al- and associated malformations (VCUAM) (1). Gen- though magnetic resonance imaging (MRI) is con- ital malformations have an incidence of up to ~7% VLGHUHGWKHJROGVWDQGDUGIRUGH¿QLWLYHGLDJQRVLVDQG in the general population (2). FODVVL¿FDWLRQRIJHQLWRXULQDU\DQRPDOLHVHVSHFLDOO\ for complex cases, hysterosalpingography is still an Congenital anomalies of the cervix are rarely important tool in the early evaluation of infertility. isolated, and are more commonly associated with Contrast medium which is slowly injected into the other uterine and vaginal anomalies. Cervical uterus through the cervical canal, provides good out- anomalies may manifest as agenesis, dysgenesis, lines of the uterine cavity and fallopian tubes, as well obstruction, abnormal length, inadequate width, as the cervical canal and isthmus (6). and hypertrophy (3); however, complete or partial duplication of the cervix with a normal uterus and We retrospectively reviewed 38574 hysterosal- an unusual Müllerian anomaly have been reported pingograms (HSGs) performed over a 29-year pe- (4, 5). Cervical atresia usually presents with pri- ULRG -DQXDU\'HFHPEHU E\RQHDXWKRU mary amenorrhea and cyclic abdominal pain. De- (G.Sh.). The indications for HSG included infer- pending on the type and degree of maldevelopment tility, abnormal uterine bleeding, and symptoms Received: 22 Feb 2016, Accepted: 29 Oct 2016 *Corresponding Address: P.O.Box: 16635-148, Department of Re- productive Imaging, Reproductive Biomedicine Research Center, Royan Institute for Reproductive Biomedicine, ACECR, Tehran, Iran Royan Institute Email: [email protected] International Journal of Fertility and Sterility Vol 11, No 2, Jul-Sep 2017, Pages: 71-78 71 =DIDUDQLHWDO UHODWHGWRXWHULQH¿EURLGV7KLVUHYLHZLOOXVWUDWHV *\QDHFRORJLFDO(QGRVFRS\ (6*( FODVVL¿FDWLRQ the various radiographic appearances of congeni- V\VWHPV 0RVWRIWKHVHFODVVL¿FDWLRQV\VWHPV tal malformations of the utero-cervix with a brief seem to be associated with limitations, especially overview of the embryologic features. in the diagnosis of unusual and complex malfor- mations (12). (PEU\RORJ\ 7KH$)6FODVVL¿FDWLRQLVEDVHGPDLQO\RQWKHGH- The female genital tract develops from a pair of scription of uterine changes and most widely used Müllerian ducts between 6 and 12 weeks of ges- DVWKHPDLQFODVVL¿FDWLRQV\VWHPIRULWVVLPSOLFLW\ tation. The process involves three main stages: i. friendliness and clinical usefulness. However, this Development of both Müllerian ducts that form V\VWHP LV DVVRFLDWHG ZLWK OLPLWDWLRQV LQ HI¿FLHQW the fallopian tubes, uterus, and cervix and upper categorization of female genital anomalies. The ac- two thirds of the vagina, whereas failure of this companying malformation (such as duplex vagina), stage results in agenesis/hypoplasia or a unicornu- unusual or/and complex malformations especially ate uterus, ii. Fusion of the lower Müllerian ducts WKRVH LQ JURXS , K\SRSODVLDDJHQHVLV QRW ¿W DQG leads to formation of the uterus and cervix while DUHGHVFULEHGFRPSOHWHO\LQWKH$)6FODVVL¿FDWLRQ defects in this phase result in a bicornuate uterus, DQGVRRIWHQIDLOVWRFRUUHFWO\LGHQWL¿HGDQGWUHDWHG and iii. Canalization and septal resorption of the (12). In addition, some other cases of utero-vaginal central septum which results in a single uterine DQRPDOLHV KDYH EHHQ FODVVL¿HG LQ QLQH VXEW\SHV cavity and cervix, whereas failure of this stage of septate and bicornuate communicating uteri leads to a septate or arcuate uterus. Mesonephric schemes, as suggested by Toaff et al. (13). RU:ROI¿DQGXFWVSOD\DUROHDVLQGXFWRUVIRUDG- equate development, fusion, and resorption of the 7KHXVHRIHPEU\RORJLFDOFOLQLFDOFODVVL¿FDWLRQ walls of Müllerian ducts (7). There is controversy of genitor-urinary malformations seems to unify over formation of the vagina. Recent studies have the current embryological and pathogenic