Cecile A. Unger1 and Marie Fidela R. Paraiso1
Department of Obstetrics and Gynecology, Women’s Health Institute, Center for Urogynecology and Pelvic Reconstructive Surgery, Cleveland Clinic, Cleveland, OH, USA
Cecile A. Unger (Corresponding author)
Marie Fidela R. Paraiso
Creation of a neovagina is usually necessary in the following cases: congenital absence of the vagina, gender assignment surgery, vaginal contracture and stenosis, and reconstruction following neoplastic resective surgery or radiotherapy. While there is no standard procedure for neovaginal reconstructive surgery, there exist many surgical and nonsurgical techniques that are often used to create the vagina. These techniques include vaginal dilation methods, the McIndoe vaginoplasty procedure with the use of split-thickness skin grafts, modified McIndoe procedures using full-thickness skin and mucosal grafts, transpositional skin graft techniques, laparoscopic techniques including the Davydov and Vecchietti operations, myocutaneous and fasciocutaneous pedicled flap surgeries, and intestinal flap surgeries. The ideal reconstructive method should provide a patent vaginal canal of adequate length, width, and texture that will allow for sexual intercourse, provide a cosmetically appealing appearance with minimal morbidity of both the recipient and donor surgical sites, and have a low incidence of overall complications. Construction of the neovagina can be very complex and challenging. Each method of repair has its advantages and disadvantages, which should be carefully weighed with the desired treatment goals as well as the surgeon’s experience with various surgical techniques.
Creation of a neovagina is usually necessary in the following cases: congenital absence of the vagina, gender assignment surgery, vaginal contracture and stenosis, and reconstruction following neoplastic resective surgery or radiotherapy.
Approximately 1 in 4,000 to 1 in 10,000 female newborns are born with congenital absence of the vagina . The most common cause of this congenital malformation is Mayer–Rokitansky–Kuster–Hauser Syndrome (MRKH). The anomaly results from hypoplasia or agenesis of the mullerian duct system. The phenotype of this anomaly exists on a wide spectrum and may include partial or total vaginal agenesis with a hypoplastic or rudimentary uterus and fallopian tubes. Patients have a normal 46,XX karyotype, normal female phenotype, and normal ovarian hormonal and oocyte function. On exam, patients have normal external genitalia with a normal-appearing hymenal ring and a small vaginal pouch with a dimple. These patients often present in the setting of primary amenorrhea or once sexual intercourse proves to not be possible. Up to 10 % of patients have a functional endometrium, which can lead to cyclic pain and associated hematometra . These adolescent patients most often present in the setting of primary amenorrhea and painful hematocolpos. Concomitant urologic anomalies such as unilateral renal agenesis, pelvic or horseshoe kidney, and anomalies of the collecting duct system occur in up to 50 % of patients, while 10–15 % of patients also have skeletal anomalies .
A shortened vagina, or blind vaginal pouch, is also found in genetic syndromes such as Morris syndrome and Turner’s syndrome , as well as disorders of sex development including Androgen Insensitivity Syndrome (AIS) and Congenital Adrenal Hyperplasia (CAH). These conditions often require sex assignment surgery in either childhood or adolescence. AIS is an X-linked disorder caused by a mutation in the androgen receptor gene that leads to peripheral androgen resistance . The complete form of AIS occurs in approximately 1:20,000 of individuals who are born 46,XY with normal-appearing female genitalia but with sparse pubic hair and a blind vaginal pouch . CAH is a result of several different inherited defects of the steroid synthesis pathway. In cases of 17 α-hydroxylase deficiency, 46,XY individuals will present with normal-appearing female external genitalia, a blind short vaginal pouch, no uterus or fallopian tubes, and intra-abdominal testes . This phenotype is referred to as “complete male pseudohermaphroditism.” These patients frequently have abnormal-appearing external genitalia in addition to a shortened vagina, and their reconstructive surgical needs may be extensive.
An immediate partial or total vaginal reconstruction is frequently necessary in cases of extirpative or extensive radical pelvic surgery for cancer treatment. Anterior exenteration procedures are commonly performed for invasive bladder carcinoma , while total exenteration procedures are considered salvage operations for recurrent gynecologic cancers such as cervical, uterine, vulvar, and vaginal cancer . Exenteration procedures involve removal of the pelvic organs en bloc and result in significant tissue defects that cannot be closed primarily.
While there is no standard procedure for neovaginal reconstructive surgery, there exist many surgical and nonsurgical techniques that are often used to create the vagina. These techniques include the following:
All of these techniques will be described in detail in this chapter.
Preoperative Assessment and Planning
Patient evaluation begins with a detailed physical exam. In adolescence and young adulthood, office examination with pelvic and/or rectal exam is the first step of evaluation. In the pediatric population, vaginoscopy is the diagnostic standard for evaluating the lower reproductive tract, and is usually performed in the operating room. Patients who are suspected of having a congenital anomaly or disorder of sex differentiation should have a karyotype and hormonal evaluation. Additionally, imaging is important to help elucidate which mullerian structures are present and which are absent. T2-weighted magnetic resonance imaging (MRI) of the vagina can show a high intensity central mucosa surrounded by a low intensity submucosal layer . With a marker on the perineum, the distance of an obstructed vagina from the perineum can be determined , delineating the length of the agenesis, which is helpful for preoperative planning. MRI is also helpful in determining the presence of the uterus, cervix, and ovaries. MRI has been shown to have 100 % sensitivity and specificity in diagnosing MRKH syndrome in patients who are suspected of having vaginal agenesis on physical exam . Transabdominal ultrasonography is useful for evaluating the presence of pelvic organs as well as detecting associated urologic anomalies. Transperineal ultrasound can be used to measure the length of the agenesis as well, especially in the setting of hematocolpos. Understanding the anatomy is the key to successful neovagina reconstruction, which is why the preoperative assessment is so important.
Goals of Therapy
Timing for nonsurgical or surgical creation of a neovagina is elective; however, it is best planned when the patient is emotionally mature. Many of these surgeries are performed in adolescence or in young adulthood. Additionally, it is important to ensure that the patient has a strong support system. The best predictor of favorable emotional outcomes after diagnosis and surgery is a positive relationship between the patient and her guardians and the patient’s feeling of self-efficacy with regard to being able to share her feelings with family and friends .
The ideal reconstructive method should provide a durable, stable vaginal canal of adequate size and texture that will allow for sexual intercourse, provide a natural aesthetic appearance while simultaneously minimizing morbidity of both the recipient and donor sites, and have a low incidence of overall complications. The most preferable methods also preclude the need for subsequent long-term dilation or need for stents or obturators, as well as lubricants, and can be done in a single-stage fashion . Conservative management is an option for most patients and should strongly be considered as first-line therapy. There are also several surgical procedures that are indicated for neovaginal reconstruction. There is currently no consensus in the literature about the best approach for this type of surgery. The approach should be based on the experience of the surgeon performing the surgery, taking into consideration that the initial surgery is the most likely to be effective and that repeat surgeries may become more challenging with less successful outcomes over time .
Conservative approaches to the creation of a neovagina can be attempted in patients with congenital absence of the vagina. When vaginal development fails, a soft, pliable span of skin is usually left between the urethral meatus and the anus . Nonoperative creation of a vagina can be achieved through progressive dilation and invagination of the perineal epithelium. To achieve this, the patient must have a shallow vaginal pouch that can be stretched to lengthen the canal . Conservative therapy becomes most challenging in patients with no vagina or only a small vaginal dimple, but should still be attempted as success has been demonstrated in these patients as well . The American Congress of Obstetrics and Gynecology recommends dilation as first-line therapy for vaginal lengthening in patients with agenesis of the vagina, as it is the least invasive approach, is very safe with significantly less morbidity than surgical alternatives, is not associated with disfiguring scarring, and has highly favorable outcomes . In 1938, Frank  was the first to formally describe vaginal formation with progressive dilation by using Pyrex glass molds and applying persistent pressure to the perineum. Today, Frank’s method includes the use of silicon dilators to invaginate the vaginal pouch with progression of dilator size until a functional vagina is created. Daily dilation, usually performed in lithotomy position, is required for at least 30 min each day . The Ingram  modification of Frank’s technique involves having the patient sit on a bicycle seat stool for progressive dilation, using body weight to maintain adequate dilator pressure. This method has also showed very favorable outcomes , but is associated with more discomfort and less patient compliance. Patients should be counseled on the need for good compliance, as well as the anatomic and functional expectations once the process is complete. Anatomic and functional success rates are as high as 90 % if there is good compliance with dilator use . Roberts et al.  followed 51 patients, finding 92 % success in those adhering to a vaginal dilation regimen using Frank’s technique. Mean time to successful dilation was 11.8 months, with a range of 3–33 months. Younger patients have been found to have the poorest compliance with dilator use  as the technique is time-intensive and uncomfortable, especially at the beginning of the process. These patients especially must be counseled about the commitment required for this technique, as well as the projected length of time it will take to achieve a vagina of adequate length and caliber. Figure 18.1 is an image of vaginal dilators, showing that they come in variable lengths and sizes.
