Societies for Pediatric Urology Societies for Pediatric Urology
Dialogue Archives: The High Urogenital Sinus
(Volume 21, Number 7, July 1998)


Previous Issue | Table Of Contents | Next Issue

Guest Editor: Richard C. Rink, MD
VAGINOPLASTY IN SEVERELY VIRILIZED CAH FEMALES
Giacomo Passerini Glazel, MD
POSTERIOR PRONE SAGITTAL APPROACH TO THE HIGH VAGINA
Mark C. Adams, MD Richard C. Rink, MD
THE TRANSANORECTAL APPROACH FOR THE TREATMENT OF UROGENITAL SINUS
Alberto Pefia, MD
SURGICAL REPAIR OF THE HIGH VAGINA W. Hardy Hendren, III, MD
GUEST EDITOR'S NOTES:
One of the most challenging procedures the genitourinary reconstructive surgeon faces is repair of the high urogenital sinus. Regardless of the age of the child, defining the anatomy can at times be challenging, eg, vision of the critical portions of the operation poor, the vagina can be extremely difficult to separate, and the risks to the adjacent structures high. Because of the problems with exposure and my growing respect for the difficulty of many of these procedures, I have gathered a group of four experts in this issue to help all of us with these reconstructions. While many surgeons have contributed to various aspects of the techniques used today, this group has vast experience with this difficult problem and are true pioneers in the field.
Timing of the vaginoplasty always is a controversial subject. We have elected to do these repairs in one stage in the first few months of life. Giacomo Passerini Glazel has given us reason to continue to do so by noting that the vagina is thicker, more vascularized and closer to the perineum during this time. He points out two important causes of postoperative vaginal stenosis: outer compression and inadequate opening of the narrowed distal vagina. He now does nearly all of his repairs perineally and only rarely does he find the need to approach the vagina transtrigonally. I have found the use of this flap quite helpful but have used it only anteriorly. I have avoided complete tubularization for fear of stenosis.
Mark Adams and I delineate the advantages of our posterior prone approach in terms of exposure for the separation of the vagina from the sinus and for reconstruction of the urethra. Opening the entire length of the sinus posteriorly is extremely helpful and has not led to problems. We have struggled with whether to call this an "anterior" or "posterior" sagittal approach but now will defer to Alberto Pefia's suggestion that it is "posterior" to the UG sinus. You must marvel at Dr. Pefia's vast experience with this problem. I have not divided the entire rectum because of the need for colostomy and have not found this necessary for exposure. I am very intrigued by his Total Urogenital Mobilization of the urogenital sinus. This is extremely attractive if there is no consequence to sphincteric musculature or innervation. This may obviate the need for vaginal separation anteriorly, which is the most difficult part of the procedure.
Editor
Richard M. Ehrlich, MD
Clinical Professor of Surgery/Urology
School of Medicine
University of California, Los Angeles
Publisher
William J. Miller
SUBJECT OF CONTROVERSY: HIGH UROGENITAL SINUS
I asked W. Hardy Hendren, my mentor and the father of these reconstructions, to discuss the history of this surgery. He has provided us with a fascinating account of his experience. We all are indebted to his perseverance in sorting out this difficult problem, in refining his technique, and his willingness to teach us all.
I would like to thank all of our coauthors in this issue for their splendid cooperation, and also to Rick Ehriich, Editor of Dialogues, for the opportunity to be Guest Editor of this state-of-the-art issue on high urogenital sinus.
Richard C. Rink, MO
Robert A. Garrett Professor of Pediatric Urologic Research; Chief, Pediatric Urology, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine
GIACOMO PASSERINI GLAZEL, MD
Professor of Urology, University of Padova Medical School, Italy
Vaginoplasty in Severely Virilized CAH Females
Five major points must be taken into consideration when discusssing surgical reconstruction of severely virilized CAH girls: age at treatment; one- or two-stage procedures; anatomy of the region; surgical approach to the vagina, and the final cosmetic appearance. Point two represents a consequence of point one while points four and five are consequences of point three.
The anatomy of the perineal region is influenced by hormonal exposure. High corticosteroid levels during fetal life will convert the anatomy of the female perineum into a progressively virilized structure. On the other hand, estrogen production by the ovaries under the influence of maternal gonadotropins will counterbalance the virilizing effects. Under their influence, the vaginal walls become thicker and more vascularized (good surgical material) and the vagina moves closer to the perineal floor (superficial). This happens in the first two or three months of life or around puberty. In the years in between when estrogen levels are low, the vaginal wall becomes thinner and less vascularized (poor vascular material) and the entire structure deepens into the perineum. As a consequence, the vaginal reconstruction is easier (and the results probably better) in the first two to three months of life or around puberty. Therefore, a one-stage vaginoplasty is better carried out in the first months of life while later, a two-stage vaginoplasty (clitoral reduction with preservation of the entire phallic skin followed by a late vaginoplasty) can be taken into consideration.
A good comprehension of the anatomy of the perineal structures represents the key for the best surgical approach to the vagina. We must understand clearly that the cases we are discussing have little to do with the perineal anatomy of more complex situations like cloacal anomalies (or exstrophies). In CAH severely virilized females, the anatomy is very much like that of a male perineum with normal muscular structures and well-established distances between sacrum, rectum, and urogenital sinus (which is very much like a male urethra) and the pubic bones. As a consequence, there is a good space, anteriorly to the rectum, to approach the vagina, to pull it down, and to place it properly.
