High imperforate transverse vaginal septum with vaginal cicatrisation: a surgical tribulation

Abstract

An early adolescent girl was referred to us with cryptomenorrhoea, and pelvic pain consistent with obstructed menstruation. Originally presumed to be a case of imperforate hymen, she was referred to our centre after two failed surgical misadventures at correcting the obstruction. MRI revealed a haematometrocolpos, high transverse complete vaginal septum and an occluded vagina. She underwent a laparoscopic drainage of the collection, septal resection and a vaginoplasty with an absorbable Interceed graft. Postoperative recovery was smooth and she was sent with instructions to use a vaginal mould daily. Successful surgical treatment requires precise preoperative planning with MRI. A vaginal-assisted laparoscopic approach turned out to be advantageous in resecting the septum to a large extent due to the associated cicatrised vagina. The use of Interceed, a novel mould and harnessing system, ensured a favourable postoperative outcome by bolstering patient motivation due to its less challenging technique of use.

Background

Müllerian anomalies being clinically less common, pose a daunting challenge to gynaecologists with respect to management. Transverse vaginal septa is a complex and rare variety of Müllerian anomaly. Its accurate incidence is not known and is thought to be between 1/2100 and 1/72 000.¹

It is quite usual for young girls with pelvic pain to approach centres that may not be equipped to manage such complex paediatric gynaecological cases. Prompt recognition and referral to a higher centre are vital in these situations.

The available knowledge and particulars in managing such a case is meagre. Determining the ideal route and technique of surgery integrated with a few idiosyncratic modifications is called for, in productively managing an uncommon condition as this.

Case presentation

We present a case of cryptomenorrhoea in an early adolescent girl who was referred to our hospital after two failed surgical explorations at other centres. At presentation, the girl complained of cyclical, lower abdominal pain, lasting for a year which had gradually worsened over the past 6 months, coupled with the absence of menstruation. She was of normal stature for her age with adequately developed secondary sexual characteristics. On local examination, external genitalia was normal. However, the vagina was as a 1 cm blind pouch.

Investigations

A transabdominal ultrasonography revealed the presence of a huge collection in the pelvis. MRI confirmed the presence of a 1.4 cm thick imperforate high transverse vaginal septum, distended endometrial and upper vaginal cavities with altered signal intensity collection T1 high and T2 low suggestive of haematometra and haematocolpos and a cicatrised vagina below (figures 1 and 2). The uterus and bilateral adnexa were normal (figure 3). A U0C0V3 Müllerian anomaly was designated to her as per the latest European Society of Human Reproduction and Embryology (ESHRE) classification.²

Figure 1.

T2 MRI image in sagittal section showing haematometra and a huge haematocolpos with a structurally normal uterus.

Figure 2.

T2 MRI image in sagittal view with the transverse vaginal septum (thickness 1.4 cm). The haematometrocolpos is seen above the septum closely abutting the rectum posteriorly.

Figure 3.

Transaxial T2 shows the dilated vagina with chronic haemorrhagic contents. The rectum is compressed and displaced to the left side.

Differential diagnosis

Some important differentials that need to be considered in one such case are cervical atresia and vaginal agenesis. Although imperforate hymen also presents with a similar picture, it is easy to identify an imperforate hymen on perineal examination.

Treatment

Due to the narrow and scarred vagina secondary to previous failed explorations and as the septum was thick and high in location, we planned a laparoscopy-assisted vaginal approach. While awaiting the scheduled surgery, she was put on continuous progestin (Dienogest) for menstrual suppression.

Surgery was performed in the lithotomy position. The bladder was catheterised. A 10 mm camera port and three 5 mm accessory ports were made. A distended lower part of the uterus and upper vagina was visualised suggesting a haematometrocolpos. Both the tubes and ovaries were normal. (figure 4). The vesicouterine fold of the peritoneum was opened and the bladder was pushed caudally to avoid injury. A 2 cm transverse incision was given on the ballooned-out vagina and around 800 mL of haematocolpos was drained (figure 5A,B). Visualised portion of the cervix appeared normal. A thick transverse septum was seen through this vaginal incision just below the cervix.

Figure 4.

