- Email: [email protected]
An anomaly of the external genitalia in female patients with exstrophy of the bladder
An anomaly of the external genitalia in female patients with exstrophy of the bladder HOWARD Baltimore,
W.
JONES,
JR.,
M.D
Maryland
Exstrophy of the bladder is a rare anomaly which affects both males and females. Some affected females have an associated anomaly of the external genitalia which, while allowing menstruation, is sufficiently disabling to prevent sexual activity. Hitherto little attention has been given to the genital portion of this anomaly and its correction, probably due to the early death of patients with exstrophy. With improved prognosis, the anomaly of the external genitalia has become of importance. This article estimates its frequency and describes the anomaly and its surgical correction.
F E M A I. E patients with exstrophy of the bladder sometimes have an associated anomaly of the external genitalia which prevents normal vaginal function. It is remarkable that this anomaly has received only passing attention in the literature-has had neither its gynecologic significance emphasized nor its correction described. This is perhaps understandable because (1) not all patients with exstrophy have the associated anomaly in question, (2) until recently the treatment of exstrophy was so unsatisfactory, and the defect so compromised longevity, that vaginal function was until now of only secondary importance, and (3) the surgical correction of the anomaly is relatively simple so that no doubt it has been carried out without formal documentation. The purposes of this paper are ( 1) to describe the anomaly of the external genitalia sometimes associated with exstrophy of the
bladder, (2) to estimate the frequency of this associated anomaly, and (3) to describe its surgical correction. The material consists of five patients who sought gynecologic care because of a nonfunctioning vagina and of 25 female patients with exstrophy seen at The Johns Hopkins Hospital from January, 1952, through Dec. 30, 1971, a 20 year period, used in an effort to estimate the frequency of the genital malformation. The
anomaly
In the female child with exstrophy, the bladder lies open and everted on the surface of the lower abdomen. The urethral canal lies completely unroofed. There is, therefore, no urethral orifice. The clitoris is bifid. The labia minora, which normally arise as a continuation of the prepuce and flow posteriorly around the vaginal orifice, are by virtue of the cleft clitoris separated anteriorly but can be traced posteriorly in a rather normal manner around the vaginal orifice. If that vaginal orifice be displaced anteriorly, as in the anomaly under discussion, the labia minora are quite insignificant, especially in the newborn (Fig. 1). The labia majora are separated anteriorly but otherwise seem to be unaffected. The size and placement of the vaginal
From the Department of Gynecology and Obstetrics, The Johns Hopkins University School of Medicine. Presented at the Ninety-sixth Annual Meeting of the American Gynecological Society, Colorado Springs, Colorado, May 2-5, 1973. Reprint requests: Dr. Howard W. Jones, Jr., Johns Hopkins Hospital, 601 North Broadway, Baltimore, Maryland 21205. 748
Volume Number
117 6
External
orifice are sometimes quite abnormal and sometimes normal. However, our special interest is in those patients in whom the orifice is small and anteriorly located in such a manner that the vagina is quite nonfunctional (Figs. 2 and 3). Injection of radiopaque material through the displaced orifice will give an estimate of the size of the unusable vagina (Figs. 4 and 5). Whether the displacement of the vaginal orifice is simply a forward mechanical shift secondary to the serious defects located even more anteriorly or whether the defect is caused by abnormal fusion of the scrotolabial folds in the embryo is a moot point. The latter explanation would imply some abnormal androgenic activity active during embryonic life, but there is really no substantial evidence for this. All five patients in this series exhibited the complete or classical type of exstrophy according to the classifications of Uson and associates’ and Marshall and Muecke,2 respectively. All were treated by urinary diversion and excision of the bladder and all enjoyed good general health. It is remarkable that two of the five had excellent results with respect to the upper urinary tracts with the now obsolete operation of uterosigmoidostomy (Table I ) . The
incidence
of the
vaginal
anomaly
The incidence of the vaginal anomaly is difficult to ascertain from the literature. As previously mentioned, there has been remarkably little attention directed to the reproductive tract of female patients with exstrophy. In an effort to determine the frequency of the anomalously located vaginal orifice, a retrospective examination was undertaken of the charts of all female patients with exstrophy admitted to The Johns Hopkins HOSpita1 in the 20 year period from 1952 to 1971. There were 25 such patients. Unfortunately, there was absolutely no mention of the external genitalia in seven of these patients, sometimes in spite of multiple admissions. Among the remaining 18, the description of the external genitalia was often far from adequate. Thus, our conclusions, based on such data, are at best estimates. Nevertheless,
genital
anomaly
with bladder
exstrophy
749
Fig. 1. Patient T. K. Exstrophy of bladder in child aged l& years. Note absence of vaginal orifice at expected site. it appears as if 13 patients could be considered to have had a small and anteriory displaced vaginal orifice, while the remaining five patients had a vaginal orifice which was normal in size and location, or even more patulous than normal in a few. Thus it seems as if about two thirds of female patients with exstrophy have a nonfunctioning vagina and require a relatively simple gynecologic procedure in order to provide vaginal function. The surgical correction of the anomaly The correction of the anomaly is relatively simple. In all instances the bladder will surely have been previously removed, as it had been in all of our patients, and the urine diverted by means of an ilial loop or, in a few instances, by a ureterosigmoidostomy. The operative procedure consists of two steps: ( 1) the enlargement and relocation of the vaginal orifice and (2) the approximation in the midline of the two halves of the cleft clitoris and associated tissue. The enlargement and relocation of the
750
November Am. J. Obstet.
