Dilation and Surgical Management in Vaginal Agenesis: A Systematic Review

Dr. Sarah K. McQuillan

MD, FRCSC
Paediatric and Adolescent Gynaecology Fellow
The Royal Children’s Hospital – Melbourne, Australia
University of Melbourne

Dr. Sonia R. Grover

MBBS, MD, FRANZCOG
Head of Department of Paediatric and Adolescent Gynaecology
The Royal Children’s Hospital – Melbourne, Australia
Associate Professor – Department of Paediatrics University of Melbourne
Consultant Gynaecologist – Mercy Hospital of Melbourne

Corresponding Author:
Dr. Sarah K. McQuillan
[email protected]
Mobile: 0438 919 551
Fax: (03) 8458 4889
The Royal Children’s Hospital - Melbourne
50 Flemington Rd., Parkville, Vic, Australia, 3052

FINANCIAL DISCLAIMER/CONFLICT OF INTEREST: NONE

SK McQuillan: Data collection or management, Data analysis, Manuscript writing/editing

SR Grover: Project development, Data analysis, Manuscript writing/editing

1
Abstract

INTRODUCTION: The management of vaginal agenesis currently is determined by

geographical location and surgeon preference. The optimal treatment is unknown and the
majority of articles on technique and outcome focus on personal case series with little
standardization of reporting and follow up.
METHODS: 6691 articles concerning the management of vaginal agenesis were
systematically reviewed, with 162 fitting the inclusion criteria.
RESULTS: Only 1 randomized control trial was included with the remaining articles made
up of case series or case reports. The bowel vaginoplasty method was most commonly
reported historically with 945 patients and 45 articles included. The Vecchietti procedure
had the shortest operative time but the most number of urological injuries (2.1%). The
split thickness procedure had the highest infection rate (4.2%) and re-operative rate
(7.84%).
CONCLUSION: Overall, the conservative method using dilation had the least number of
complications, with an average vaginal length of 6.65 cm (+/- 1.39cm). However, with an
operative procedure full consent and understanding of the need for postoperative dilation
with the majority of techniques is imperative.

Keywords: management, vaginal agenesis

Brief Summary: A systematic review of the management of vaginal agenesis in MRKH and
AIS to determine the optimal outcome.

Word Count
Abstract: 171 words
Manuscript Text: 4,047 words

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Introduction

Vaginal agenesis can occur in a number of different settings. It can be a congenital

condition where it may be an isolated anomaly, or as part of a specific disorder of sexual

development (DSD) such as in androgen insensitivity syndrome (AIS), or it may occur in

association with a number of other anomalies. An absent or small vagina may also occur

secondary to radiotherapy or surgery. Vaginal agenesis resulting from either Mayer-

Rokitansky-Kuster-Hauser Syndrome (MRKH) or AIS affects between 1 in 4000 and 1 in

13000 girls respectively, worldwide(1). There is considerable confusion caused by

inaccurate or differing uses of a number of expressions relating to an absent or small

vagina (Table 1). Absent vagina refers to vaginal agenesis and AIS for the purpose of this

paper and is generally diagnosed in adolescents presenting with primary amenorrhea or

more rarely primary infertility. But even in these 2 diagnoses, some vagina may be present,

and this may vary from no vagina and no hymen (in some women with vaginal agenesis), to

3 -5 cm length of vagina as is usually the case in AIS.

Currently, there are many methods described in the literature for creating a

functional neovagina. However, the best method has yet to be determined using a rigorous

randomized control trial. The majority of articles published review outcomes of personal

case series with no standardized assessment of indications, initial vaginal length,

intraoperative or postoperative complications, or outcome measures both from a

functional and anatomic perspective. Without standardized approaches to outcomes and

follow up the comparison of methods of vaginoplasty is difficult.