Image showing a ballooned-out upper vagina secondary to haematocolpos.

Figure 5.

(A) Separation of uterovesical peritoneal fold. (B) Chocolate coloured fluid draining out of the vagina.

At the perineal end, a horizontal incision was made at the blind end of the vagina and a neovagina was created, with the rectum posteriorly and the bladder anteriorly (figure 6A).

Figure 6.

(A) A blind and cicatrised distal end of the vagina. (B) Making of the neovagina.

Sharp dissection was done with Metzenbaum scissors where fibrous tissue was encountered (figure 6b). A transrectal and a transvaginal probe was held in position to guide the excision of the septum laparoscopically using a harmonic scalpel (figure 7).

Figure 7.

Resection of the septum under the guidance of a vaginal probe.

At this point, the septum was noted to be in extremely close proximity with the rectum. This deviation in anatomy was presumed to be secondary to previously attempted procedures. In light of this, the septum was only partially excised more on the anterior aspect under the guidance of the transvaginal probe. The septum was situated at 6 cm from the introitus measured through the neovagina. The redundant edges of the septum were approximated with the proximal vagina, laparoscopically with the use of a 2–0 polyglactin suture. A Foley catheter was positioned through the resected septum and its bulb inflated to 50 mL to prevent stenosis at the level of the septum. The anterior vaginal incision that was originally made to drain the haematocolpos was approximated laparoscopically with absorbable polyglactin.

A vaginal mould made of aquaplast material measuring 6 cm in length and 2 cm in width was wrapped with two sheets of Interceed and inserted into the neovagina. The mould with its harnessing system was attached to the patient’s waist, which facilitated easy ambulation (figure 8). Urinary catheter, vaginal mould and the trans-septal Foley catheter were retained postoperatively.

Figure 8.

The novel mould with its harness (a circumferential band to fit around the waist and two supports – one from the front and the other from the back).

Outcome and follow-up

Postoperatively she was placed on complete bed rest with no oral intake for 48 hours. Clear fluids were started on postoperative day 3 and was gradually shifted to a normal diet. Adequate parenteral antibiotic coverage was provided. On postoperative day 6, ultrasound revealed no collection in the vagina. On postoperative day 8, the urinary catheter and vaginal mould were removed, while the trans-septal catheter was retained. Saline irrigation of the neovagina was done, and a new mould was positioned coated with oestrogen cream. Ambulation and daily activities were resumed. On postoperative day 13, the patient had her first menstrual cycle lasting for 3 days. Following this, a repeat ultrasound revealed no collection (figure 9). On discharge, she was advised to use the mould regularly with oestrogen cream.

Figure 9.

A postoperative transabdominal sonogram showing a normal uterus and a cervical canal.

Discussion

The success of any Müllerian abnormality correction depends on accurate diagnosis. A thorough clinical examination is always necessary, although a complete examination may not be possible in all cases. Over the last few decades, a variety of imaging modalities have come up and we are now at a point where an accurate diagnosis is possible. The initial diagnostic tool is usually the two-dimensional ultrasound (US2D) due to its ready availability and simplicity. A three-dimensional ultrasound (US3D) is slightly better than a US2D as both the sensitivity and the agreement between observers are better in a US3D. In addition, a US3D allows the evaluation of the cervix and vagina.³ However, MRI is the current gold standard as it provides high-resolution images and intricate interpretation of the uterine contour, cavity, cervix and vagina. In addition, any concomitant anomalies such as those related to the urinary tract can also be identified. Disadvantages of MRI are few but include high cost and potentially lengthy examination time relative to other imaging modalities.⁴

