eMedicine Specialties > Pediatrics: Surgery > Gynecology
Vaginal Atresia: Treatment
Updated: Dec 11, 2008
Treatment
Medical Therapy
The role of medical management of vaginal atresia is limited. In patients with functioning endometrium and an obstructed outflow tract, temporary hormonal manipulation of the menstrual cycle may be necessary until a patent genital tract can be created.
In 1938, Frank described a nonsurgical technique developed especially for patients with Rokitansky-Mayer-Küster-Hauser (RMKH) syndrome.12 In this clinical situation, absence of the uterus and proximal vaginal tract obviates complex reconstructive operations. The goal is to create a blind-ending vaginal pouch. The technique involves forceful dilation of a shallow rudimentary vaginal pit with the sequential application of progressively wider and longer dilators. In 1980, Hendren and Donahoe reported that 7 of 8 patients treated with this technique achieved a pouch within 2-4 months.13 In another study, vaginal dilation was attempted in 37 of 51 patients with vaginal agenesis, with a 92% success rate.14
Success depends on the presence of at least a vaginal dimple and requires a highly motivated patient who, wishing to avoid extensive surgical procedures, is willing to continue long-term dilation. Problems of stenosis dyspareunia (20%), and decreased vaginal lubrication have made this option unattractive for many patients.
Surgical Therapy
As for other congenital anomalies, the multiple reconstructive options available for vaginal reconstruction indicate that no single technique has gained uniform approval.15,16 Pediatric surgeons, gynecologists, and plastic surgeons have traditionally contributed to the literature on the subject, and specialists in each field continue to be involved in the treatment of patients with vaginal atresia. A multidisciplinary approach is recommended to best meet the challenge of providing good cosmetic results with optimal functional and reproductive outcomes.
The various surgical approaches can be classified according to the tissues used to create a neovagina. Reconstruction involves the use of extra-abdominal tissues or tissues from the abdominal cavity.
Reconstruction by using extra-abdominal tissues
Split-thickness skin grafts are the basis for many reconstructive procedures, such as the Abbe-McIndoe operation. With this procedure, a split-thickness skin graft is taken from the buttock and used to create the neovagina. Afterward, a mold is inserted to shape the vagina. Advantages are related to the low morbidity and mortality rates of this type of reconstruction. Disadvantages include the tendency to develop vaginal stenosis, the lack of natural lubrication, the development of fistulas, and the need for continuous dilation.
Musculocutaneous flaps provide a reliable means of creating a neovagina.17 Although they provide a sensate surface, they are bulky. The rectus and gracilis muscles are the primary muscle groups used. As with fasciocutaneous and subcutaneous pedicled flaps, operations involving these flaps tend to cause scarring and use hair-bearing skin.18 Bilateral flaps have been used to create a neovagina for vaginal atresia, but they are primarily reserved for reconstruction after ablative oncologic surgery. A unilateral flap is often appropriate for reconstruction after repair of rectoneovaginal fistulas or urethral defects.
In vulvovaginoplasty, tissue expanders are used to increase the available skin from the labia. This tissue is used to create a posteriorly directed pouch. Functional assessment reportedly has yielded unsatisfactory results. Several groups report the use of amnion as a homograft, without evident rejection. This technique maintains a vaginal space for future dissection, but it has not gained wide acceptance.
Reconstruction by using tissues of the abdominal cavity
Intestinal segments, typically derived from the sigmoid colon,19,20 provide notable advantages over the skin grafts used in the Abbe-McIndoe operation. Sections of the ileum, cecum, and rectosigmoid colon have also been used. The advantages are principally related to the distensibility and self-lubricating nature of the conduit and the reduced natural contraction resulting in neovaginal stenosis, which allow the patient to avoid the discomfort of long-term use of dilators. Disadvantages are related to the potential complications encountered in laparotomy and bowel resection and to the continuous production of mucous secretions that may require the use of an absorptive pad or tampon.
The peritoneum has been used to maintain a cavity until the surfaces become epithelialized.
Laparoscopic Vecchietti procedure is gaining acceptance in the management of hymenal atresia.21 This procedure aims to create a neovagina by invagination by using an acrylic olive that is placed against the vaginal dimple. The olive is attached to a traction device mounted on the abdomen with laparoscopically placed subperitoneal sutures. Then, traction is applied to the olive to produce 1-1.5 cm of invagination per day, creating a neovagina in approximately 7-9 days. After the neovagina is created, active dilation is required until regular sexual activity is started.
One advantage of this technique over the Frank procedure is that uninterrupted traction is applied. In addition, prolonged hospitalization is unnecessary because the traction can be completed on an outpatient basis. However, this is a relatively new technique, and the long-terms results have yet to be reported.
