Acta Obstet Gynecol Scand 57: 89-94, 1978
VAGINAL AGENESIS: An Analysis of Ninety Cases
Carlos Albert0 Salvatore and Orlando Lodovicci
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From the Gynecology Clinic of the University of Srio Paulo Medical School, Srio Paulo, Brasil
Abstract. This study of 90 cases of vaginal agenesia showed the following results: 50% of the patients sought medical advice when between 16 and 20 years of age; 71.1 % of the patients were not married; amenorrhea was present in 100% of the cases, with hypogastric pains in 20%; the uterus was not palpable clinically in 77.7% of the cases; 77.7% of the patients had total agenesis, 1 1 . 1 % had partial agenesis and 7.7 % had homatometra; through laparotomy, 75.5 % were found to have normal ovaries, 92.5% of the cases were histologically normal; laparotomy showed the uterus as solid rudimentary in 55.5% of the cases and absence of the uterus in 24.4% of the cases; the Fallopian tubes were normal in 32.22% of the cases and rudimentary in 46.6%; excretion urography disclosed renal anomalies in 17.5 %; surgical treatment by the McIndoe technique, carried out in 90 cases, gave 90.5 % satisfactory results; the follow-up made between one and 5 years, in which the long-term use of an acrylic mould is recommended in those who do not have sexual relations, showed permanence of the satisfactory results in 91.3% of the cases, of which 83.3% were after one operation only; neovaginal cytology, done between one and 6 years, showed 72.2% of the cases with acidophyl cells between 5 and 20% with acidophily between 21 and 40%.
Vaginal agenesis is a legal, social and medical problem of great importance, demanding treatment at the right period of the woman’s sexual evolution. It is a malformation that sometimes goes unnoticed up to the time of puberty, when it is realized that menstruation has failed to appear. Vaginal agenesis is a result of arrested development of the Miillerian ducts, hence its frequent association with uterine aplasia. According to most authors (e.g. 2, 9, 12), the external genital organs have a normal appearance, with or without the presence of the hymenal membrane and a small depression where the vaginal opening should be. Vaginal agenesis is easily diagnosed and is seldom connected with the form of intersexuality associated with sex chromosome abnormalities. It is
frequently associated with changes in the urinary system. Surgery is the treatment that offers the best results, demonstrated in the practice of “neovaginoplasty” according to McIndoe (2), but the time of the woman’s reproduction life at which its should be carried out is still under discussion. Williams’ (21) recent technique prepares the neovagina in a situation unfavourable for coitus. Consequently we are still using the McIndoe operation. In previous works (17, 18), it was shown that according to several authors neovaginoplasty is indicated immediately when there is hematometra and in those patients who have attempted sexual intercourse ( 1 , 2, 5 , 13, 15). We have reported previously 2 patients with hematometra and 18 who have tried to accomplish coitus, of whom 16 were married. Generally speaking, however, neovaginoplasty must be done in cases where the wedding is imminent and, according to the case, after 17 years of age, so as to facilitate the integral development of the personality. If the operation is performed-sometimes before intercourse is likely to occur-there is an increased risk of stenosis of the neovagina. The methods used in the making of an artificial vagina were reported at length in a previous work, in which we studied 42 cases (17) and in which we stressed the efficacy of the McIndoe method. In the present study we report a total review of the cases we have treated including those quoted previously and those reported by Lodovicci (10).