On the contrary, in most cloacal anomalies, the perineal region, with its main structures, is rotated anteriorly and the space available for vaginal reconstruction is diminished. As a
consequence, a simple perineal approach is advisable in CAH girls even in case of high urethrovaginal confluence, while a posterior saggital approach is the best in the case of cloacal anomalies.
Since my original paper (J Urol 142:565,1988), the technique has been partially modified. Its main steps now can be summarized below.
A Fogarty balloon catheter is inserted into the vagina. If possible, the catheter is inserted laterally to the cystoscope. Its distal end can be seen and, by gentle manipulation, the inflatable tip generally can be inserted into the vagina under direct vision. It is important to mention that, in most cases, the vaginal conjunction does not appear as a hole but as flourishing mucosa. Vaginal insertion is accomplished by pushing the catheter into the center of this area.
It must be kept in mind that the distal part of the vagina has a conic shape and is rather narrow and fibrotic; therefore, the Fogarty balloon should not be filled to more than 0.75 ml. This level allows the balloon to be retracted near the urethrovaginal confluence. Balloons inflated with higher volumes remain high into the wider part of the vagina and would be virtually useless for more difficult distal dissections. A Foley catheter also is inserted into the bladder. This is useful for monitoring diuresis, which is particularly important in the case of the CAH patient, but also to recognize the urethra in situations of particularly difficult dissections.
The operation is started at the level of the labioscrotal folds with an inverted Y incision. The urogenital sinus is approached exactly as you would do to expose the bulbomembranous urethra in the male. Following its posterior wall, the perineal body is detached and a wide window is made through the fibers of the elevator ani. This wide space represents the major point in order to avoid later vaginal stenosis by outer constriction. The urethrovaginal confluence is then approached. A clear cleavage plane between the vagina and urethra often is not present and the emergence of the vagina from the the urogenital sinus may not be evident. In these cases, it is suggested that the vagina be incised longitudinally where the Fogarty balloon can be felt. Once the vagina has been entered from its posterior (inferior) wall, its separation from the urogenital sinus and urethra can be completed safely. The posterior longitudinal incision should be generously extended to maximize the vaginal opening. In fact, it is very important to move away from the narrow and dysplastic distal opening of the vagina in order to avoid intrinsic vaginal stenosis.
After suturing the distal vaginal stump, which is connected to the urethrovaginal confluence, a wet sponge is inserted temporarily into the cavity so that attention can be paid to the previously described phallic reduction. In this procedure, a circular incision is made around the balanic sulcus approximately 1 cm distal to the glans, and the phallus is degloved. The ventral part of the phallic skin cylinder is incised vertically from the circular incision down to the inverted U perineal incision.
An incisbn is carried out just lateral to each corpus cavernosum, exposing the albuginea. After this, a plain dissection is carried out all around the corpora cavernosa. The neurovascular bundle is elevated as a wide strip on the dorsal side. On the ventral side, the urogenital sinus is freed from its insertion point at the level of the urogenital diaphragm
up to the glans, where it is dissected free. The glans also is dissected free, moving around the tips of the corpus cavernosa within a virtually avascular plane. The corpora are ligated and dissected at their meeting point. The glans is trimmed by removing two wedges on each side of what previously was the urethral meatus. This excision was adopted after it was realized that the neurovascular supply of the glans first runs laterally, and then converges towards the urethral meatus. (P. Mollard and S. Juskiewensky). After being sutured into a conic shape, the reduced glans is fixed to the corporal stumps. A portion of the foreskin that had been attached to the glans is sutured vertically in order to partially cover it.
The urogenital sinus has been preserved intact during the clitoral reduction. The dorsal aspect is now incised longitudinally, ending in a Y at the level of the desired opening of the "female" urethra, thereby developing a small V flap. This V flap is turned up and sutured to the base of the reduced clitoris while the remaining portion of the split urogenital sinus hangs down. The phallic skin is incised at the midline of its dorsum down to its base, producing two rectangular flaps. These are rotated down and their medial edges are sutured to the lateral edges of the opened urogenital sinus. A wide rectangle is created with its central portion represented by the mucosal aspect of the split urogenital sinus. The combined phallic flaps and the urogenital sinus are then progressively sutured to the vagina, starting from the 12 o'clock position. A Fortunoff-type flap, previously created from the perineal skin, is then sutured at 6 o'clock on the vagina, completing the posterior wall of the distal vagina.
Before the labioscrotal folds that will become the major labia are sutured to the lateral side of the inverted phallic skin, they can be partially defatted. Indeed, in case of excessive "scrotalization" of the labioscrotal folds, the final appearance of the major labia might maintain a somewhat scrotal appearance. Part of the fatty tissue of one side is removed, and that of the opposite side is only partially detached and interposed between the urogenital sinus and the reconstructed vagina. This will help to get more distance between the "urethral" and vaginal opening and avoid the risk of a urethrovaginal fistula.
Only if the vagina cannot be detached from the perineum (as it is in the most severe cases or if the surgeon lacks experience with a perineal approach), a transtrigonal detachment of the vagina can be performed. In these cases, the mucocutaneous rectangular flap, formed by the urogenital sinus and the phallic skin, is first converted into a tube and then inverted into the perineum to be anastomosed transtrigonally to the vagina. This solution, however, can permit the risk of distal stenosis.
