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Certain rare congenital defects can occur during the prenatal development of the female reproductive system, resulting in an abnormal or absent vagina.1 It is a part of the female genital tract. It is muscular and an elastic organ with a soft epithelial lining making it susceptible to disruption during crucial times of organogenesis.1,2 There are different malformations that occur in the female genital organ, many of which are classified under the broader term “Müllerian duct anomalies”. 1,2 Müllerian agenesis is also referred to as congenital absence of the uterus and vagina (CAUV), müllerian aplasia (MA), genital renal ear syndrome (GRES), and Mayer-Rokitansky-KüsterHauser syndrome (MRKH). It has been considered as a sporadic anomaly, but the increase in familial cases now supports the hypothesis of a genetic cause characterised by a failure of the Müllerian ducts to develop, resulting in a missing uterus and variable malformations of the vagina.1-7 According to scientific data, its incidence is reported to be 1 in 4,000 to 10,000 females occurring due to a heterozygous transformation of WNT4 gene on chromosome 1p36.1-7 Additionally, traumatic vaginal injury in females, which is not uncommon, results in vaginal stenosis.5 This results in formation of scar tissue causing stricture of the vagina.5 Based on the family history, the patient must undergo medical examination supported by gynaecologic assistance to provide a sensitive and specific means of diagnosis.1,2 It is essential for the patients and their families to attend counselling before proceeding for treatment as this syndrome can cause severe psychological stress in a young woman. Patients with vaginal agenesis in MRKH syndrome can be treated by both surgical and non-surgical procedures and there has been no evidence of the superiority of one technique over another.6,11 Abbe–McIndoe operation is a common surgical method using split thickness skin graft for reconstruction and Frank’s dilators is a common nonsurgical method for the creation of neovagina.3,7 Frank's initial technique used hand-held vaginal dilators in the lithotomy position and had technical limitations such as uncomfortable positioning, loss of time spent during the process of dilation, and fatigue of patient's hands. Ingram's method overcame these difficulties by using the sitting position to take advantage of the patient's body weight.8,9 This method used vaginal dilators mounted on a specially designed bicycle seat stool that provided perineal pressure via the narrow and elevated anterior portion of the bicycle seat.10 Despite the favourable clinical outcome of this method, there were some practical difficulties in making and handling these in an outpatient clinic. However, irrespective of the procedure opted, prosthetic vaginal dilators or long-term vaginal stents are required to prevent the possible contraction of the reconstructed neovagina, to maintain vaginal width, depth and to avoid vaginal stenosis.6
Certain rare congenital defects can occur during the prenatal development of the female reproductive system, resulting in an abnormal or absent vagina.1 It is a part of the female genital tract. It is muscular and an elastic organ with a soft epithelial lining making it susceptible to disruption during crucial times of organogenesis.1,2 There are different malformations that occur in the female genital organ, many of which are classified under the broader term “Müllerian duct anomalies”. 1,2 Müllerian agenesis is also referred to as congenital absence of the uterus and vagina (CAUV), müllerian aplasia (MA), genital renal ear syndrome (GRES), and Mayer-Rokitansky-KüsterHauser syndrome (MRKH). It has been considered as a sporadic anomaly, but the increase in familial cases now supports the hypothesis of a genetic cause characterised by a failure of the Müllerian ducts to develop, resulting in a missing uterus and variable malformations of the vagina.1-7 According to scientific data, its incidence is reported to be 1 in 4,000 to 10,000 females occurring due to a heterozygous transformation of WNT4 gene on chromosome 1p36.1-7 Additionally, traumatic vaginal injury in females, which is not uncommon, results in vaginal stenosis.5 This results in formation of scar tissue causing stricture of the vagina.5 Based on the family history, the patient must undergo medical examination supported by gynaecologic assistance to provide a sensitive and specific means of diagnosis.1,2 It is essential for the patients and their families to attend counselling before proceeding for treatment as this syndrome can cause severe psychological stress in a young woman. Patients with vaginal agenesis in MRKH syndrome can be treated by both surgical and non-surgical procedures and there has been no evidence of the superiority of one technique over another.6,11 Abbe–McIndoe operation is a common surgical method using split thickness skin graft for reconstruction and Frank’s dilators is a common nonsurgical method for the creation of neovagina.3,7 Frank's initial technique used hand-held vaginal dilators in the lithotomy position and had technical limitations such as uncomfortable positioning, loss of time spent during the process of dilation, and fatigue of patient's hands. Ingram's method overcame these difficulties by using the sitting position to take advantage of the patient's body weight.8,9 This method used vaginal dilators mounted on a specially designed bicycle seat stool that provided perineal pressure via the narrow and elevated anterior portion of the bicycle seat.10 Despite the favourable clinical outcome of this method, there were some practical difficulties in making and handling these in an outpatient clinic. However, irrespective of the procedure opted, prosthetic vaginal dilators or long-term vaginal stents are required to prevent the possible contraction of the reconstructed neovagina, to maintain vaginal width, depth and to avoid vaginal stenosis.6
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