Vaginal dilators. Vaginal dilators come in variable lengths and sizes (Used with permission from Ridgeway BR, Attaran M. Embryology and Congenital Anomalies of the Urinary Tract, Rectum, and Female Genital System. In Walters MD, Karram MM eds.: Urogynecology and Reconstructive Pelvic Surgery, 4th ed. Philadelphia: Elsevier; 2014)
Physical therapy can also be used as an adjunct to vaginal dilators in the creation of the neovagina. Vaginal dilators are widely used by physical therapists for the treatment of pelvic floor disorders such as pelvic floor hypertonicity causing pelvic pain, vaginismus, vulvodynia, and dyspareunia . They are used in desensitization therapy using graded exposure with a progressive increase in size of the dilator in order to treat dyspareunia . Physical therapy using various heat modalities to make the tissues more pliable in conjunction with manual stretching by a therapist while the patients continues to use dilators on her own is associated with a shorter length of treatment to attain a functional vagina  and may be a good option for some patients and should be considered.
Surgery is indicated for patients who are unsuccessful with dilators or patients who opt for surgical management after they have been thoroughly counseled about the risks and the benefits of surgery. The patient should be counseled that surgical management with vaginoplasty is not necessarily a “quick fix” and that she will need to use vaginal dilators postoperatively to maintain her surgically created vagina. Again, the goals of therapy involve the creation of a vaginal canal that is of adequate length and caliber, in the correct axis, with some secretory capacity that will allow for sexual intercourse to occur without the need for lifelong dilation. The timing of the surgery depends on the patient and the type or procedure planned. Surgeries often are performed in late adolescence when the patient is more mature and better able to adhere to postoperative dilation or instructions .
The most common surgical procedure performed in the United States to create a neovagina is the McIndoe operation, which is commonly used to treat patients with congenital absence of the vagina. The primary goal is creation of a functional vagina. The technique involves creating a canal within the connective tissue between the bladder and the rectum and using a mold to line the vagina with a split-thickness skin graft (STSG) obtained from the patient’s thigh, inguinal region, or buttocks  followed by progressive vaginal dilator use to achieve maximal vaginal length and caliber. In order to perform this technique, the patient is placed in dorsal supine lithotomy position. Laparoscopy may be performed first, even in cases when an intraabdominal graft is not used, in order to delineate organs such as the bladder and rectum prior to commencing the rectovesical dissection from below. Next, the vaginal dimple or foreshortened vagina is identified and a 3 cm transverse incision is made across it. Dissection is then done, using mostly blunt technique, first creating two channels on either side of the median raphe of the perineum (Fig. 18.2a). Gentle pressure is applied cephalad during dissection to create the canal with a goal depth of approximately 10–12 cm (Fig. 18.2b). Care should be taken during the dissection to avoid entry into the bladder, rectum, and posterior cul-de-sac. An EEA sizer may be used in the rectum to help with dissection. Prior to dissection, a split-thickness graft is harvested and should be approximately 10 × 20 cm in size and kept moist using normal saline during the canal dissection. The graft is passed through a skin mesher, which perforates the tissue, expanding the surface area of the graft while permitting egress of blood and fluid from the surgical site postoperatively. Once dissection is complete, the graft is placed over a dilator-like mold in an “inside-out” fashion so that the external portion of the skin lies against the mold (Fig. 18.2c). Placement should be symmetric so that the tip of the mold is at the middle of the graft with the long axis of the graft draped along the long axis of the mold on both sides. The mold is then placed inside the dissected vaginal canal and the edges of the graft are then everted and sutured in an interrupted fashion to the distal opening of the neovagina using 3-0 or 4-0 delayed absorbable suture (Fig. 18.2d, e). Patients are advised about modified or complete bed rest to avoid having the mold or stent fall out and placing tension on the distal sutures. The labia majora are often sewn together over the stent to keep it in place. A foley catheter is left in place for 5–7 days postoperatively, and is removed at the time of mold or stent removal, which is done carefully during an exam under anesthesia so as to not avulse the graft from the underlying connective tissue. Patients subsequently undergo a dilation process that can last several months to a year depending upon patient and tissue compliance.
(a–e) McIndoe procedure. (a) The initial step of the McIndoe procedure involves the identification of the vaginal dimple and the creation of a 3 cm transverse incision across it. (b) Blunt dissection can be done in the space between the rectum and the bladder in order to create the canal with a goal depth of approximately 10–12 cm. (c) Once dissection is complete, the graft is placed over a dilator-like mold in an “inside-out” fashion so that the external portion of the skin lies against the mold. (d) The mold is then placed inside the dissected vaginal canal. (e) Sagittal view of the mold inside the vaginal canal (All: Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
Studies have shown excellent results after the McIndoe operation. After 12 months, Seccia et al.  found that out of 32 patients, 90 % of patients presented with complete skin graft take and 84 % reported normal sexual activity with good sensitivity. The most common postoperative complications were anxiety (~6 % of patients) related to possible pain during insertion of the dilator and keloid scarring on the donor site of the skin grafts (~3 % of patients). Klingele et al.  looked at patient satisfaction with the McIndoe procedure and reported that 79 % believed that the procedure improved their quality of life, and 91 % were sexually active. Other potential complications reported in the literature include graft rejection, contraction of the graft, hematoma, infection, fistula formation, and excess granulation tissue [26–28].
Modifications of the McIndoe
A modification to the McIndoe procedure is the technique used to open the perineum prior to dissection. A triangular-shape inferiorly based flap approximately 3–4 cm in size can be created as the initial incision, which can then be sutured to the graft placed on the stent . This method of opening can provide additional length to the neovagina, and also help create a tension-free re-approximation of the graft to the neointroitus.
A second modification to the standard McIndoe procedure is the addition of a laparoscopic intraperitoneal repair. Laparoscopically, the bladder is retrograde filled to facilitate visualization of its margins. The peritoneum is grasped at the superior edge of the bladder margin and opened and then dissected off of the underlying bladder muscularis. Dissection can be facilitated with injection of normal saline to create a hydrodissection plane. A retropubic dissection is sometimes necessary to further mobilize the peritoneum and to release the bladder from the pubic symphysis, which brings the peritoneum closer to the graft implant site. After the rectovesical dissection is complete and the mold is placed with the graft in its desired location, the peritoneal flap can be used to cover the mold. Bowel epiploica as well as the omentum, if it can be mobilized, can also be used to cover the mold.
The McIndoe procedure also has several modifications related to the type of tissue or material that is used to line the neovagina. While the split-thickness skin graft has been described as a safe and low-morbidity technique [25, 27], it has significant disadvantages. The use of a STSG can be associated with a high contracture rate, even when patients are compliant with vaginal dilation, and this can lead to inadequate vaginal length and caliber [19, 30]. Reported modifications of the McIndoe technique include replacement of the STSG with various alternatives, including autologous full-thickness skin grafts (FTSG), human amnion, peritoneum, bladder mucosal grafts, xenografts, and synthetic graft material [28, 31–33]. Techniques using FTSG are less likely to lead to neovaginal contracture and stenosis and do not require prolonged stenting [33, 34]. In addition, sebaceous and sweat glands are better preserved in these grafts, which can help with lubrication of the neovagina in some patients . Akin describes a technique using a FTSG from the inner groin areas  (Fig. 18.3a). These grafts are used in a similar fashion to the STSG used with the traditional McIndoe procedure (Fig. 18.3b). Younger patients who require neovagina reconstruction may have limited potential graft sites for FTSG harvesting. Techniques such as tissue expander placement in sites such as the bilateral groins have been described with good outcomes and limited morbidity to the donor sites . While FTSG confer many advantages when compared to STSG, the disadvantages of these grafts include skin texture mismatch and unwanted hair growth. Additionally, donor-site morbidity is slightly higher than with STSG and short-term dilation is still required with this technique.