It is important to understand that an extensive anatomic dissection by detaching the perineal body and by entering the perineum through the elevator ani always is advised before making the decision to approach the vagina from above through the bladder. As previously noted, the perineal dissection is important also because it prevents one of the major causes of vaginal stenosis.
In conclusion, the advantages of this surgical approach are considered as follows:
• It may be performed as a one-stage procedure starting from
the age of two months;
• It results in a normal-appearing vulvar region;
• It avoids vaginal stenosis and all of its incumbent psychological problems provided that all of the appropriate steps are followed. Consequently, there no longer are reasons to postpone vaginoplasty in order to avoid stenosis at the "urethral" meatus, which may occur if the urogential sinus is simply transected.
Other than surgical skill, gender assignment offers the most difficult challenge. A wrong choice can be a life-long disaster for specific patients. Therefore, it must be considered with great responsibility.
MARK C. ADAMS, MD
Associate Professor, Pediatric Urology, Vanderbilt University School of Medicine, Knoxville, TN
RICHARD C. RINK, MD
Professor and Chief of Pediatric Urology, Indiana University School of Medicine, Bloomington
Posterior Prone Sagittal Approach to the High Vagina
Unquestionably, our thoughts about the high vagina have been profoundly influenced by training with Dr. Hendren. It is then not surprising that we favor a sagittal approach to the high vagina in patients with a cloacal malformation of urogenital sinus. With the latter malformation, we have routinely used a sagittal approach, anterior to the rectum. Dr. Hendren first described separation of the high vagina from a urogenital sinus and "pull-through" vaginoplasty for patients with a suprasphincteric confluence of the two. While we do agree that such patients are better served by surgical correction based on similar principles and would do poorly with a simple flap vaginoplasty no matter how much mobilization of the posterior vagina is done, we have very seldom appreciated a distinct external sphincter in such patients. We do not recall recognizing any thickening of the intrinsic urethral musculature or a diaphragm through which the urethra passed that we would have called an external sphincter mechanism other than in a few patients severely virilized from adrenal hyperplasia. In girls with CAH, the urinary sphincter may virilize just as does the clitoris and urogenital sinus. The vagina may end up proximal to most of the sphincter mechanism. Otherwise, our perception of the external urinary sphincter has been similar to one we recently heard from Mr. Richard Turner-Warwick in talking about his long experience in reconstructive female urology. He described a female external sphincter mechanism that is an intrinsic tubular structure along the entire length of the urethra and much thicker anteriorly. This concept makes a posterior approach into the urogenital sinus logical.
We have been very pleased with the sagittal approach to the high vagina in patients with a urogenital sinus. This approach does not preclude the use of a posteriorly based, midline perineal skin flap that is quite useful in ultimately getting the vagina to the perineum. In fact, we have used that flap routinely in such cases. After mobilization of the thick skin flap, dissection is carried strictly in the midline. The urogenital sinus is routinely split in the posterior midline from the meatus back to the confluence of the sinus and vagina. The dissection has allowed for superb exposure to this critical
SUBJECT OF CONTROVERSY: HIGH UROGENITAL SINUS
area where the separation of the vagina from the proximal urethra, bladder neck, or bladder is difficult. This is particularly true with the patient in the prone position when the surgeon can look down on the area of interest. In cases when the dissection has been started with the patient in a supine lithotomy position, the improvement in exposure has been startling simply by turning of the patient. With the prone approach, we have been able to perform the vaginal portion of the reconstruction with good exposure and good results in very young girts. This allows reconstruction at the same time as the reconstruction of the external genitalia and clitoris when there is greater flexibility in the use of skin flaps to meet the high vagina. We have been so pleased with the prone approach that we now circumferentially prepare and drape the lower body of all patients undergoing vaginoplasty if the position of the vagina is at all equivocal on endoscopy so that we may turn them to the prone position if necessary.
In most cases, it has not been necessary to split the anterior aspect of the rectal sphincter to improve exposure. In only one case have we split the anterior wall of the rectum. In our experience, incising the posterior aspect of the entire urogenital sinus up to the vagina improves proximal exposure. The length of the perineal incision in the anterior-posterior axis is increased by the diameter of the urogenital sinus when such an incision is made and may help avoid posterior dissection into the rectal sphincter or rectum. This is particularly true of the large urogenital sinus that is more vaginal-like in size and shape. One may ask whether incision of the entire length of the urogenital sinus is necessary since it might increase the length over which a stricture of fistula between the repaired urethra and vagina may occur. That concern is theoretically true, although we have never seen any fistula distally and expect that they should be very rare as it is generally easier to achieve a sound closure of the distal urogenital sinus to create a urethra. We would recommend a multilayer closure of tissue between the urethra and vaginal distally. Virtually all strictures or fistulae, if they are to occur, will do so proximally at the level of the previous confluence where the previous goals are much more difficult to reach unless careful, yet complete, mobilization of the vagina away from the urethra is achieved. We believe that anything done to increase the exposure and the quality of the repair at that critical proximal level will increase the chances of achieving a good result. Incising the entire length of the urogenital sinus does this and also allows the surgeon to taper the distal urogenital sinus to a proper urethral circumference. This might actually improve external sphincter function in some cases if that sphincter is indeed an intrinsic tubular structure, thicker anteriorly yet attenuated posteriorly in a vaginal like sinus.