METHOD AND MATERIAL Our series consists of 90 cases, observed at the Gynecologic Clinic of the University of SBo Paulo Medical School. The patients underwent a gynecological examinaActa Obsrer Gynecol Scand 57 (1978)
90
C. A . Salvatore a n d 0 .L o d o v i c i
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tion (rectal palpation under narcosis), laparotomy (36 cases) or laparoscopy (54 cases) excretion urography (40 cases) and determination of genetic sex (40 cases). The operating technique is as follows (19). The patient must be placed in a semigynecologicalposition. We prefer the McIndoe technique for the building of the neovagina, using a free skin graft taken from the abdomen. The operation has two fundamental parts: the preparation of the graft, followed by construction of the tunnel between the rectum and the urethra and bladder. We usually operate together with a plastic surgeon. The abdominal stage After preparing the abdominal skin, the plastic surgeon takes a skin layer, with the dermatome, big enough to cover the acrylic mould. The raw part of the abdomen is protected with gauze and cotton and bandaged. The plastic surgeon then prepares the mounting of the epidermis over the acrylic mould, sewing the edges with nylon thread, while the gynecologist prepares the neovaginal tunnel. The vaginal stage An incision is made with the scalpel transverse to the vestibule or to the retrohymenal fossette, between the anus and the urethral meatus. One tries to cut first, transversely, the bundles of the “fibrous perineal nucleus”, found between the urethra and the anus-rectum. The incision is deepened and two small tunnels are formed on either side of the urethra. These are subsequently united in the mid-line. By means of the scalpel or its handle, or else with curved-tip scissors, the urethra and bladder are separated from the rectum up to the point where the base of the peritoneal bladder-rectal sac is reached. In this stage of the procedure we never open the urethra, the bladder, or the rectum-an accident which often happens when the surgeon attempts to advance the dissection through the mid-line. The tunnel must be large enough to take a medium-sized acrylic mould. Absolute haemostasis of all small vessels is obtained with plain catgut number 00 ligatures. According to the case, a partial dissection should be done at the sides of the pillars of the elevator ani muscle, so as to avoid subsequent stenosis of the vaginal introitus. After the mould is prepared and haemostasis is confirmed and after the tunnel has been soaked with physiological serum, the acrylic mould covered with the epidermis taken from the abdomen is introduced. The mould is fixed with cloth bandages passing through the mould‘s screw and attached to the abdominal bandages with sticking plaster. The patient must remain in bed until the seventh or eighth day, when she is allowed to walk and to take out the mould. She must subsequently undergo re-dressing every 48 hours, the mould being removed and re-inserted. She must be instructed concerning the long-term hygiene of the neovagina in order to avoid stenosis. Details of the post-opreative management are to be found in other papers (10, 17). An acrylic mould is inserted soon after surgery and it remains in place for 8 days. The dressing is changed every Acta Obsfet Gynecol Scand 57 (1978)
other day and, after discharge, about the 14th day, the patient herself will prepare and apply the dressings, consisting of removing and reinsetting the mould into the neovagina over a period of 6 months.
ANALYSIS OF THE MATERIAL Age (90 cases of vaginal agenesis) 1lL15 years, 7 cases 16-20 years, 46 cases (50%) 21-25 years, 24 cases 26-30 years, 10 cases 31-35 years, 4 cases Civil status Unmarried, 64 cases (71.1 %) Married, 26 cases (28.9%) Symptoms Amenorrhea, 90 cases (100 %) Impossibility of coitus, 22 cases (24.4%) Hypogastric pain, 18 cases (20%) Gynecological examination (Rectal) Non-palpable uterus, 70 cases (77.7%) Rudimentary palpable uterus, 14 cases Uterus increased in volume, 4 cases unrecognizable ovaries, 80 cases (88.8 %) Palpable ovaries, 8 cases Diagnosis Total agenesis, 70 cases (77.7%) Partial agenesis, 10 cases (1 1.1 %) Male pseudo-hermaphroditism, 3 cases Total agenesis plus haematometra, 3 cases (7.7%) Partial agenesis, haematometra and haematocolpos, 2 2 cases (7.7%) Total agenesis, haematometra and haematosalpinx, 2 2 cases (7.7%) Internal genital organs observed through Laparoscopy (54 cases) and Laparotomy (36 cases) ( a ) Ovaries: Normal, 68 cases (75.5 %) Polycystic, 6 cases Hypoplastic, 12 cases Rudimentary, 4 cases ( b ) Histology of the gonads (biopsy) (40 cases): Normal ovaries, 37 cases (92.5%) Rudimentary testicles, 3 cases (c) Uterus: Hypoplastic, 2 cases Increased in volume (haematometra), 4 cases Solid rudimentary, 50 cases (55.5%) Double uterus, 1 case Bicornuate uterus, 3 cases Hemi-uterus, 4 cases Two rudimentary uteri, 4 cases Lack of uterus, 22 cases (24.4%) ( d ) Tubes: Normal, 29 cases (32.2%)
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Fig. 1 . Collin speculum in the neovagina.
