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.

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Abstract Cervical abnormalities may be congenital or acquired. Congenital anomalies of the cervix 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 potential 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 malformations (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 considered 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 hysterosalpingography, two symmetric separate cavities and two cervical canals are present; a double vagina is often present (Fig.1). Didelphys uterus is usually asymptomatic, while cases with unilateral vaginal obstruction may manifest with hematometrocolpos and dysmenor- rhea at menarche.

Fig.1:ŝĚĞůƉŚǇƐƵƚĞƌƵƐŝŶĂϯϭǇĞĂƌͲŽůĚͲǁŽŵĂŶǁŝƚŚϯǇĞĂƌƐŽĨ ƉƌŝŵĂƌǇŝŶĨĞƌƟůŝƚǇ͘,ǇƐƚĞƌŽƐĂůƉŝŶŐŽŐƌĂŵĚĞŵŽŶƐƚƌĂƚĞƐƚǁŽƐǇŵ- ŵĞƚƌŝĐĂůƐĞƉĂƌĂƚĞĐĂǀŝƟĞƐ͕ƚǁŽĐĞƌǀŝĐĂůĐĂŶĂůƐĂŶĚƉƌĞƐĞŶĐĞŽĨ ĚŽƵďůĞǀĂŐŝŶĂ͘ & %LFRUQXDWHXWHUXV The bicornuate uterus represents approximately 25% of MDAs and results from incomplete fusion of the Müllerian ducts at the level of the uterine fundus (2). The two separate uterine cavities are fused caudally and communicate 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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6HSWDWHXWHUXV & A septate uterus results from partial or complete failure of resorption of the uterovaginal septum after fusion of the paramesonephric ducts, which occurs in approximately 35% of MDAs (2). Hysterosalpingography of a septate uterus represents varying degrees of the midline septum, extending from the fundus to the cervix and upper vagina, and yielding a V-shaped FRQ¿JXUDWLRQRIWHQZLWKDQDQJOH EHWZHHQ the two uterine horns (Fig.3). In 25% of cases, complete extension of the septum to the upper vagina is present (Fig.3C) (14).

Fig.3: ,ǇƐƚĞƌŽƐĂůƉŝŶŐŽŐƌĂƉŚǇŽĨƐĞƉƚĂƚĞƵƚĞƌƵƐĚĞŵŽŶƐƚƌĂƚĞƐĂ ǀĂƌŝĂďůĞĚĞŐƌĞĞƐŽĨĐĞƌǀŝĐĂůƐĞƉƚĂƟŽŶŝŶĚŝīĞƌĞŶƚƉĂƟĞŶƚƐ;&^ ĐůĂƐƐsͿ;ϭϮͿ͘A.ǆƚĞŶƐŝŽŶŽĨƚŚĞŵŝĚůŝŶĞƐĞƉƚƵŵƚŽƚŚĞůŽǁĞƌ ŝŶƚĞƌŶĂůŽƐ͕B.^ĞƉƚĂƚĞƵƚĞƌƵƐǁŝƚŚĞǆƚĞŶƐŝŽŶŽĨƐĞƉƚƵŵƚŽƚŚĞ ůĞǀĞůŽĨĞǆƚĞƌŶĂůŽƐĂŶĚƉƌŽĚƵĐƟŽŶŽĨƚǁŽĐĞƌǀŝĐĂůĐĂŶĂůǁŝƚŚ ŽŶĞŽƉĞŶŝŶŐ͕ĂŶĚC.ŽŵƉůĞƚĞƐĞƉƚĂƚĞƵƚĞƌƵƐǁŝƚŚƚǁŽƐĞƉĂƌĂƚĞ ĐĞƌǀŝĐĂůĐĂŶĂů;ƉƐĞƵĚŽĚŝĚĞůƉŚǇƐͿ͘