In the few patients with a very high confluence at the bladder neck or the bladder in whom we have not been satisfied with proximal exposure using the techniques previously described, we have used a transtrigonal approach to further mobilize the vagina rather than dividing the anterior wall of the rectum. There has been no short- or long-term morbidity from approaching the vagina through the bladder.
Bibliography
Rink RC, Pope JC, Kropp BP et al: Reconstruction of the high urogenital sinus: early perineal prone approach without division of the rectum. J Urol 158:1293-1297, 1997.
Rink RC, Adams MC: Feminizing genitoplasty: state of the art. World J Urol (In Press), 1998.
ALBERTO PENA, MD
Chief, Division of Pediatric Surgery, Schneider Children's Hospital, Long Island Jewish Medical Center, New Hyde Park, NY
The Transanorectal Approach for the Treatment of Urogenital Sinus
Many operations have been described for the treatment of urogenital sinus and intersex malformations. The procedures described by Drs. Hendren and Donahoe deserve a special mention. Most urogenital sinus malformations can be treated successfully with those operations. However, it is my conviction that, when dealing with a very high confluence of the vagina to the urogenital sinus, the long-term results are less than satisfactory unless the vagina is fully mobilized down to the perineum. It is not easy to do this, and consequently, different techniques have been designed to bring skin up into the pelvis to be connected to the vagina. Such a maneuver is frequently doomed to failure, and often results in a stricture or a fistula to the urethra.
It is very important to keep in mind that the urogenital sinus presents as a wide spectrum of defects. Therefore, while some techniques may work for the low vaginal confluence type, the very high malformations require technical variants that have not been fully described.
My experience in the surgical repair of cloacas in more than 225 patients has provided insight into this problem. After all, during the repair of a cloaca, once the rectum has been separated from the genitourinary tract, you are left with urogenital sinus that is indistinguishable from the malformation that we are discussing. The idea of repairing a urogential sinus with a high vaginal implantation in a more radical and improved manner came naturally.
Being directly exposed to this area provides a unique opportunity to become familiar with the real anatomy of the spectrum. For instance, the urinary external sphincter is frequently depicted as a little sling, and these malformations have been classified as "above the sphincter" or "below the sphincter." In reality, an isolated sling does not exist. Instead, stimulation along the entire length of the urogenital sinus elicits contraction of the striated muscle. This striated muscle can be seen to be in continuity with the levator mechanism.
The Transanorectal Approach. The goal of the treatment of the urogenital sinus is to create a urethral opening, separate from the vaginal opening, and a nonstrictured, capacious vagina. One should try, as much as possible, to preserve urinary control and to achieve the capacity to have sexual function and bear children. In cases of high vaginal implantation in which the common channel is more than 2.5 cm in length, the transanorectal aproach has several advantages, the greatest of which is exposure. To achieve this, the rectum is split exactly in the midline and retracted laterally to each side. Visualization of the urogenital sinus is excellent. We have experimental and clinical evidence that such a maneuver does not jeopardize bowel control. The rectum must be reconstructed meticulously after the vagina has been reconstructed.
The patient is placed in the prone position with the pelvis elevated. The incision extends from the middle portion of the sacrum through the anus, the perineum, and down to the urogenital sinus opening. The entire sphincter mechanism of the rectum and the anus is divided. As you keep opening, silk
stitches are placed on both sides of the midline on the rectal wall, which will help to bring together corresponding structures at the time of reconstruction. An electrical stimulator helps to determine where the midline is by checking the magnitude of the muscle contraction on both sides. The perineal fat also is divided, the posterior wall of the urogenital sinus is identified and opened.
In the past, the vagina was then separated from the urinary tract. This was the most technically demanding and delicate part of this method since the urethra and vagina share a common wall and you were obligated to create two walls out of one. Multiple 6-0 silk stitches were placed along the anterior border of the vaginal mucosa. These sutures were used to apply traction and the common wall is split transversely using needle tip electrocautery. Uniform traction is very important for it helps the surgeon to identify the correct plane and prevents damage to the vagina. The dissection must continue until you gain enough vaginal length to be able to suture the vagina to the perineum without tension. At that point, what used to be the urogenital sinus was reconstructed to become the neourethra. This reconstruction was performed using two layers of interrupted 6-0 long-term absorbable suture. The first layer included the mucosa and the wall of the urogential sinus and the second layer consisted of the striated muscle previously mentioned. The vagina was then sutured to the skin of the perineum. The surgeon must be sure not to leave a damaged vaginal wall in front of the urethral suture which may lead to a urethrovaginal fistula.
Total Urogenital Mobilization. More recently, we have modified this approach by the use of a maneuver called total urogenital mobilization. This maneuver has been applied for the repair of the urogenital sinus in 43 patients with cloacas and also has proved to be very advantageous in my last three cases of urogenital sinus with normal rectum.