Hypoplastic, 6 cases Rudimentary (long) 42 cases (46.6 %) One rudimentary tube, 2 cases Lack of tubes, 10 cases Hematosalpinx, 1 case Excreting urography (40 cases) Ureters and normal kidneys, 33 cases (82.5%) Double pelvis-calyces, 3 cases Lack of left kidney, 4 cases Genetic sex (40 cases) Presence of the female sexual chromatin, 36 cases Lack of the female sexual chromatin (male), 4 cases Treatment (90 cases) Total neovagina, 80 cases (88.8%) Partial neovagina, 10 cases Neovagina and draining of the haematometra through the vagina, 3 cases Partial neovagina and draining of the haematometra and haematocolpos (through the vagina), 2 cases Neovagina and draining of the haematometra through the abdomen (hysterotomy), 1 case Neovagina and draining of the haematometra and haematosalpinx (through the abdomen), 1 case Neovagina and clitoris amputation, 2 cases Results (90 cases) Good graft integration and good vaginal permeability, 75 cases (83.3 %) Vaginal stenosis (fibrous ring) (2 re-operated with satisfactory results), 9 cases Partial disintegration of the graft due to a haematoma (all re-operated with satisfactory results), 6 cases Total satisfactory results including the 5 who were reoperated, 81 cases (90.0%)
Follow-up (between 1 and 6 years) (70 cases) Good results (penetration of the Collins speculum, medium sized) (Figs. 1, 2), 64 cases (91.3%) Partial stenosis, 6 cases With normal sexual relations, 54 cases With dyspareunia, 4 cases With normal menses, 4 cases Pregnancies (caesarean), 2 cases Cytology of neovagina (25 cases) (between 1-6) (Fig. 3) From 5 to 20% of acidophil cells, 18 cases (72.0%) From 21 to 40% of acidophil cells, 7 cases (28.0%) Biopsy of the neovagina showed atrophic epidermis (Fig. 4).
COMMENTARY As may be observed from an analysis of the cases, most patients sought advice in the age range 16 to 20 years (50% of the cases), coinciding with a wait of 3-5 years for the onset of the menarche. As a matter of fact, primary amenorrhea was the predominant symptom (100% of the cases). Hypogastric pains occurred in 20% of the cases and attempts at sexual intercourse among those who had tried had been unsuccessful in around 24.4 % of the cases. Of the casrs examined, 28.9% of the patients were married and had serious problems just after the marriage. Because of such problems, most authors think that the ideal age for the making of the neovagina is before the marriage. As we have stated Actu Obstet Gynecol Scand 57 (1978)
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C. A . Salvatore and 0 .Lodovici
Fig. 2. Collin speculum in the neovagina (cul-de-sac).
in an earlier work (18), libido was present in 79.1 % of the 49 cases previously studied. The diagnosis of vaginal agenesis is an easy one. The gynecological examination by rectal palpation allows the non-identification of the uterus in 77.5 % of the cases. Moreover, 77.7% of our 90 cases were of total vaginal agenesis and 11.1% of partial agenesis. There was haematometra in 7 cases (7.7%). The exploration of the internal genitalia by means of laparoscopy and laparotomy is unavoidable, but
shows that the ovaries are normal in 75.5 % of the cases. In 3 of the cases (pseudo-hermaphroditism) the biopsy revealed rudimentary testicles, confirmed by histological study. The psychological analysis in these cases was more important than the analysis of the sexual chromatin-hence the indication for a neovagina. A solid rudimentary uterus was found in 55.5 % of the cases, lack of uterus in 24.4% and functioning uterus with haematometra in only 7 cases (7.7%). These results agree with those of Counseller (5) (5
Fig. 3. Acidophil cells of the
neovagina. Acta Obstet Gynecol Scand 57 (1978)
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Fig. 4. Atrophic epidermis in the neovagina 3 years after the operation.