Historically, surgical creation of a neovagina was described as early as 460-377BC

by Hippocrates(2). However, it wasn’t until 1898 when Abbe described the use of a mold

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following the opening of the perineum by a medical practitioner in 2 women to maintain a

vaginal opening for intercourse(3). Over the course of the first half of the 20th century both

surgical and non-surgical methods of neovagina creation were described (Baldwin (bowel

segment)(4), Frank’s dilation(5), McIndoe’s modification of Abbe’s split thickness graft(6)).

Towards, the middle of the 20th century modifications of traditional methods such as

Sheares modification of the McIndoe procedure (7), Creatsas modification of William’s

vaginoplasty(8) and new methods (Vecchietti(9), and the plastic surgery flaps were

introduced) were described (Table 2). Additionally, case series of vaginoplasty also

included summaries of complications and an attempt to qualify functional outcome. The

end of the 1980s marked a change in surgical practice with the introduction of laparoscopic

assisted procedures, which decreased the morbidity the patient experienced from surgery,

and the length of inpatient stay. Finally in 2012, the first attempt at a prospective

randomized control trial was published by Cao et al. comparing the outcomes of

laparoscopic peritoneal (traditionally the Davydov method) and laparoscopic sigmoid

vaginoplasty for 40 patients(10).

The purpose of this review is to summarize objectively all available evidence

regarding techniques of vaginoplasty in women with vaginal agenesis or AIS, including

intraoperative and postoperative (both short and long term) complications and outcomes

of surgical and nonsurgical techniques from the last 20 years in an attempt to guide clinical

practice. Currently, the type of neo-vaginal creation is dictated not by rigorous evidence but

solely by surgeon experience and preference and thus where in the geographical world the

patient is residing. Other conditions where there is an absent vagina in the context of

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multiple other anomalies (such as a cloacal anomaly) or an atretic vagina (secondary to

surgery or radiotherapy) require special consideration of the additional problems, which

make any attempt at comparison between operative techniques even more difficult.

Conditions where there is a uterus present also require very careful consideration

regarding the possibility of allowing a fertility potential – and in many parts of the world

this option will then influence the management of the absent vagina (and/or cervix)(11).

Methods:

A search of all published literature restricted to the management of vaginal agenesis

in patients with Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH) or Androgen

Insensitivity Syndrome (AIS) since 1898 was conducted using pubmed, medline (on a

weekly basis), ovid as well as hand searches for relevant articles not initially identified

using the search parameters. Results were then restricted to systematic reviews,

randomized control trials, controlled clinical trials, observational studies and case series

(of 5 or more cases) since 1992. Non-English articles, case reports of operative description,

repeat vaginoplasty after failed primary procedure, double reporting of results and where

there were associated urogenital (including lower urinary or anorectal) anomalies were all

excluded. However, case reports of rare complications were reviewed for possible

inclusion. All potential studies identified were assessed for inclusion and any disagreement

between the reviewing authors was resolved through discussion. The two review authors

then independently assessed the included articles looking for the following extracted data:

patient population, intraoperative complications (i.e. injury to bowel or bladder,

hemorrhage requiring transfusion, conversion to an open procedure if a laparoscopic

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procedure), operative time, objective length and width of neo-vagina and short and long

term postoperative complications (i.e. short term: length of stay, infection, hematoma,

anastomotic leak, versus long term: failure, prolapse, cancer, colitis, stenosis, scarring,

vaginal discharge, venous thrombo-embolic event, death). Results of sexual function and

satisfaction will be included in another published review article (to be submitted to the

International Journal of Urogynecology and pelvic floor reconstruction with the title

Systematic Review of Sexual Satisfaction following the Management of Vaginal Agenesis).

For included studies we have assessed results reported including the possibility of

inherent author biases and patient attrition. Given the potential of variation in results

reported, we attempted to dichotomize data for analysis. Data were then synthesized and

analysis was carried out using statistical software.

