Jurnal Anomali Vagina Word.docx

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JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION

JULY, 1970

Management of Congenital Anomalies of the Vagina JOHN H. NILES, Maj., MC

Chief, Obstetrics and Gynecology BRAVO, col., MC

Chief, Surgery and Professional Service,

U. S. Kirk Army Hospital, Aberdeen Proving Ground, Maryland LEROY JACKSON, M.D., F.A.C.O.G. Clinical Instructor in Obstetrics and Gynecology, Howard University College of Medicine CASE REPORTS The gynecologist has a formidable problem in the management of congenital anomalies of the Case No. 1 : Patient F.R. was a 21-year-old G O P vagina. In many instances correction of the O who presented with the chief complaint: "I am planning to be married and went to my doctor for a defect is a form of transplantation with problems check up." She gave a history of no episodes of of selection of the patients, the time of menstrual bleeding. She and her fiance had attempted correction, the surgical procedure used, the intercourse but penetration was found to be management of the patient in the critical ee impossible. Reportedly, the patient visited a physician rejection phase," the psychological support of at age 15 concerning amenorrhea and was told no the patient and her family and the investigation investigation of her problem was needed at this time. of possible associated congenital abnormalities She denied lower abdominal pain, urinary tract and gastrointestinal tract symptoms. Past history and in other organ systems. systems review were negative. Physical examination The number of cases of congenital anomalies revealed a 5' 2" female weighing 124 lbs. with normal of the vagina in the literature is relatively vital signs. General physical examination was • modest although it has been increased rapidly in within normal limits. Pelvic examination revealed the last few years. There are probably about 200 normal appearing clitoris and labia. There was no cases recorded and published, most of them in vaginal opening. The urethra was in the normal series of one, two or three cases. The largest location. On rectal examination, no pelvic masses group of patients reported in our review of the were palpated. A uterus and cervix were not palpated. Hematocrit, hemoglobin and urinalysis were within literature is that of Angelo Lodi l in 1951, who normal limits. The BUN was 18, mg. c/o. The reviewed and reported 110 cases of various chromatin pattern was positive with a karyotype of 46 vaginal abnormalities that had been studied over chromosomes and normal female XX pattern. a 44 year period in Milan, Italy. Among these Intravenous pyelogram revealed complete absence of cases were 42 of transverse vaginal septum, the the kidney and ureter on the left side. On the right most common of all vaginal abnormalities. side, there was the impression of pelvic kidney. The This paper will present two cases managed patient was subjected to an exploratory laparotomy. Examination of the abdomen revealed the right recently by two of the authors at Freedmen's kidney palpable at the level of the sacral prominence, Hospital in Washington, D.C., congenital congenital absence of the left kidney, two grossly absence of the vagina and transverse vaginal normal appearing ovaries, two rudimentary horns, septum. Also a discussion of the developmental two streaks of apparent Mullerian tissue extending anatomy and anatomical description of the blindly down to the apex of the pelvic floor. Biopsies anomalies, cytogenetic findings, abnormalities were taken from both ovaries. The abdomen was then of the urinary tract and skeleton, genetic aspects, closed and the patient placed in the lithotomy a historical review and description of surgical position. A one inch incision was made in the mucosa just below the urethral meatus and by blunt dissection techniques and post operative care will be a 4 cm. x 8 cm. cavity was formed, extended both presented. inferiorly down toward the rectum and then