in 76), of Bryan et al. (2) (4 in a loo), Jackson (7) (5 in 128), Cordier (4) (1 in 23), Lodovici (10) (1 in 16) and Jeffcoate (8). These findings indicate its existence in 10% of cases. Normal tubes were, in the same way, found in only 32.2% of the cases. The practice of excretion urography is an important one as was shown by us and several other authors. The frequency of malformation of the urinary system is very high in cases of vaginal agenesis. Thus the absence of one of the kidneys (4 cases) and double pelvis-calyces (2 cases) was observed in 17.5 % of the cases. In a previous work, in an analysis of 21 cases, 15 (71.4%) were normal and the rest (28.6%) showed anomalies of the unnary system as represented by the lack of one of the kidneys, dilation of the ureters, of pelvis, double ureters, anomalous implantations, rotation displacement of one of the kidneys, or dislocated kidney, thus confirming the reports of others (5, 7, 10). In the experience of many authors (5, 7, 9, 10, 12, 13, 17, 18) the choice of treatment is the making of the neovagina by the McIndoe techniqueMcIndoe having, in 1950-59 established the basic principles for the formation of the neovagina from free skin grafts. Eighty complete neovaginas were made (88.8 %) and 10 partial ones, i.e. the vagina was completed in those cases where the agenesis was partial. In two of these cases there was agenesis of the exterior half
of the vagina, the inner half being full of menstrual blood (haematocolpos). The remaining 7 cases showed the presence of a vagina in its exterior third. In the 6 patients who had a functioning uterus, perforated moulds were used. As we have stated in a previous work (18), we recommend the use of an acrylic mould by the patient until the beginning of regular sexual activity, returning to its use in cases where a long intermption occurs. The results of the McIndoe technique are good, as graft integration occurred in 83.3% of the cases. If we add 6 cases where the integration was partial but re-operation was necessary, we achieved 90% of optimum graft integration and optimum condi tions of permeability. We observed the presence of a fibrous ring in only 9 of the 90 cases. Page & Owley (14) claim 81 % good results. The late follow-up observed between one and six years showed 91.3% satisfactory results in 70 cases, of which 54 had normal sexual relations, two had become pregnant and undergone caesarian section. Only 6 cases showed a small vaginal stenosis and 4 complained of dyspareunia. We have not so far found enterocele. In our experience, therefore, the McIndoe technique for the making of an artificial vagina is the one offering the best results and the long-term use of an acrylic mould guarantees the late success of the operation. Acta Obstet Gynecol Scand 57 (1978)
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REFERENCES 1. Barrows, D. N.: Am J Obstet Gynecol73: 609, 1957. 2. Bryan, A. L., Nigro, J. A. & Counseller, V. S.: Surg Gynecol72: 504, 1958. 3. Cali, R. W. & Pratt, T. H.: Am J Obstet Cynecol 100:752, 1968. 4. Cordier, G.: C. R. SOCFranc Gynecol22: 156, 1952. 5. Counseller, V. S.: JAMA 136: 861, 1948. 6. Evans, T. N.: Am J Obstet Gynecol99: 944, 1967. 7. Jackson, I.: J Obstet Gynaecol Br Emp 72: 335, 1965. 8. Jeffcoate, T. N. A.: J Obstet Gynaecol Br Comm 76:%1, 1969. 9. Jones, H. & Scott, W. W.: Hermaphroditism, Genital Anomalies and Related Endocrine Disorders. Williams & Wilkins, Baltimore, 1958. 10. Lodovici, 0.: NeoXJaginoplastia metodizada no tratamento de ausCncia congsnita da vagina. Tese. Fac. Med. Univers. Sio Paulo, 1966. 11. Lodovici, 0. & Salvatore, C. A,: An Bras Ginec 64:251, 1967. 12. McIndoe, A. H.: Proc R SOCMed52:952, 1959. 13. McIndoe, A. H.: Br J Plast Surg2: 254, 1960. 14. Page, E. U. & Owsley, Jr, J. Q.: Am J Obstet Gynecol 105: 774, 1969.
Actu Obsret Cvnecol Sccind 57 (1978)
15. Riva, H. L. & Harding, R. L.: Obstet Cynecol4: 517, 1954. 16. Salvatore, C. A , , Halbe, H. W., Cruz, D. S., Braga, L. F. & Gallucci, J . : Arq Obst Ginec Sio Paulo, 4 : 151, 1963. 17. Salvatore, C. A , , Spina, Lodovici, 0. & Faure, R.: Rev Paulista de Medicina65: 63, 1964. 18. Salvatore, C. A., Lodovici, 0. & Gallucci, J.: AnBras Ginec64: 317, 1967. 19. Salvatore, C. A.: Ginecologia Operatbria. Guanabara Koogan, Rio de Janeiro, 1974. 20. Ulfelder, H.: Am J Obstet Gynecol 100: 745, 1968. 21. Williams, E. A.: J Obstet Gynaecol Br Comm 79: 1147, 1972. Submitted for publication Febr. 27, I977 C. A. Salvatore Dept. of Obst. and Gyn. Hospital las Clinicas Srio Paulo Brad
VAGINAL AGENESIS: An Analysis of Ninety Cases
Carlos Albert0 Salvatore and Orlando Lodovicci
Acta Obstet Gynecol Scand Downloaded from informahealthcare.com by Nyu Medical Center on 04/28/15 For personal use only.