'LHWK\OVWLOEHVWUROH[SRVHGXWHUXV Exposure to DES in utero results in multiple, benign abnormalities of the genital tract and clear cell adenocarcinoma of the vagina. DES has been associated with T-shaped and irregular configurations of the endometrial cavity, con- strictive bands, structural cervical changes, and % cervical anomalies that include hypoplasia, cervical ridges, and cervical collars (15). Hysterosalpingography is an excellent screening tool to diagnose DES-related uterine anomalies. The radiographic appearance includes an irregular, narrowed endocervical canal with a shortened upper uterine segment and small, typically irregular cavity WKDW\LHOGVD7VKDSHGXWHULQHFRQ¿JXUDWLRQ )LJ

&RPPXQLFDWLQJXWHUXV ,Q7RDIIHWDO SURSRVHGDFODVVL¿FDWLRQ scheme for nine subtypes of septate and bicornuate XWHULWKDWLGHQWL¿HGWKHSUHVHQFHRIDFRPPXQLFDWLRQ between two separate uterocervical cavities. All types of communicating uteri have an isthmic communication, except for type 9, which has a low cervical communication.

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Fig.4: ^ͲĞǆƉŽƐƵƌĞƵƚĞƌƵƐŝŶĂϮϴͲǇĞĂƌͲŽůĚŝŶĨĞƌƟůĞƉĂƟĞŶƚ͘EŽƚĞ dͲƐŚĂƉĞĚĂƉƉĞĂƌĂŶĐĞŽĨƚŚĞĞŶĚŽŵĞƚƌŝĂůĐĂǀŝƚǇǁŝƚŚĂůŽŶŐŶĂƌ- ƌŽǁĞĚŝƌƌĞŐƵůĂƌĞŶĚŽĐĞƌǀŝĐĂůĐĂŶĂů͘ During >29 years of one author’s experience (G.SH) in performing HSGs, some rare cases of communicating uteri have been observed and reported (Fig.5). Of & these, three represented communications at unusual VLWHV7KH\ZHUHLGHQWL¿HGDVQRQLVWKPLFFRPPXQL- FDWLQJXWHULDQGFODVVL¿HGDVDQHZVXEFODVVRIW\SH 7KH¿UVWFDVHZDVDFRPSOHWHVHSWDWHXWHUXVZLWK a mid-corporeal communication, in which the septum ended inferiorly several millimeters above the external os (Fig.6). Another case was a bicornuate uterus with two sites of communication, at the mid-cervical and isthmic levels (Fig.7), and a third case with communication that involved the bicornuate uterus with a low cervical communication, left cervico-vaginal atresia, and left renal agenesis (Fig.8).

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Fig.6: ,ǇƐƚĞƌŽƐĂůƉŝŶŐŽŐƌĂƉŚǇŝŶĂϮϯǇĞĂƌͲŽůĚͲǁŽŵĂŶǁŝƚŚϱǇĞĂƌƐ E ŽĨƉƌŝŵĂƌǇŝŶĨĞƌƟůŝƚǇ͘dŚĞƵƚĞƌƵƐŝƐƐĞƉƚĂƚĞǁŝƚŚĂŵŝĚĐŽƌƉŽƌĞĂů ĐŽŵŵƵŶŝĐĂƟŽŶ;ƐƚƌĂŝŐŚƚĂƌƌŽǁͿ͘dŚĞĐĞƌǀŝǆŝƐƉĂƌƟĂůůǇƐĞƉƚĂƚĞĂŶĚ ƚŚĞĐĞƌǀŝĐĂůƐĞƉƚƵŵĞŶĚƐĂĨĞǁŵŝůůŝŵĞƚĞƌƐĂďŽǀĞƚŚĞƐŝŶŐůĞĞǆƚĞƌ- ŶĂůŽƐ;ŽƉĞŶĂƌƌŽǁͿ;ϭϲͿ͘

