Laparoscopic sigmoid vaginoplasty: a salvage option for genitourinary fistula after failed McIndoe’s repair

Abstract

A 30-year-old woman presented with vesicovaginal fistula after a forceful intercourse. She was diagnosed as a case of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome 14 years ago and had underwent McIndoe’s vaginoplasty using amniotic membrane. Similarly, another 14-year-old girl presented with an iatrogenic urethral and bladder neck injury during an attempt at McIndoe’s vaginoplasty 4 months ago at another centre leading to urinary fistula with absent vagina. A laparoscopic salvage was done for both the cases along with repair of genitourinary fistula using sigmoid colon. There was no further requirement of vaginal dilation. Both patients remain fully continent at 1 year follow-up. Laparoscopic sigmoid vaginoplasty is a worthy minimally invasive salvage method for the patients of MRKH who develop fistulous complication after a previous attempt at neovagina creation. The bowel wall provides a structurally strong layer to withstand the repeated sexual trauma of the vagina.

Background

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterised by the absence of vagina as well as various abnormalities of the uterus and the urinary tract, but functional ovaries.^{1 2} The incidence of MRKH syndrome has been estimated as 1 in 4500 female births.³ The exact cause of MRKH syndrome is still unknown as the person’s karyotype is normal (46, XX) and any chromosomal abnormalities are extremely rare. In most cases, diagnosis is made during adolescence due to primary amenorrhoea with normal puberty.^{4 5}

MRKH is classified into two types: type I is an isolated form characterised by the absence of uterus and vagina with normal bilateral ovaries. Type II or MURCS association (Müllerian duct aplasia, Renal dysplasia and Cervical Somite anomalies) has other associated anomalies like urological anomalies in 15%–40% of cases and skeletal anomalies in 20%–40% of cases, while auditory and cardiac defects are rare.⁶

Various surgical options for reconstruction of vagina in these cases include Frank’s perineal dilatation, McIndoe’s vaginoplasty, William’s vulvar tube vaginoplasty, various rotational flap (perineal/thigh) procedures, Vecchietti’s pull up technique, Davydov’s peritoneal flap vaginoplasty and intestinal neovagina techniques.⁷

McIndoe’s vaginoplasty remains the most common procedure for reconstruction because of its minimal invasiveness and reasonable outcomes in terms of a functional vagina.⁸

The urethra and bladder may be at risk of injury during space creation for neovagina in any of these procedures. Also because the neovaginal lining provided by McIndoe’s vaginoplasty is not tough enough unlike a natural vagina, it remains prone to erosion either due to repeated forceful vaginal dilatation with moulds or during sexual activity. This may lead to formation of a concomitant genitourinary fistula posing a significant surgical challenge. A sigmoid neovagina provides a good alternative as a salvage procedure in this setting. The sigmoid tube also provides an intact healthy layer of tissue to cover the fistula. We present two cases of genitourinary fistula after a previous McIndoe’s vaginoplasty that were salvaged using minimally invasive sigmoid vaginoplasty.

Case presentation

A 30-year-old woman presented with a single episode of haematuria and continuous urine leak per vagina following forceful sexual intercourse. She was earlier diagnosed as a case of MRKH type 1 at the age of 14 when she had presented with primary amenorrhea. She underwent McIndoe’s vaginoplasty using amniotic membrane. Since then, patient was regularly using vaginal glass moulds and was also sexually active for last 10 years. Another 14-year-girl diagnosed as a case of MRKH type 1 presented with continuous urine leak after an attempt at McIndoe’s vaginoplasty at another centre 4 months ago.

Investigations

Local examination revealed normal external urinary meatus. Cystoscopy showed normal bladder capacity with small trigonal vesicovaginal fistula in the first case (figure 1A) and a complex bladder neck/urethrovaginal fistula in the second case (figure 1B). Vaginoscopy confirmed short vaginas (5 and 2.5 cm, respectively) and the corresponding fistulous openings at vaults of neovagina.