From the Gynecology Clinic of the University of Srio Paulo Medical School, Srio Paulo, Brasil
Abstract. This study of 90 cases of vaginal agenesia showed the following results: 50% of the patients sought medical advice when between 16 and 20 years of age; 71.1 % of the patients were not married; amenorrhea was present in 100% of the cases, with hypogastric pains in 20%; the uterus was not palpable clinically in 77.7% of the cases; 77.7% of the patients had total agenesis, 1 1 . 1 % had partial agenesis and 7.7 % had homatometra; through laparotomy, 75.5 % were found to have normal ovaries, 92.5% of the cases were histologically normal; laparotomy showed the uterus as solid rudimentary in 55.5% of the cases and absence of the uterus in 24.4% of the cases; the Fallopian tubes were normal in 32.22% of the cases and rudimentary in 46.6%; excretion urography disclosed renal anomalies in 17.5 %; surgical treatment by the McIndoe technique, carried out in 90 cases, gave 90.5 % satisfactory results; the follow-up made between one and 5 years, in which the long-term use of an acrylic mould is recommended in those who do not have sexual relations, showed permanence of the satisfactory results in 91.3% of the cases, of which 83.3% were after one operation only; neovaginal cytology, done between one and 6 years, showed 72.2% of the cases with acidophyl cells between 5 and 20% with acidophily between 21 and 40%.
Vaginal agenesis is a legal, social and medical problem of great importance, demanding treatment at the right period of the woman’s sexual evolution. It is a malformation that sometimes goes unnoticed up to the time of puberty, when it is realized that menstruation has failed to appear. Vaginal agenesis is a result of arrested development of the Miillerian ducts, hence its frequent association with uterine aplasia. According to most authors (e.g. 2, 9, 12), the external genital organs have a normal appearance, with or without the presence of the hymenal membrane and a small depression where the vaginal opening should be. Vaginal agenesis is easily diagnosed and is seldom connected with the form of intersexuality associated with sex chromosome abnormalities. It is
frequently associated with changes in the urinary system. Surgery is the treatment that offers the best results, demonstrated in the practice of “neovaginoplasty” according to McIndoe (2), but the time of the woman’s reproduction life at which its should be carried out is still under discussion. Williams’ (21) recent technique prepares the neovagina in a situation unfavourable for coitus. Consequently we are still using the McIndoe operation. In previous works (17, 18), it was shown that according to several authors neovaginoplasty is indicated immediately when there is hematometra and in those patients who have attempted sexual intercourse ( 1 , 2, 5 , 13, 15). We have reported previously 2 patients with hematometra and 18 who have tried to accomplish coitus, of whom 16 were married. Generally speaking, however, neovaginoplasty must be done in cases where the wedding is imminent and, according to the case, after 17 years of age, so as to facilitate the integral development of the personality. If the operation is performed-sometimes before intercourse is likely to occur-there is an increased risk of stenosis of the neovagina. The methods used in the making of an artificial vagina were reported at length in a previous work, in which we studied 42 cases (17) and in which we stressed the efficacy of the McIndoe method. In the present study we report a total review of the cases we have treated including those quoted previously and those reported by Lodovicci (10).