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Fig.7: ŝĐŽƌŶƵĂƚĞƵƚĞƌƵƐŝŶĂϮϲǇĞĂƌͲŽůĚͲǁŽŵĂŶǁŝƚŚϯǇĞĂƌƐŽĨ ƉƌŝŵĂƌǇ ŝŶĨĞƌƟůŝƚǇ͘ ,ǇƐƚĞƌŽƐĂůƉŝŶŐŽŐƌĂŵ ƐŚŽǁƐ ƚŚĞ ďŝĐŽƌŶƵĂƚĞ ƵƚĞƌƵƐǁŝƚŚƚǁŽƐŝƚĞƐŽĨĐŽŵŵƵŶŝĐĂƟŽŶďĞƚǁĞĞŶƚǁŽƵƚĞƌŽĐĞƌ- ǀŝĐĂů ĐĂŶĂů͕ ŵŝĚĐĞƌǀŝĐĂů ;ĐƵƌǀĞĚ ĂƌƌŽǁͿ ĂŶĚ ŝƐƚŚŵŝĐ ;ĂƐƚĞƌŝƐŬͿ͖ ƐĞƉƚƵŵŽĨƚŚĞĐĞƌǀŝǆĞŶĚƐƐĞǀĞƌĂůŵŝůůŝŵĞƚĞƌƐĂďŽǀĞƚŚĞƐŝŶŐůĞ ĞǆƚĞƌŶĂůŽƐ;ƐƚƌĂŝŐŚƚĂƌƌŽǁͿ;ϭϲͿ͘

Fig.5: ^ŽŵĞĐĂƐĞƐŽĨĐŽŵŵƵŶŝĐĂƟŶŐƵƚĞƌŝǁŝƚŚĚƵƉůŝĐĂƟŽŶŽĨĐĂƌ- ǀŝĐĂů ĐĂŶĂů ;ϭϮͿ͘ A. hƚĞƌƵƐ ĐŽŵŵƵŶŝĐĂŶƚƐ ƐĞƉƚƵƐ͕ ĐĞƌǀŝǆ ĚƵƉůĞǆ͕ ǀĂŐŝŶĂ ƐĞƉƚĂ ;ƚǇƉĞ ϭĂͿ͘ sĂŐŝŶĂů ƐĞƉƚƵŵ ǁĂƐ ŽďƐĞƌǀĞĚ ŝŶ ǀĂŐŝŶĂů ĞǆĂŵŝŶĂƟŽŶ͕B.hƚĞƌƵƐĐŽŵŵƵŶŝĐĂŶƚƐƐĞƉƚƵƐ͕ĐĞƌǀŝǆĚƵƉůĞǆ͕ǀĂ- ŐŝŶĂƐĞƉƚĂƵŶŝůĂƚĞƌĂůŝƐĂƚƌĞƟĐĂ;ƚǇƉĞϮĂͿ͕ C. hƚĞƌƵƐĐŽŵŵƵŶŝĐĂŶƚƐ ƐĞƉƚƵƐ͕ ĐĞƌǀŝǆ ƐĞƉƚĂ͕ ǀĂŐŝŶĂ ƐĞƉƚĂ ;ƚǇƉĞ ϯĂͿ͘sĂŐŝŶĂů ƐĞƉƚƵŵ ǁĂƐ ǀŝƐƵĂůŝǌĞĚŝŶǀĂŐŝŶĂůĞǆĂŵŝŶĂƟŽŶ͕D.hƚĞƌƵƐĐŽŵƵŶŝĐĂŶƚƐďŝĐŽƌŶŝƐ͕ ĐĞƌǀŝǆ ĚƵƉůĞǆ͕ ǀĂŐŝŶĂ ƐĞƉƚĂ ;ƚǇƉĞ ϰĂͿ͘ sĂŐŝŶĂů ƐĞƉƚƵŵ ǁĂƐ ƐĞĞŶ ŝŶǀĂŐŝŶĂůĞǆĂŵŝŶĂƟŽŶ͕ E. hƚĞƌƵƐĐŽŵŵƵŶŝĐĂŶƚƐďŝĐŽƌŶŝƐ͕ĐĞƌǀŝǆ ĚƵƉůĞǆ͕ǀĂŐŝŶĂƐĞƉƚĂƵŶŝůĂƚĞƌĂůŝƐĂƚƌĞƟĐĂ;ƚǇƉĞϱĂͿ͕ĂŶĚ F.hƚĞƌƵƐ ĐŽŵŵƵŶŝĐĂŶƚƐďŝĐŽƌŶŝƐ͕ĐĞƌǀŝǆƐĞƉƚĂ͕ǀĂŐŝŶĂƐŝŵƉůĞǆ;ƚǇƉĞϲͿ͘