Jones
Fig. 2. Patient orifice
has been
T. N., displaced
aged
of lateral of vaginal orifice. B, Drawing normal anterior displacement
orifice
can
be
Exstrophied
bladder
has
been
removed;
tiny
vaginal
far anteriorly.
Fig. 3. A, Drawing
vaginal
20 years.
15, 1973 Gynecol.
accomplished
view of patient with exstrophy of bladder of lateral view of patient with exstrophy of vaginal orifice.
by
mid-
line incision dividing what seems to be fused scrotolabial folds. The incision needs to be carried posteriorly far enough to establish the vaginal orifice in its proper position just anterior to the anus. In the more posterior aspect of the incision, the septum may have a thickness of up to 2 cm. or even more, so
with normal of bladder
location with ab-
that the vaginal epithelium may have to be undermined posteriorly and laterally to secure good approximation of the vaginal epithelium to the skin edge. Interrupted stitches are convenient. A vaginal pack to hold apart the new vaginal outlet completes this portion of the operation (Fig. 6, A and B) . The second step of the operation is purely
Volume Number
117 6
External
genital
anomaly
with bladder
exstrophy
751
Fig. 5. Patient Fig. 4. Patient T. radiography after soluble radiopaque serted into orifice.
N. Oblique view of vagina by injection of 8 C.C. of watermaterial through catheter in-
T. N. Anterior view of vagina by radiography after injection of 8 C.C. of radiopaque material and after removal of catheter. Note absence of pubic rami characteristic of patients with exstrophy of bladder.
Fig. 6, A and B. A, Drawing of situation prior to first step midline incision beginning at vaginal orifice and continuing view through displaced vaginal orifice. Note increasing incision is carried posteriorly. cosmetic the
and
midline
associated sue.
While
not
necessary
consists the
labia
cleft
of
minora
this for
bringing
clitoris
and, and
together thereby,
surrounding
portion
of
the
procedure
vaginal
function,
it has
in
of
of operation and showing location of posteriorly. B, Drawing of sagittal thickness of perineal structures as
interest
the
independently
tis-
something
is been
to
us
that
and could
each
of
spontaneously not
abnormal appearance To bring together
be
done
the
patients
inquired to
if
improve
of the genitalia. the two halves
the of
the
752
November Am. J. Obstet.
Jones
Table I. Summary
of five case reports
Type of Patient T. N. 09433 C. F. T. K. 246418 Z. B. 38155 J. M. 40153
Fig. area.
15, 1973 Gynecd.
exstrophy
Associated -
Complete Complete Complete
Menses
anomalies
Rectal
Complete Complete
6, C and D. D, Drawing
Webbing
fingers
and
toes
Comment
As mentioned above, the etiology of the displaced diminutive vaginal orifice is probably associated with and secondary to the
diversion
11, regular
%!
Onset 12, regular Premenarchial
1?4, 1 %a
Ureterosigmoidostomy Ileal loop
Onset
16, regular
10
Ileal
loop
Onset
12, regular
9
Ileal
loop
C, Drawing of second step of operation showing showing central approximation of divided halves
clitoris, a diamond-shaped area of skin and subcutaneous tissue is excised. The medial aspect of each side of the clitoris is denuded and the latter flaps are undermined, so that the defect may be closed from side to side. Because of the scar tissue resulting from the previous excision of the bladder, there may be considerable tension on the lateral flap, so that approximation may be less than complete (Fig. 6, C and D) Vaginal function after the procedure described has been quite satisfactory (Fig. 7).
Type of urinary
Ureterosigmoidostomy
Onset
prolapse
Age at bladder excision
excision of diamond-shaped of clitoris.
basic defect causing the exstrophied bladder. Thus the difficulty may be simply a mechanical anterior displacement due to the major nearby defect affecting not only the bladder but the urethra, clitoris, and even the underlying bone. Alternately, it is possible that the anterior location of the vaginal orifice is due to abnormal fusion of the scrotolabial folds during embryonic life. This seems unlikely, as such fusion would imply some abnormal androgenie stimulus and in no patient has there been an identifiable androgenic source, nor has there been observed any other androgenic effect. Furthermore, and most importantly, androgenic fusion involves the scrotolabial folds from which derive the labia majora and in
Volume Number
Age urinary
117 6
at diversion
External
Age genital
seen for anomaly
Upper status
y$?