closed and the patient was placed in the lithotomy position. A cruciate incision was made in the mucosa in the area where the introitus should be located. Following blunt dissection through approximately 3 cm. of connective tissue, an apparently normal mucosa was reached, with the release of approximately 75cc of a dark mucoid material. A small cervix was then observed with a small amount of mucoid material exuding from the external os. A "Z plasty"-like procedure was performed at the edges of the vaginal muccsa. To these edges, the mucosa at the area of the introitus was attached to the upper mucosa with 2-0 chromic suture. The introitus formed a VOL. 62, No. 4 Congenital Anomalies of the Vagina 275 p p e The post-operative course was uneventful. The graft was not rejected. The patient later married and reportedly had satisfactory ared adequate. The vagina was then packed. The post sexual intercourse. It was later ascertained that this patient's 12operative course was uneventful. On discharge, the year-old sister also had congenital absence of vagina. newly formed vaginal opening was intact with no Case No. 2: Patient C.B. was a 12-year-old female with no evidence of infection. Six weeks, six months and one previous menses. She presented with a history of vague year following surgery, the patient was noted to be lower abdominal pain for three months prior to admission. having normal menses and no evidence of stricture. This pain was noted during the middle of each month. The DISCUSSION patient was admitted to another hospital one month prior to this admission with the diagnosis of acute appendicitis. A To better understand the congenital vaginal procedure was performed at this time followed by abnormalities of the vagina, one needs to two days of bright red bleeding. A diagnosis of 2 consider its embryology. The vagina is derived hematocolpos secondary to imperate hymen was made and a vaginal cream was given to the patient on discharge. by a joining of the distal fused Mullerian ducts Approximately one month after discharge, the previous to the urogenital sinus. By the eleventh week of symptoms recurred and no menses had been noted. The gestation, the distal portion of the two patient was admitted to Freedmen's Hospital. On physical Mullerian ducts have fused to form a single examination the patient was well developed and well nourished. Her height was 61 3/4" and her weight was 103 cavity. The wall of the superior portion of this lbs. Adequate breast development was present for her age cavity rapidly thickens and sharply demarcates group. A moderate amount of pubic and axillary hair were itself, forming the uterine corpus and cervix. present. The general physical examination was within normal The inferior portion elongates caudaly and fuses limits. The abdomen was flat with no palpable masses. A with the two columns of cells, the sinovaginal question of slight tenderness was noted in the midline in the lower quadrant but no rebound. On pelvic examination, no bulbs, which are derived from the urogenital vaginal orifice was noted. The labia and clitoris were within sinus. After 17 to 18 weeks, the core of the normal limits. On rectal examination a sausage shaped mass sinovaginal bulbs degenerates and forms a was palpated in the area between the bladder and the rectum. cavity which connects the fused inferior No uterus or adnexal structures were palpated. The Mullerian ducts to the urogenital sinus which at hematocrit, hemoglobin and urinanalysis were within normal this stage of growth remains only as the limits. Twenty-four hour pituitary gonadotraphins were 5 mouse units. Chromosome studies revealed 46 chromosomes vestibule. The band of tissue circumscribing the with XX pattern. Buccal smear showed 8% bar bodies. IVP aperture between the sinovaginal bulbs and the revealed a double kidney and collecting system on the left vestibule becomes the hymen. The percentage and the impression of a mass in the upper pole of the right of vagina derived from each of these sources is kidney. The collecting system on the right appeared normal. debatable, however, it appears that the majority Renotcmogram and renoarteriogram demonstrated a cystic mass in the upper pole of the right kidney. Laparotomy was is Mullerian in origin. then performed. Examination of the pelvis revealed grossly Understanding this embryological normal internal genitalia, but a markedly distended cystic development of the vagina, one can easily see vagina with uterus and adnexa setting on top of this mass. A the possible abnormalities that can occur: 1) biopsy was obtained from the ovaries for pathological and chromosome studies. On exploration of the upper abdomen complete absence of the vagina due to failure of the cyst in the area of the upper pole of the right kidney was both Mullerian and sinovaginal bulbs to found and decortication of the simple 4 x 4 cm. cyst was develop, 2) absence of the upper portion of the performed. The wall of the cyst was smooth. A flank drain vagina due to failure of proper Mullerian was placed and hemostasis maintained. The abdomen was superiorly upward toward the bladder. A skin graft was made from the inner aspect of the right thigh. This was then placed over an acrylic mold measuring 10 cm. in length and 3 cm. in diameter. The mold with skin graft was then sutured loosely with 4-0 chromic catgut within the previously made cavity. The labia were then closed around the mold using 2-() black silk. A foley was then inserted. Hemostasis was adequate. The patient tolerated this three hour and fifteen minute operation successfully. The pathology report on the ovarian biopsy was within normal limits.

