Total Urogenital Mobilization- An Easier Way to Repair Cloacas

By Albert0 Petia New Hyde Park, New York

l The surgical treatment of persistent cloaca is a serious challenge. The operation is technically difficult and the final results for urinary and fecal function are far from excellent. The repair of a cloaca includes, among other maneuvers, the separation of the vagina from the urinary tract. This step is a serious technical challenge and is very time consuming. Devascularization of these structures is the main source of complications such as urethro-vaginal fistula, vaginal stric- ture, and acquired vaginal atresia. To avoid these complica- tions and to facilitate the cloaca1 repair, a new technical variation using total urogenital mobilization was performed in 11 patients. In this procedure, after the rectum is sepa- rated from the vagina, both the urethra and the vagina are mobilized together as a single unit. The surgical time spent during the reconstruction was reduced by approximately 70%. All patients recovered well from the operations and have been followed up for 1 to 14 months. The blood supply of the vagina and urethra in all cases remained excellent. No patient developed urethrovaginal fistula, vaginal stricture, or acquired vaginal atresia. The cosmetic appearance in these patients is superior to the one achieved with previous techniques. Although this maneuver may not render better urinary or fecal control, the urethra is more accessible for catheterization. These preliminary results suggest that the total urogenital mobilization maneuver provides a definite technical advance in the repair of cloaca malformations. Copyright Q 1997 by W.B. Saunders Company

INDEX WORDS: Cloaca, anorectal malformations, urogenital sinus, total urogenital mobilization.

U SUALLY. the repair of a cloaca includes a posterior sagittal approach, separation of the rectum from

the vagina, and the separation of the vagina from the urinary tract. This last maneuver is a technical challenge and can be very time consuming. Furthermore. the maneuver used to separate the vagina from the urinary tract seems to be the source of most complications including urethrovaginal fistula (7% to 1 lci’c), vaginal strictures (10%) and acquired vaginal atresia (2%).‘,’ To avoid these complications and improve results, a new technical variant called “total urogenital mobilization” has been used since December, 1994. This surgical maneuver avoids the separation of the vagina from the urinary tract with associated morbidity, and achieves the important goal of leaving the patient with a distinct urethra, vagina. and rectum.

MATERIALS AND METHODS

Until February. 1996. 177 patients with a persistent cloaca have been operated on by this author: 139 of them underwent a prtmary repair. and 38 of them underwent a secondary repair. The new maneuver (total urogenital mobihzatton) described here was used in the last 1 I patients who had a primary cloaca repair. Eight patients from this last group had a common channel shorter than 3 cm. enablmg the entire repair to be

Journal of Pediatric Surgery, Vol 32, No 2 (February), 1997. pp 263-268

accomphshed by the posterior sagrttal approach and the total urogenital mobihzation. without opening the abdomen and without usmg any other extra maneuvers. In the other three patients in whom the common channel was longer than 3 cm. a total urogemtal mobilizatton through a posterior sagittal approach was not sufficient to complete the repair. In these three patients. additional maneuvers were used to complete the repair, eg. the traditional separation of the urethra and vagina.

The ages of the patients at the time of operation was 3 months to 3 years. Follow-up has been between 3 and 16 months.

Operative Technique

Each patient had a dtvertmg colostomy at berth. Four of them also had urinary diversion, by cuprapubic cystostomy (n = I ). ureterostomy (n = I ). vesicostomy and nephrostomy (n = I ). and vesicostomy and ureterostomy (n = I ).

The posterior aagittal approach was used mittally m all patients. The separation of the rectum was completed as previously described (Fig I).’ ’ With the rectum \ucces\fully separated from the vagma, no attempt wa\ made to separate the vagina from the urmary tract; rather the entire urogemtal smu\ was dissected and mobilized. Multiple fine silk sutures were placed at the edge of the vagma and the common channel (Figs 2 and 3). Then another series of very fine 6-O sulk sutures were placed across the common channel (urogenital sinus) between 5 and IO mm dorsal to the chtoris (Figs 3 and 4). Umform traction on all those sutures avoids any tissue damage by dtstributmg the tension on as many sutures as possible (Figs 4 and 5). The wall of the urogemtal smus is divided completely. ventral to the silk sutures (Ftg 1).