Figure 1.

(A) Vaginoscopic view of vesicovaginal fistula in the first case with McIndoe’s vaginoplasty done 14 years ago. (B) Operative image showing large bladder neck/urethrovaginal fistula in the second case after a failed attempt at McIndoe’s vaginoplasty 4 months ago. Space has been created between the bladder and rectum to subsequently accommodate the sigmoid segment as neovagina, as shown with full depth insertion of a Sim’s speculum.

Differential diagnosis

No urinary, skeletal, cardiac or auditory anomalies were identified in both patients. MRI of the abdomen showed normal solid organs, bilateral ovaries with the absence of uterus and short blind-ending neovagina. The chromosomal analysis confirmed 46, XX pattern. Taking all findings together, the differential diagnosis of simple vaginal aplasia, androgen resistance syndrome and testicular feminisation syndrome was ruled out.

Treatment

Transvaginal genitourinary fistula repair with laparoscopic isolation of sigmoid colon and sigmoid vaginoplasty was done. Initially, the fistula was circumscribed all around and space was created between bladder and rectum to accommodate the neovagina (figure 1B). The existing neovagina was de-epithelised by scraping the epithelium. The fistula was then closed over a 16 Fr urethral catheter. Three ports were then placed after creation of pneumoperitoneum as depicted in figure 2A. Incisions were given on sigmoid mesentery on either side just medial to the ureters. Sigmoid mesentery was thus lifted along with the colon. It is important to mobilise it maximally from its origin at the inferior mesenteric artery till the lowermost point of rectum easily approachable and amenable to circular stapling device (figure 2B). This helps to transport the isolated segment easily till the introitus without any tension. Using endocutter linear stapler (Ethicon, USA), the rectum was transected. A 3 cm Pfannenstiel incision was given and the end of sigmoid colon was pulled out through it. Approximately 12 cm of sigmoid colon segment was isolated by using another linear cutter stapler device (figure 2C). An anvil of circular stapler device was inserted into the proximal colon end and a purse string suture was taken around it using polypropylene suture (figure 3A). A stay suture was taken at the distal end of isolated segment for better identification and the bowel was then pushed back in to the abdomen and Pfannenstiel incision was closed. A circular stapler (CDH33A, Ethicon) was used for anastomosis of descending colon with rectum. Then an incision was made over the peritoneum behind the bladder and space created to meet the rectovesical space earlier created from the vaginal side (figure 3B). The distal end of isolated sigmoid segment was pulled into the vaginal introitus and edges sutured (figure 3C). The second case also required a concomitant prophylactic autologous pubovaginal sling for stress incontinence. Proximal end of the isolated segment is fixed over the sacral promontory and the pelvic peritoneum is closed to completely extraperitonealise the sigmoid neovagina.

Figure 2.

(A) Port placement for sigmoid vaginoplasty. Additional 3 cm Pfannenstiel incision is required to pull out the sigmoid segment for placing anvil of circular stapling device. (B) Complete mobilisation of sigmoid mesentery saving the ureters on either side. (C) Endolinear cutter stapler device used to transect the rectum.

Figure 3.

(A) Isolated sigmoid segment taken out via Pfannenstiel incision and insertion of anvil in the end of descending colon for use of circular stapler for colorectal anastomosis. (B) Creation of space in pelvis with finger inserted in vagina. (C) Final picture showing neovaginal introitus.

Outcome and follow-up

Postoperative period was uneventful. Blood loss was minimal. Both cases were allowed orally on third postoperative day. Patients were discharged on fifth postoperative day. They were advised to use vaginal mould 6 weeks after surgery and were allowed to have intercourse after that. After 1 year of follow-up, the first case is sexually active and there is no urine leak per vagina. The second case is still unmarried but is able to easily pass glass vaginal mould and has no urine leak.