con- stated that the mesonephric ducts together with the cepts and appear to be the most clinically useful Müllerian tubercle form the vagina (7, 8). %DVHGRQWKH$)6FODVVL¿FDWLRQVFKHPHWKH FHUYL[LVFODVVL¿HGDVIROORZV,% XQGHUVHJPHQWDO %\ZHHNWKHSURFHVVRIGHYHORSPHQWLVFRP- Müllerian duct agenesis or hypoplasia), III (com- pleted. Development of both Müllerian ducts and plete non-fusion of MDAs), IV (incomplete fusion the urinary tract occurs from a common ridge of of the superior segments of the uterovaginal ca- the mesoderm; hence, anomalies of the urinary nal), V (partial or complete non-resorption of the tract are commonly observed in females with geni- uterovaginal septum), and VII [MDAs related to tal malformation. diethylstilbestrol (DES) exposure in utero]. &ODVVL¿FDWLRQV\VWHP The accurate diagnosis of such cases is consid- ered essential for optimal treatment and in support $FFXUDWHFODVVL¿FDWLRQRIDIHPDOHJHQLWDOWUDFW of the embryologic concept. malformation is necessary to prevent inadequate surgery and achieve optimal treatment. Until now, &HUYLFDODJHQHVLVG\VJHQHVLV VHYHUDO FODVVL¿FDWLRQ V\VWHPV IRU IHPDOH JHQLWDO tract anomalies have been proposed: the American Agenesis/dysgenesis of the cervix is rare and )HUWLOLW\ 6RFLHW\ &ODVVL¿FDWLRQ 6\VWHP FXUUHQWO\ usually occurs in association with complete or par- American Society for Reproductive Medicine, WLDOYDJLQDODJHQHVLV ,WLVGLI¿FXOWWRGLDJQRVH AFS/ASRM) (9); the embryological-clinical clas- cervical agenesis. Clinical examination has limited VL¿FDWLRQV\VWHPRIJHQLWRXULQDU\PDOIRUPDWLRQV diagnostic value and in most cases hysterosalpin- SURSRVHGE\$FLpQDQG$FLpQ WKHYDJLQD gography is impossible. cervix, uterus, adnexae and associated Malforma- tions system based on the tumor node metastasis 8WHURFHUYLFDODQRPDOLHV (TNM) principle in oncology (1), and the new European Society of Human Reproduction and 8WHUXVGLGHOSK\V Embryology (ESHRE) and European Society for Uterus didelphys results from complete fail- Int J Fertil Steril, Vol 11, No 2, Jul- Sep 2017 72 +6*RI&RQJHQLWDO&HUYLFDO$QRPDOLHV ure of Müllerian duct fusion and accounts for $ approximately 5% of MDAs (2). On hystero- salpingography, two symmetric separate cavi- ties and two cervical canals are present; a dou- ble vagina is often present (Fig.1). Didelphys uterus is usually asymptomatic, while cases with unilateral vaginal obstruction may mani- fest with hematometrocolpos and dysmenor- rhea at menarche. % Fig.1:ŝĚĞůƉŚLJƐƵƚĞƌƵƐŝŶĂϯϭLJĞĂƌͲŽůĚͲǁŽŵĂŶǁŝƚŚϯLJĞĂƌƐŽĨ ƉƌŝŵĂƌLJŝŶĨĞƌƟůŝƚLJ͘,LJƐƚĞƌŽƐĂůƉŝŶŐŽŐƌĂŵĚĞŵŽŶƐƚƌĂƚĞƐƚǁŽƐLJŵ- ŵĞƚƌŝĐĂůƐĞƉĂƌĂƚĞĐĂǀŝƟĞƐ͕ƚǁŽĐĞƌǀŝĐĂůĐĂŶĂůƐĂŶĚƉƌĞƐĞŶĐĞŽĨ ĚŽƵďůĞǀĂŐŝŶĂ͘ & %LFRUQXDWHXWHUXV The bicornuate uterus represents approxi- mately 25% of MDAs and results from incom- plete fusion of the Müllerian ducts at the level of the uterine fundus (2). The two separate uterine cavities are fused caudally and com- municate in the lower segment, mostly at the uterine isthmus; a single cervix and vagina are present. Hysterosalpingography demonstrates separate fusiform uterine horns, often with DQ LQWHUFRUQXDO DQJOH RI ! 7KHUH are various degrees of separation between the two horns, as follows: a complete bicornuate uterus, in which the failure to fuse extends the length of the uterine body inferiorly to the in- Fig.2:ŝĐŽƌŶƵĂƚĞƵƚĞƌƵƐǁŝƚŚǀĂƌŝŽƵƐĚĞŐƌĞĞƐŽĨĚƵƉůŝĐĂƟŽŶŽĨ ƚŚĞĐĞƌǀŝdžŝŶĚŝīĞƌĞŶƚƉĂƟĞŶƚƐ;&^ĐůĂƐƐ/sͿ͘A.ŝĐŽƌŶƵĂƚĞƵƚĞƌ- ternal os; and lesser degrees of a bicornuate ƵƐ͕ĐŽŶƐŝƐƟŶŐŽĨƚǁŽƐLJŵŵĞƚƌŝĐƵƚĞƌŝŶĞĐĂǀŝƟĞƐǁŝƚŚĐŽŵŵƵŶŝ- uterus, in which the partial interfering cleft is ĐĂƟŽŶĂƚƚŚĞƵƚĞƌŝŶĞŝƐƚŚŵƵƐ͕ĂŶĚĂůƐŽŝŶƚĞƌĐŽƌŶĞĂůĂŶŐůĞŐƌĞĂƚĞƌ ƚŚĂŶϭϬϱ͘^ŝŶŐůĞĐĞƌǀŝdžĂŶĚǀĂŐŝŶĂĂƌĞƉƌĞƐĞŶƚ͕B. /ŶƚĞƌǀĞŶŝŶŐ variable in length, extending from the fundus ĐůĞŌĞdžƚĞŶĚƐƚŽƚŚĞĞŶĚŽĐĞƌǀŝĐĂůĐĂŶĂů͕ĂŶĚC.džƚĞŶƐŝŽŶƚŽƚŚĞ to the cervix (Fig.2). ůĞǀĞůŽĨĞdžƚĞƌŶĂůŽƐ͘
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