Hysterosalpingography in The Assessment of Congenital Cervical Anomalies

The female genital tract develops from a pair of Müllerian ducts between 6 and 12 weeks of gestation. The process involves three main stages: i. Development of both Müllerian ducts that form the fallopian tubes, uterus, and cervix and upper two thirds of the vagina, whereas failure of this stage results in agenesis/hypoplasia or a unicornuate uterus, ii. Fusion of the lower Müllerian ducts leads to formation of the uterus and cervix while defects in this phase result in a bicornuate uterus, and iii. Canalization and septal resorption of the central septum which results in a single uterine cavity and cervix, whereas failure of this stage leads to a septate or arcuate uterus. Mesonephric or Wolffian ducts play a role as inductors for adequate development, fusion, and resorption of the walls of Müllerian ducts (7). There is controversy over formation of the vagina. Recent studies have stated that the mesonephric ducts together with the Müllerian tubercle form the vagina (7, 8).

By week 20, the process of development is completed. Development of both Müllerian ducts and the urinary tract occurs from a common ridge of the mesoderm; hence, anomalies of the urinary tract are commonly observed in females with genital malformation.

Accurate classification of a female genital tract malformation is necessary to prevent inadequate surgery and achieve optimal treatment. Until now, several classification systems for female genital tract anomalies have been proposed: the American Fertility Society Classification System (currently American Society for Reproductive Medicine, AFS/ASRM) (9); the embryological-clinical classification system of genito-urinary malformations proposed by Acién and Acién (8, 10), the vagina, cervix, uterus, adnexae and associated Malformations system based on the tumor node metastasis (TNM) principle in oncology (1), and the new European Society of Human Reproduction and Embryology (ESHRE) and European Society for Gynaecological Endoscopy (ESGE) classification systems (11). Most of these classification systems seem to be associated with limitations, especially in the diagnosis of unusual and complex malformations (12).

The AFS classification is based mainly on the description of uterine changes and most widely used as the main classification system for its simplicity, friendliness and clinical usefulness. However, this system is associated with limitations in efficient categorization of female genital anomalies. The accompanying malformation (such as duplex vagina), unusual or/and complex malformations especially those in group I (hypoplasia/agenesis) not fit and are described completely in the AFS classification and so often fails to correctly identified and treated (12). In addition, some other cases of utero-vaginal anomalies have been classified in nine subtypes of septate and bicornuate communicating uteri schemes, as suggested by Toaff et al. (13).

The use of embryological-clinical classification of genitor-urinary malformations seems to unify the current embryological and pathogenic concepts and appear to be the most clinically useful (10). Based on the AFS classification scheme, the cervix is classified as follows: IB (under segmental Müllerian duct agenesis or hypoplasia), III (complete non-fusion of MDAs), IV (incomplete fusion of the superior segments of the uterovaginal canal), V (partial or complete non-resorption of the uterovaginal septum), and VII [MDAs related to diethylstilbestrol (DES) exposure in utero].

The accurate diagnosis of such cases is considered essential for optimal treatment and in support of the embryologic concept.

Agenesis/dysgenesis of the cervix is rare and usually occurs in association with complete or partial vaginal agenesis (2). It is difficult to diagnose cervical agenesis. Clinical examination has limited diagnostic value and in most cases hysterosalpingography is impossible.

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, constrictive 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 that yields a T-shaped uterine configuration (Fig .4).

In 1984, Toaff et al. (13) proposed a classification scheme for nine subtypes of septate and bicornuate uteri that identified the presence of a communication between two separate uterocervical cavities. All types of communicating uteri have an isthmic communication, except for type 9, which has a low cervical communication.

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 sites. They were identified as non-isthmic communicating uteri and classified as a new subclass of type 9 (16). The first case was a complete septate uterus with 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).

Some unclassified cases of normal or septate uteri that communicate with double cervices with or without vaginal septa have been previously described (4, 17-19). We encountered a case of normal uterus with septate cervix and vagina (Fig .9).

In the second case, the proximal part of the cervical canal was double, but the distal portion was single and one cervical opening was present (Fig .10).

The third case was a bicornuate uterus with communication at the isthmic level, a septate cervix, and normal external cervical os and vagina (Fig .11). The patient had no history of any previous vaginal or cervical septum resection.

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