METHOD AND MATERIAL Our series consists of 90 cases, observed at the Gynecologic Clinic of the University of SBo Paulo Medical School. The patients underwent a gynecological examinaActa Obsrer Gynecol Scand 57 (1978)
90
C. A . Salvatore a n d 0 .L o d o v i c i
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tion (rectal palpation under narcosis), laparotomy (36 cases) or laparoscopy (54 cases) excretion urography (40 cases) and determination of genetic sex (40 cases). The operating technique is as follows (19). The patient must be placed in a semigynecologicalposition. We prefer the McIndoe technique for the building of the neovagina, using a free skin graft taken from the abdomen. The operation has two fundamental parts: the preparation of the graft, followed by construction of the tunnel between the rectum and the urethra and bladder. We usually operate together with a plastic surgeon. The abdominal stage After preparing the abdominal skin, the plastic surgeon takes a skin layer, with the dermatome, big enough to cover the acrylic mould. The raw part of the abdomen is protected with gauze and cotton and bandaged. The plastic surgeon then prepares the mounting of the epidermis over the acrylic mould, sewing the edges with nylon thread, while the gynecologist prepares the neovaginal tunnel. The vaginal stage An incision is made with the scalpel transverse to the vestibule or to the retrohymenal fossette, between the anus and the urethral meatus. One tries to cut first, transversely, the bundles of the “fibrous perineal nucleus”, found between the urethra and the anus-rectum. The incision is deepened and two small tunnels are formed on either side of the urethra. These are subsequently united in the mid-line. By means of the scalpel or its handle, or else with curved-tip scissors, the urethra and bladder are separated from the rectum up to the point where the base of the peritoneal bladder-rectal sac is reached. In this stage of the procedure we never open the urethra, the bladder, or the rectum-an accident which often happens when the surgeon attempts to advance the dissection through the mid-line. The tunnel must be large enough to take a medium-sized acrylic mould. Absolute haemostasis of all small vessels is obtained with plain catgut number 00 ligatures. According to the case, a partial dissection should be done at the sides of the pillars of the elevator ani muscle, so as to avoid subsequent stenosis of the vaginal introitus. After the mould is prepared and haemostasis is confirmed and after the tunnel has been soaked with physiological serum, the acrylic mould covered with the epidermis taken from the abdomen is introduced. The mould is fixed with cloth bandages passing through the mould‘s screw and attached to the abdominal bandages with sticking plaster. The patient must remain in bed until the seventh or eighth day, when she is allowed to walk and to take out the mould. She must subsequently undergo re-dressing every 48 hours, the mould being removed and re-inserted. She must be instructed concerning the long-term hygiene of the neovagina in order to avoid stenosis. Details of the post-opreative management are to be found in other papers (10, 17). An acrylic mould is inserted soon after surgery and it remains in place for 8 days. The dressing is changed every Acta Obsfet Gynecol Scand 57 (1978)
other day and, after discharge, about the 14th day, the patient herself will prepare and apply the dressings, consisting of removing and reinsetting the mould into the neovagina over a period of 6 months.
ANALYSIS OF THE MATERIAL Age (90 cases of vaginal agenesis) 1lL15 years, 7 cases 16-20 years, 46 cases (50%) 21-25 years, 24 cases 26-30 years, 10 cases 31-35 years, 4 cases Civil status Unmarried, 64 cases (71.1 %) Married, 26 cases (28.9%) Symptoms Amenorrhea, 90 cases (100 %) Impossibility of coitus, 22 cases (24.4%) Hypogastric pain, 18 cases (20%) Gynecological examination (Rectal) Non-palpable uterus, 70 cases (77.7%) Rudimentary palpable uterus, 14 cases Uterus increased in volume, 4 cases unrecognizable ovaries, 80 cases (88.8 %) Palpable ovaries, 8 cases Diagnosis Total agenesis, 70 cases (77.7%) Partial agenesis, 10 cases (1 1.1 %) Male pseudo-hermaphroditism, 3 cases Total agenesis plus haematometra, 3 cases (7.7%) Partial agenesis, haematometra and haematocolpos, 2 2 cases (7.7%) Total agenesis, haematometra and haematosalpinx, 2 2 cases (7.7%) Internal genital organs observed through Laparoscopy (54 cases) and Laparotomy (36 cases) ( a ) Ovaries: Normal, 68 cases (75.5 %) Polycystic, 6 cases Hypoplastic, 12 cases Rudimentary, 4 cases ( b ) Histology of the gonads (biopsy) (40 cases): Normal ovaries, 37 cases (92.5%) Rudimentary testicles, 3 cases (c) Uterus: Hypoplastic, 2 cases Increased in volume (haematometra), 4 cases Solid rudimentary, 50 cases (55.5%) Double uterus, 1 case Bicornuate uterus, 3 cases Hemi-uterus, 4 cases Two rudimentary uteri, 4 cases Lack of uterus, 22 cases (24.4%) ( d ) Tubes: Normal, 29 cases (32.2%)
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Fig. 1 . Collin speculum in the neovagina.