15
Normal
16/12
15 6
Normal Moderate
10
20
9
16
Moderate nephrosis Normal
1%
urinary when
genital
tract seen
anomaly
with bladder
General when Good
bilateral
hydronephrosis
to severe bilateral hydrowith chronic infection
hearth Seen
exstrophy
753
Age operated on i for genital anomaly 20
Good Good
17 -
Good
21
Good
17
Fig. 7B. Patient Z. B. Photograph 10 days postoperatively. Vaginal function subsequently proved to be quite
Fig. 7A. Patient tion. Note tiny and separation
Z. B. Photograph prior to operaanteriorly displaced vaginal orifice of two halves of clitoris.
the exstrophied cases the labia majora clearly are not immediately involved in the fusion. In this connection, it is noteworthy that in exstrophy patients in general, and in our series in particular, normal pubertal development took place at the expected time and the menarche appeared when expected. There remains for discussion the question of the most suitable time for the vaginal reconstruction. It might seem that this could be correlated with the onset of sexual activity
satisfactory.
which would, of course, vary from patient to patient. On the other hand, one patient who had an extremely small vaginal outlet, which admitted only a uterine sound, had to be operated upon at the age of 20 before her vaginal outlet was corrected for a unilateral tubo-ovarian abscess which conceivably could have been associated with poor vaginal drainage. However, it is interesting that no patient had symptoms related to obstruction to the outflow of menstrual blood. It is our current belief, therefore, that operation can in general be delayed until the onset of sexual activity, except perhaps where the vaginal orifice is quite small and, therefore, potentially obstructive, in which case the operation should probably be carried out some time prior to the menarche.
754
Jones
REFERENCES
1. Uson, A. C., Lattimer, J. K., and Melicow, M. M.: Pediatrics 23: 927, 1959.
Discussion C. BENSOK, Portland, Oregon. Exstrophy of the bladder, one of the most dread of all congenital anomalies, continues to challenge the urologist. Now, enter the gynecologist, who may have better surgical luck, because serious genital maldevelopments which often accompany exstrophy are not as formidable. Dr. Jones deserves much commendation for presenting another fine paper. There is an extensive and complicated anatomic and urologic literature pertaining to exstrophy of the bladder. The ratio of males to females with this abnormality is about 3: 1. Dozens of imaginative, ingenious operations to correct or relieve this problem have been handsomely illustrated. Some successes have been reported but many failures are admitted. The broad picture is still blurred but Dr. Jones is helping to bring it into focus. The true incidence of significant associated gynecologic anomalies is still moot. With regard to our present concern, birth certificate statistics are superficial and worthless; pediatric reporting is prejudiced and inaccurate; the urologic listings are preoccupied and incomplete. I cannot accept Dr. Jones’ hospital incidence of gynecologic anomalies associated with bladder ectopy as a measure of their general frequency, any more than our own. He is referring to 18 females in 20 years whose gynecologic abnormalities were recorded; we have seven such in 19 years. This only tells us that many patients with bladder exstrophy and some patients with an additional gynecologic problem gravitate to centers. This is even more confusing when we consider a related problem of 100 patients with congenital absence of the vagina referred to the Mayo Clinic; no individual had had exstrophy of the b1adder.l The completeness of our over-all recording and reporting obviously is faulty. Moreover, a generous sampling of the literature during the past 20 years has not been very helpful in the identification of the types of gynecologic anomalies to be expected with exstrophy of the bladder. Surprisingly, only a few series are available. Higgins” reported briefly on 147 patients with exstrophy of the bladder. (He did not give the DR.
RALPH
November Am. J. Obstet.
15, 1973 Gynecol.
2. Marshall, V. F., and Muecke, E. C.: J. Ural. 88: 766. 1962.
number of females but stated that males were three times more prevalent.) The associated gynecologic anomalies included three patients with vesicorectal fistula, one with vesicorectovaginal fistula, and one with a double vagina and bicornuate uterus. Gynecologic corrective measures were omitted. (It is obvious on review of this article that only the more gross abnormalities were recorded.) Uson and associates3 reported on 23 females (and 69 males) with bladder exstrophy. Seventeen of the 23 girls had other anomalies. Of these, gynecologic maldevelopments included two with absence of the vagina, one had a double vagina, and one had a complete uterine prolapse. There was no mention of gynecologic correction. Many case reports describing one or two patients with bladder ectopy are available in the journals, but most of these do not include COexistent gynecologic abnormalities. The University of Oregon series of seven females (and 19 males) with exstrophy previously reported by Stagner and Hodges4 comprise two patients who had “agenesis of the genitalia” (actually the problem described by Dr. Jones), complete absence of the vagina in one and a bicornuate uterus in another. I do not believe that we can generalize using the data available. Nevertheless, partial gynetresia (ci la Dr. Jones), absence of the vagina, and double vagina seem to be some of the major gynecologic anomalies associated with bladder exstrophy. One of our patients, aged 20 when first seen at our institution, had been told by her local physician that she could “have neither sex nor children” because of a vagina which would not even admit the examiner’s little finger. The clitoris was bitid and the labia were mere flattened folds. In infancy, the patient had sustained excision of an exstrophied bladder and a bilateral ureterosigmoidostomy. The diversion had been very successful and good kidney function had been maintained. At gynecologic surgery, the situation was as pictured by Dr. Jones. A shallow bilateral mediolateral episiotomy was accomplished and reapproximation of the more superficial tissues anteroposteriorly was effected. The bifid clitoris was
Volume Number
117 6
External