Discussion

MRKH syndrome arises due to Müllerian agenesis and is a rare disorder but is reported to be the second most common cause of primary amenorrhea, next only to gonadal dysgenesis.⁵ Various surgical techniques are available to correct the anomaly by creation of neovagina either by surgical or non-surgical dilatation techniques.^9–13 Currently, there is no consensus in the literature regarding the best procedure for neovagina formation with regards to functional outcome and sexual satisfaction.¹⁴ Non-surgical method like sustained vaginal self-dilation (Frank’s method) over several months is tedious, painful and prone to secondary vaginal shrinkage and urethral injury.¹³ Vaginal lengthening by pull from inside (Vecchietti’s vaginoplasty) using a pluggable segmented dummy, which is connected to two threads that are kept on constant tension, is another option but limited by same downsides of possible urethral or bladder erosion with limited vaginal length.¹² Surgical methods which create vesicorectal space and cover it with autogenic tissues like skin (McIndoe’s vaginoplasty) or peritoneum (Davydov’s vaginoplasty) or vulval flap (William’s vaginoplasty) are limited by the length of neovagina that can be fashioned besides risk of scarring.⁹ The McIndoe’s vaginoplasty is the most commonly performed surgery for MRKH patients in our country because it is simple to perform and has acceptable success rates besides no abdominal or peritoneal access. But such graft procedures are prone to erosion over long term because only epithelium is recreated. Vulval flaps are limited by the external genital mutilation and cosmesis. Other important limitations of all these repairs are graft rejection (<1%), necrosis, dehiscence, hair growth, dyspareunia, secondary stenosis (4.9%) as well as the risk of developing squamous cell carcinoma.9 10 15–17

Despite providing a long and lubricated vagina, enterovaginoplasty techniques are usually reserved as salvage procedures for vaginoplasty. They are not frequently used for primary repair because they entail use of bowel, thereby increasing the invasiveness of surgery. However, in the salvage setting enterovaginoplasty can be easily used for any kind of previous vaginoplasty irrespective whether the failure is due to inadequate vaginal length or scarring or fistula formation. The laparoscopic approach to enterovaginoplasty retains all these advantages with the added benefits of a minimally invasive surgery.

Fistula after McIndoe or any other vaginoplasty is a devastating complication for the patient that threatens to takedown the neovagina as well. The repair of fistula in this setting is very challenging as the standard native tissue repairs of fistula surgery are not applicable here with no native vaginal wall available for any advancement or tissue repair. The limited available options include complete occlusion of vagina (total colpocleisis), various vascularised vulval or other fasciocutaneous flaps and enterovaginoplasty. Using colpocleisis ends up with loss of a functional neovagina, while vascularised vulval flaps are limited by the absence of a robust vaginal wall under which a tunnel can be made for transposition of flap. The neovaginal functionality is also hindered and affected. The use of bowel as in our cases (enterovaginoplasty) scores over other options in that it not only provides a layer of intact healthy tissue cover over the fistula (with no suture lines that can overlap at fistula site) but also provides a neovagina which is generally better in terms of depth and width compared with any other technique of neovaginoplasty.

Both small and large intestines can be used for the creation of neovagina. Small intestinal segment (usually ileum) is less preferred as it has more excoriating secretions and small lumen. Complications like stenosis, prolapse or necrosis of bowel segment are rare. The use of intestinal segment after failed McIndoe’s vaginoplasty like in this case is a fine salvage technique for genitourinary fistulas after primary vaginoplasty for MRKH syndrome cases. The minimally invasive laparoscopic technique has potential to reduce per-operative morbidity in such cases.

Learning points.

• The McIndoe’s vaginoplasty is most commonly performed surgery for Mayer-Rokitansky-Küster-Hauser syndrome with high success rates but a small risk of fistula formation.

• Sigmoid vaginoplasty offers a good salvage technique for urinary fistula after failed prior vaginoplasty.

• Laparoscopic approach to sigmoid vaginoplasty is feasible and offers all advantages of minimally invasive surgery.