Hypoplastic, 6 cases Rudimentary (long) 42 cases (46.6 %) One rudimentary tube, 2 cases Lack of tubes, 10 cases Hematosalpinx, 1 case Excreting urography (40 cases) Ureters and normal kidneys, 33 cases (82.5%) Double pelvis-calyces, 3 cases Lack of left kidney, 4 cases Genetic sex (40 cases) Presence of the female sexual chromatin, 36 cases Lack of the female sexual chromatin (male), 4 cases Treatment (90 cases) Total neovagina, 80 cases (88.8%) Partial neovagina, 10 cases Neovagina and draining of the haematometra through the vagina, 3 cases Partial neovagina and draining of the haematometra and haematocolpos (through the vagina), 2 cases Neovagina and draining of the haematometra through the abdomen (hysterotomy), 1 case Neovagina and draining of the haematometra and haematosalpinx (through the abdomen), 1 case Neovagina and clitoris amputation, 2 cases Results (90 cases) Good graft integration and good vaginal permeability, 75 cases (83.3 %) Vaginal stenosis (fibrous ring) (2 re-operated with satisfactory results), 9 cases Partial disintegration of the graft due to a haematoma (all re-operated with satisfactory results), 6 cases Total satisfactory results including the 5 who were reoperated, 81 cases (90.0%)
Follow-up (between 1 and 6 years) (70 cases) Good results (penetration of the Collins speculum, medium sized) (Figs. 1, 2), 64 cases (91.3%) Partial stenosis, 6 cases With normal sexual relations, 54 cases With dyspareunia, 4 cases With normal menses, 4 cases Pregnancies (caesarean), 2 cases Cytology of neovagina (25 cases) (between 1-6) (Fig. 3) From 5 to 20% of acidophil cells, 18 cases (72.0%) From 21 to 40% of acidophil cells, 7 cases (28.0%) Biopsy of the neovagina showed atrophic epidermis (Fig. 4).
COMMENTARY As may be observed from an analysis of the cases, most patients sought advice in the age range 16 to 20 years (50% of the cases), coinciding with a wait of 3-5 years for the onset of the menarche. As a matter of fact, primary amenorrhea was the predominant symptom (100% of the cases). Hypogastric pains occurred in 20% of the cases and attempts at sexual intercourse among those who had tried had been unsuccessful in around 24.4 % of the cases. Of the casrs examined, 28.9% of the patients were married and had serious problems just after the marriage. Because of such problems, most authors think that the ideal age for the making of the neovagina is before the marriage. As we have stated Actu Obstet Gynecol Scand 57 (1978)
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92
C. A . Salvatore and 0 .Lodovici
Fig. 2. Collin speculum in the neovagina (cul-de-sac).
in an earlier work (18), libido was present in 79.1 % of the 49 cases previously studied. The diagnosis of vaginal agenesis is an easy one. The gynecological examination by rectal palpation allows the non-identification of the uterus in 77.5 % of the cases. Moreover, 77.7% of our 90 cases were of total vaginal agenesis and 11.1% of partial agenesis. There was haematometra in 7 cases (7.7%). The exploration of the internal genitalia by means of laparoscopy and laparotomy is unavoidable, but
shows that the ovaries are normal in 75.5 % of the cases. In 3 of the cases (pseudo-hermaphroditism) the biopsy revealed rudimentary testicles, confirmed by histological study. The psychological analysis in these cases was more important than the analysis of the sexual chromatin-hence the indication for a neovagina. A solid rudimentary uterus was found in 55.5 % of the cases, lack of uterus in 24.4% and functioning uterus with haematometra in only 7 cases (7.7%). These results agree with those of Counseller (5) (5
Fig. 3. Acidophil cells of the
neovagina. Acta Obstet Gynecol Scand 57 (1978)
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Fig. 4. Atrophic epidermis in the neovagina 3 years after the operation.