revised also. An adequate introitus and vagina was the result. The patient married soon thereafter and pregnancy followed promptly. Regrettably, the patient aborted twice at two months and we do not have a recent follow-up. One of our bladder ectopy patients also had a congenital absence of the vagina and a vestigeal cervix and uterus. Cystectomy and ileal loop ureteral diversion were accomplished. A patulous anus, because of virtual absence of the anal sphincter, necessitated a p&manent colostomy. The patient married and reports satisfactory coitus using the blind anorectal segment. More traditional (e.g., split-thickness skin graft) vaginoplasty now is employed for construction of a vagina when congenital absence is the indication. Nonetheless, Carlson has successfully converted the inverted exstrophic bladder into a functional vagina after urinary diversion.4 Be this as it may, assuming normal internal genitalia and ovarian function, pregnancy and even vaginal delivery should be considered likely in patients born with bladder ectopy who have had reconstructive gynecologic surgery, as suggested by Dr. Jones and others.5, G REFERENCES
1. Bryan, A. L., Nigro, J. A., and Counseller, V.: Gynecol. Obstet. 88: 79, 1949. 2. Higgins, C. C.: J. Urol. 80: 279, 1958. 3. Uson, A. C., Lattimer, J. K., and Melicow, M. M.: Pediatrics 23: 927, 1959. 4. Stagner, R. V., and Hodges, C. V.: J. Urol. 89: 53, 1963. 5. Rubovitz, F. E., and Arnkoff, M.: J. Urol. 62: 52, 1949. 6. Nabity, S. F.: Nebraska State Med. J. 51: 284, 1966. DR. FRANK C. GREISS, JR., Winston-Salem, North Carolina. I enjoyed Dr. Jones’ paper immensely because I have always been interested in the area of genital anomalies. I do not know how many cases of exstrophy of the bladder it takes to make a specialist but since I just carried such a patient through a pregnancy, I thought I should tell you about her. The patient was a 22-year-old primigravida who had repair of her exstrophy and bilateral ureterosigmoidostomy at the age of four. The repair was very successful and an IVP obtained during the fourth month of pregnancy showed a normal upper urinary tract. As Dr. Jones pointed out, the rami of the pubis are usually absent in such patients. In addition, prolapse of the uterus is often associated with exstrophy. The most
genital
anomaly
with
bladder
exstrophy
755
notable observations during this patient’s pregnancy related to these associated complications. The patient experienced extreme pelvic pressure and discomfort frequently requiring bed rest. We tried to support the pubic area with a corset but this was not really successful. The infant had a vertex presentation and the head could be felt just under the examining finger in what would have been the pubic area. Her labor was perfectly normal except, as one should have expected, the normal mechanism of labor did not occur. The presentation was LOT but the absence of normal anatomic structures which make for a normal mechanism of labor caused a persistent transverse presentation. Midforceps delivery was a delight because, in the absence of normal bony structures, there was all the room in the world. Forceps application and rotation were performed without the usual difficulties. DR. ROBERT B. JAFFE, Ann Arbor, Michigan. We have been troubled in both patients with the adrenogenital syndrome and patients with the type of anomaly which Dr. Jones described about the concept of waiting until they are ready for coitus before embarking upon surgical correction. 1 say this not so much from the surgical point of view, because this would be an optimal time for surgery, but from a psychic point of view. It seems to me that there is a good deal of psychic trauma connected with both of these types of lesions and that perhaps we should consider earlier correction to obviate some of the psychic difficulties that these individuals have as they go through pubescence. DR. CLYDE L. RANDALL, Buffalo, New York. I was impressed by many things about the author’s presentation. Among those points was the fact that he aroused our suspicions as to how a patient with no functional vagina would develop a PID and then was very careful not to tell us what the culture showed. In the days when bacteria still had an opportunity to develop interesting pathology, there used to be a very fascinating entity of pneumococcic salpingitis. It was quite dramatic. It often followed respiratory infection. And I do not know what all happens to Dr. Jones’ patients before he sees them, but I would like to know the results of the culture. DR. JONES (Closing). Dr. Jaffe has made a very important point. Certainly in the adrenogenital syndrome we think reconstruction at a very early time is the thing to do for the reason
756
Jones
that he said We regard 18 months of age as the upper limit for reconstruction. That philosophy certainly could be applied to this situation. We just have not had the opportunity to apply that except in one child who was four years old and who came because she noted that her genitalia did not correspond to what she saw in her schoolmates. We have, indeed, offered reconstruction to her and to her mother but have not done it yet because, after the problem was explained to them, this did not seem to be important. Also, I think it is well to recall that in the exstrophy cases the seriousness of the exstrophy per se usually involves the first several years of life. So that the situation is somewhat different here, I think, than in the adrenogenital syndrome. Dr. Randall asks about the cuture in the pa-
November Am. J. Obstet.
15, 1973 Gynecol.
tient with salpingitis. It was a foul-smelling colon bacillus. It was not a pneumococcus at all. This patient had rather foul-smelling vaginal contents, actually, when it was opened. We thought that there was probably an ascending infection from poor hygiene in the vagina due to that rather long, narrow tract. She is the one who required a No. 16 catheter to feed down through the sinus before we could get an x-ray. It could be that, based on this experience, these narrow orifices should be opened at an earlier time than when a functioning vagina for a functioning vagina’s sake seems to be necessary. I realize that the vaginal orifice in some cases varies a little bit and can be posterior so that dilatation might be possible rather than surgical correction.