Another laparoscopic approach is an adaptation of the Davydov procedure. This technique is characterized by a 3-stage operation that includes dissection of the rectovesical space, abdominal mobilization of the peritoneum to create the vaginal fornices, and attachment of the peritoneum to the introitus. With use of the laparoscopic approach, the abdominal end of the neovagina is closed with a purse-string suture. This laparoscopically assisted operation lowers the rate of intraoperative complications, shortened operating times and hospital stays, and minimizes external scars. After surgery, sexual function (composite score for desire, arousal, lubrication, orgasm, satisfaction, pain) approaches that of matched controls without gynecologic disorders.
At present, no consensus has been reached regarding the ideal method for creating a functional vagina. At present, the most common operation is McIndoe vaginoplasty. Relatively noninvasive laparoscopic surgery seems promising. However, additional data about results and long-term complications are required for it to gain general acceptance.
Preoperative Details
Psychological preparation of the patient is paramount. A well-established patient-physician relationship must be established.
Standard mechanical and antibiotic bowel preparation is appropriate when colon resection is contemplated for vaginal reconstruction. When extra-abdominal tissues are used for reconstruction, preoperative use of enemas may be prudent to evacuate the rectum. Perioperative antibiotics should be provided.
Intraoperative Details
Abbe-McIndoe operation
With the patient in the lithotomy position, an H -shaped or Z -shaped incision is made on the perineum. Sharp and blunt dissection of the tissues interposed between the urinary tract and the rectum results in a cavity 10-12 cm in length. A Foley catheter in the urethra guides the dissection away from this structure. The proximity to the rectum is best gauged by placing an examining finger or Hegar dilator in the rectum.
A split-thickness skin graft is harvested from the inner thigh or buttocks. Although a full-thickness graft tends to limit contraction, it transfers undesirable epithelial appendages. The authors know of no controlled prospective evaluation performed to determine the benefits of meshed grafts versus unmeshed grafts to guide this decision.
A suitably shaped piece of polyethylene foam measuring approximately 5 cm in width and 15 cm in length serves as a mold for the neovagina. This is covered with adhesive plastic. The skin graft is sutured to the mold using catgut, with the skin surface facing the obturator. This construct is inserted into the soft-tissue pouch. The Foley catheter is left in place, and a T-binder holds the device stable. The retainer is removed after 7-10 days, by which time the graft should have taken. However, the retainer must be replaced with a silicone retainer, which must to be worn for at least 6 months. Sexual activity can begin after the epithelial cells form a lining in the cavity.
Correction of vaginal atresia using tissue from the intestine
The patient is placed in a lithotomy position to allow access to the abdomen and perineum. The abdomen is entered through a Pfannenstiel incision, and the status of the uterine remnant is evaluated. For the patient with müllerian agenesis, rudimentary uterine horns may be present with remnant fallopian tubes. The value of these structures, in terms of subsequent fertility, must be determined.
The ovaries are typically normal and undisturbed. Patients with androgen insensitivity syndrome have male gonads, which are removed to prevent virilization. A segment of the sigmoid colon is chosen, with a major vascular pedicle supplying the mesenteric arcade. This segment is divided, and the adjacent intestinal tract is placed in continuity. Then, whether the graft is moved to the perineum in an isoperistaltic or reverse peristaltic manner is decided on the basis of the length of the mesenteric pedicle.
The chosen proximal end is closed in 2 layers. The perineal dissection requires a circular or cruciate incision at the hymenal ring. Then, blunt dissection from below toward the peritoneal reflection allows this incision to be opened and the sigmoid to be passed into the newly created tract. A single-layer anastomosis is created to the hymenal regions by using absorbable sutures. Attempts are made to extraperitonealize the sigmoid. A petroleum jelly (Vaseline) pack is placed in the neovagina to maintain apposition to the dissected tissues. The use of closed-suction drains is optional.
In patients presenting with variants of urogenital sinus, imperforate anus, and cloaca, a single-stage reconstructive pull-through procedure is an option. Because this procedure is not used in patients with pure vaginal agenesis, it is not discussed in detail in this article.22,13
Postoperative Details
Management after colon interposition vaginoplasty entails the routine postoperative care performed after bowel resection. Vaginal packing covered with a sheet of petroleum gauze is left in place for approximately 48 hours. Radhakrishnan (1987) recommends examination performed under anesthesia after 1 week to separate any synechiae.23 Progressive dilations are begun 3 weeks after surgery to prevent stricture at the distal anastomosis.
Postoperative care after an Abbe-McIndoe operation differs because no intra-abdominal concerns are present. The obturator maintaining the neovaginal cavity remains in place for 7-10 days to optimize graft adherence. It is then replaced with a silicone dilator. Examination can often be accomplished in lithotomy stirrups without the need for general anesthesia.