Results:

A total of 6691 articles were identified using the key search words “vaginal

agenesis”, “Müllerian agenesis” plus “management” up to July 2013. A total of 162 articles

were identified and reviewed (Figure 1). The studies included were carried out in a

number of countries including Australia, China, Italy, Great Britain, Canada, USA and Brazil.

In the majority of the studies, no information was provided on the indication for the

particular treatment offered, there was no blinding of either the patients or the clinicians

with only one study having randomized care. The majority of studies were observational

case series with no standardization of objective outcomes and need for ensuring adequate

long term follow. There was evidence of papers containing a certain amount of author bias

due to triage practices within the country where patients are referred directly for surgical

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correction to tertiary centers such as in Poland, Italy and Germany(12). Additionally, for

these same centers, many women may not ever be referred as they have already been

successful in neo-vaginal creation using dilation or intercourse, and there is no way of

accounting for these patients and measuring their outcomes.

Overall, 162 articles were included accounting for 4326 patients (3 articles were

included in 2 different vaginoplasty methods because of an attempt to compare techniques

(10, 13)). The vaginoplasty method using the bowel was the most published with 45

different peer reviewed articles with a total of 945 patients(10, 13-58). The full thickness

flap method (including muscle flaps, rotational flaps and bladder tissue) was the least

researched in the English language literature with only 168 patients fulfilling the inclusion

criteria (59-70).

Intraoperative Experience:

Overall, the shortest operative time utilizes the Vecchietti Procedure (19 papers, 934

patients) with an average time of 44.45 minutes (+/- 17.7 minutes) (71-90) versus the

average time of 181.2 minutes (+/- 59.8 minutes) with bowel vaginal reconstruction

(including both laparoscopic and open approaches). For the split thickness graft methods

(including McIndoe technique (of skin), buccal mucosa, oxidative cellulose and amnion)

operative time varied greatly between 15 to 20 minutes and 245 minutes (91)((92-127).

Postoperative Length of Stay:

The length of postoperative stay has significantly reduced since the introduction of a

laparoscopic approach, decreasing to a mean of two days compared to the 8 or 9 days in

the past with the Vecchietti, peritoneal and bowel methods. Both graft techniques (full and

split thickness) required the longest length of stays with an average of 14 days.

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 Complications were split into 3 time frames for simplicity: intraoperative,

immediate and long-term postoperative complications (Figure 2). Only 2 deaths were

reported and occurred with the bowel and split thickness vaginoplasty methods. Prior to

the advent of routine prophylactic antibiotic use, many more deaths had occurred in the

bowel vaginoplasty group.

Intraoperative Complications:

Intraoperative complications were obviously non-existent in the conservative

(dilation) approach, though one mention of neo-vaginal vault rupture was reported during

vaginal lengthening using coitus (128). Urological injuries, including injury to bladder or

ureters or postoperative urinary retention, (2.1%) occurred most commonly using the

Vecchietti approach whereas bowel injuries occurred more commonly with peritoneal

approach (2.6%)(83, 129-141). Bleeding was associated more often with the bowel

approach with 19 transfusions reported, an overall higher estimated blood loss (EBL) of

around 302.5ml and 9 postoperative hematomas were also described.

The remaining complications focus on the postoperative period. Postoperative ileus

(1.1%, n=10/945) and anastomotic leak (n=3/945) occurred more frequently following

bowel vaginoplasty but was followed closely by the split thickness method of repair. Given

that the success of the split thickness procedure is dependent upon graft integration it is

worth noting that it has the highest infection rate (n=29/688) at 4.2%, which is not

statistically significant compared with the other methods of vaginal creation using ANOVA

analysis. The split thickness method had the most number of reoperations (7.84%, n=54),

although this was not statistically significantly different. The re-operative rate included

scar revisions (from harvesting of the graft site), graft failure and dehiscence, which

8
required surgery to either repair the initial graft or placement of a brand newly harvested

graft.