Fig.8: ,ǇƐƚĞƌŽƐĂůƉŝŶŐŽŐƌĂƉŚǇ ŝŶ Ă ϮϳͲǇĞĂƌͲŽůĚ ǁŽŵĂŶ ǁŝƚŚ ƉƌŝŵĂƌǇ ŝŶĨĞƌƟůŝƚǇŽĨϱǇĞĂƌƐ͘dŚĞƵƚĞƌƵƐŝƐďŝĐŽƌŶƵĂƚĞǁŝƚŚĂůŽǁĐĞƌǀŝĐĂůĐŽŵ- ŵƵŶŝĐĂƟŽŶĂŶĚůĞŌĂƚƌĞƟĐŚĞŵŝĐĞƌǀŝǆ͘dŚĞĐĂŶŶƵůĂŝƐůŽĐĂƚĞĚŝŶƚŚĞ ƌŝŐŚƚŚĞŵŝĐĞƌǀŝǆ͘dŚĞƌŝŐŚƚĞǆƚĞƌŶĂůŽƐŝƐǀŝƐŝďůĞ͘/ŶũĞĐƟŽŶŽĨĐŽŶƚƌĂƐƚ ŝŶƚŽƚŚĞƌŝŐŚƚŚĞŵŝĐĞƌǀŝǆǁĂƐŶŽƚĞĚƚŽŽƉĂĐŝĨǇƚŚĞůĞŌŚĞŵŝƵƚĞƌƵƐĂŶĚ ĐĞƌǀŝǆƚŚƌŽƵŐŚƚŚĞůŽǁĐĞƌǀŝĐĂůĐŽŵŵƵŶŝĐĂƟŽŶ;ĂƌƌŽǁͿ;ϭϲͿ͘

8QXVXDOFHUYLFDODQRPDO\ 6RPHXQFODVVL¿HGFDVHVRIQRUPDORUVHSWDWH uteri that communicate with double cervices with or without vaginal septa have been previ- ously described (4, 17-19). We encountered a case of normal uterus with septate cervix and vagina (Fig.9).

Int J Fertil Steril, Vol 11, No 2, Jul- Sep 2017 76 +6*RI&RQJHQLWDO&HUYLFDO$QRPDOLHV

(Fig.11). The patient had no history of any previ- ous vaginal or cervical septum resection.

Fig.9: ,ǇƐƚĞƌŽƐĂůƉŝŶŐŽŐƌĂŵŽĨĂϯϲǇĞĂƌͲŽůĚǁŽŵĂŶƐŚŽǁƐĂŶŶŽƌ- ŵĂůƵƚĞƌƵƐǁŝƚŚƐĞƉƚĂƚĞĐĞƌǀŝǆĂŶĚǀĂŐŝŶĂ͘