Follow-up
Follow-up care should emphasize maintenance of an adequate vaginal opening and length. Reassessment and progressive sizing of the dilators occur until the patient is regularly sexually active.
Complications
Creation of a neovagina by using skin grafts requires long-term use of vaginal dilators to avoid stenosis. Dilator trauma places the posterior wall abutting the rectum at particular risk for neovaginal-rectal ulcers and fistulas that may require additional surgery. Schult et al (2001) report a standardized surgical evaluation and repair technique that avoids a diverting colostomy in most patients.24 A perineal approach to fistulectomy with 2-layer closure of the rectum and levatorplasty resulted in good functional outcomes in this series. The mean hospital stay was 13 days, and only 2 patients required additional surgery for late relapse of a fistula.
The potential for malignant transformation of the neovaginal epithelium is always present. This complication has been documented in at least one case report of incidental squamous cell carcinoma manifesting as a fistula.25
Complications related to the sigmoid neovagina tend to be related to excess mucous drainage and the potential for prolapse.
More on Vaginal Atresia |
| Overview: Vaginal Atresia |
| Workup: Vaginal Atresia |
 Treatment: Vaginal Atresia |
| Follow-up: Vaginal Atresia |
| References |
| « Previous Page | Next Page » |
References
Burgis J. Obstructive Mullerian anomalies: case report, diagnosis, and management. Am J Obstet Gynecol. Aug 2001;185(2):338-44. [Medline].
Bryon A, Nigro J, Counsellor V. One hundred cases of congenital absence of the vagina. Surg Gynecol Obstet. 1949;88:79-85.
Reindollar RH, Byrd JR, McDonough PG. Delayed sexual development: a study of 252 patients. Am J Obstet Gynecol. Jun 15 1981;140(4):371-80. [Medline].
Banerjee R, Laufer MR. Reproductive disorders associated with pelvic pain. Semin Pediatr Surg. Feb 1998;7(1):52-61. [Medline].
Rock JA. Te Linde's operative gynecology. In: Rock JA, Thompson JD, eds. Surgery for Anomalies of the Mullerian Ducts. 8th ed. Philadelphia, PA: Lippincott-Raven; 1997.
Nazir Z, Rizvi RM, Qureshi RN. Congenital vaginal obstructions: varied presentation and outcome. Pediatr Surg Int. Sep 2006;22(9):749-53. [Medline].
Acien P, Acien M, Sanchez-Ferrer M. Complex malformations of the female genital tract. New types and revision of classification. Hum Reprod. Oct 2004;19(10):2377-84. [Medline].
Stoler JM, Herrin JT, Holmes LB. Genital abnormalities in females with Bardet-Biedl syndrome. Am J Med Genet. Jan 30 1995;55(3):276-8. [Medline].
David A, Bitoun P, Lacombe D, et al. Hydrometrocolpos and polydactyly: a common neonatal presentation of Bardet-Biedl and McKusick-Kaufman syndromes. J Med Genet. Aug 1999;36(8):599-603. [Medline].
Carranza-Lira S, Forbin K, Martinez-Chequer JC. Rokitansky syndrome and MURCS association--clinical features and basis for diagnosis. Int J Fertil Womens Med. Sep-Oct 1999;44(5):250-5. [Medline].
Economy KE, Barnewolt C, Laufer MR. MRI sensitivity in detecting uterine agenesis in cases of vaginal agenesis. Paper presented at: Annual Meeting of the North American Society of Pediatric and Adolescent Gynecology;. 1998.
Frank RT. The formation of an artificial vagina without operation. Am J Obstet Gynecol. 1938;35:1053-5.
Hendren WH, Donahoe PK. Correction of congenital abnormalities of the vagina and perineum. J Pediatr Surg. Dec 1980;15(6):751-63. [Medline].
Dharamsi N, Sheldon C. Management quandary. Case 2005: management of vaginal agenesis. J Pediatr Adolesc Gynecol. Oct 2005;18(5):359-62. [Medline].
Michala L, Cutner A, Creighton SM. Surgical approaches to treating vaginal agenesis. BJOG. Dec 2007;114(12):1455-9. [Medline].
Miller RJ, Breech LL. Surgical correction of vaginal anomalies. Clin Obstet Gynecol. Jun 2008;51(2):223-36. [Medline].
Wang X, Qiao Q, Burd A, et al. A new technique of vaginal reconstruction with the deep inferior epigastric perforator flap: a preliminary report. Plast Reconstr Surg. May 2007;119(6):1785-90; discussion 1791. [Medline].