(a, b) Modification of the McIndoe procedure. (a) Technique using full-thickness skin grafts (FTSG) from the inner groin. (b) FTSG placed over a mold (All: Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
The ideal lining for the vagina is a moist mucosa; however, there are limited donor sites for this type of graft. An option includes lining the neovaginal cavity with multiple full-thickness buccal mucosal grafts. The advantage of this type of grafting is that the neovagina is lined with mucosa, which is moist and may facilitate pleasurable intercourse. Additionally, the donor site heals well with virtually no morbidity . The use of autologous buccal mucosa to reconstruct the vagina was presented in 2003 in two separate publications. Lin et al.  used complete pieces of full-thickness harvested mucosal grafts (approximately 6–7 cm × 2–3 cm) from both cheeks to line the neovagina; each graft was expanded in size by making stab incisions throughout the grafts, which were sutured over a stent and then placed in an inside-out fashion into the dissected vesicorectal space. The stent is removed once the graft takes to the underlying tissue. Ozgenel et al.  described a similar technique of harvesting the same mucosal grafts, expanding them with stab incisions, but then dividing them into several smaller pieces to cover a larger area over the stent. Another technique involves harvesting two buccal grafts, expanding them and then mincing the grafts into tiny pieces and then spreading the micromucosa graft onto the surfaces of gelatin sponge strips which are then placed over a stent and introduced into the dissected cavity, left in place until the graft takes to the underlying tissue . Biologic grafts may also be used to line the neovagina and obviate the need for autologous tissue, which confers many advantages in terms of donor-site morbidity. Acellular dermal allografts such as Alloderm® (LifeCell Corps., Woodlands, TX), porcine dermal grafts such as Permacol® (Covidien, Mansfield, MA), and porcine intestinal submucosa grafts such as Surgisis® (Cook Medical Inc., Bloomington, IN) may be options in neovagina reconstruction. All these grafts are composed of an acellular collagen scaffold that provides a bridge for tissue incorporation and neovascularization. Research on the role of these materials for reconstruction is sparse, with the exception of Alloderm®, which has yielded successful outcomes in vulvovaginal reconstructive cases .
Tissue engineering to generate vaginal cells is being studied as an alternative approach to lining the neovagina at the time of the McIndoe procedure. Construction of a functional vagina using autologous cells expanded from a small vaginal biopsy was successful in a rabbit model . And, in 2007, Panici et al.  reported the first case of neovaginal construction using autologous in-vitro cultured vaginal tissue. A small skin biopsy can be used to culture vaginal tissue, which can be used as a graft at the time of McIndoe vaginoplasty. Early results demonstrate a vagina with normal length and depth with vaginal tissue present on biopsy . Further research is needed in tissue engineering, in its use for vaginoplasty, and long-term outcomes associated with this type of procedure.
The Williams Procedure is a vaginoplasty technique that has been employed in vaginal agenesis patients that involves creating a vulvar skin flap that is then sutured in place to create a neovagina. Williams  describes placing allis clamps on the vulvar tissue and applying gentle traction. A U-shaped incision is made extending across the perineum and up to the medial side of the labia (Fig. 18.4a). The upper edge of the incision is made 4 cm laterally and up to the level of the external urethral meatus. The skin flap is sharply dissected off of the underlying tissues creating a flap that can be mobilized inwards, creating a vaginal pouch (Fig. 18.4b). Once the tissues are mobilized, a first layer of sutures is placed between the inner skin margins using interrupted 0 delayed absorbable sutures, starting posteriorly and moving anteriorly (Fig. 18.4c). A second layer of sutures of the same material is used to approximate the subcutaneous fat and the perineal muscles (Fig. 18.4d). Lastly, the external skin is sutured with interrupted stitches (Fig. 18.4e). The Creatsas modification of the Williams vaginoplasty involves using electrocautery to open the hymen at the 3, 6, and 9 O’clock positions, which further opens the introitus and helps to create adequate vaginal caliber and prevents hemorrhage due to rupture of hymenal vessels during the first sexual intercourse . Follow-up of these patients reveals overall subjective satisfaction with vaginal lengths of 10–12 cm and widths of 4–5 cm . This procedure is considered to be superior to the McIndoe procedure as it is can be performed in less time and there is less need for postoperative vaginal dilators, which reduces the psychological impact of the treatment .
(a–e) Williams procedure. (a) The initial step of the Williams procedure is a U-shaped incision extending across the perineum and up to the medial side of the labia. (b) The skin flap is sharply dissected off of the underlying tissues creating a flap that can be mobilized inwards, creating a vaginal pouch. (c) Once the tissues are mobilized, a first layer of suture is placed between the inner skin margins using interrupted 0 delayed absorbable sutures. (d) A second layer of sutures of the same material is used to approximate the subcutaneous fat and the perineal muscles. (e) The external skin is sutured with interrupted stitches (All: Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
The Vecchietti and Davydov techniques for vaginal reconstruction were first performed as open procedures, but advances in minimally invasive surgery have allowed these procedures to be performed laparoscopically. The main advantage of a laparoscopic approach is the ability to bridge the need to perform an indicated abdominal procedure with the ease of recovery for the patient. The postoperative phase of these surgeries is usually very involved, and therefore, length of hospital stay is usually not significantly shortened, as is seen with most laparoscopic operations. However, once discharged from the hospital, patient recovery is easier as postoperative pain is less and patients are able to return to their daily activities faster.
The Vecchietti procedure involves gradual mechanical stretching of the patient’s vaginal skin to create a full-length vagina. This procedure is most appropriate for patients presenting with vaginal agenesis and no prior reconstructive surgery . In this procedure , the vesicorectal space is carefully dissected laparoscopically to reflect the bladder anteriorly. An EEA sizer can be placed inside the rectum for mobilization and visualization of the rectovesical space in order to avoid entry into the rectum at the time of dissection. A 2 cm olive-like acrylic bead is placed on the vaginal dimple and is sutured in place to the perineum. Under direct visualization with the laparoscope, a guide needle is used to pass permanent sutures through the acrylic bead and vagina and into the dissected rectovesical space in the pelvis. A guide needle is then inserted suprapubically and used to pull the sutures out of the body (Fig. 18.5a). The sutures are connected to a traction device that is secured to the patient’s abdomen (Fig. 18.5b). Sutures are tightened on a regular schedule, placing traction on the vaginal epithelium, and gradually increasing the length of the vaginal canal. Once adequate vaginal length is achieved, the traction device is removed and the sutures are cut and freed from the body. Patients are advised to practice daily dilation in order to help stretch the vaginal epithelium and maintain vaginal caliber and length during the traction phase of the procedure, and for a limited amount of time once the device is removed. As with the Frank dilation method, patient compliance with vaginal dilation and routine follow-up of the traction device are paramount to the success of the surgery. Data on long-term anatomic outcomes as well as sexual health and quality of life outcomes are favorable with this procedure .
(a, b) Vecchietti procedure. (a) Under direct laparoscopic visualization, a guide needle is used to pass permanent sutures through the acrylic bead and vagina and into the dissected rectovesical space in the pelvis. A guide needle is then inserted suprapubically and used to pull the sutures out of the body. (b) Sutures are connected to a traction device that is secured to the patient’s abdomen (a: Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved; b: Used with permission from Fedele L, Bianchi S, Berlanda N, Fontana E, Bulfoni A, Borruto F. Laparoscopic creation of a neovagina with the laparoscopic Vecchietti operation: comparison of two instrument sets. Fertility and Sterility 2006; 86(2):429-432)
The Davydov procedure  is a technique used to create a neovagina using the patient’s own peritoneum. Good candidates for this procedure include patients with disorders of sex differentiation, such as XY females, who have undergone prior feminizing genitoplasty procedures, but have had poor outcomes, or are not satisfied with vaginal length or caliber. Several modifications of the procedure exist. We recommend first making a U-shaped perineal incision to serve as a landmark for where the peritoneal edges are to be sutured later in the case. Laparoscopic dissection is then done in the rectovesical space, similar to the technique described for the Vecchietti procedure. This is also done using an EEA sizer in the rectum, to delineate the correct dissection plane. Releasing peritoneal incisions in the pouch of Douglas are then made either laparoscopically or transvaginally, freeing and mobilizing the peritoneum caudally so that it can be sutured to the previously made perineal incision. Closure of the abdominal end of the neovagina is done laparoscopically with a purse string suture (Fig. 18.6). A vaginal mold is left in place for several weeks and, once removed, it is replaced with daily dilation until maximal vaginal length is created .
