Congenital Anomalies: A Colour Atlas of Ileal Neovagina (Volume 33) Narendra Malhotra, Arun Nagrath, Manju Nagrath
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IntroductionChapter 1

Vaginoplasty (Ileal Neovagina)
Vaginal agenesis is an uncommon condition, with an incidence ranging from 1 in 4,000 to 1 in 10,000. Congenital absence of uterus and vagina, also called müllerian agenesis or Mayer-Rokitansky-Küstner-Hauser (MRKH) syndrome, is the most common factor requiring creation of neovagina. Müllerian development is distinct from ovarian development and thus müllerian agenesis generally occurs without affecting the ovaries and normal external genitalia.
However, creation of neovagina may also be considered after vaginectomy, as from carcinoma, after extensive scarring from trauma or infection or in the case of male to female trans-sexual surgery.
Data in the literature estimate an incidence between 1:4,000 and 1:5,000 female live births. Associated renal anomalies may include unilateral agenesis of the kidney, ectopic kidney(s), horseshoe kidney and crossed-fused ectopia (occur in 30% of these patients).
Associated skeletal anomalies may include anomalies found in the Klippel-Feil syndrome (i.e. aberrations of the cervicothoracic somite development), which manifests as fused vertebrae or other variants. Anomalies of the ribs and limbs are also encountered.
Signs and Symptoms
A woman with this condition is hormonally normal; that is, she will enter puberty with development of secondary sexual characteristics including thelarche and adrenarche (pubic hair). Her chromosome constellation will be 46XX. Ovaries are intact and ovulation usually occurs. Typically, the vagina is shortened and intercourse may, in some cases, be difficult and painful. Medical examination supported by gynaecologic ultrasonography demonstrates a complete or partial absence of the cervix, uterus and vagina.
If there is no uterus, women with MRKH cannot carry a pregnancy. Women with MRKH typically discover the condition when, during puberty years, the menstrual cycle does not start (primary amenorrhoea). Some women find out earlier through surgeries for other conditions, such as a hernia.
Bowel Vaginoplasty
Creation of a space for the neovagina in the interlabial space lined with a split skin graft was tried (Mc Indoe, 1938; M Parikh, 2000). Fedele (1994), replaced Vecchietti's method of positioning an olive at the place of the neovagina and a traction suture passed extraperitoneally through a pfannenstiel incision lateral to the rectus muscle, attached to a device 2attached on the abdominal wall for traction, with a laparoscope. Various other methods are all exquisite ways to create a neovagina. Due to the anatomic closeness of the rectum and bladder, injuries to these organs occur now and then, and at times bleeding in the neovaginal space is a problem.
Many surgeons have used parts of large intestine like caecum and sigmoid colon to create a neovagina. The advantage claimed is the lesser incidence of scar formation and no chances of rejection (as it is autograft) and good vascular uptake. But the procedure requires laparotomy and manipulation of a normal and very essential organ structure (the large intestine), which is a major deterrent in presence of less extensive procedures with good results.
Colon, caecum or ileum may be used for bowel vaginoplasty, but the sigmoid colon is preferred over the others because it satisfies the following criteria:
Sigmoid vaginoplasty provides an aesthetically pleasing neovagina with a good length, natural lubrication and obviating the need for stenting and/or dilatation. Laparoscopic approaches for sigmoid vaginal reconstruction were tried. Karateke et al. advised that the preparation of the sigmoid flap should be individualised according to the length of the sigmoid flap and mesosigmoid together with the distribution of the sigmoid arteries and their relation with the left colic and superior rectal arteries
Sigmoid vaginoplasty is a safe and acceptable procedure to treat the patients of vaginal agenesis with acceptable cosmetic results and complication rate.
Sigmoid colon vaginoplasty is a better treatment modality because of its large lumen, thick wall resistant to trauma, adequate secretion allowing lubrication, not necessitating prolonged dilatation and short recovery time.
Vaginal reconstruction has been performed for more than a century. Main complications are vaginal stenosis requiring dilatation, dyspareunia, excessive mucus secretion, and poor aesthetic and functional outcome. Because of balanced liquid resorption and mucus secretion with sufficient vessel length in the terminal ileum, this intestinal segment was chosen. A J-pouch of distal ileum was constructed pedicle on the ileocolic artery and accompanying nervous plexus, transferred into the lower pelvis and sutured to the vaginal stump. One year follow-up showed a highly satisfied, sexually active patient, with adequate vaginal size, optimal lubrication and no molesting faecal odour. Terminal ileum J-pouch vaginoplasty is an optimal method for vaginal reconstruction providing a sufficient vaginal lumen and lubrication and thereby restoring patients’ sexual life and increasing life quality.3
Advantages and Disadvantages of Bowel Vaginoplasty
Regardless of the above, it does appear that after a few years the method of creating the neovagina is relatively unimportant since the functional reaction patterns become identical, including behaviour during arousal and orgasm, as well as lubrication.
Ileal J-Pouch Vaginoplasty: Reconstruction of a Physiologic Vagina with an Ileal J-Pouch
The distal of the transferred ileal segment is 15 cm apart from the ileocaecal junction. The ileum continuity was restored immediately by end-to-end anastomosis and the distal oral of the transplant was closed using a curved intraluminal stapler. Meanwhile, a neovaginal tract was completed to dissect from the perineum into the peritoneum and the tract widened laterally. Then the ileum transplant is reversed to reach the perineum through the peritoneal incision at the top of the neovaginal tract without subjecting the mesenteric neurovascular pedicle to undue tension and subsequent necrosis. The oral edge of the ileum transplant was sutured to the perineal skin.
The advantages of using a laparoscopic ileum colpopoiesis are that:
Ileum necrosis at donor site requiring ileum resection and bilateral ileostomy is a rare, but possible major complication. Mild stenosis responsive to finger dilatation may be encountered with sigmoid vaginoplasty. Excess mucous production, long operation time and shortness of mesentery of ileum may draw the surgeon away from adopting this technique in favour of a sigmoid vaginoplasty.
The neovaginas remain patent and functional. The additional advantages of sigmoid colon vaginoplasty as the treatment of choice may be its large lumen, thick walls resistant to trauma, adequate secretion allowing lubrication, not necessitating prolonged dilatation, short recovery time compared with ileum vaginoplasties; and in patients’ reluctance to prolonged use of dilatators or in those who experienced previous failure of the other treatment modalities.
All said and done the results of an operation of an Ileal vagina are very rewarding, less complicated than using an ileocaecal or a sigmoid colon segment with a trouble free postoperative period with a satisfactorily functioning vagina even after many years of surgery. One such case is presented.