With respect to long-term outcomes, the vaginal length achieved with surgery is

longest using the bowel method (with a weighted average of 12.87cm). We did not

calculate statistical significance between the different surgical techniques due to the

heterogenicity not only between the surgical techniques but also between the surgical and

conservative methods in terms of starting vaginal length (which ranged from flush with the

perineum to more than 3 cm in 43/200 patients(8, 101, 142-151) to omission of

information in the majority of papers). Additionally, there was heterogeneity in terms of

attempts at using dilation prior to surgery and the duration of postoperative follow-up,

which ranged from 4 weeks up to 50 years(152). Dilation resulted in the shortest average

vaginal length of 6.65cm (13, 101, 122, 128, 133, 153-173). All vaginas were at least 2

finger-breadths wide.

Urinary incontinence (not defined) occurred primarily with graft vaginoplasty,

however, the data was slightly skewed by the inclusion of 1 article by Borkowski et al. in

2008 which included 37 patients with bladder flaps for vaginoplasty where 19 described

symptomatic urinary incontinence postoperatively(62).

Less frequent long-term complications included vaginal hair growth, vaginal

prolapse, condylomata and vaginal carcinomas (both squamous cell and adenocarcinoma).

Vaginal hair growth only occurs with techniques, which mobilized the vulvar hair-bearing

areas into the vagina (i.e. Creatsas (3/200) or full thickness flaps using the pudendal area

(n=19/168)). Neo-vaginal prolapse was reported to occur with all techniques except for

the Vecchietti, Davydov and Creatsas’s modification of William’s vaginoplasty methods.

9
When prolapse occurred using bowel vaginoplasty, both mucosal and entire neo-vaginal

prolapse were described. Several different methods have been described to repair neo-

vaginal prolapse but this is beyond the scope of this article. The carcinomas (n=23)

described included both squamous cell and adenocarcinoma, with adenocarcinoma only

occurring when bowel mucosa was transpositioned for creation of a neovagina(148).

Carcinoma occurred most commonly using the split thickness method, however that may

be because the split thickness method has the longest reported follow up (up to 50 years).

Individually, condylomata and vaginal cancer have been reported in the neo-vaginal

literature. Looking specifically at that relationship, a review of 33 women following neo-

vaginal creation primarily using Vecchietti’s method (and the bowel method) were referred

for evaluation of human papilloma virus (HPV) lesions; 27 tested positive for the low-risk

HPV, 6 for high-risk HPV, 3 had vaginal intra-epithelial neoplasm (174) grade 1, 2 grade 2

VAIN and 1 adenocarcinoma was diagnosed(175). Of these lesions, 17 were located on the

vulva and 16 within the neo-vagina. All the vulvar lesions and 10 of the 16 neo-vaginal

lesions were condylomata. These findings suggest that papanicolaou testing guidelines

that are set out by individual regulatory bodies throughout the world should apply to

sexual active women with neo-vaginas as well, keeping in mind that the majority of low

risk HPV subtype will spontaneously resolve (176) and that the interpretation of results

may be difficult due to the altered tissue. Only 3 case report of vaginal colitis in the

literature on vaginal agenesis(177) could be found and is much more commonly reported

in the setting of bowel vaginoplasty as a long term complication in the congenital adrenal

hyperplasia (CAH) and cloacal repair vaginoplasty literature(178) (Table 2).

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Discussion:

This review was initially planned as a systematic review to determine the optimal

management of isolated vaginal agenesis due to MRKH or AIS. However, unfortunately, the

literature available was non-standardized and heterogenous with differing amounts of

details available regarding patient characteristics, follow up and complications discussed.

Because of the diverse range of details available with mixed information and follow-up, the

use of the recommended methodology from the Preferred Reporting Items from Systematic

reviews and Meta-Analyses (PRISMA) checklist for meta-analyses was unfortunately not

possible(179).