W.
JONES,
JR.,
M.D
Maryland
Exstrophy of the bladder is a rare anomaly which affects both males and females. Some affected females have an associated anomaly of the external genitalia which, while allowing menstruation, is sufficiently disabling to prevent sexual activity. Hitherto little attention has been given to the genital portion of this anomaly and its correction, probably due to the early death of patients with exstrophy. With improved prognosis, the anomaly of the external genitalia has become of importance. This article estimates its frequency and describes the anomaly and its surgical correction.
F E M A I. E patients with exstrophy of the bladder sometimes have an associated anomaly of the external genitalia which prevents normal vaginal function. It is remarkable that this anomaly has received only passing attention in the literature-has had neither its gynecologic significance emphasized nor its correction described. This is perhaps understandable because (1) not all patients with exstrophy have the associated anomaly in question, (2) until recently the treatment of exstrophy was so unsatisfactory, and the defect so compromised longevity, that vaginal function was until now of only secondary importance, and (3) the surgical correction of the anomaly is relatively simple so that no doubt it has been carried out without formal documentation. The purposes of this paper are ( 1) to describe the anomaly of the external genitalia sometimes associated with exstrophy of the
bladder, (2) to estimate the frequency of this associated anomaly, and (3) to describe its surgical correction. The material consists of five patients who sought gynecologic care because of a nonfunctioning vagina and of 25 female patients with exstrophy seen at The Johns Hopkins Hospital from January, 1952, through Dec. 30, 1971, a 20 year period, used in an effort to estimate the frequency of the genital malformation. The
anomaly
In the female child with exstrophy, the bladder lies open and everted on the surface of the lower abdomen. The urethral canal lies completely unroofed. There is, therefore, no urethral orifice. The clitoris is bifid. The labia minora, which normally arise as a continuation of the prepuce and flow posteriorly around the vaginal orifice, are by virtue of the cleft clitoris separated anteriorly but can be traced posteriorly in a rather normal manner around the vaginal orifice. If that vaginal orifice be displaced anteriorly, as in the anomaly under discussion, the labia minora are quite insignificant, especially in the newborn (Fig. 1). The labia majora are separated anteriorly but otherwise seem to be unaffected. The size and placement of the vaginal
From the Department of Gynecology and Obstetrics, The Johns Hopkins University School of Medicine. Presented at the Ninety-sixth Annual Meeting of the American Gynecological Society, Colorado Springs, Colorado, May 2-5, 1973. Reprint requests: Dr. Howard W. Jones, Jr., Johns Hopkins Hospital, 601 North Broadway, Baltimore, Maryland 21205. 748
Volume Number
117 6
External
orifice are sometimes quite abnormal and sometimes normal. However, our special interest is in those patients in whom the orifice is small and anteriorly located in such a manner that the vagina is quite nonfunctional (Figs. 2 and 3). Injection of radiopaque material through the displaced orifice will give an estimate of the size of the unusable vagina (Figs. 4 and 5). Whether the displacement of the vaginal orifice is simply a forward mechanical shift secondary to the serious defects located even more anteriorly or whether the defect is caused by abnormal fusion of the scrotolabial folds in the embryo is a moot point. The latter explanation would imply some abnormal androgenic activity active during embryonic life, but there is really no substantial evidence for this. All five patients in this series exhibited the complete or classical type of exstrophy according to the classifications of Uson and associates’ and Marshall and Muecke,2 respectively. All were treated by urinary diversion and excision of the bladder and all enjoyed good general health. It is remarkable that two of the five had excellent results with respect to the upper urinary tracts with the now obsolete operation of uterosigmoidostomy (Table I ) . The
incidence
of the
vaginal
anomaly
The incidence of the vaginal anomaly is difficult to ascertain from the literature. As previously mentioned, there has been remarkably little attention directed to the reproductive tract of female patients with exstrophy. In an effort to determine the frequency of the anomalously located vaginal orifice, a retrospective examination was undertaken of the charts of all female patients with exstrophy admitted to The Johns Hopkins HOSpita1 in the 20 year period from 1952 to 1971. There were 25 such patients. Unfortunately, there was absolutely no mention of the external genitalia in seven of these patients, sometimes in spite of multiple admissions. Among the remaining 18, the description of the external genitalia was often far from adequate. Thus, our conclusions, based on such data, are at best estimates. Nevertheless,
genital
anomaly
with bladder
exstrophy
749
Fig. 1. Patient T. K. Exstrophy of bladder in child aged l& years. Note absence of vaginal orifice at expected site. it appears as if 13 patients could be considered to have had a small and anteriory displaced vaginal orifice, while the remaining five patients had a vaginal orifice which was normal in size and location, or even more patulous than normal in a few. Thus it seems as if about two thirds of female patients with exstrophy have a nonfunctioning vagina and require a relatively simple gynecologic procedure in order to provide vaginal function. The surgical correction of the anomaly The correction of the anomaly is relatively simple. In all instances the bladder will surely have been previously removed, as it had been in all of our patients, and the urine diverted by means of an ilial loop or, in a few instances, by a ureterosigmoidostomy. The operative procedure consists of two steps: ( 1) the enlargement and relocation of the vaginal orifice and (2) the approximation in the midline of the two halves of the cleft clitoris and associated tissue. The enlargement and relocation of the
750
November Am. J. Obstet.