In this method, the urogenital sinus is mobilized together with the vagina and the two are brought down as a unit and sutured to the perineum. Multiple 6-0 silk stitches are placed in both edges of the open urogenital sinus, the vaginal edges and approximately 5 mm posterior to the clitoris. All these stitches are used to exert traction and allow dissection and mobilization of the entire urogenital sinus. There is a natural plane of separation between the urogenital sinus and the posterior aspect of the pubis. After dissecting the urogenital sinus away from the pubis, you can feel that the avascular suspensory ligaments are the only remaining structures preventing advancement of the urogenital sinus and bladder. These ligaments are divided and 2-4 cm of length is gained. The dissection continues in a circumferential manner, trying not to damage the wall of the urogenital sinus nor bladder, which will guarantee that we do not provoke nerve damage. When the length of the common channel is between 2-4 cm, this maneuver is enough to repair the entire malformation. The greatest advantage of this is that we can save approximately 60 to 80% of operative time. The blood supply of both vagina and urethra remain intact and excellent. There are no complications such as urethrovaginal fistula and, so far, we do not have a single case of vaginal stricture.
The Long Common Channel. You must always keep in mind that we are dealing with a spectrum, and therefore, may be dealing with a urogential sinus with a common channel longer than 2-4 cm. Under those circumstances, additional
techniques must be used to complete the separation of the vagina from the urinary tract. The vagina must be separated from the urinary tract as previously described. However, this separatbn happens to be much easier when performed after the total urogenital mobilization simply because the entire maneuver is performed outside the wound. This separation may give some extra length to the vagina which may be enought to have a satisfactory repair. On the other hand, if the vagina will still not reach the perineum, then we have to go into the abdomen to complete the separation.
The Abdominal Approach. The posterior sagittal wound is packed temporarily and the patient is turned to perform a midline laparatomy. The bladder is opened in the midline and feeding catheters are introduced in each one of the ureters in order to protect them during the separation of the vagina and bladder. You must remember that both ureters pass through the common wall that separates vagina and bladder before reaching the trigone. There is a high chance of injuring one or both of the ureters during the separation of the vagina from the bladder. The bladder is then pushed anteriorly in order to expose the uterus and the Fallopian tubes. Careful dissection is carried out to separate the vagina and uterus from the bladder. These patients have a high incidence of abnormalities of the Muellerian structures. You should expect to find two hemiuteri and hemivaginas or some sort of vaginal septum which may be total or partial. It also is usual for these patients to have hydrocolpos, a problem that we see commonly in cloacas. The presence of hydrocolpos early in life may represent a problem because it could provoke ureteral obstruction. At the time of the main repair, on the other hand, the presence of a hydrocolpos may represent a technical advantage because it means the surgeon has more tissue with which to work.
The Vaginal Switch. There is one specific type of defect in which the patient has two hemivaginas with giant hydrocolpos and, at the same time, a very high confluence between the vagina and the urinary tract. In this situation, the transverse diameter of both hemivaginas together is often longer than the vertical length. A maneuver called a vaginal switch can be used to reconstruct this malformation. One hemiuterus and Fallopian tube is sacrificed, being careful to preserve the blood supply to the ovary. The vagina is separated from the urinary tract on the same side. The vaginal septum is resected and both hemivaginas together are tubularized. Then, what used to be the dome of the hemivagina on the side where the hemiuterus was resected is switched down to the perineum. This maneuver has been very effective in 17 patients with cloaca and in one patient with urogenital sinus with a normal rectum.
Vaginal Replacement. Unfortunately, sometimes we find that the patient has a very small and high vagina. In fact, we have seen patients where the vagina is tiny and attached to the posterior wall of the bladder. In these situations, a vaginal replacement with intestine is indicated. Sigmoid colon is the material of choice, but small bowel also can be utilized.
Long Common Channel with No Bladder Neck. There is one specific type of defect in which the vagina opens in the bladder neck and those patients have no bladder neck. In those cases, I prefer to permanently close the lower end of the bladder. A Mitrofanoff principle for intermittent catheterization through the umbilicus is created and the vagina is reconstructed. I believe that trying to create the
SUBJECT OF CONTROVERSY: HIGH UROGENITAL SINUS
bladder neck and to create a new urethra is technically demanding. More important, the patients frequently leak urine. The surgeon always has to find the right balance between making a very tight urethra that is difficult to catheterize or a little loose that leaks; It is very difficult to find just the right approach. In addition, girls are very reluctant to be catheterized through the genitalia, which is a very sensitive area, and they tolerate catheterization through the umbilicus much better.
Colostomy. The question of whether or not to use a protective colostomy must be answered according to the specfic case. If you want to be absolutely sure that the patient will not suffer any complication, you could prepare the entire intestine with Go-Lytely and then open a protective colostomy at the time of the definitive repair. I have done this several times. Lately, I also have done the same procedure without the protective colostomy. You must be very strict in the bowel preparation one or two days prior to the procedure. During this operation, we insert a central line with parenteral nutrients and the patient stays 7-10 days with nothing by mouth.
At the present time, our routine includes asking for consent to create a protective colostomy, if necessary, to insert a central line, and to repair the malformation with or without a laparotomy. During the operation, and depending on my own clinical impression, I decide to leave the patient without a protective colostomy or to open a protective colostomy.
My experience includes eight cases. Two patients enjoy full urinary control. Three patients are too young to be evaluated but they show a normal voiding pattern (voiding episodes and dry in between). Two patients had no bladder neck and received continent diversion. They remain dry with intermittent catheterization. One patient was tost.
Final Comments. Recently, several authors have been presenting their experience with what they call "anterior approach" to the surgical treatment of urogenital sinus. Basically, they refer to anterior as related to the rectum; in other words, they open between the urogential sinus orifice and the rectum, and sometimes they include the anterior rectal wall. However, this approach, from my point of view, is still posterior sagittal because it is posterior to the urogenital sinus. This may be a purely semantic problem.