The only RCT regarding vaginoplasty compared the use of bowel versus a laparoscopic

peritoneal approach with a significant smaller intraoperative blood loss (65 versus 200ml),

shorter operative time (100.4 versus 217.8 minutes) and decreased duration of inpatient

stay (6 versus 10.5 days) with the laparoscopic peritoneal approach. Postoperatively, the

mean vaginal length was not statistically significantly different between the 2 groups,

however, abdominal discomfort and foul vaginal secretions during intercourse were

increased in the bowel vaginoplasty group.

Never the less, given the current choice of correction of vaginal agenesis differs

depending on where in the world the patient is geographically located, it was important to

examine the currently available literature to determine the pros and cons of the individual

methods and recommend an initial course of action for correction of vaginal agenesis so

that the patient’s informed consent for vaginal creation is as accurate as possible in respect

to possible options and complications.

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 The American Congress of Obstetricians and Gynecologists in 2013 recommends

that vaginal dilation be recommended as first line for correction of vagina agenesis. The

articles focusing on dilation have high successes rates ranging between 43-94.5%(172),

with a success rate of 94.5% being reported in the largest cohort group (159). Never the

less, there are only 28 papers and 802 patients in the English literature with reasonable

methodology. It is worth noting that dilation is the preferred technique in the UK,

Australia, USA and parts of Russia suggesting that it is a widely used technique that should

have more follow-up data relating to it. As well, Routh et al. reviewed the cost of dilation

versus vaginoplasty and found that even with dilation failures requiring surgery in the

future, dilation was overall more cost effective by $US 17,724 (180) with only one article

publishing their exact estimated average costs of $US 2,397 for length of stay and use of

tissue-engineered biomaterial for the graft (127). Although many surgical techniques

imply that they are used for failed dilatation, the criteria for failure are not defined, neither

in terms of length of time trialing dilatation, timing of commencement of dilatation in terms

of patient maturity nor support given for dilatation. Furthermore, the time required for

successful creation of a vagina through dilation ranges considerably between 2 months to

11 months(159) with the average in most articles around 5 to 6 months. Another issue

pertains to vaginal agenesis in an adolescent who may not be emotionally ready to commit

to daily dilation, does she constitute a failure if a vagina is not created through dilation at

her 6-month follow-up? These same papers describing surgical techniques often state the

negative aspect of dilatation and the reason for the failure of this approach, as the need to

use the dilator daily. Yet they fail to acknowledge that dilation or the use of a post

operative vaginal mold is required for their surgical technique often for at least 3 months in

12
the postoperative period. Others routinely use surgical techniques as first line approach. In

Creatsas’ series of 200 women, surgical vaginoplasty is offered at the first visit if the patient

is interested in sexual activity and dilation is only offered if the vaginal dimple is more than

2.5cm(144). In contrast, in Jasonni’s retrospective review, a vagina of 3 to 5 cm was

considered a failure of dilation and patients were offered William’s vaginoplasty(101). It is

worth noting that in some areas of the world patients with absent vagina present with

primary infertility and are only then discovered to have uterine agenesis (and not vaginal

agenesis as they have already created their vagina through intercourse). As these women

have been sexually active, it is difficult to know how much vagina had been present before

intercourse began (personal communication between Dr. Y. Sharma and Dr. S. Grover). An

article from Norway describes repeated coitus as a method for vagina creation(181). Again

re-iterating, the majority of the surgical technique papers mention the need for

postoperative dilation or use of a vaginal mold (except in the case of Creatsas’s

modification of William’s vaginoplasty and the bowel vaginoplasty). This can be required

from 6 to 8 hours a day with the peritoneal technique(83) and up to 24 hours a day for up

to 3 to 6 months followed by nightly insertion with the split-thickness technique(102) or

daily dilation (sometimes upwards of 3 times per day) and then on a regular ongoing basis

or until regular sexual activity commences. The question to pose is, if patients were non

compliant with dilation for neo-vagina creation, that is, they had failed the dilation

technique, how can surgeons ensure compliance postoperatively, especially considering

that failure of the split thickness technique is directly attributed to non compliance with

mold insertion. Furthermore, McVearry et al. describe faster time to intercourse (around 6

13
weeks) with a combination of dilation and physiotherapy support giving more credit to the

success of dilation with a multidisciplinary support team(182).

