An alternative approach to vaginal dilation in patients with Meyer-Rokitanski-Küster-Hauser syndrome: two case reports

Ogliari, Kátia Crys Moura; Rios, Salete da Silva; Chen, Ana Carolina Rios; Chen, Juliana Rios; Resende, C.N.; Júnior, Edward Araujo

doi:10.1080/09513590.2020.1719061

Cited by 3 publications

(1 citation statement)

References 11 publications

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“…Management of vaginal atresia or agenesis includes surgical and non-surgical approaches based on the clinical presentation. Non-surgical vaginal dilation is considered as the first line of therapy in a narrow vaginal canal, that provides an anatomical and functional success rate of more than 90% ( 1 ). A surgical approach is required in patients presenting with complete vaginal agenesis, and/or unsuccessful vaginal dilation using non-surgical vaginal dilators.…”

Section: Introductionmentioning

confidence: 99%

Customised Vaginal Stent – The Phases in Management of Vaginal Agenesis in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome

Raza¹,

Raju²,

Babu³

et al. 2023

JFRH

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Objective: Vaginal agenesis or atresia in females suffering from MRKH syndrome is more common and management involves both surgical and non-surgical approaches. Use of prefabricated stents to maintain the patency of the canal may not fit appropriately during the initial surgical phase and are not economical. This case report discusses a series of modifications in a custom-made vaginal dilator to improve the retention for expansion after surgical management of MRKH syndrome. Case report: A 28-year-old female diagnosed with MRKH syndrome with characteristic Mullerian agenesis was referred for customised vaginal stent. Customised surgical stent was fabricated with loops for orientation and retention, which was later modified into interim expansion and passive stent. Conclusion: The customisation of the vaginal stent, provision of a retentive loop that positioned the stent in the proper orientation, and gradual increase in the size of the stent, ensured dilatation in a patient with vaginal agenesis.

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Section: Introductionmentioning

confidence: 99%

Customised Vaginal Stent – The Phases in Management of Vaginal Agenesis in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome

Raza¹,

Raju²,

Babu³

et al. 2023

JFRH

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Hollow Prosthetic Vaginal Dilator - A Novel Technique

Mohamed¹,

Krishnamurthy²,

K³

et al. 2021

jemds

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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

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Variability in Design and Materials of Vaginal Stent or Moulds for Vaginal Agenesis - A Systematic Review

Jain,

Bansal

et al. 2024

Journal of Human Reproductive Sciences

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Background: Vaginal stents play a crucial role in both non-surgical and surgical management of vaginal agenesis. Different types of stents with variable designs and materials have been described in the literature. However, a summary of various stents described in the existing data and their outcome measures needs to be studied. Objectives: The objective of the study was to identify and summarise different types of vaginal stents used for patients with vaginal agenesis, to identify the design and material of different vaginal stents and to assess the benefit of using that specific vaginal stent. Materials and Methods: The literature search was conducted in the PubMed database for articles from 2000 to 2024 in the English language. An exploration of grey literature was also included through Google Scholar. The articles were included only if they clearly described vaginal stent, its material, design and advantage of using that particular stent, and any study not directly related to the research question or in which vaginal stent was used for cases other than vaginal agenesis/Mayer–Rokitansky–Kuster–Hauser syndrome/Mullerian aplasia or agenesis were not included in this study. Risk of bias assessment was done using an eight-item tool that included domains of selection, ascertainment, causality and reporting. Results: A total of 133 abstracts appeared in PubMed, of which, 26 titles/abstracts were related to the research question. Based on inclusion and exclusion criteria, seventeen articles were selected for the review. A total of 905 articles were identified from Google Scholar, of which, 7 were chosen to be included. The literature reports the usage of variable vaginal stents which can be hard, soft, solid or hollow with the option of customisation available. Interpretation: Based on the results of this review, soft stents are better than rigid stents due to increased patient compliance, a critical factor contributing to the success of the treatment. The resilient nature, comfort and lightweight of the silicone material are more suitable than commonly used acrylic material. However, the choice in a particular case may vary. Limitations: The majority of articles included in this review were case reports. As the duration and frequency of usage of stents affect the outcome, studies need to be done to evaluate the effectiveness of different types of stents to come to identify the best design and material for vaginal stents. PROSPERO Registration: CRD42024554676.

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An alternative approach to vaginal dilation in patients with Meyer-Rokitanski-Küster-Hauser syndrome: two case reports

Cited by 3 publications

References 11 publications

Customised Vaginal Stent – The Phases in Management of Vaginal Agenesis in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome

Customised Vaginal Stent – The Phases in Management of Vaginal Agenesis in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome

Hollow Prosthetic Vaginal Dilator - A Novel Technique

Variability in Design and Materials of Vaginal Stent or Moulds for Vaginal Agenesis - A Systematic Review

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