In the second case, the proximal part of the cervi- Fig.11: ϮϵͲǇĞĂƌͲŽůĚǁŽŵĂŶǁŝƚŚĂŚŝƐƚŽƌǇŽĨϱǇĞĂƌƐŽĨƉƌŝŵĂƌǇ cal canal was double, but the distal portion was sin- ŝŶĨĞƌƟůŝƚǇ͘,^'ƌĞƉƌĞƐĞŶƚƐĂďŝĐŽƌŶƵĂƚĞƵƚĞƌƵƐǁŝƚŚĐŽŵŵƵŶŝĐĂ- JOHDQGRQHFHUYLFDORSHQLQJZDVSUHVHQW )LJ ƟŽŶĂƚƚŚĞůĞǀĞůŽĨŝƐƚŚŵƵƐ͕ĂƐĞƉƚĂƚĞĐĞƌǀŝǆ͕ĂŶĚŶŽƌŵĂůĐĞƌǀŝĐĂů ŽƐĂŶĚǀĂŐŝŶĂ͘dŚĞƉĂƟĞŶƚŚĂƐŶŽŚŝƐƚŽƌǇŽĨƉƌĞǀŝŽƵƐǀĂŐŝŶĂůĂŶĚ ĐĞƌǀŝĐĂůƐĞƉƚƵŵƌĞƐĞĐƟŽŶ͘

In the last case, HSG demonstrated a left blind hemivagina (atretica) with left renal agenesis (Herlyn-Werner- Wunderlich syndrome, Fig.12).

Fig.10: /ŶĨĞƌƟůŝƚǇŽĨĂϮϳǇĞĂƌͲŽůĚǁŽŵĂŶŝŶǀĞƐƟŐĂƚĞĚ͘,^'ĚĞŵ- ŽŶƐƚƌĂƚĞƐŝŶǀĂŐŝŶĂƟŽŶŽĨĚŝƐƚĂůƉĂƌƚŽĨĐĞƌǀŝĐĂůĐĂŶĂůǁŝƚŚŝŶƉƌŽǆŝ- ŵĂůƉĂƌƚŽĨĐĞƌǀŝǆ͘/ŶƚŚŝƐƉĂƟĞŶƚƉƌŽǆŝŵĂůƉĂƌƚŽĨĐĞƌǀŝĐĂůĐĂŶĂů ŝƐĚŽƵďůĞďƵƚƚŚĞĚŝƐƚĂůƉŽƌƟŽŶŝƐƐŝŶŐůĞĂŶĚƚŚĞƉĂƟĞŶƚŚĂƐŽŶĞ ĐĞƌǀŝĐĂůŽƉĞŶŝŶŐ͘

The third case was a bicornuate uterus with Fig.12: ϮϰͲǇĞĂƌͲŽůĚǁŽŵĂŶǁŝƚŚĂŚŝƐƚŽƌǇŽĨϯǇĞĂƌƐŝŶĨĞƌƟůŝƚǇ͘ communication at the isthmic level, a septate cer- ,^'ƐŚŽǁƐĂƐĞƉƚĂƚĞƵƚĞƌƵƐǁŝƚŚĂůĞŌďůŝŶĚŚĞŵŝǀĂŐŝŶĂ;ĂƚƌĞƟĐĂͿ vix, and normal external cervical os and vagina ĂŶĚůĞŌƌĞŶĂůĂŐĞŶĞƐŝƐ;,ĞƌůǇŶͲtĞƌŶĞƌͲtƵŶĚĞƌůŝĐŚƐǇŶĚƌŽŵĞͿ͘