Davydov procedure. The mobilized peritoneum is sutured to the perineal incision and closure of the abdominal end of the neovagina is done laparoscopically with a purse string suture (Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
There are significant advantages to these two laparoscopic procedures when compared to nonsurgical dilation methods. Lengthening of the vagina is accomplished at the time of the procedure, and does not require long-term dilation that can be very uncomfortable initially and time-consuming. The dilation that is then required postoperatively is usually much easier as the vagina has already been created and the main goal of dilation is maintenance of length and caliber. Patients tend to be very compliant with these steps. The main disadvantage is that both techniques require surgical intervention, and while they are performed in a minimally invasive fashion, require extensive dissection into the rectovesical space, which can be associated with rectal, bladder, nerve, and vascular injury. Therefore, meticulous technique is required by an experienced surgeon.
Myo- and Fascio-Cutaneous Flap Procedures
The principle of a myo- and fasciocutaneous flap is the creation of an island flap that depends on the underlying muscle or fascia for its vascular supply. The flap is made up of muscle with or without fascia or fascia alone and the overlying subcutaneous and cutaneous tissues.
Two main techniques can be described when reconstructive surgery is performed using flaps: (1) the standard local or regional flap technique which is based on a vascular pedicle that remains intact while the flap is being mobilized and (2) the more sophisticated microvascular free flap, which involves ligation of the vascular pedicle and reanastomosis to the vasculature of the recipient site. With a few exceptions, the pedicled flap is the most commonly employed flap technique for reconstruction of the neovagina and is usually used after extirpative pelvic surgery or when initial skin-graft techniques have failed in patients with congenital anomalies.
Flap orientation and dimensions of the skin paddle harvested are designed to achieve adequate perfusion through the muscular portion of the flap while achieving adequate skin for vaginal reconstruction, as well as primary donor-site closure. All pedicled flaps have their limitations in terms of the arc of rotation, the size, the tissue volume, and the restriction of mobility. These factors sometimes make it difficult to tailor the flap to the defect that needs repair . Advantages of myo- and fasciocutaneous flaps include the mobilization of a substantial amount of tissue to repair pelvic dead space while providing a source for revascularization for the surrounding tissues. The disadvantages of these flaps are that they can sometimes be very bulky which can affect cosmetic outcome and make the neovaginal cavity narrow, the skin paddles that line the vagina do not provide any lubrication for intercourse, and there can be significant morbidity from the donor site .
Rectus Abdominis Flap
Rectus abdominis musculocutaneous flaps are based off of the deep inferior epigastric vessels and can be harvested in two different orientations: transverse (TRAM) and vertical (VRAM). The VRAM flap is usually the preferred method of harvesting for exenteration procedures when stomas are created for bowel and urologic reconstruction [49, 51]. The VRAM flap can be taken from either the patient’s left or right side and is developed above the level of the arcuate line (Soper) (Fig. 18.7a). The skin paddles typically measure 12 × 8 cm in size, which is usually sufficient for the creation of a functional vagina . The horizontal dimension is usually limited by the ability to close the skin primarily; a flap width extending 2 cm lateral to the palpable edges of the rectus can be closed easily in most cases . Once the course of the deep inferior epigastric vessels is identified with Doppler, the flap is elevated from the costal margin to the level of the inguinal fold. Dissection is carried down to the rectus sheath and the lateral border is opened sharply. The skin island with the underlying subcutaneous tissue is mobilized off of the anterior rectus fascia, and the fascia is incised in a slightly smaller ellipse mirroring the skin island in order to leave a smaller fascial defect . The muscle is elevated off of the posterior fascia after the intercostal neurovascular bundles are ligated. The flap is freed superiorly by dividing the muscle at the costal margin. The superior epigastric vessels are identified and ligated. The deep inferior epigastric vessels remain as the vascular supply to the flap, and are identified inferiorly on the posterolateral surface of the muscle, crossing the lateral border of the muscle at approximately the level of the arcuate line. The flap is then elevated carefully so as to not shear the underlying branches of the pedicle. Interrupted absorbable sutures can be used to secure the muscle edges to the overlying subcutaneous tissue to prevent shearing during flap transfer  (Fig. 18.7b, c). TRAM flaps are raised in an elliptical fashion as well, below the umbilicus, from one anterior superior iliac spine to the contralateral iliac spine (Fig. 18.8a). In a similar fashion, the dissection is made underneath the anterior rectus fascia, which is preserved in a transverse orientation. The epigastric vessels are identified, and the superior vessels are ligated to allow for mobilization of the pedicled flap in a similar method used for the VRAM (Fig. 18.8b, c).
(a–c) VRAM flap. (a) Orientation of the vertical rectus abdominis musculocutaneous (VRAM) flap. (b) Mobilization of the VRAM flap on its vascular pedicle. (c) Coronal view of the mobilization of the VRAM flap on its vascular pedicle (All: Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
(a–c) TRAM flap. (a) Orientation of the transverse rectus abdominis musculocutaneous (TRAM) flap. (b) Mobilization of the TRAM flap on its vascular pedicle. (c) Coronal view of the mobilization of the TRAM flap on its vascular pedicle (All: Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
Once the flap is raised, it is folded into a tube by approximating the edges in a 2-layer closure using absorbable sutures. VRAM flaps are folded into a tube such that the proximal and distal ends of the flap form the introitus once placed in proper position . TRAM flaps are folded such that the lateral border is approximated to the medial border and the cranial edge of the flap is used to form the introitus . The tube is then mobilized into the pelvis through an opening in the posterior rectus fascia, and then brought beneath the pubic ramus, without placing tension on the pedicle. Closed suction drains are placed in the abdomen and pelvis to prevent hematoma and seroma formation. The rectus fascia at the donor site is closed with heavy running sutures and the overlying skin is closed in a manner that limits the distortion of the umbilicus.
A modification to the VRAM flap is the inferior-based VRAM flap. This flap has been shown to meet reconstructive needs in cases of vulvar and perineal defects after resective surgery. Traditional myocutaneous flaps used for reconstruction following radical vulvectomy can cover the large perineal defects but do not provide a “functional” reconstruction that can preserve anal and vaginal patency. The inferior-based VRAM flap is marked and raised in a similar fashion to the standard VRAM flap. The muscle is then split distally in the midline with care taken to avoid transection of the muscular branches of the superior epigastric artery that anastomose with the deep inferior epigastric artery, and supply important perforators to the muscle and skin of the flap. These muscular branches can easily be identified and separated on the underside of the muscle by using spreading dissection, each supplying a tongue of overlying skin and subcutaneous tissue . Division of the distal flap produces well-vascularized myocutaneous fork flaps that can be draped around the vaginal cuff and crossed inferiorly over the perineal body to create a fourchette. This also provides a skin edge for attachment of anal mucosa if extensive perianal dissection was performed.
Several series have looked at the complication rates and outcomes of rectus abdominis myocutaneous flaps [50, 52, 54, 55]. One of the larger series found that 38 % of patients developed flap-specific complications including stricture/stenosis (13 %), pelvic abscess or hematoma (6 %), and rectovaginal fistula (6 %), while only two (6 %) patients experienced complete flap loss . Donor-site complications included fascial dehiscence (5 %) and superficial separation (13 %). A major criticism of the VRAM flap is that synthetic mesh is often necessary to repair the fascial defect to prevent the risk of ventral wall hernia . However, the VRAM orientation is often preferable for certain exenteration procedures, as it allows for easier stoma and conduit creation on the contralateral side. Additionally, techniques that focus on reducing the size of the fascial defect to less than 4–6 cm in comparison to the entire flap size can significantly reduce the risk of future hernia. In their case series, Soper et al.  found that there were no significant differences between the TRAM and VRAM groups in the distribution of donor-site, recipient-site, or overall flap-specific complications; VRAM flaps were not more likely to be complicated by ventral wall hernia.