Jones
Fig. 2. Patient orifice
has been
T. N., displaced
aged
of lateral of vaginal orifice. B, Drawing normal anterior displacement
orifice
can
be
Exstrophied
bladder
has
been
removed;
tiny
vaginal
far anteriorly.
Fig. 3. A, Drawing
vaginal
20 years.
15, 1973 Gynecol.
accomplished
view of patient with exstrophy of bladder of lateral view of patient with exstrophy of vaginal orifice.
by
mid-
line incision dividing what seems to be fused scrotolabial folds. The incision needs to be carried posteriorly far enough to establish the vaginal orifice in its proper position just anterior to the anus. In the more posterior aspect of the incision, the septum may have a thickness of up to 2 cm. or even more, so
with normal of bladder
location with ab-
that the vaginal epithelium may have to be undermined posteriorly and laterally to secure good approximation of the vaginal epithelium to the skin edge. Interrupted stitches are convenient. A vaginal pack to hold apart the new vaginal outlet completes this portion of the operation (Fig. 6, A and B) . The second step of the operation is purely
Volume Number
117 6
External
genital
anomaly
with bladder
exstrophy
751
Fig. 5. Patient Fig. 4. Patient T. radiography after soluble radiopaque serted into orifice.
N. Oblique view of vagina by injection of 8 C.C. of watermaterial through catheter in-
T. N. Anterior view of vagina by radiography after injection of 8 C.C. of radiopaque material and after removal of catheter. Note absence of pubic rami characteristic of patients with exstrophy of bladder.
Fig. 6, A and B. A, Drawing of situation prior to first step midline incision beginning at vaginal orifice and continuing view through displaced vaginal orifice. Note increasing incision is carried posteriorly. cosmetic the
and
midline
associated sue.
While
not
necessary
consists the
labia
cleft
of
minora
this for
bringing
clitoris
and, and
together thereby,
surrounding
portion
of
the
procedure
vaginal
function,
it has
in
of
of operation and showing location of posteriorly. B, Drawing of sagittal thickness of perineal structures as
interest
the
independently
tis-
something
is been
to
us
that
and could
each
of
spontaneously not
abnormal appearance To bring together
be
done
the
patients
inquired to
if
improve
of the genitalia. the two halves
the of
the
752
November Am. J. Obstet.
Jones
Table I. Summary
of five case reports
Type of Patient T. N. 09433 C. F. T. K. 246418 Z. B. 38155 J. M. 40153
Fig. area.
15, 1973 Gynecd.
exstrophy
Associated -
Complete Complete Complete
Menses
anomalies
Rectal
Complete Complete
6, C and D. D, Drawing
Webbing
fingers
and
toes
Comment
As mentioned above, the etiology of the displaced diminutive vaginal orifice is probably associated with and secondary to the
diversion
11, regular
%!
Onset 12, regular Premenarchial
1?4, 1 %a
Ureterosigmoidostomy Ileal loop
Onset
16, regular
10
Ileal
loop
Onset
12, regular
9
Ileal
loop
C, Drawing of second step of operation showing showing central approximation of divided halves
clitoris, a diamond-shaped area of skin and subcutaneous tissue is excised. The medial aspect of each side of the clitoris is denuded and the latter flaps are undermined, so that the defect may be closed from side to side. Because of the scar tissue resulting from the previous excision of the bladder, there may be considerable tension on the lateral flap, so that approximation may be less than complete (Fig. 6, C and D) Vaginal function after the procedure described has been quite satisfactory (Fig. 7).
Type of urinary
Ureterosigmoidostomy
Onset
prolapse
Age at bladder excision
excision of diamond-shaped of clitoris.
basic defect causing the exstrophied bladder. Thus the difficulty may be simply a mechanical anterior displacement due to the major nearby defect affecting not only the bladder but the urethra, clitoris, and even the underlying bone. Alternately, it is possible that the anterior location of the vaginal orifice is due to abnormal fusion of the scrotolabial folds during embryonic life. This seems unlikely, as such fusion would imply some abnormal androgenie stimulus and in no patient has there been an identifiable androgenic source, nor has there been observed any other androgenic effect. Furthermore, and most importantly, androgenic fusion involves the scrotolabial folds from which derive the labia majora and in
Volume Number
Age urinary
117 6
at diversion
External
Age genital
seen for anomaly
Upper status
y$?