development, usually associated with absence of the uterus, 3) absence of the lower portion of the vagina due to the improper sinovaginal bulbs to develop, 4) absence of the septum separating the upper and lower vagina due to the failure of the fusion of the Mullerian ducts to the sinovaginal bulb, and 5) imperforate hymen resulting from lack of rupture of the sinovaginal bulbs into the vestibule. In terms of anatomical description, patients with congenital absence of the vagina usually also have absence of the uterus. The usual lesions include absence of the middle and the

abnormalities, an incidence of 48 per cent. None of the patients had symptoms referable to the urinary tract. Five patients had solitary pelvic kidneys, five had solitary kidneys, two situated normally and three in various ectopic sites. One patient demonstrated malrotation of the kidney. Cali's6 study showed 40 per cent of the patients with congenital absence of the vagina also had major urinary tract abnormalities. Stabler7 found only seven of 19 patients with congenital absence of vagina to have two kidneys and ureters in normal position.

The various skeletal abnormalities with abse 276 JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION JULY, 1970 nce of upper vagina. The ovaries are usually normal. the vagina have been given insuffcient attention. The external genitalia are normal except that the Studies by Novak and Jones in 1961 and Bryan vaginal opening is either absent or very shallow. in 1949 shows a significant incidence of skeletal Septa in the vagina may be congenital or may abnormalities in cases of absence of the vagina. develop later as a result of trauma or Chawla in his study found four patients with pathological processes. Congenital septa may be skeletal abnormalities, three of whom had in the long axes of the vagina, either anteriorsolitary kidneys. These findings were posterior, called a sagitial septum or from side to hemivertebrae and spina bifida of the cervical side, called a coronal septum. They may lie spine, lumbarization of Sl, scalous or butterfly perpendicular to the long axes, a transverse vertebrae with cervical spine fusion of DI and septum. Most transverse septa occur at the lower D2, multiple abnormalities of ribs and arms and end of the vagina. They rarely do lie at the spina bifida at Sl. higher level. If a high transverse septum is There is evidence that at least one form of complete the patient is likely to present with a transverse vaginal septum has a genetic basis and is case of cryptomenorrhea. If it is incomplete the inherited on a simple autosomal recessive trait. septum may first come to notice as an obstetric McKucich et al. 8 in the study of the Amish of problem.3 As with imperforate hymen, large Pennsylvania, a highly inbred community, described amounts of menstrual blood may accumulate two sisters with the condition. McElroy and Ward 9 with relatively minor symptoms, although lower reported a siblingship of three sisters. The three abdominal pain recurring at monthly intervals is sisters were diagnosed because the eldest at age 20 characteristic. A patient with a transverse had not menstruated. vaginal septum may be mistaken as one with In terms of a historical review 10 in 1904 Baldwin intersexuality or congenital absence of the transplanted an ileal loop to function as a vagina and vagina and uterus if the examiner interprets the in 1910 Papoff used a similar technique involving septum as the vaginal apex. In most instances the the rectosigmoid. This was difflcult surgery and was septum is less than a centimeter in thickness, but associated with a high morbidity. Prolapse of the in some cases the vaginal plate has failed to loop was frequent and its mucosal discharge often canalize for a much longer distance so that a offensive. The use of simple intermittent pressure by portion of the vagina seems to be congenitally means of a mold was suggested by Frank in 1932. absent.4 This still claims some advocates, even though the We were unable to find any reports in the method is tedious and the results are often literature on cytogenetic changes in patients with inadequate. Counseller established that in some cases congenital absences of the vagina. All were the tract would eventually epithelialize if kept patent reported as 44 autosomes and 2X chromosomes. long enough. Chawla5 reported 23 patients with vaginal Skin grafting techniques were established as early anomalies. Eleven showed urological as 1870. Until this concept was understood and until

materials appeared from the building of vaginal molds that were non-reactive to tissue, the grafting method fell into disrepute and disuse for repairing the vagina. In construction of the artificial vagina, it has been established that split-thickness skin grafting is the best method, at present, producing a high degree of success and long range satisfaction. This is referred to as the Mclndoe procedure. It is also a more desirable technique because of the simplicity of the procedure and the relatively short time involved in achieving a functional result. In the Mclndoe procedure the graft is taken from the buttock or thigh with a Paget dermatone set at about .018 inches thickness. It is kept moist with saline while inserting it over the obturator by stretching, turning and suturing with 5-0 chromic catgut to the mold. The dissection of the vaginal canal is done carefully to a size that accepted the graft-covered mold snuggly. This is accomplished mainly by blunt separation of the bladder and