Anterior or ventral to the urogenital sinus. a well-defined space and plane separates thts sinus from the pubis (Fig 6) The dissection must proceed lateral and ventral to the urogenital sinus to reach the retropubtc space. This maneuver is easily accomplished Then traction ts exerted on the vagmal edges as the dissection continues m a circumferential manner mcludmg vagma and urethra together (Figs 1 and 6). The surgeon can feel that urethra and vagma are held m the pelvis by avascular tibrous hgaments attached to the pelvic rtm. These ligaments must be divided to free the v’agma, bladder. and urethra without the need for dlvtdmg the blood supply of these structures. The dissection contmues cncumferentially around the lateral and postenor walls of the vagma. and the anterior wall of the bladder and urethra, until enough length has been gamed to connect the vaginal edges to the permeum Thus. a urethral and vaginal openmg of a near normal appearance IS created (FIN:, 7 and 8). The vaginal edges are then sutured to the skm or the labia of the permrum wtth Interrupted 5 0 long-term absorbable sutures. The urethral opening ~111 then be located 5 to 8 mm from the clitoris. and the tissue of the urogenital sinus at the level of the urethra will he sutured at that particular place. The above procedure was

Prewrltrd ut the 27th Amu~d Meeting oj the Anteram Pedratrw .Sur,gz~~~l Aswctntion, Stm Dtego. Calijormu, May 20-23, 1996.

Address reprim quests to A/bet-to Perill. MD, Chtej; Pediatrrc Sur~yely Schneider Chddren :F Hosprtul, I %Y-01 76th Ave. Near Hyde Purk. NY 110-10

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Fig 1. Diagram showing the most common anatomical presenta-

tion of a cloaca. (A) Preoperatively. (B) The rectum has been sepa- rated from the urogenital sinus.

performed easily in all eight patients with a cloaca that had a common channel shorter than 3 cm.

Patients with longer common channels. however, may require additional maneuvers for reconstruction because total urogemtal mobi- lization may not be enough to bring a wide vaginal opening down to the perineum. This happened in three of our cases. In such cases, after the urogenital sinus has been mobilized as described above, the vagina still will be located too high to be sutured to the perineum. and the urethral opening will be too high to be sutured at the normal location. Then the vagina should be separated from the urinary tract as previously described.‘-3 Because the urogenital sinus has already been mobihzed, the separation of vagina and urethra IS a much easier affan, performed basically outside the wound. The surgeon has more control and the procedure is more likely to be successful. Also, the amount of dissection required between vagina and urethra is less exten.slve after total mobilization of the urogemtal sinus. Once the urethra and vagma have been separated, the old common channel can be reconstructed as a neourethra as described in previous publications.‘-3 If the mobihzed vagina cannot be sutured to the perineum, the distal vagina can be

reconstructed ~lth small bowel. colon, a vaginal switch or creation of a vagmal flap.

The rest of the repalr is not different from previous descrlptlons.‘~” The permeal body should he reconstructed tahmg as a landmark the antenor hmit of the external sphincter, wtuch must be electrically determmed and marked with temporary stitches. The posterior limlt of the external sphincter is also electrically determined and marked with silk stitches The levator mechamsm and the posterior edge of the muscle complex are sutured behind the rectum. The rectum will be located withm the limits of the external sphincter and the muscle complex.

RESULTS

All patients recovered very well from these operations, but none are old enough for evaluation of urinary or fecal control. Those children that had a common channel shorter than 3 cm and a normal sacrum, however, have voiding and bowel movement patterns consistent with potential urinary and bowel control. The three children with absent sacrum or severe sacral dysplasia as well as longer common channels require intermittent bladder catheterization: in addition, their bowel movement pat- tern indicates that they probably will need a bowel management program for the treatment of fecal inconti- nence.