The majority of the articles fail to mention the issue of timing of surgery if

undertaken, in terms of ensuring adequate maturity of the individual to undertake the very

important follow-up care. The clinicians undertaking the Vecchietti in Germany do use a

screening technique with psychological assessment prior to undertaking surgery although

do not inform the reader of the rate of delay/deferring surgery due to lack of psychological

readiness(74).

The endpoint in terms of the type of skin lining the vagina is mentioned in the

literature though the importance of this finding in terms of outcome is not completely

understood. Vaginal mucosa-like lining of the neo-vagina is achieved by using peritoneal

lining, traction, dilation, and various graft-like methods. Although the technique of

achieving this is different for both Vecchietti and dilation, the process is the same for

creation of a space (one by traction, the other by pressure). Davydov is not dissimilar,

although lined by peritoneum (which is very similar to the use of an amnion graft), which

converts to vaginal mucosa as seen through biopsies of the neo-vagina by Fedele et al.

showing iodine positive vaginal epithelium(83). In contrast, the use of bowel results in an

intestinal-like mucosa, which universally produces a significant amount of discharge in the

first three to six months. Of the grouped patients having bowel vaginoplasty in our analysis

7.5% (n=70) describe prolonged or bothersome (foul smell, having to wear a pad daily,

daily douching) vaginal discharge, which was listed as a long-term complication potentially

interfering with surgical satisfaction in some cases. Some proponents of bowel

vaginoplasty report decreased problems with lubrication during intercourse with the

14
bowel technique and that this discharge initially is not an issue. Here we must wait for the

other promised review by the authors.

Secondly, many of the surgical advocates comment on the inadequate vaginal length

through the use of dilation, however, the average length in this review of vaginal dilation

was 6.65cm, with the largest cohort by Edmonds et al. quoting 232 of 245 patients with

lengths over 6 cm. The literature would suggest that 6.6 cm is ideally the length of vagina

necessary for satisfactory sexual activity, which poses the question regarding what should

be the final goal of vaginal length(183)? Furthermore, is measuring a vaginal length a valid

outcome measure or should it be satisfactory sexual intercourse without pain? Finally,

sexual partners can change throughout life meaning that the initial vagina created may be

just right at the time of creation but may need to be smaller or larger later on. Thus with a

surgical technique this is difficult to alter and fraught with risks, whereas coitus or dilation

allows for patient controlled creation.

With regard to the risk of prolapse of the bowel segment neovaginas, Lenaghan et al.

reported a very high rate of prolapse at 10% following sigmoid vaginoplasty (with 6

mucosal prolapses in 60 patients (only one MRKH, the remaining with gender

dysphoria)(38), whereas O’Connor et al. failed to describe any prolapse in their series of 10

cases(46). Prolapse of vaginal mucosa has also been reported to occur following the

dilation technique. No reports of prolapse have been reported following the Vecchietti or

peritoneal (Davydov) methods– either due to lack of reports, the relatively short

postoperative follow up interval or possibly because the traction thread tracts offer some

scarring and thus support.

15
 The problem with many of the long-term complications is that the articles published

are only starting to look at these complications more remote from surgery. Thus the next

25 years of literature will give us better ability to counsel patients regarding the potential

long term complication of surgery. As well, only since 1992 has laparoscopy started to be

used for neo-vaginal creation and is now starting to become the standard of care in MRKH

and AIS patients where operative approaches are being used. The major complication rates

(i.e. death and venous thromboembolic events) anecdotally have decreased since

laparotomy has been phased out or only reserved for very difficult procedures or

intraoperative complications.

The limitations of this review include differences in technical terms used to describe

surgical techniques making grouping of methods challenging, the split thickness method

has changed substantially since Abbe’s first description in 1898(3), and finally

complications and follow up are not uniformly described.