should be performed so as to define exactly the nature of the abnormality before excising the septum from below. Radical excision or total incision of the septum may not produce adequate caliber initially and often is followed by stricture and a circular scar but the technique utilizing the so called multiple e e Y plasties" is presented as a means of avoiding post operative stricture. In this operation, described by Owen, no tissue is excised. The septum is dissected into two layers, and two or three incisions are made in each leaf of the septum. Three incisions are made in alternating positions and then they extend into the normal vaginal wall for two or three centimeters. The resulting triangular flaps of mucosa are then interdigitated and sutured in place with 3-0 chromic catgut, thus incorporating the septum into the wall of the vagina, actually increasing the caliber at this point. The vagina is packed with iodoform or petroleum gauze to prevent hematoma formation. After a few days the gauze is removed and then coit VOL. 62, No. 4 Congenital Anomalies of the Vagina 277 us is per rectum through an incision in the vulvar space. mitted a few weeks later. ll Meticulous hemostasis and asepsis were considered In conclusion, these key points should be mandatory. The inserted graft was held in place by a remembered in the management of congenital double T-binder. The distal edges of the graft were anomalies of the vagina. sutured to the cut edges of the epithelium within the 1. The patient should be mentally mature newly established introitus with 3-0 black silk. The to understand what is needed to keep necessary preoperative preparation has to be done mainly with dilation artificially if she is not married. the bowel. A low residue diet was instituted, 2. There should be a complete cleansing enemas were given and the patient investigation of the genito-urinary tract and obstipated to a point wherein defication was skeletal systems. prevented for the fist seven to 10 days post 3. Cytogenic studies must be performed. operative. At this time it was proved safe to remove 4. Obtaining an accurate family history the obturator. It is preferable to do this under and examination of the sibling females is also general anesthesia, cleaning the cavity with normal necessary. saline. Post oper atively the patient is kept at 5. There should be a complete physical absolute bed rest for the first 10-14 days. The mold examination in young patients presenting with a must be kept in the canal most of the time until the history of amenorrhea and/or abdominal pain. contractile phase is past. This varies from case to case and with differing circumstances. If the take is almost 100 per cent constant, wearing of the obturator for eight to 10 weeks would be minimal. If the take was less, this period would be prolonged. If the patient is unmarried the application should be worn at night for a long period of time. Few cases are available in the literature to act as a guide to the treatment of complete transverse septum of the vagina. Excision appears to have the best results. If, however, the septum is thick, laparotomy

CONCLUSION

Two case reports, demonstrating congenital absence of the vagina and transverse septum presenting with abdominal pain are presented. •The embryology, anatomical features, the surgical approach, and preoperative and post operative management of the two lesions are reviewed.

LITERATURE CITED

1. JONES, H. W. and R. H. HELLER. Pediatric and Adolescent Gynecology. The Williams and Wilkins Company, Baltimore, Md., 1966, p. 62. 2. NORTHCUTT, R. C. Med. Ann. D. C. 35:316-9, June 1966. 3. WHITE, A. J. Obstet. & Gynec. 27:695-8, May 1966. 4. JONES, H. W. and R. H. HELLER. The Williams and Wilkins Company, Baltimore, Md., 1966, p. 65. 5. CHAWLA, S. and K. BERG. Brit. Med. J. 5500:1398400, June 1966.

COLI, R. W. and J. H. PRATT. Am. J. Obstet. & Gynec. 100:752-578, March 1968. 7. STABLER, F. J. Obstet. Gynec. Brit. Comm. 73:4636, June 1966. 8. McKUSICH, V. A. and R. L. BAUER. J. Amer. Med. Assoc. 189:813, 1964. 9. MCILROY, C. and 1. V. WARD. Proc. Roy. soc. Med. 23•.633, 1930. 10. WHITELY, J. M. Amer. J. Obstet. & Gynec. 89:37785, June 1969. 11. OWENS, W. L. Obstet. & Gynec. 23:940-941, June, 1969. 6.

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