A new classification of Müllerian anomalies was suggested by ESHRE and the European Society for Gynecological Endoscopy in 2013, according to which the transverse vaginal septum is categorised as a subclass V3 anomaly.² Information about other aspects such as septal thickness, location and the presence of a perforation are however not part of this classification. This was overcome by Williams et al when they categorised vaginal septum as perforate and imperforate. They further categorised them as high, when the distance from the introitus to the septum was more than 6 cm, mid when it was between 3 and 6 cm and low when it was less than 3 cm. Finally, they also suggested that any septa more than 1 cm is to be designated as thick and the rest as thin. This information was widely accepted and was found to be extremely useful in choosing the best operative approach.¹ Based on these various characteristics of the septum, laparoscopic, vaginal or abdominoperineal approach of correction may be used. For mid and high septa, the laparoscopic approach is suggested, as these are highly complex and approaching such septal blindly through the vagina increases the risk of trauma to adjacent bowel and bladder.⁵ On account of this, we decided to proceed with a laparoscopic approach to drain the collection followed by a septal resection with the help of a probe in the neovagina. As a complete resection of the septum did not seem feasible, intraoperatively a decision to retain a trans-septal inflated Foleys catheter was made.

Various forms of vaginoplasty have been described in the literature. As this was a peculiar case of complete vaginal cicatrisation secondary to previous failed surgeries, the obvious choice was a modified McIndoe vaginoplasty. A skin graft is linked with higher chances of stenosis. Amnion grafts are not readily available and are notoriously connected with postoperative infections.⁶ In recent years use of a synthetic absorbable adhesion barrier for vaginoplasty has come into light. Motoyama et al and Anagani et al used Interceed for vaginoplasty in more than 10 patients and had no operative or postoperative complications.^{7 8} At par with these authors, we found vaginoplasty with Interceed to be a less invasive and an effective alternative to the conventional grafts.

A strict follow-up plan post-surgery is crucial since postoperative results depend on it. Vaginal dilations are recommended in all of these cases. Sessions should ideally commence no later than a few days after surgery and should be continued in order to maintain the established patency.^{9 10} While the regular use of vaginal mould is perceived as cumbersome, daily vaginal dilatation sessions require utmost sincerity and dedication from the patient and her family. To overcome this, we had originally designed a vaginal mould from aquaplast material with a harnessing system containing a waist belt and two perineal straps. In our experience with vaginoplasties, this novel mould and harness made continuous use of the mould much easier as it caused little restriction in daily activities.¹¹ We found similar observations in this case as well with our patient using the mould continuously for 8 months now.

In addition to the mould, we advocated liberal use of local oestrogen cream every day. Oestrogen assists in promoting re-epithelialisation, stabilising collagen synthesis and degradation and by facilitating a quicker rate of epidermal keratinocyte proliferation.¹²

Patient’s perspective.

I had never taken a sick leave from school but that changed when one day suddenly I had stomach pain. I was crouching on the floor, everybody thought I had eaten something bad, they gave me some medicine, the pain got better but the dull ache and the heaviness remained. This kept happening month after month, going to school amidst the multiple doctors’ appointments became a task. Even on normal days, I was scared when the pains might come back. Nobody told me what I had, I thought I was going to die. My mother finally decided to come to a higher centre everything was the same as before, the questions, the scan, the examination table but this time they explained to me and my mom what was going on, that my menstrual blood was not able to come out, it was getting collected and that’s why I was experiencing all the pain. I was told I would require two operations. The process of both the surgeries was difficult, I was scared but I really wanted to get better. My doctors always answered my questions, they told me about the female body, about how there were other girls who had experienced similar problems. I always felt more motivated after each visit. After my second surgery they gave me a belt to wear. It took some getting used to, and if my friends ask I tell them what my doctors have taught me. I wear it every night. On my last follow-up my doctors asked me about how I was doing at school and I told them that I hadn’t taken any more sick leaves.

Learning points.

• It is imperative to differentiate a septum from other simpler causes of obstructed menstruation before attempting correction as a suboptimal outcome can render the definitive surgery much more abstruse due to revamped anatomy.

• Failure to excise the septum completely can be overcome by positioning a trans-septal catheter to prevent stenosis and re-obstruction.

• An enormous degree of motivation and compliance from the patient is a cardinal requirement in successfully managing such a case. The choice between regular vaginal dilation and uninterrupted mould use is one that the patient can best adhere to.

• Interceed is an easy and safe alternative to amnion. A well-tailored harnessing system as described before helps in easy ambulation and conduct of daily activities while also ensuring good compliance.

Ethics statements

Patient consent for publication

Consent obtained from parent(s)/guardian(s).