15
Normal
16/12
15 6
Normal Moderate
10
20
9
16
Moderate nephrosis Normal
1%
urinary when
genital
tract seen
anomaly
with bladder
General when Good
bilateral
hydronephrosis
to severe bilateral hydrowith chronic infection
hearth Seen
exstrophy
753
Age operated on i for genital anomaly 20
Good Good
17 -
Good
21
Good
17
Fig. 7B. Patient Z. B. Photograph 10 days postoperatively. Vaginal function subsequently proved to be quite
Fig. 7A. Patient tion. Note tiny and separation
Z. B. Photograph prior to operaanteriorly displaced vaginal orifice of two halves of clitoris.
the exstrophied cases the labia majora clearly are not immediately involved in the fusion. In this connection, it is noteworthy that in exstrophy patients in general, and in our series in particular, normal pubertal development took place at the expected time and the menarche appeared when expected. There remains for discussion the question of the most suitable time for the vaginal reconstruction. It might seem that this could be correlated with the onset of sexual activity
satisfactory.
which would, of course, vary from patient to patient. On the other hand, one patient who had an extremely small vaginal outlet, which admitted only a uterine sound, had to be operated upon at the age of 20 before her vaginal outlet was corrected for a unilateral tubo-ovarian abscess which conceivably could have been associated with poor vaginal drainage. However, it is interesting that no patient had symptoms related to obstruction to the outflow of menstrual blood. It is our current belief, therefore, that operation can in general be delayed until the onset of sexual activity, except perhaps where the vaginal orifice is quite small and, therefore, potentially obstructive, in which case the operation should probably be carried out some time prior to the menarche.
754
Jones
REFERENCES
1. Uson, A. C., Lattimer, J. K., and Melicow, M. M.: Pediatrics 23: 927, 1959.
Discussion C. BENSOK, Portland, Oregon. Exstrophy of the bladder, one of the most dread of all congenital anomalies, continues to challenge the urologist. Now, enter the gynecologist, who may have better surgical luck, because serious genital maldevelopments which often accompany exstrophy are not as formidable. Dr. Jones deserves much commendation for presenting another fine paper. There is an extensive and complicated anatomic and urologic literature pertaining to exstrophy of the bladder. The ratio of males to females with this abnormality is about 3: 1. Dozens of imaginative, ingenious operations to correct or relieve this problem have been handsomely illustrated. Some successes have been reported but many failures are admitted. The broad picture is still blurred but Dr. Jones is helping to bring it into focus. The true incidence of significant associated gynecologic anomalies is still moot. With regard to our present concern, birth certificate statistics are superficial and worthless; pediatric reporting is prejudiced and inaccurate; the urologic listings are preoccupied and incomplete. I cannot accept Dr. Jones’ hospital incidence of gynecologic anomalies associated with bladder ectopy as a measure of their general frequency, any more than our own. He is referring to 18 females in 20 years whose gynecologic abnormalities were recorded; we have seven such in 19 years. This only tells us that many patients with bladder exstrophy and some patients with an additional gynecologic problem gravitate to centers. This is even more confusing when we consider a related problem of 100 patients with congenital absence of the vagina referred to the Mayo Clinic; no individual had had exstrophy of the b1adder.l The completeness of our over-all recording and reporting obviously is faulty. Moreover, a generous sampling of the literature during the past 20 years has not been very helpful in the identification of the types of gynecologic anomalies to be expected with exstrophy of the bladder. Surprisingly, only a few series are available. Higgins” reported briefly on 147 patients with exstrophy of the bladder. (He did not give the DR.
RALPH
November Am. J. Obstet.
15, 1973 Gynecol.
2. Marshall, V. F., and Muecke, E. C.: J. Ural. 88: 766. 1962.
number of females but stated that males were three times more prevalent.) The associated gynecologic anomalies included three patients with vesicorectal fistula, one with vesicorectovaginal fistula, and one with a double vagina and bicornuate uterus. Gynecologic corrective measures were omitted. (It is obvious on review of this article that only the more gross abnormalities were recorded.) Uson and associates3 reported on 23 females (and 69 males) with bladder exstrophy. Seventeen of the 23 girls had other anomalies. Of these, gynecologic maldevelopments included two with absence of the vagina, one had a double vagina, and one had a complete uterine prolapse. There was no mention of gynecologic correction. Many case reports describing one or two patients with bladder ectopy are available in the journals, but most of these do not include COexistent gynecologic abnormalities. The University of Oregon series of seven females (and 19 males) with exstrophy previously reported by Stagner and Hodges4 comprise two patients who had “agenesis of the genitalia” (actually the problem described by Dr. Jones), complete absence of the vagina in one and a bicornuate uterus in another. I do not believe that we can generalize using the data available. Nevertheless, partial gynetresia (ci la Dr. Jones), absence of the vagina, and double vagina seem to be some of the major gynecologic anomalies associated with bladder exstrophy. One of our patients, aged 20 when first seen at our institution, had been told by her local physician that she could “have neither sex nor children” because of a vagina which would not even admit the examiner’s little finger. The clitoris was bitid and the labia were mere flattened folds. In infancy, the patient had sustained excision of an exstrophied bladder and a bilateral ureterosigmoidostomy. The diversion had been very successful and good kidney function had been maintained. At gynecologic surgery, the situation was as pictured by Dr. Jones. A shallow bilateral mediolateral episiotomy was accomplished and reapproximation of the more superficial tissues anteroposteriorly was effected. The bifid clitoris was
Volume Number
117 6
External