Fig 2. Surgical view of the urogenital sinus, with multiple silk sutures placed at the vaginal edge after the rectum has been separated.

rOTAL UROGENITAL MOBILIZATION 265

Fig 4. Diagram showing stitches placed on the urogenital sinus,

near the clitoris. The sinus has been divided, traction is applied, the whole structure is being dissected.

Fig 3. Surgical view showing a series of sutures placed across the urogenital sinus, very close to the clitoris. The urogenital sinus will then be divided anterior to these sutures.

At the time of colostomy closure. vaginoscopy and cystoscopy was performed. All patients had excellent- looking vaginal and urethral openings (Figs 7 and 8). There were no cases of urethrovaginal fistula. The blood supply of vagina and urethra in all cases appeared excellent. The cosmetic appearance in these patients is superior to the one achieved with previous techniques. The surgical time spent during the cloaca1 reconstruction was reduced approximately 70%.

DISCUSSION

The treatment of cloaca1 malformations has evolved rapidly during the last 40 years. Until recently, the most common approach consisted of repair of the rectal component, leaving the urogenital sinus untouched so a secondary operation could be attempted at a later time.lmx One author referred to the fact that Dr. Orvar Swenson once achieved a complete repair of a cloaca.’ Raffensperger and Ramenofsky’” in 1973 proposed an abdomino-perineal vaginal and simultaneous rectal pull- through. Hardy Hendren “-rJ has written very authorita- tive reports on the subject. Reading Hendren’s reports, one can appreciate the evolution of his concepts and the

Fig 5. Surgical view showing the traction exerted on the urogeni tal sinus during the dissection.

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Fig 6. Surgical view of the dissection. The urogenital sinus has been lifted up to see the retropubic space.

complexity of the subject. Since 1982 Pefia has treated this condition with an operation called posterior sagittal anorectovaginourethroplasty. This procedure allows excel- lent exposure and, therefore, more technical altematives.‘“~‘” The surgical time was between 5 and 18 hours, with much of that time spent in the separation of vagina from the urinary tract and in the reconstruction of the vagina. As described in previous publications’.’ complications were secondary to devascularization of the vagina; and in- cluded urethrovaginal fistula, which required a complete operation (7% to 11% of the patients), vaginal stricture (lo%), and acquired vaginal atresia (2%). These compli- cations can be avoided by the use of this new technical variation.

In the previous experience of this author,” 66.5% of the patients with a common channel longer than 3 cm have voluntary bowel movements by the age of 3 years and 69% of them require intermittent catheterization. In patients with common channels shorter than 3 cm, 76% of them have voluntary bowel movements by the age of 3 years and only 19% require intermittent catheterization. A significant number of patients require intermittent catheterization to remain dry and bowel management to remain clean enabling social acceptability. This new

i

Fig 7. Surgical view showing the mobilized sinus after the dissec- tion has been completed. The urethral orifice has been sutured near the clitoris. The vaginal edges have been sutured to the skin of the labia and perineum.

maneuver is unlikely to change these functional results because the underlying muscle and nerve function is unchanged. However the performance of uncomplicated intermittent catheterization requires a urethral opening

Fig 8. Diagram showing the completed maneuver. Urethra and vagina are sutured to the skin of the labia or perineum.

TOTAL UROGENITAL MOBILIZATION 267

located in a visible position and a smooth urethra. With previous techniques, a suprapubic cystostomy tube was the new technique, the patient preserves her own smooth placed in most patients to guarantee bladder drainage. urethra and the meatus is placed in a very visible position With the new technique a urethral Foley catheter was for catheterization. With previous techniques this author used in the eight patients who had urogenital mobiliza- attempted to create a smooth urethra. but irregularities tion only, because a dislodged catheter is very easy to often made intermittent catheterization difficult. With reinsert.