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Conclusion SK\LQWKHGHWHFWLRQRILQWUDXWHULQHDEQRUPDOLWLHVDFRP- SDULVRQZLWKK\VWHURVFRS\$-5$P-5RHQWJHQRO Although congenital anomalies of the utero- 7. $FLpQ3(PEU\RORJLFDOREVHUYDWLRQVRQWKHIHPDOHJHQLWDO cervix in the setting of MDAs are rare, the im- WUDFW+XP5HSURG pact on a woman’s reproductive potential can be 8. $FLpQ3$FLpQ0,7KHKLVWRU\RIIHPDOHJHQLWDOWUDFWPDO- VLJQL¿FDQW$QRPDOLHVRIWKHFHUYLFRXWHUXVDUH IRUPDWLRQFODVVL¿FDWLRQVDQGSURSRVDORIDQXSGDWHGV\V- WHP+XP5HSURG8SGDWH widely diagnosed by HSG. The diagnostic value 9. 7KH $PHULFDQ )HUWLOLW\ 6RFLHW\ FODVVL¿FDWLRQ RI DGQH[DO of HSG in the detection of anomalies varies, de- adhesions, distal tubal occlusion, tubal occlusion second- pending on the type of malformation. Accurate DU\WRWXEDOOLJDWLRQWXEDOSUHJQDQFLHVPOOHULDQDQRPD- OLHVDQGLQWUDXWHULQHDGKHVLRQV)HUWLO6WHULO diagnosis of these cases, especially the cases 944-955. ZLWKDQ\FODVVL¿FDWLRQV\VWHPLVLPSRUWDQWIRU 10. $FLpQ3$FLpQ07KHSUHVHQWDWLRQDQGPDQDJHPHQWRI optimal treatment and categorization of each FRPSOH[IHPDOHJHQLWDOPDOIRUPDWLRQV+XP5HSURG8S- GDWH anomaly. 11. *ULPEL]LV*)*RUGWV6'L6SLH]LR6DUGR$%UXFNHU6'H $QJHOLV&*HUJROHW0HWDO7KH(6+5((6*(FRQVHQ- Acknowledgements VXVRQWKHFODVVL¿FDWLRQRIIHPDOHJHQLWDOWUDFWFRQJHQLWDO DQRPDOLHV*\QHFRO6XUJ 7KHUH LV QR ¿QDQFLDO VXSSRUW DQG FRQÀLFW RI 12. $FLpQ3$FLpQ06iQFKH])HUUHU0&RPSOH[PDOIRUPD- WLRQVRIWKHIHPDOHJHQLWDOWUDFW1HZW\SHVDQGUHYLVLRQ interest in this article. RIFODVVL¿FDWLRQ+XP5HSURG 13. 7RDII0(/HY7RDII$67RDII5&RPPXQLFDWLQJXWHULUH- References YLHZDQGFODVVL¿FDWLRQZLWKLQWURGXFWLRQRIWZRSUHYLRXVO\ 1. 2SSHOW35HQQHU63%UXFNHU66WULVVHO3/6WULFN52S- XQUHSRUWHGW\SHV)HUWLO6WHULO SHOW3*HWDO7KH9&8$0 9DJLQD&HUYL[8WHUXV$GQH[ 14. 7URLDQR 51 0F&DUWK\ 60 0XOOHULDQ GXFW DQRPDOLHV DVVRFLDWHG0DOIRUPDWLRQ FODVVL¿FDWLRQDQHZFODVVL¿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¿FDWLRQ)HUWLO6WHULO H SURG 18. 'XQQ5+DQWHV-'RXEOHFHUYL[DQGYDJLQDZLWKDQRUPDO 5. 2NHNH&$QHOH72Q\HMHODP&%LFHUYLFDOQRUPDOXWHUXV XWHUXVDQGEOLQGFHUYLFDOSRXFKDUDUHPXOOHULDQDQRPDO\ ZLWK QRUPDO YDJLQD $QQ 0HG +HDOWK 6FL 5HV )HUWLO6WHULO 6XSSO6 19. 7DYDVVROL)8QXVXDOXWHULQHPDOIRUPDWLRQ'RXEOHFHUYL[ 6. 5RPD'DOIy$8EHGD%8EHGD$0RQ]yQ05RWJHU5 ZLWK VLQJOH FRUSXV DQG VLQJOH YDJLQD 2EVWHW *\QHFRO 5DPRV5HWDO'LDJQRVWLFYDOXHRIK\VWHURVDOSLQJRJUD-

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