According to published reports, the prevalence of utero-vaginal aplasia ranges from 0.025% to 0.01%. The frequency, according to the most recent epidemiological study, is 1/5,000 female births [4]. 85% of vaginal aplasias are caused by MRKH syndrome, which is also the second most common cause of primary amenorrhea after gonadal dysgenesis [5,6].

Inquiries show a personal or familial history of abnormalities, particularly skeletal and renal [7]. A hymenal flange may make the vulva appear normal from the outside in babies and young girls. A vaginal cup that is blind can only be seen by catheterization using a tiny, flexible probe.

According to the pubertal evaluation, the adolescent is developing normally for their age.

With the patient's consent, a vulva examination verifies that the external genitalia appear normally and that there is a basic vaginal cup, the depth and depressibility of which can vary from nearly zero to a few centimeters. The cup's depressibility and depth can vary, ranging from nearly nothing to a few millimeters. According to Creatsas et al., 78.5% of 200 patients had a vaginal cup that measured between 3 and 20 mm. Complete vaginal aplasia without any cup depth might occasionally occur. The urethra may appear circular and slightly gaping, and it may be more posterior than usual, especially if there is no vaginal cup (19% of instances in the series by Khen-Dunlop et al.) [8]. The rectal examination, rarely performed, shows the absence of a median uterine structure. Clinical examination may reveal skeletal anomalies, inguinal hernia, hypoacusis, facial asymmetry, and extremity abnormalities [9].

In our case, the clinical indicators were primary amenorrhea with cyclical pelvic pain; a clinical examination showed normal external female genitalia and a well-developed look; however, there is no need for a clinical examination under general anesthesia. An objective analysis and accurate anatomical assessment of this syndrome are provided by MRI, the high-performance imaging tool of choice for diagnosis that is crucial for any surgical management.

Age- and pubertal-development-appropriate ovarian function is confirmed by measurements of FSH, LH, estradiol, androgens (delta-4 androstenedione and testosterone), and 17-OHP.

An increased FSH can be utilized to rule out gonadal dysgenesis or to draw attention to the uncommon occurrences of ovarian failure linked to MRKH syndrome.

Tests for testosterone also aid in ruling out total androgen insensitivity. Other abnormalities are not always routinely looked for in a series of MRKH patients, with the exception of urinary tract abnormalities. Clinical evaluation served as the starting basis for the search for related abnormalities.

The blood karyotype, 46 XX, is normal; no anomalies are apparent. Particularly prior to puberty, when the hormonal work-up is ineffective in differentially diagnosing androgen insensitivity syndrome, it removes MRMKH syndrome.

Parsons et al. [10] reported two transitory subocclusions, four mucosal prolapses, four introital stenosis, and perineal hematomas in a group of 28 patients with an average follow-up of 6.2 years. None of the patients thought these issues affected their quality of life.

A functioning vagina without significant postoperative stress is achieved after sigmoidal colpoplasty in 4–6 weeks. There aren't many published studies on sigmoidal colpoplasty's problems and functional outcomes [11]. The neovagina was 11.5 cm long on average and did not shorten over time. The neocavity does not gradually narrow, and some have even argued against autodilation altogether. The neovagina is adequately lubricated because the colon secretes very little, in contrast to ileal transplant procedures. Aside from the occlusion that comes with any laparotomy, the two main hazards are stenosis of the sigmoido-vestibular orifice, which can be avoided by autodilatation, and peritonitis through an anastomotic fistula [12]. There is a chance that any rectovesical cleavage, an operation that is a component of any procedure for uterovaginal aplasia [13].

Regardless of the method used, including vaginal sigmoidoplasty, the patient's participation and comprehension are essential and call for a level of maturity that can only be attained with thorough medical information and a patient who is very driven [14-16].