Gracilis musculocutaneous flaps are based off of the medial circumflex femoral artery. A skin flap matching the dimensions of the defect is outlined on the proximal two-thirds of the inner thigh overlying the gracilis muscle, the most superficial muscle of the inner thigh (Fig. 18.9a). Once the course of the medial circumflex femoral artery has been identified with Doppler, a full-thickness elliptical island of skin and subcutaneous tissue approximately 14–20 cm in length and 8–10 cm in width is raised. The proximal skin incision and the arc of rotation are based approximately 6–8 cm from the pubic tubercle, at the point of entry of the neurovascular bundle into the gracilis muscle . Using sharp and blunt dissection, the underlying gracilis muscle is mobilized with care taken to identify and preserve the dominant neurovascular pedicle  (Fig. 18.9b, c). Incising the fascia over the adductor magnus muscle and dissecting this fascia medially with a blunt instrument facilitates identification of the bundle. Once the muscle is completely dissected out and separated from the surrounding inner thigh muscles, it is transected proximally close to the ischial pubic ramus and then sutured to the overlying subcutaneous tissue using 3-0 or 4-0 absorbable sutures in an interrupted fashion . The raised flap is then rotated posteriorly through a subfascial perineal tunnel. If complete vaginal reconstruction is necessary, bilateral flaps are raised. Flaps are then formed into tubes by approximating the skin edges such that the distal edge of each flap becomes the apex of the neovagina. The neovaginal tube is then rotated posteriorly into the pelvic defect and the proximal skin edges approximated to the introitus. The donor site is closed in layers with synthetic absorbable suture and a closed end drain is left in place at this site.
(a–c) Gracilis flap. (a) Orientation of the gracilis musculocutaneous flap. (b) Identification of the gracilis muscle. (c) Mobilization of the gracilis muscle (All: Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
The gracilis musculocutaneous flap is commonly used to repair vaginal defects after extirpative surgery. The main advantage of the flap is that, with proper technique, it is easy to raise and also less difficult to tunnel to the vaginal or vulvar defect. However, when compared to rectus abdominis flaps, the gracilis flap has been associated with a higher rate of flap loss (14 % versus 3 %) . Casey et al.  performed one of the largest outcome studies evaluating myocutaneous flaps. They compared 41 VRAM, 13 gracilis, and 45 pudendal thigh flaps. They determined that the VRAM had the lowest overall and flap-related complication rates following complete vaginal reconstruction. However, the flap and donor-site complication rates for the gracilis and pudendal thigh flaps were acceptable enough to consider these flaps good alternatives if a VRAM flap is not possible. They also found that patient age and preoperative sexual activity were good predictors of postoperative sexual activity following vaginal reconstruction; therefore, this should be assessed preoperatively and considered when deciding upon vaginal reconstruction methods.
Posterior Thigh Flap (Fig. 18.10)
An alternative to the gracilis flap is the posterior thigh flap, based off of the inferior gluteal artery. The posterior femoral cutaneous nerve of the thigh is usually preserved at the time of this flap dissection; therefore, a portion of the flap is usually partially sensate. Friedman et al.  describe this technique. The course of the inferior gluteal artery is confirmed with Doppler and the skin island is centered over the vascular pedicle along the length of the posterior thigh. Flap dimensions are marked according to the defect needing repair. The distal most aspect of the flap should be marked a few centimeters superior to the popliteal crease, to avoid potential scar contracture deformity and wound-healing problems. Dissection is first begun at the distal aspect of the flap and carried out laterally and medially until the distal portion of the flap is reached. The flap is then elevated in continuity with the underlying fascia of the posterior compartment of the thigh, from a distal to proximal direction. Absorbable sutures are placed through the fascia and dermis to prevent shearing of the vascular pedicle and to facilitate mobilization of the flap. The flap is completely raised once it is dissected proximally to the inferior border of the gluteus maximus muscle. The flap is mobilized to the pelvis through a subcutaneous tissue tunnel, above the underlying fascia, between the posterior thigh and the adjacent defect. Bilateral flaps are usually raised and mobilized for creation of the neovagina. Once mobilized, they are tubularized and positioned in a similar fashion as the gracilis flap. Drains are placed in the pelvis and at the donor site, which is usually closed primarily. The advantages and disadvantages of the posterior thigh flap reflect those of the gracilis and rectus abdominis flaps . It remains a good alternative in cases that do not allow for the use of more common reconstructive procedures.
Posterior thigh flap. Mobilization of the posterior thigh flap, based off of the inferior gluteal artery (Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography ©2013. All Rights Reserved)
Pudendal Thigh Flap
The pudendal thigh flap is a vulvoperineal fasciocutaneous flap and is also known as the Singapore or Malaga flap, and can also be used for reconstruction of the vagina. The pudendal thigh flap is based off of the posterior labial arteries, which are a continuation of the perineal vessels, which are the terminal vessels of the internal pudendal artery. These arteries anastomose with branches of the deep external pudendal artery, the medial circumflex femoral, and the anterior branch of the obturator artery over the proximal portion of the adductor muscle. The posterior portion of this flap is innervated by the posterior labial branches of the pudendal nerve. As a result, the proximal portion of the flap often times maintains some degree of sensation. The superficial perineal nerve is often preserved as well with this flap, which adds additional sensation. The vulvoperineal skin island is marked vertically in a rectangular shape with the longitudinal axis overlapping the lateral limit of the labia majora; the base of the flap is inferior to the posterior border of the neovaginal introitus, extending from the labia majora across the groin crease to the medial thigh and measures up to 12 × 6 cm  (Fig. 18.11a). Sharp dissection is done to raise the flap starting at the superior most margins where the deep external pudendal artery branches with the superficial perineal artery. This anastomosis is ligated and the superficial perineal artery is preserved with the flap. The flap is dissected down to the level of the adductor muscles, the fascia over the muscles is raised with the flap, and the superficial perineal muscles can be identified . The flap is detached medially by transecting the insertion of the adductor aponeurosis, which inserts into the ischiopubic ramus. Bilateral flaps are usually raised and then tunneled subcutaneously under the labia majora into the previously dissected rectovesical space. The strip of flap that passes under the labia is de-epithelialized and sutured to the overlying tissue. Lastly, the medial, distal, and lateral margins of the flaps are approximated, creating a tubular pouch, and the skin edges are sutured to the neointroitus and cutaneous edges of the labia majora (Fig. 18.11b, c). The donor site is then closed primarily in several layers. Long-term follow-up of these patients shows positive anatomic and functional outcomes .
(a–c) Pudendal thigh flap. (a) Pudendal thigh flap based off of the posterior labial arteries. (b) The strip of flap that passes under the labia is de-epithelialized and sutured to the overlying tissue. (c) The medial, distal, and lateral margins of the flaps are approximated, creating a tubular pouch, and the skin edges are sutured to the neointroitus and cutaneous edges of the labia majora (All: Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
Martius Bulbocavernosus Flap
The Martius bulbocavernosus flap was first described in 1928 by Martius  and has been used frequently in the repair of complex vaginal fistulas. It can also be a useful source for the construction of a partial or complete neovagina [63–65]. For larger defects such as complete vaginectomy in the setting of total pelvic exenteration, bilateral Martius flaps can be used by tubularizing the flaps to create a complete, full-length vagina with normal caliber. When marking and mapping these flaps, the primary goal should be to create flaps that are as large as possible with the ability to close the donor site primarily without tension. This technique has been described by Green et al. . The Martius flaps should extend from the level of the clitoris superiorly to a level just above the perineal body inferiorly. The medial margin is the sulci between the labia majora and minora. The lateral margin is the lateral edge of the labia majora. The flap is sharply developed and raised, preserving the vascular pedicle, which is the posterior labial branch of the internal pudendal artery. The flaps are tunneled through the paravaginal windows into the abdomen. The portion of the flap that is to become the posterior vagina is sewn to the top of the vagina. The two flaps are joined on the posterior aspect with interrupted sutures. The anterior aspects are sewn to the vaginal cuff and then joined anteriorly, creating a tube with vulvar epithelium lining the neovagina. This method of reconstruction is an excellent choice for patients who have vulvar anatomy that is conducive to creating large flaps that can be easily mobilized. In the appropriate patient, this procedure for neovaginal reconstruction is associated with minimal blood loss, short operative time, decreased pain, and less disfigurement than other types of flaps and has very favorable anatomic outcomes .