I think that the main message of this presentation must be that the urogenital sinus is a spectrum of defects that can be repaired utilizing a spectrum of techniques. Short, common channels can be repaired with a simple perineal flap-type approach. If the patient has a higher confluence of the vagina, you may extend the incision in the perineum; if it is still not enough, one can include the anterior rectal wall, and if that is not enough, we can continue dividing the posterior rectal wall and the posterior sphincter mechanism. The length of the incision depends on the exposure requirement, which depends on the specific malformation. If that is not enough, you have to go finally into the abdomen to separate the vagina from the urinary tract. In the extreme fo the spectrum, you have to replace the vagina with a piece of intestine. The patient should go to the operating room with a completely clean bowel and with consent for a transanorectal approach, a central line, a possible laparotomy, a possible colostomy, and a possible vaginal replacement. The idea of a spectrum of this malformation is the most realistic one. If the surgeon is not prepared to accept this concept, he is going to suffer a
significant degree of frustration and more importantly, many of his patients with suffer as well.
The advantages of the transanorectal approach for the urogenital sinus are significant. Excellent exposure of the sinus is obtained and operative time is significantly decreased. However, because the rectum is divided in the midline posteriorly and anteriorly, there is considerable reluctance from the surgical community to accept this maneuver. I remain convinced, however, that, when indicated, this is a very useful maneuver.
W. HARDY HENDREN, III, MD
Department of Surgery, Children's Hospital and Harvard Medical School, Boston, MA
Surgical Repair of the High Vagina
It is interesting to read the preceding comments of colleagues and friends, Drs. Rink, Adams, Pefia, and Passerini on various ways to manage the high vagina. When I first became interested in these problems in 1955 there was very little interest in the subject and certainly nothing in the literature describing several options for repair of a high vagina. Managing a low vagina is fairly straightforward. In one where there is simply labial fusion, a Heineke Mikulicz procedure to the posterior fourchette is adequate. More often the vagina is tow, but there is substantial tissue blocking the entrance. Making an inverted U-shaped flap which is advanced into the posterior wall of the vagina will usually suffice. This is true in many circumstances, such as the majority of girls with the adrenogenital syndrome, and some girls who have had exstrophy of the bladder.
A high vagina which enters the urogenital sinus close to the bladder neck is a special problem. It occurs in three clinical settings: 1) Most common is the infant female with a cloacal malformation involving all three organ systems, ie, bladder, vagina(s) and rectum; 2) Isolated urogenital sinus malformation where the anorectal canal is normal, or nearly normal, but there is a persisting urogenital sinus into which empties the bladder and the vagina(s); 3) Females with the adrenogenital syndrome and masculinization of the tower genitourinary tract.
My interest in the entity of cloacas began when an infant was referred to the Boston Children's Hospital in 1955 when I was a resident. None of the senior surgical staff or the resident staff had any experience dealing with such an anomaly, except for performing a colostomy. The baby died from dysplastto kidneys. Autopsy permission was given which afforded me an opportunity to dissect the pelvic organs. From this it was clear that a functional repair should be possible and would require 1) separating the rectum from its entry to the urogenital sinus (UGS) and pulling it through to the perineum; 2) separating the vagina (often two of them) from the UGS and pulling it through to the perineum, and 3) utilizing the UGS as a urethra. My first opportunity to do this came in 1962 when a two-month-old female infant with a typical cloaca was referred after colostomy, vesicostomy and vaginostomy elsewhere. The repair worked out well. She is now a lovely 36-year-old woman, happily married for 10 years, and most recently seen by me to perform cholecystectomy for gallstones! My developing thoughts on surgical reconstruction of cloaca have been recorded in a series of papers,18 the most recent including 195 patients
with cloaca! malformations (154 "regular" cloacas, and 41 cloacal exstrophy cases).9 These papers, spanning 31 years, have stressed the wide range or spectrum of anatomy that the surgeon may encounter. My late friend Dr. Stephen Gans, Editor of the Journal of Pediatric Surgery, encouraged me to include before and after anatomic diagrams of many of the cases in the Journalto serve as a reference source for other surgeons who may be confronted by a case and have a need to review the spectrum of pathology which they may encounter.
Each case with a cloaca or a persisting UGS should be studied thoroughly by radiological imaging10 and endoscopy. Any retained fecal material which may be present in the defunctioned colon should be washed out. Go-Lytely bowel prep is routine. A total body prep is done so we can work in 1) posterior sagittal position (prone); 2) do a laparotomy (supine), or 3) lithotomy position by picking up the wrapped legs. I usually start prone in the posterior sagittal position as described by Pefia and deVries in 198211 to separate the three-organ systems and often use multiple position changes if needed, ie, prone- supine- lithotomy-supine-prone, for example.
The isolated UGS case with high vagina and no anorectal anomaly is a different problem. They can be divided into two groups: a) those with no endocrine disorder, and b) those females with the adrenogenital syndrome who have severe masculinization of the genitalia and the lower urinary tract.