In terms of concluding the best method of neovaginal creation, this review has

failed. However, the review does provide an overall summary of surgical complications

and highlights the need for long-term follow up of these patients. It also highlights what

would be the most valuable now would be more formal randomized control trials (RCT) or

given the difficulty with the practicality of such an undertaking a more standardized

approach to patient evaluation and decision to undertake a surgical procedure, accurate

description of operative procedure (which with the advent of laparoscopy allows for easy

dissemination of video recording of procedures), and thorough long-term follow up

utilizing similar assessment tools.

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 As the only RCT between bowel vaginoplasty and peritoneal approaches, suggests

fewer problems with the peritoneal approach combined with the fact that it does seem that

undertaking significant bowel surgery when a less invasive procedure offers similar

outcomes, that the less invasive approach should be used. Futhermore, consideration of

enhanced support for the dilation technique may improve success negating the need for

surgery only to very rare instances

The review further reiterates, that while awaiting guidance of the optimal approach,

the need for conservative approaches for the initial management, with dilation being

considered the first line option. Surgical procedures should be utilized for failure or refusal

to dilate with full understanding of the need for long-term dilation or use of a vaginal mold

postoperatively similar to the length of time required initially for vaginal creation. The

review also highlights the need for more standardized reporting of indications for surgery,

complications of surgery and long-term follow-up. Ultimately the decision to proceed with

neo-vagina creation rests upon both the young affected and her treating (surgical) team.

17
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Legend of Figures:

Figure 1: Literature Review of Management of Vaginal Agenesis

Table 1: Standardization of Nomenclature in Vagina Agenesis

Table 2: Definition of Vaginoplasty Techniques

Table 3: Summary of Short and Long-Term Outcomes of Management of Vaginal Agenesis

31
 Figure 1: Literature Review of Management of Vaginal Agenesis

6691
articles

1569
non
english
1769
prior
to 1992
3108 non
MRKH/AIS
Previous
surgery 83
Case
reports

162
included

10 Sheares/
28 Dilation 19 Vecchietti Williams/ 50 graft 45 bowel
13 Davydov
Creatsas (peritoneal) technique technique

12 Full
thickness 38 split
flaps thickness

32
1 Table 1: Standardization of Nomenclature in Vagina Agenesis

Nomenclature Definition Commentary

Vaginal agenesis/vaginal Congenital absence – Authors using this
aplasia although this can still expression often do not
Agenesis or aplasia = Non include the presence of report the size of the
formation of a part the lower 1/3 of the original vagina. The
vagina which is thought to minimal/maximal length
develop from caudal that a surgical approach
growth of the vaginal would or would not be
plate. The distal most used is usually not defined
portion of the sinovaginal
bulb forms the hymenal
tissue and some vaginal
agenesis patients may
have a hymen(184).
MRKH (Mayer- Described independently Specifically refers to
Rokitansky-Kuster-Hauser by these clinicians in vagina agenesis with or
Syndrome) 1829(185), 1838(186), without associated
1910 and 1961 with all 4 abnormalities. Hauser
descriptions referring to differentiated AIS from
congenital absence of the MRKH(188).
vagina and an absent or
rudimentary uterus and
associated renal/skeletal
anomalities (Kuster)(187)
due to arrest of Mullerian
duct development.
Vaginal hypoplasia Hypoplasia from the greek Some vaginal agenesis do
plasis/plassein to have some vagina so it
mold/form meaning under would be best to avoid

33
 formation of an organ or this expression. Most
tissue. women with AIS have 3-5
cm of vagina.
Vaginal atresia From the Merriam- Best used when referring
Webster dictionary to vaginal obliteration
meaning “absence or secondary to trauma,
closure of a normal body radiation or previous
part”(189). surgery.