While the greater omentum-pedicled graft is not a musculocutaneous flap, it has been extensively used for coverage of perineal and other soft-tissue defects but has also been used successfully in vaginal reconstruction ; for these reasons, it is worth mentioning. Case series have shown that the flap can be used in conjunction with an absorbable graft such as a vicryl mesh that is first positioned into proper location using a vaginal stent and sutured to the remaining edges of either the posterior or anterior vagina. The omentum-pedicled graft is based off of the left gastro-epiploic artery, which is mobilized down along the left paracolic gutter, and then draped over all parts of the mesh and sutured to the graft  (Fig. 18.12). While this type of flap is not commonly used for vaginal reconstruction, it may confer important benefits. The omentum has a rich vascular supply and it unlikely to necrose, even after mobilization. Additionally, it is easily mobilized without significant morbidity. Lastly, the flap itself is much less bulky than other commonly used myocutaneous flaps and may have better cosmetic results. Its major disadvantage is that a mesh graft may be necessary for placement as there are no reported cases of omental flaps used for vaginal reconstruction without the use of a mesh graft. There are currently no studies examining the safety of synthetic nonabsorbable grafts in neovaginal reconstruction with omentum-pedicled flaps.
Omentum-pedicled flap. Based off of the left gastro-epiploic artery, which is mobilized down along the left paracolic gutter to the site of reconstruction (Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
Different bowel segments, including the ileum, jejunum, cecum, and sigmoid colon, have been used for neovaginal reconstruction. The procedure involves resecting a segment of the bowel approximately 10–12 cm in length with its vascular pedicle intact, reanastomosing the bowel, mobilizing the resected segment into the pelvis, suturing the edges to the obliterated vagina after creation of a proper space, and suturing to close the proximal end of the segment, forming a pouch  (Fig. 18.13a–d). The main advantages of intestinal flaps are that the risk of contracture and stenosis is significantly reduced, molds or stents are not required to ensure patency, and lubrication is not a problem, facilitating intercourse . Procedures are usually performed via a laparotomy; however, there are some case reports and series describing laparoscopic techniques . The most significant disadvantages of intestinal flaps include the morbidity of laparotomy such as infection and wound dehiscence, shrinkage with intestinal stenosis, anastomotic dehiscence, possible need for colostomy, and persistent copious secretion of colonic mucus. These procedures are more complicated than other neovaginal procedures and are limited to surgeons with a very unique skill set.
(a–d) Creation of a neovagina with an intestinal flap. (a) A segment of the bowel is resected with its vascular pedicle intact. (b) A segment of the bowel is resected with its vascular pedicle intact. (c) The bowel is reanastomosed and the resected portion is mobilized into the pelvis. (d) A sagittal view of the bowel flap sutured to the obliterated vagina, creating a neovaginal pouch (All: Reprinted with permission, Cleveland Clinic Center for Medical Art & Photography © 2013. All Rights Reserved)
The sigmoid is the most commonly used piece of colon for neovaginal reconstruction because of its location in the pelvis and ease of mobilization . Its vascular supply consists of the sigmoidal arteries that branch off of the inferior mesenteric artery. In a large series, Kwun Kim et al.  showed that the sigmoid neovagina achieved a very low contraction rate, was able to maintain adequate vaginal length and width without the use of stenting, mucous secretion helped with sexual intercourse, there was low incidence of malodor, and patients were satisfied with the cosmetic appearance of the reconstruction. Additionally, sexual satisfaction has been reported to be as high as 78 % after sigmoid vaginoplasty . While these cases have been done laparoscopically , the vast majority are performed via laparotomy, which has its risks and associated comorbidities. In addition, there are reports of diversion colitis, ulcerative colitis, patient dissatisfaction with copious malodorous neovaginal discharge, prolapse, flap failure, and defecatory dysfunction . While very rare, primary adenocarcinoma of the colon has been reported in the sigmoid neovagina as well .
The ileum and cecum have both been used successfully in the creation of the neovagina and are based off of the ileal branches of the superior mensenteric artery (SMA). The main advantage of these flaps is that there is excellent blood supply to those portions of the bowel and the vascular pedicle is long enough to be mobilized to the pelvis. Additionally, reports show that there is the least mucous production of all of the intestinal flaps and therefore, there is less vaginal discharge . The main disadvantage of the ileal flap is that compared to the jejunum and sigmoid, the ileum wall is much more fragile and delicate and more likely to sustain trauma with subsequent bleeding at the time of mobilization and is also associated with a higher rate of stenosis and intestinal obstruction . Similar to the sigmoid flaps, there are reported cases of laparoscopic ileal vaginoplasty, which have been successful and confer the advantage of less morbidity .
This pedicled flap is also based off of a branch of the SMA. The jejunum has a smaller lumen than the rectosigmoid and can provide favorable cosmetic and functional outcomes . Another significant advantage over the sigmoid flap is that there is less dissection needed for mobilization and patients report significantly less defecatory dysfunction . A disadvantage of the flap is copious intestinal secretion but this can be rectified by performing the surgery as a free flap procedure with anastomosis rather than as a pedicled flap procedure, as temporary ischemia of the flap during mobilization leads to decreased mucous production . When a free flap procedure is performed, the SMA branches that are mobilized with the flap are anastomosed using a microvascular technique to the inferior epigastric vessels. There are fewer laparoscopic cases that have been reported, as mobilization of the jejunum is done most easily via a laparotomy, and is obligatory when a free flap technique is employed.
Prolapse of the Neovagina
There are few reported cases of vaginal vault prolapse in women who have undergone neovaginal reconstruction for vaginal agenesis [75–78]. In seven previous case reports, four cases of vaginal vault prolapse occurred after mechanical dilation and three occurred after surgical management with either the McIndoe or Williams procedures. The vagina is composed of three segments: upper and middle and lower. The upper and middle segments are derived from the mullerian ducts and are suspended to the surrounding structures by connective tissue fibers to the sacrum and fascia of the pelvic sidewall, respectively. The lower segment of the vagina is derived from the urogenital sinus and is fused to the perineum as well as the fascia of the levator ani muscles and perineal body. Patients with mullerian agenesis are born without the fibrous connections of the upper and middle segments of the vagina. In these patients, the vagina is a blind pouch that opens at the introitus. A mechanically created neovagina or a surgically constructed one lacks the fibrous supports that suspend the vagina to the bony pelvis, and as a result, are at risk for prolapse of the vaginal apex and its lateral supports. Based on existing published case reports, successful outcomes can be achieved with vaginal vault prolapse surgeries. Abdominal sacrocolpopexy with synthetic mesh and paravaginal repair has been performed successfully on patients with prolapse of the neovagina . There is also a case report showing a successful sacrospinous ligament colpopexy used in the case of vaginal vault prolapse in a MRKH patient . In this particular case, the vagina was not long enough to be directly sutured to the sacrospinous ligament, and so, a fascia lata allograft was sutured to the anterior and posterior vaginal walls and then secured to the right sacrospinous ligament to help bridge the gap between the vaginal apex and ligament. The prevalence of vaginal vault prolapse in vaginal agenesis patients who have undergone neovaginal construction is not known. However, as we continue to perform reconstructive neovaginal procedures for these patients, prolapse procedures for some patients will continue to be necessary.
Patients with congenital anomalies of the vagina usually present in adolescence while children with sex differentiation disorders present with an intersex phenotype in the early period of life. Once these disorders are identified, proper evaluation is required in order to identify all anatomic abnormalities and to help with accurate diagnosis. Evaluation includes all or some of the following: office physical exam and exam under anesthesia if necessary, karyotype, hormonal panel, and imaging with MRI and/or ultrasonography. Once this information is obtained, proper management planning can be done. Treatment takes place once the patient is mature enough to understand their disorder, to commit to the treatment plan, and as long as adequate social supports are in place. First-line management for patients with vaginal agenesis is any version of Frank’s method of progressive vaginal dilation. It should be the first therapeutic procedure because it is the least invasive and has fewer serious complications.