In the group, those with no endocrine disorder, I have positioned them prone and made a vertical incision in the perineum up to the anterior wall of the anus and rectum. The rectum can then be retracted posteriorly, giving excellent access to separate the vagina and pull it down. I have not found it necessary to split the rectum in front and back as described by Dr. Pefia to attain that exposure. In some, I have incised through the sphincter into the anal canal or lower rectum, then closing that primarily without a protective colostomy. All vaginoplasty cases have a full bowel prep preoperatively using 1) clear liquids for one to two days, 2) Go-Lytely bowel lavage, and 3) endoscopic inspection just before surgery with antibiotic lavage of the lower colon if it is not pristine clean. A Betadine-soaked gauze pack up the rectum is an added safeguard.
I regard the adrenogenital syndrome cases with masculinized genitourinary anatomy as a special group. A historical note might be of interest about this. In February of 1968, Dr. John Crawford, Chief of Pediatric Endocrinology at Massachusetts General Hospital, asked me to evaluate the anatomy of a 5 1/2-year-old female with the adrenogenital syndrome.
At birth, an abdominal exploration by another surgeon had shown normal female pelvic organs. Urethrogram had disclosed a male-appearing urethra but no vagina. Clitorectomy had also been performed. I cystoscoped the child and saw a male-like urethra. There was a clear-cut external urethral sphincter, a normal bladder neck, and a typical veru between the two. Inserting a ureteral catheter into the prostatic utricle at the summit of the veru and injecting contrast medium disclosed an excellent vagina! Imagine my surprise! My good friend, Dr. Wyland F. Leadbetter, then Chief of Urology at Braintree Laboratory, MGH, was operating in an adjacent OR. I asked if he would cystoscope the patient and tell me what he thought. He remarked, "It
looks like a normal male urethra." I then showed him the x-ray film. He was as surprised as I. He told me that he had never seen such an entity, although he trained at Hopkins where much of our knowledge about pseudohermaphroditism originated.
I then called my former Chief, Dr. Robert E. Gross at Boston Children's Hospital, and asked if he had ever seen a high vagina which entered the urogenital sinus close to the bladder neck. He answered that he had never done a cystoscopy on one of those patients and so he could not answer the question. He had taught me to exteriorize the vagina in adrenogenital patients with a flap vaginoplasty. I asked him if he had ever encountered one where he had to dissect very high to find entry to the vagina to lay in a flap and then see stress incontinence after that. Dr. Gross thought he had seen that situation.
I called next a senior surgeon at Hopkins to pose the same question. He had a large experience with the adrenogenital syndrome. When I asked about a vagina entering the urethra above the level of the external sphincter in the female the reply was that the external sphincter does not exist in a female! When I replied with some temerity that the female does have an external sphincter and it is that sphincter which keeps her dry when we do a YV-plasty to the bladder neck (a frequently performed operation in the 1960s) it was clear that the discussion could not go any further. It did not contribute to the conversation that a recent issue of the Hopkins Bulletin described autopsy findings of two high vagina cases. It was clear that the high vagina entity was not appreciated clinically at that time.
Soon after that Dr. Steve Gans called from Los Angeles to discuss a problem case. It was a newborn "male" with a normal looking penis and undescended testicles. The baby had been given a male name and circumcised, but at about a week of age went into shock. It was a female infant with the adrenogenital syndrome and masculinization. Abdominal exploration showed normal female pelvic anatomy. Urethrogram and endoscopy showed an apparently normal male urethra. I felt certain that this must be another similar case. I flew to Los Angeles and operated upon the baby at Cedars Sinai Medical Center. We started with endoscopy, catheterization of the verumontanum, and injection of contrast. Thank heaven there was a beautiful vagina up there. I brought it down in the presence of a sizable audience of interested surgeons, which included my good friend, Dr. Willard Goodwin, Chairman of Urology at UCLA. I felt like Daniel in the lion's den and have often reflected on how embarrassing it would have been had a vagina not been present!
I was visiting professor at The National Children's Medical Center as a guest of Dr. Judson Randolph. Another similar case was presented of a very masculinized little girl about age 10 years, who had been explored and found to have normal female pelvic anatomy but no vagina visible on a urethrogram or previous cystoscopy. I felt sure it must be another of these high vaginas and volunteered to operate on the child. Dr. Philip Exelby, Dr. Randolph's Chief Resident at that time, scrubbed. Catheterization of the veru and dye injection disclosed a vagina, which we brought down. By then I had collected seven of these cases, and submitted a paper to the program Committee of the Surgical Section of the American Academy of Pediatrics. My good friend, Dr. Tom
SUBJECT Of CONTROVERSY: HIGH UROGENITAL SINUS
Holder was the program chairman. He called to discuss the abstract I had submitted. With his usual good humor, Tom remarked, "I donl believe this, because I have never seen it, but we will put this on the program anyway, because you will make a good show of it!" The presentation was not easily disbelieved because it included a movie strip of one of the cases. The first case was operated from a posterior parasacral approach, retracting the rectum laterally for access to the high vagina. It was an awkward exposure, although the case was completed successfully. The next case I did in lithotomy position, opening the perineum through an inverted U flap, the exposure frequently used for perineal prostatectomy. Placing a Fogarty catheter in the vagina and dissecting upward to the Fogarty catheter greatly facilitated finding the junction of the vagina with the urethra. Although the vagina can be mobilized maximally from this position, it will often not easily reach the perineum, because it is developmentally only the proximal 2/3 of the vagina. Four local perineal flaps are fashioned to sew to the lower end of the vagina. We have described all of these details in subsequent papers.1215 Our first paper on the subject in The New England Journal™ described the first four cases and included a summary of the literature on the subject. The entity of a high vagina had been noted in several previous papers, but we did not find any reference to repair of the entity. Since our last paper on this subject in 1995 which reported 16 of these unusual cases,17 we have corrected six others (five in a single week at the Aga Khan Hospital in Karachi, Pakistan). It is a sound operation which can be duplicated safely by other surgeons. Technical details and follow-up results have been described in our papers.