Monstrey S, Blondeel P, Van Landuyt K, et al. The versatility of the pudendal thigh fasciocutaneous flap used as an island flap. Plast Reconstr Surg. Mar 2001;107(3):719-25. [Medline].
Ekinci S, Karnak I, Ciftci AO, Senocak ME, Tanyel FC, Buyukpamukcu N. Sigmoid colon vaginoplasty in children. Eur J Pediatr Surg. Jun 2006;16(3):182-7. [Medline].
Kapoor R, Sharma DK, Singh KJ, Suri A, Singh P, Chaudhary H, et al. Sigmoid vaginoplasty: long-term results. Urology. Jun 2006;67(6):1212-5. [Medline].
Brucker SY, Gegusch M, Zubke W, Rall K, Gauwerky JF, Wallwiener D. Neovagina creation in vaginal agenesis: development of a new laparoscopic Vecchietti-based procedure and optimized instruments in a prospective comparative interventional study in 101 patients. Fertil Steril. Nov 30 2007;[Medline].
Graivier L, McKay DL Jr, Katz A. Hydrocolpos, vaginal atresia, and urethrovaginal fistula in a neonate: abdomino-perineal-vaginal pull-through. J Pediatr Surg. Aug 1977;12(4):605-7. [Medline].
Radhakrishnan J. Colon interposition vaginoplasty: a modification of the Wagner-Baldwin technique. J Pediatr Surg. Dec 1987;22(12):1175-6. [Medline].
Schult M, Wolters HH, Lelle RJ, et al. Outcome of surgical intervention for rectoneovaginal fistulas in Mayer- Rokitansky-Kuester-Hauser syndrome. World J Surg. Apr 2001;25(4):438-40. [Medline].
Schult M, Hecker A, Lelle RJ, et al. Recurrent rectoneovaginal fistula caused by an incidental squamous cell carcinoma of the neovagina in Mayer-Rokitansky-Kuster-Hauser syndrome. Gynecol Oncol. Apr 2000;77(1):210-2. [Medline].
De Philippo RE, Bishop CE, Filho LF, Yoo JJ, Atala A. Tissue engineering a complete vaginal replacement from a small biopsy of autologous tissue. Transplantation. Jul 27 2008;86(2):208-14. [Medline].
Breech LL, Laufer MR. Obstructive anomalies of the female reproductive tract. J Reprod Med. Mar 1999;44(3):233-40. [Medline].
Croak A, Gebhart JB. Congenital abnormalities of the female urogenital tract. J Pelvic Med Surg. 2005;11(4):165-81. [Full Text].
Darai E, Toullalan O, Besse O, Potiron L, Delga P. Anatomic and functional results of laparoscopic-perineal neovagina construction by sigmoid colpoplasty in women with Rokitansky's syndrome. Hum Reprod. Nov 2003;18(11):2454-9. [Medline].
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. Apr 2002;15(2):101-4. [Medline].
Heller DS. Lower genital tract disease in children and adolescents--a review. J Pediatr Adolesc Gynecol. Apr 2005;18(2):75-83. [Medline].
Malasanos TH. Sexual development of the fetus and pubertal child. Clin Obstet Gynecol. Mar 1997;40(1):153-67. [Medline].
Masson JC. Discussion of a simple method for making an artificial vagina. Am J Obstet Gynecol. 1940;40:915-6.
McIndoe A. The treatment of congenital absence and obliterative conditions of the vagina. Br J Plast Surg. Jan 1950;2(4):254-67.
Palmer JS. Genitourinary manifestations in boys and girls associated with genetic diseases [article online]. J Men's Health. 2006;3(1).
Rajimwale A, Furness PD, Brant WO, Kyle MA. Vaginal construction using sigmoid colon in children and young adults. BJU Int. Jul 2004;94(1):115-9. [Medline].
Reinhold C, Hricak H, Forstner R, et al. Primary amenorrhea: evaluation with MR imaging. Radiology. May 1997;203(2):383-90. [Medline].
Wesley JR, Coran AG. Intestinal vaginoplasty for congenital absence of the vagina. J Pediatr Surg. Jul 1992;27(7):885-9. [Medline].
Further Reading
Keywords
vaginal atresia, vaginal agenesis, Rokitansky-Mayer-Küster-Hauser syndrome, RMKH, Rokitansky-Mayer syndrome, Rokitansky syndrome, Rokitansky's syndrome, müllerian agenesis, McKusick-Kaufman syndrome, MKKS, Bardet-Biedl syndrome, BBS, absent vagina, uterovaginal outflow tract obstruction, vaginal obstruction, transverse vaginal septum, müllerian-inhibiting substance, MIS, amenorrhea, hydrometrocolpos, postaxial polydactyly, imperforate anus, congenital heart defects, secondary endometriosis