If this method fails to create a proper vagina, or patients decline this method, surgical neovaginal construction can be performed. The surgical option often depends on the experience and preference of the surgical team, and sometimes requires a multidisciplinary approach with gynecologists, pediatric surgeons, urologists, and plastic surgeons. The technique most often employed for patients with vaginal agenesis or disorders of sex differentiation is the McIndoe operation, a modification of this surgery, or the Williams vaginoplasty. Laparoscopic techniques such as the Vecchietti and Davydov operations are also minimally invasive options for patients and have yielded favorable outcomes. For failed procedures or more complex reconstruction, various flap procedures can be performed. Many factors direct the type of flap that is used. These include the size and type of defect that needs to be repaired, the availability of certain flaps, the morbidity associated with flap harvesting and repair of the donor site, and the number and types of prior reconstructive procedures the patient has already had. Patients with vaginal agenesis or disorders of sex differentiation require construction of a patent vaginal tube that is functional and cosmetically appealing. Patients who have undergone resective surgeries or have undergone radiation therapy for malignancy have these same requirements, in addition to larger vulvar and perineal defects that require repair. Many different flaps have been described in this chapter, including those that are musculo- and fasciocutaneous and intestinal. The majority of these flaps are pedicle-based and rotational in nature and do not require microvascular surgery. Additionally, the use of biologic allografts and tissue engineering to create the neovagina was briefly discussed and may be promising minimally invasive techniques for the future, but need to be investigated further. The ideal reconstructive method should provide a patent vaginal canal of adequate length, width, and texture that will allow for sexual intercourse, provide a cosmetically appealing appearance with minimal morbidity of both the recipient and donor surgical sites with a low incidence of overall complications.
Reconstruction of the neovagina can be very complex and challenging. Each method of repair has its advantages and disadvantages, which should be carefully weighed with the desired treatment goals as well as the surgeon’s experience with various surgical techniques.
Evans TN, Poland ML, Boving RL. Vaginal malformations. AJOG. 1981;141(8):910–7.
Silber CG, Magness RL, Farber M. Duplication of uterus with non-communicating functional horn. Mt Sinai J Med. 1990;57:374–7.PubMed
Griffen JE, Edwards C, Madden JD, Harrod MJ, Wilson JD. Congenital absence of the vagina. The Mayer-Rokitansky-Kuster-Hauser syndrome. Ann Intern Med. 1976;85(2):224.CrossRef
Seccia A, Salgarello M, Sturla M, Loreti A, Latoree S, Farallo E. Neovaginal reconstruction with a modified McIndoe technique: a review of 32 cases. Ann Plast Surg. 2002;49(4):379–84.PubMedCrossRef
Burgu B, Duffy PG, Cuckow P, Ransley P, Wilcox DT. Long-term outcome of vaginal reconstruction: comparing techniques and timing. J Pediatr Urol. 2007;3:316–20.PubMedCrossRef
Ozkan O, Akar EM, Ozkan O, Dogan NU. Reconstruction of vaginal agenesis. Ann Plast Surg. 2011;66(6):673–8.PubMedCrossRef
Marshall FF. Radical cystectomy in the female. AUR Update Ser. 1997;27:1–8.
Eifel PJ, Berek JS, Thigpen JT. Cancer of the cervix, vagina and vulva. In: DeVita VT, Hellman S, Rosenberg SA, editors. Cancer principles and practice of oncology. 5th ed. Philadelphia: Lippincott Williams & Wilkins; 1997. p. 1433–78.
Economy KE, Barnewolt C, Laufer MR. A comparison of MRI and laparoscopy in detecting pelvic structures in cases of vaginal agenesis. J Pediatr Adolesc Gynecol. 2002;15:101–4.PubMedCrossRef
Church DG, Vancil JM, Vasanawala SS. Magnetic resonance imaging for uterine and vaginal anomalies. Curr Opin Obstet Gynecol. 2009;21:379–89.PubMedCrossRef
Poland ML, Evans TN. Psychologic aspects of vaginal agenesis. J Reprod Med. 1985;30:340–4.PubMed
Laufer MR. Congenital absence of the vagina: in search of the perfect solution. When, and by what technique, should a vagina be created? Curr Opin Obstet Gynecol. 2002;14:441–4.PubMedCrossRef
Lappohn RE. Congenital absence of the vagina: results of conservative treatment. Eur J Obstet Gynecol Reprod Biol. 1995;59:183–6.PubMedCrossRef
Williams EA. Congenital absence of the vagina. J Obstet Gynaecol Br Commonw. 1972;79:1147–8.PubMedCrossRef
Gargollo PC, Cannon GM, Diamond DA, Thomas P, Burke V, Laufer MR. Should progressive perineal dilation be considered first line therapy for vaginal agenesis. J Urol. 2009;182:1882–91.PubMedCrossRef
Nonsurgical diagnosis and management of vaginal agenesis. ACOG Committee Opinion. No. 274, July 2002. Obstet Gynecol. 2002;100(1):213–7.
Frank RT. The formation of an artificial vagina without operation. AJOG. 1938;35:1053–5.
Ingram JM. The bicycle seat stool in the treatment of vaginal agenesis and stenosis: a preliminary report. AJOG. 1981;140:867–73.
Roberts CP, Haber MJ, Rock JA. Vaginal creation for mullerian agenesis. AJOG. 2001;185:1349–52.CrossRef
Klingele CJ, Gebhart JB, Croak AJ, DiMarco CS, Lesnick TG, Lee RA. McIndoe procedure for vaginal agenesis: long-term outcomes and effect on quality of life. AJOG. 2003;189:1569–73.CrossRef
Bo K, Berghmans B, Morkved S, Van Kampen M. Evidence-based physical therapy for the pelvic floor: bridging science and clinical practice. 1st ed. Philadelphia, PA: Elsevier; 2007. p. 263–4.
McGuire H, Hawton KKE. Interventions for vaginismus. Cochrane Database Systematic Reviews [Internet] 2003 [cited 12 Dec 2012] Available from http://www.mrw.interscience.wiley.com/cochrane/clsysrev/articles/CD001760/frame.html
McVearry ME, Warner WB. Use of physical therapy to augment dilator treatment for vaginal agenesis. FPMRS. 2011;17(3):153–6.
Vaginal agenesis: diagnosis, management, and routine care. ACOG Committee Opinion No. 355. Obstet Gynecol. 2006;108:1605–9.
McIndoe AH, Banister JB. An operation for the cure of congenital absence of the vagina. J Obstet Gynecol Br Emp. 1938;45:490–6.CrossRef
Farber M, Mitchell GW. Surgery for congenital absence of the vagina. Obstet Gynecol. 1978;51:364–6.PubMedCrossRef
Buss JG, Lee RA. McIndoe procedure for vaginal agenesis: results and complications. Mayo Clin Proc. 1989;64:758–62.PubMedCrossRef
Martinez-Mora R, Isnard R, Castellvi A, Lopez OP. Neovagina in vaginal agensis: surgical methods and long-term results. J Pediatr Surg. 1992;27:10–4.PubMedCrossRef
Akin S. Experience with neovaginal construction using the full-thickness skin graft in vaginal agenesis. Ann Plast Surg. 2004;52(4):391–6.CrossRef
Ozgenel GY, Ozcan M. Neovaginal construction with buccal mucosal grafts. Plast Reconstr Surg. 2003;111(7):2250–4.CrossRef
Ashworth MF, Morton KE, Dewhurst J, Lilford RJ, Bates RG. Vaginoplasty using amnion. Obstet Gynecol. 1986;67:443–6.PubMed
Jackson ND, Rosenblatt PL. Use of interceed absorbable barrier for vaginoplasty. Obstet Gynecol. 1994;84:1048–50.PubMed
Sadove RC, Horton CE. Utilizing full-thickness skin grafts for vaginal reconstruction. Clin Plast Surg. 1988;15:443–8.PubMed
Chen YB, Cheng T, Lin HH, Yang YS. Spatial W-plasty full-thickness skin graft for neovaginal reconstruction. Plast Reconstr Surg. 1994;94:727–31.PubMedCrossRef
Alessandrescu D, Peltecu GC, Buhimschi CS, Buhimschi IA. Neocolpopoiesis with split-thickness skin graft as a surgical treatment of vaginal agenesis: retrospective review of 201 cases. AJOG. 1996;175:131–8.CrossRef
Loveless M, Gutman R, Cundiff G. Tissue expanders optimize harvesting full-thickness skin grafts in select patients undergoing McIndoe neovagina. J Pelvic Med Surg. 2008;14(3):185–90.CrossRef
Zhao M, Li P, Li S, Li Q. Use of autologous micromucosa graft for vaginoplasty in vaginal agenesis. Plast Reconstr Surg. 2009;63(6):645–9.