In a follow-up of my discussion with the senior surgeon at Hopkins in 1968,1 received a very nice letter from him some years later. He wanted permission to use the operative diagrams of correcting the high vagina12 in a new edition of a book. I was pleased and honored to be asked. I did not mention my being the young surgeon on the phone in 1968. We met some time after that and both enjoyed reminiscing about it.
If no vagina is present, bowel makes a good substitute. We reported recently an experience in 65 patients.18 Additionally, bowel can be used as an extention of a vagina which is high but too short to reach the perineum, using a combined approach through the abdomen and perineum.
If the patient lacks a urethra, one can be constructed using the anterior vaginal wall and a buttocks flap as described in a recent paper on 40 such cases.191 would add that little girls in my experience adapt perfectly well to catheterization from below when we can make them dry with an operation on the bladder neck and construction of a urethra when that is lacking.
Regarding the urethral sphincter in females, I agree it is not as discrete a structure as in the male, except in those with very masculinized anatomy. Its depiction as a narrow muscle sling by our medical artist in various papers through the years is to show anatomic relationships, not details of anatomy.
There is one final thought that I would like to express on the subject of high vagina, whether it is in a patient with a cloaca an isolated urogenital sinus, or the severely masculinized adrenogenital syndrome patient. There is more than one way to do most operations to achieve a satisfactory result. So it is
8
with a high vagina. I would encourage young surgeons who are entering this field to try different approaches and settle on what ever works best in their hands.
References
1. Hendren WH: Surgical management of urogenital sinus abnormalities. JPediatrSurg 12:339-357, 1977.
2. Hendren WH: Urogenital sinus and anorectal malformations: Experience with 22 cases. J Pediatr Surg 15:628-641,1980.
3. Hendren WH: Further experience in reconstructive surgery for cloacal anomalies. JPediatrSurg 17:695-717, 1982.
4. Hendren WH: Repair of cloacal anomalies: Current technique. J Pediatr Surg 21:1159-1176,1986.
5. Hendren WH: Cloacal malformations: Experience with 105 cases. J Pediatr Surg 27:890-901. 1992.
6.  Hendren WH: Urogenital sinus and cloacal malformations. J Pelvic Surg 3:149-160, 1995.
7. Hendren WH: Urogenital sinus and cloacal malformations. Semin Pediatr Surg 5:72-79, 1996.
8. Hendren, WH: Management of cloacal malformations. Semin Pediatr Surg 6:217-227, 1997.
9. Hendren, WH: Cloaca: The most severe degree of imperforate anus: Experience with 195 cases. Annals Surg (In Press) 1998.
10. Jaramillo D, Lebowitz RL, Hendren WH: The cloacal malformation: Radiologic findings and imaging recommendations. Rad 177:441-448,1990.
11. Pena A, deVries PA: Posterior sagittal anorectoplasty: Important technical considerations and new applications. JPediatrSurg 17:796-811, 1982.
12.  Hendren WH, Crawford JD: Adrenogenital syndrome: The anatomy of the anomaly and its repair. J Pediatr Surg 4:49-58,1969.
13. Hendren WH, Donahoe PK: Correction of congenital abnormalities of the vagina and perineum. J Pediatr Surg 15:751-769, 1980.
14.  Hendren WH: Reconstructive problems of the vagina and the female urethra. Clinics in Plastic Surg 7:207-234, 1980.
15. Hendren WH: Surgical approach to intersex problems. Semin Pediatr Surg 7:8-18, 1998.
16. Rosenberg B, Hendren WH, Crawford JD: Posterior urethrovaginal communication in apparent males with congenital adrenocortical hyperplasia. NewEnglJMed280:131 -134, 1969.
17.  Hendren WH, Ataia A: Repair of the high vagina in girls with severely masculinized anatomy from the adrenogenital syndrome. JPediatrSurg 30:91-94, 1995.
18. Hendren WH, Atala A: Use of bowel for vaginal reconstruction. J Urol 152:752-755,1994.
19. Hendren WH: Construction of a female urethra using the vaginal wall and a buttock flap: experience with 40 cases. J Pediatr Surg 33:180-187, 1998.
WHAT'S AHEAD
Here are some of the topics to be discussed in the upcoming issues of Dialogues in Pediatric Urology and the Guest Editors of the issues.
Augmentation Ureterocystoplasty (Guest Editors: Hussein Aliabadi, MD, and Yuri Reinberg, MD)
Electrical Stimulation and Biofeedback Techniques for the
Bladder and Pelvic Floor
(Guest Editor: Anthiny H. Balcom, MD)
 
Opinions expressed in this publication are the sole responsibility of the individuals named and do not necessarily reflect the opinions of the editorial board or the publisher and members of this organization.
Copyright © 1998 by William J. Miller Associates, Inc.
All rights reserved. No part of this publication may be reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying recording or by any information retrieval system, without written permission from the publisher.