34
2 Table 2: Definition of Vaginoplasty Techniques
Techniques Definition Other Nomenclature used
Dilation Intermittent manual pressure on the Frank’s method, Ingram’s Bicycle,
perineum to gradually create a vaginal D’Alberton (coitus) (190)
canal
Vecchietti’s Upward surgical traction along the Modifications of Vecchietti’s Technique
perineum with the use of an object (i.e. change of the original olive,
including but not exclusive foley
catheter ball)
Davydov’s Surgical inlay of peritoneum to line the
vagina
Sheare’s Modification of William’s Blunt dissection to create a vulvovaginal Creatsas’ Method, Wharton (191)
Vaginoplasty pouch
Full Thickness Flap Rotation or Insertion of a harvested flap Singapore, Malaga (192), Labia
into a dissected vaginal opening
Split Thickness Flap Insertion of a harvested split thickness Abbe-McIndoe, Oxidative Cellulose,
flap into a dissected vaginal opening Tissue-Engineered Biomaterial Graft,
buccal mucosa, amniotic membrane
Bowel Harvest of bowel mucosa for Jejunem, ileum, cecum, sigmoid
transplantation into a created vaginal

35
 opening
3

36
4 Table 3: Summary of Short and Long-Term Outcomes of Management of Vaginal Agenesis

37
Analysis Dilation Vecchietti Davydov Sheares Full Split Bowel
(including (including Thickness thickness Vaginoplasty
both open and both open flap flap (including both
laparoscopy)*1 and open and
laparoscopy) laparoscopy)
Total number of 802 934 500 289 168 688 945
patients
Death 1 1

Venous Thrombo 1 3 1
Embolic Event
Intraoperative Complications
Bowel Injury 4 13 3 3

Urogenital Injury 20 10 7 3

Transfusion 2 1 7 19

Postoperative Complications
Infection 24 9 1 3 29 22
(including
urinary tract)
Hematoma 5 1 6 9

Ileus/Bowel 10
Obstruction
Abscess 1 2 5

38
Anastomotic leak 1 3

Dehiscence 1 (vault 3 1*2 10 2

laceration)
Necrosis 1 2 1 5 1 4

Need for 6 6 9 10 54 51
rehospitalization/
Sugery
Problems with 3 35 3
the graft
Fistula 1 3 3 4 11 2

LongTerm Complications
Length (in cm)*3 6.65 7.87 8.86 11.49 8.93 8.84 12.87

Incontinence 1 1 20*4 15 3

Unacceptable 5 7 55 11
Scarring (Laparotomy)
Failure 123
(+ 12
refused)
Spotting 2 1 (+ 3 2 3 14 24
hematuria)
Vaginal Discharge 1 3 9 56

39
 Granulation 5 36 1 60
Tissue
Vaginal Hair 3 21
Growth
Condyloma 46 9

Prolapse 9 1 6*5 33*6

Colitis 3

5 *1 18 Device difficulties, 1 gastric ulcer complication, 1 paralysis from laparoscopic positioning

6 *2 Intraabdominal mold migration
7 *3 All lengths weighted average including articles mentioning length with all width over 2 finger breaths when width reported
8 in cm-centimeters
9 *4 Majority of incontinence (19/20 cases) with Full Thickness Flap vaginoplasty from Borkowski et al. (61)
10 *5 Including 2 rectal prolapse
11 *6 Mucosal prolapse in 22 cases

40
Minerva Access is the Institutional Repository of The University of Melbourne

Author/s:
McQuillan, SK; Grover, SR

Title:
Dilation and surgical management in vaginal agenesis: a systematic review

Date:
2014-03-01

Citation:
McQuillan, S. K. & Grover, S. R. (2014). Dilation and surgical management in vaginal
agenesis: a systematic review. INTERNATIONAL UROGYNECOLOGY JOURNAL, 25 (3),
pp.299-311. https://doi.org/10.1007/s00192-013-2221-9.

Persistent Link:
http://hdl.handle.net/11343/223993

File Description:
Accepted version