Lin WC, Chang CY, Shen YY, Tsai HD. Use of autologous buccal mucosa for vaginoplasty: a study of eight cases. Hum Reprod. 2003;18:604–7.PubMedCrossRef
Stany MP, Sunde J, Bidus MA, Rose GS, Elkas JC. The use of acellular dermal allograft for vulvovaginal reconstruction. Int J Gynecol Cancer. 2010;20:1079–81.PubMedCrossRef
DePhilippo RE, Bishop CE, Filho LF, Yoo JJ, Atala A. Tissue engineering a complete vaginal replacement from a small biopsy of autologous tissue. Transplantation. 2008;86:208–14.CrossRef
Panici PB, Bellati F, Boni T, Francescangeli F, Frati L, Marchese C. Vaginoplasty using autologous in vitro cultured vaginal tissue in a patient with Mayer-von-Rokitansky-Kuster-Hause syndrome. Hum Reprod. 2007;22:2025–8.PubMedCrossRef
Williams EA. Congenital absence of the vagina; a simple operation for its relief. J Obstet Gynecol Commonw. 1964;71:511–4.CrossRef
Creatsas G, Deligeoroglou E, Makrakis E, Kontoravdis A, Papadimitriou L. Creation of a neovagina following Williams vaginoplasty and the Creatsas modification in 111 patients with Mayer-Rokitansy-Kuster-Hauser syndrome. Fertil Steril. 2001;76(5):1036–40.PubMedCrossRef
Deligeoroglou E, Makrakis E, Papagianni V. The Creatsas modification of Williams vaginoplasty. J Gynecol Surg. 2003;19(3):121–7.CrossRef
Ismail IS, Cutner AS, Creighton SM. Laparoscopic vaginoplasty: alternative techniques in vaginal reconstruction. BJOG. 2006;113:340–3.PubMedCrossRef
Vecchietti G, Ardillo C. La sindrome di Rokitanski-Kuster-Hauser fisiologia e clinica. Aplasia Vaginale. Corni uterini rudimentali. SEU Roma. 1970;320–7.
Fedele L, Bianchi S, Frontino G, Fontana E, Restelli E, Bruni V. The laparoscopic Vecchietti’s modified technique in Rokitansky syndrome: anatomic, functional, and sexual longterm results. AJOG. 2008;198:377.e1–6.
Crouch NS, Creighton SM. Minimal surgical intervention in the management of intersex conditions. J Pediatr Endocrinol Metab. 2004;17:1591–6.PubMedCrossRef
Ninkovic M, Dabernig W. Flap technology for reconstructions of urogenital organs. Curr Opin Urol. 2003;13(6):483–8.PubMedCrossRef
Casey III WJ, Tran NV, Petty PM, Stulak JM, Woods JE. A comparison of 99 consecutive vaginal reconstructions: an outcome study. Ann Plast Surg. 2004;52:27–30.PubMedCrossRef
Soper JT, Havrilesky LJ, Secord AA, Berchuck A, Clarke-Pearson DL. Rectus abdominis myocutaneous flaps for neovaginal reconstruction after radical pelvic surgery. Int J Gynecol Cancer. 2005;15:542–8.PubMedCrossRef
Smith H, Genesen M, Runowicz C, Goldberg GL. The rectus abdominis myocutaneous flap. Cancer. 1998;83:510–20.PubMedCrossRef
Hui K, Zheng F, Pickus E. Modification of the vertical rectus abdominis muscylocutaenous (VRAM) flap for functional reconstruction of complex vulvoperineal defects. Ann Plast Surg. 2003;51(6):556–60.PubMedCrossRef
Pursell SH, Day RG, Tobin GR. Distally based rectus abdominis flap for reconstruction in radical gynecologic procedures. Gynecol Oncol. 1990;37:234–8.PubMedCrossRef
Carlson JW, Carter JR, Saltzman AK, Carson LF, Fowler JM, Twiggs LB. Gynecologic reconstruction with a rectus abdominis myocutaneous flap: an update. Gynecol Oncol. 1996;61:364–8.PubMedCrossRef
Bakx R, Van Lanschot JJB, Zoetmulder FAN. Inferiorly based rectus abdominis flaps in surgical oncology: indications, technique and experience in 37 patients. J Surg Oncol. 2004;85:93–7.PubMedCrossRef
Soper JT, Secord AA, Havrilesky LJ, Berchuck A, Clarke-Pearson DL. Comparison of gracilis and rectus abdominis myocutaenous flap neovaginal reconstruction performed during radical pelvic surgery: flap-specific morbidity. Int J Gynecol Cancer. 2007;17:298–303.PubMedCrossRef
McGraw JB, Massey FB, Shanklin KD, Horton CE. Vaginal reconstruction with gracilis myocutaneous flaps. Plast Reconstr Surg. 1976;58:176–83.CrossRef
Friedman JD, Reece GR, Eldor L. The utility of the posterior thigh flap for complex pelvic and perineal reconstruction. Plast Reconstr Surg. 2010;126(1):146–55.PubMed
Giraldo F, Solano A, Mora M, Abehsera M, Gonzalez C, Rus JA. The Malaga flap for vaginoplasty in the Mayer-Rokitansy-Kuster-Hauser syndrome: experience and early-term results. Plast Reconstr Surg. 1996;98(2):305–12.PubMedCrossRef
Ganatra MA, Ansari NR. Pudendal thigh flap for congenital absence of vagina. J Pak Med Assoc. 2005;55(4):143–5.PubMed
Martius J. Operations for urinary incontinence. In: McCall M, Bolten KA, editors. Operative gynecology. Boston: Little, Brown; 1956.
Green AE, Escobar AF, Neubaurer N. The martius flap revisited. Int J Gynecol Cancer. 2005;15:964–6.PubMedCrossRef
Crawley WA, Grumbine FC, Dorsey JH. Flay reconstruction of the stenotic vagina. J Am Coll Surg. 1994;178:47–8.PubMed
Hatch KD. Construction of a neovagina after exenteration using the vulvobulbocavernosus myocutaneous graft. Obstet Gynecol. 1984;63:110–4.PubMed
Esrig D, Freeman JA, Stein JP, Elmajian DA, Lytton B, Skinner DG. New technique of vaginal reconstruction following anterior exenteration. Urology. 1997;49:768–71.PubMedCrossRef
Ellaffandi AH, Khalil HH, Aboul Kassem HA, El Sherbiny M, El Gemeie EH. Vaginal reconstruction with greater omentum-graft combined with a vicryl mesh after anterior pelvic exenteration. Surgical approach with long-term follow-up. Int J Gynecol Cancer. 2007;17:536–42.CrossRef
Davies MC, Creighton SM. Vaginoplasty. Curr Opin Urol. 2007;17:415–8.PubMedCrossRef
Wu JX, Li B, Liu T, Li WZ, Jiang YG, Liang JX, Wei CS, Hu HO, Zhong CX. Eighty-six cases of laparoscopic vaginoplasty using an ileal segment. Chin Med J. 2009;122:1862–6.PubMed
Li B, Wang J, Wu JX, Wang LY. Clinical analysis of vaginoplasty with sigmoid colon by laparoscopic surgery. Zhonghua Fu Chan Ke Za Zhi. 2009;44:673–5.PubMed
Kwun Kim S, Hoon Park J, Cheol Lee K, Min Park J, Tae Kim J, Chan KM. Long-term results in patients after rectosigmoid vaginoplasty. Plast Reconstr Surg. 2003;112:143–51.PubMedCrossRef
Hensle TW, Shabsigh A, Shabsigh R, Reiley EA, Meyer-Bahlburg HF. Sexual function following bowel vaginoplasty. J Urol. 2006;175:2283–6.PubMedCrossRef
Hiroi H, Yasugi T, Matsumoto K, Fujii T, Watanabe T, Yoshikawa H, Taketani Y. Mucinous adenocarcinoma arising in a neovagina using the sigmoid colon thirty years after operation: a case report. J Surg Oncol. 2001;77:61–4.PubMedCrossRef
Chen HC, Chana JS, Feng GM. A new method for vaginal reconstruction using a pedicled jejunal flap. Ann Plast Surg. 2003;51(4):429–31.PubMedCrossRef
Ramaswamy S. Mullerian agenesis with vaginal prolapse. Case report. BJOG. 1986;93:640–1.CrossRef
Peters WA, Uhlir JK. Prolapse of a neovagina created through self-dilatation. Obstet Gynecol. 1990;76:904–6.PubMed
Schaffer J, Fabricant C, Carr B. Vaginal vault prolapse after surgical and nonsurgical treatment of Mullerian agenesis. Obstet Gynecol. 2002;99(5):947–9.PubMedCrossRef
Muir TW, Walters MD. Surgical management of vaignal vault prolapse in a woman with a neovagina and pelvic kidneys. Obstet Gynecol. 2004;104(5,2):1199–201.