revised also. An adequate introitus and vagina was the result. The patient married soon thereafter and pregnancy followed promptly. Regrettably, the patient aborted twice at two months and we do not have a recent follow-up. One of our bladder ectopy patients also had a congenital absence of the vagina and a vestigeal cervix and uterus. Cystectomy and ileal loop ureteral diversion were accomplished. A patulous anus, because of virtual absence of the anal sphincter, necessitated a p&manent colostomy. The patient married and reports satisfactory coitus using the blind anorectal segment. More traditional (e.g., split-thickness skin graft) vaginoplasty now is employed for construction of a vagina when congenital absence is the indication. Nonetheless, Carlson has successfully converted the inverted exstrophic bladder into a functional vagina after urinary diversion.4 Be this as it may, assuming normal internal genitalia and ovarian function, pregnancy and even vaginal delivery should be considered likely in patients born with bladder ectopy who have had reconstructive gynecologic surgery, as suggested by Dr. Jones and others.5, G REFERENCES
1. Bryan, A. L., Nigro, J. A., and Counseller, V.: Gynecol. Obstet. 88: 79, 1949. 2. Higgins, C. C.: J. Urol. 80: 279, 1958. 3. Uson, A. C., Lattimer, J. K., and Melicow, M. M.: Pediatrics 23: 927, 1959. 4. Stagner, R. V., and Hodges, C. V.: J. Urol. 89: 53, 1963. 5. Rubovitz, F. E., and Arnkoff, M.: J. Urol. 62: 52, 1949. 6. Nabity, S. F.: Nebraska State Med. J. 51: 284, 1966. DR. FRANK C. GREISS, JR., Winston-Salem, North Carolina. I enjoyed Dr. Jones’ paper immensely because I have always been interested in the area of genital anomalies. I do not know how many cases of exstrophy of the bladder it takes to make a specialist but since I just carried such a patient through a pregnancy, I thought I should tell you about her. The patient was a 22-year-old primigravida who had repair of her exstrophy and bilateral ureterosigmoidostomy at the age of four. The repair was very successful and an IVP obtained during the fourth month of pregnancy showed a normal upper urinary tract. As Dr. Jones pointed out, the rami of the pubis are usually absent in such patients. In addition, prolapse of the uterus is often associated with exstrophy. The most
genital
anomaly
with
bladder
exstrophy
755
notable observations during this patient’s pregnancy related to these associated complications. The patient experienced extreme pelvic pressure and discomfort frequently requiring bed rest. We tried to support the pubic area with a corset but this was not really successful. The infant had a vertex presentation and the head could be felt just under the examining finger in what would have been the pubic area. Her labor was perfectly normal except, as one should have expected, the normal mechanism of labor did not occur. The presentation was LOT but the absence of normal anatomic structures which make for a normal mechanism of labor caused a persistent transverse presentation. Midforceps delivery was a delight because, in the absence of normal bony structures, there was all the room in the world. Forceps application and rotation were performed without the usual difficulties. DR. ROBERT B. JAFFE, Ann Arbor, Michigan. We have been troubled in both patients with the adrenogenital syndrome and patients with the type of anomaly which Dr. Jones described about the concept of waiting until they are ready for coitus before embarking upon surgical correction. 1 say this not so much from the surgical point of view, because this would be an optimal time for surgery, but from a psychic point of view. It seems to me that there is a good deal of psychic trauma connected with both of these types of lesions and that perhaps we should consider earlier correction to obviate some of the psychic difficulties that these individuals have as they go through pubescence. DR. CLYDE L. RANDALL, Buffalo, New York. I was impressed by many things about the author’s presentation. Among those points was the fact that he aroused our suspicions as to how a patient with no functional vagina would develop a PID and then was very careful not to tell us what the culture showed. In the days when bacteria still had an opportunity to develop interesting pathology, there used to be a very fascinating entity of pneumococcic salpingitis. It was quite dramatic. It often followed respiratory infection. And I do not know what all happens to Dr. Jones’ patients before he sees them, but I would like to know the results of the culture. DR. JONES (Closing). Dr. Jaffe has made a very important point. Certainly in the adrenogenital syndrome we think reconstruction at a very early time is the thing to do for the reason
756
Jones
that he said We regard 18 months of age as the upper limit for reconstruction. That philosophy certainly could be applied to this situation. We just have not had the opportunity to apply that except in one child who was four years old and who came because she noted that her genitalia did not correspond to what she saw in her schoolmates. We have, indeed, offered reconstruction to her and to her mother but have not done it yet because, after the problem was explained to them, this did not seem to be important. Also, I think it is well to recall that in the exstrophy cases the seriousness of the exstrophy per se usually involves the first several years of life. So that the situation is somewhat different here, I think, than in the adrenogenital syndrome. Dr. Randall asks about the cuture in the pa-
November Am. J. Obstet.
15, 1973 Gynecol.
tient with salpingitis. It was a foul-smelling colon bacillus. It was not a pneumococcus at all. This patient had rather foul-smelling vaginal contents, actually, when it was opened. We thought that there was probably an ascending infection from poor hygiene in the vagina due to that rather long, narrow tract. She is the one who required a No. 16 catheter to feed down through the sinus before we could get an x-ray. It could be that, based on this experience, these narrow orifices should be opened at an earlier time than when a functioning vagina for a functioning vagina’s sake seems to be necessary. I realize that the vaginal orifice in some cases varies a little bit and can be posterior so that dilatation might be possible rather than surgical correction.