in 76), of Bryan et al. (2) (4 in a loo), Jackson (7) (5 in 128), Cordier (4) (1 in 23), Lodovici (10) (1 in 16) and Jeffcoate (8). These findings indicate its existence in 10% of cases. Normal tubes were, in the same way, found in only 32.2% of the cases. The practice of excretion urography is an important one as was shown by us and several other authors. The frequency of malformation of the urinary system is very high in cases of vaginal agenesis. Thus the absence of one of the kidneys (4 cases) and double pelvis-calyces (2 cases) was observed in 17.5 % of the cases. In a previous work, in an analysis of 21 cases, 15 (71.4%) were normal and the rest (28.6%) showed anomalies of the unnary system as represented by the lack of one of the kidneys, dilation of the ureters, of pelvis, double ureters, anomalous implantations, rotation displacement of one of the kidneys, or dislocated kidney, thus confirming the reports of others (5, 7, 10). In the experience of many authors (5, 7, 9, 10, 12, 13, 17, 18) the choice of treatment is the making of the neovagina by the McIndoe techniqueMcIndoe having, in 1950-59 established the basic principles for the formation of the neovagina from free skin grafts. Eighty complete neovaginas were made (88.8 %) and 10 partial ones, i.e. the vagina was completed in those cases where the agenesis was partial. In two of these cases there was agenesis of the exterior half
of the vagina, the inner half being full of menstrual blood (haematocolpos). The remaining 7 cases showed the presence of a vagina in its exterior third. In the 6 patients who had a functioning uterus, perforated moulds were used. As we have stated in a previous work (18), we recommend the use of an acrylic mould by the patient until the beginning of regular sexual activity, returning to its use in cases where a long intermption occurs. The results of the McIndoe technique are good, as graft integration occurred in 83.3% of the cases. If we add 6 cases where the integration was partial but re-operation was necessary, we achieved 90% of optimum graft integration and optimum condi tions of permeability. We observed the presence of a fibrous ring in only 9 of the 90 cases. Page & Owley (14) claim 81 % good results. The late follow-up observed between one and six years showed 91.3% satisfactory results in 70 cases, of which 54 had normal sexual relations, two had become pregnant and undergone caesarian section. Only 6 cases showed a small vaginal stenosis and 4 complained of dyspareunia. We have not so far found enterocele. In our experience, therefore, the McIndoe technique for the making of an artificial vagina is the one offering the best results and the long-term use of an acrylic mould guarantees the late success of the operation. Acta Obstet Gynecol Scand 57 (1978)
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C. A . Salvatore and 0.Lodovici
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REFERENCES 1. Barrows, D. N.: Am J Obstet Gynecol73: 609, 1957. 2. Bryan, A. L., Nigro, J. A. & Counseller, V. S.: Surg Gynecol72: 504, 1958. 3. Cali, R. W. & Pratt, T. H.: Am J Obstet Cynecol 100:752, 1968. 4. Cordier, G.: C. R. SOCFranc Gynecol22: 156, 1952. 5. Counseller, V. S.: JAMA 136: 861, 1948. 6. Evans, T. N.: Am J Obstet Gynecol99: 944, 1967. 7. Jackson, I.: J Obstet Gynaecol Br Emp 72: 335, 1965. 8. Jeffcoate, T. N. A.: J Obstet Gynaecol Br Comm 76:%1, 1969. 9. Jones, H. & Scott, W. W.: Hermaphroditism, Genital Anomalies and Related Endocrine Disorders. Williams & Wilkins, Baltimore, 1958. 10. Lodovici, 0.: NeoXJaginoplastia metodizada no tratamento de ausCncia congsnita da vagina. Tese. Fac. Med. Univers. Sio Paulo, 1966. 11. Lodovici, 0. & Salvatore, C. A,: An Bras Ginec 64:251, 1967. 12. McIndoe, A. H.: Proc R SOCMed52:952, 1959. 13. McIndoe, A. H.: Br J Plast Surg2: 254, 1960. 14. Page, E. U. & Owsley, Jr, J. Q.: Am J Obstet Gynecol 105: 774, 1969.
Actu Obsret Cvnecol Sccind 57 (1978)
15. Riva, H. L. & Harding, R. L.: Obstet Cynecol4: 517, 1954. 16. Salvatore, C. A , , Halbe, H. W., Cruz, D. S., Braga, L. F. & Gallucci, J . : Arq Obst Ginec Sio Paulo, 4 : 151, 1963. 17. Salvatore, C. A , , Spina, Lodovici, 0. & Faure, R.: Rev Paulista de Medicina65: 63, 1964. 18. Salvatore, C. A., Lodovici, 0. & Gallucci, J.: AnBras Ginec64: 317, 1967. 19. Salvatore, C. A.: Ginecologia Operatbria. Guanabara Koogan, Rio de Janeiro, 1974. 20. Ulfelder, H.: Am J Obstet Gynecol 100: 745, 1968. 21. Williams, E. A.: J Obstet Gynaecol Br Comm 79: 1147, 1972. Submitted for publication Febr. 27, I977 C. A. Salvatore Dept. of Obst. and Gyn. Hospital las Clinicas Srio Paulo Brad