REFERENCES

I. PeRa A: The surgical management of persistent cloaca, Results m 5-l patients treated wtth a postrrtor sagtttal approach. .I Pedtatr Surg

24:590-598. 1989

2. Peiia A: Anorectal malformations Semin Pedtatr Sug 4:35-47. 1995

3. Petia A: Surgtcal Management of Anorectal Malformations.

Chapter 4. New York. NY. Sprmger-Verlag. Inc. 1989, pp 49-7 I 4 Gough MH: Anorectal agenests wtth perststence of cloaca Proc R

Sot Med 52886-889. 1959 5. Bock JE, Madsen CM: Anorectal atresta wtth rectocloacal fistula

Acta Chir Stand 137:284-286, 1971

6. Palken M. Johnson RJ. Derrtck W. et aI. Chmcal aspects of female pattents wtth htgh anorectal agencats. Surg Gynecol Obstet 135 Jll- -tl6. 1972

7 Cheng GK. Ftsher JH. O’Hare KH. et al Anomaly of the perststent cloaca m female Infants. AJR I20:4 1.1423. 197-t

8 Kay R. Tank ES. Prtnciples of management of the perststent cloaca m the female newborn .I Urol 117:102-104. 1977

9 Sieber EK. Klem R: Cloaca with non-adrenal female pseudoher- maphrodtsm. Pedtatrtcs 22.472477, 1958

IO Raffensperger JG. Ramenofshy ML The management of cloaca. J Pedtatr Surg 8617-657. 1973

I I. Hendren WH Surgical management of urogemtal smus abnor- mahttes. J Pedtatr Surg 12.339-357. 1977

12. Hendren WH: Urogemtal sinus and anorectal malformation: Expertence wtth 22 cases. J Pediatr Surg 15:628-611, 1980

I3 Hendren WH. Further rxpertence m reconstructtve surgery m cloaca1 anomalies J Pedtatr Surg 17 695-7 17, 1982

I-l Hendren WH. Repan of cloaca1 anomahe\. Current techmques. J Pedmtr Surg 2l,Il59-I 176, 1986

15. Penn A. DeVrtes P Posterior sagtttal anorectoplasty: Important techmcal constderations and new apphcattona. J Pediatr Surg 17,796. 81 I, 1982

Discussion

WH. Herldren (Boston, MA): Well first, I enjoyed this wonderful presentation and also the chance to study Dr Peiia’s very clear manuscript. Dr Peiia showed this idea to me in New York in February and I must say that it looks like a very good idea. I am looking forward to trying this, especially in some of the low confluence cases. I think it should be emphasized that this is not the “be-all end-all” for the high confluence case as the total reconstruction, and perhaps you would comment about what you have to do further in those cases.

I think it should be emphasized that although your title talks about an easier way to perform cloaca, you probably would agree that this is still very tough surgery and that there is never going to be an easy way to perform a cloaca. Perhaps a more practical way of doing it might be a better word than an easier way to do it.

This concept of mobilizing the urinary tract is similar to what we have to do in the genetic male with no penis, whose urinary tract goes into the rectum where we’ve had to mobilize the urinary tract away from it. That seems to be of help, and as you just presented, applying that to cloaca.

I have three questions. First. do you think the external sphincter is of any importance in these patients? And if there is any importance to it, do you think that this mobilization risks any injury to the external sphincter’?

Second, I’ve had a number of youngsters who were

referred as secondary cases in which the whole urinary tract was tethered upward by either vesicostomy or vaginostomy, and for repair of the cloaca, we had to go above and take all that down so that things could be brought down. Would you comment on your experience in that and how you think perhaps this may be of help in that particular technical problem in the secondary cases?

And third. I would pose the same question to you that I just posed to Dr Smith on the cloaca1 exstrophies, and that is how would you advise a young doctor who telephones you that they have a cloaca and they’ve never seen one or performed one before?

A. Perirt (response): Thank you very much for your comments, Dr Hendren. When I do something new. I like to present it to Hardy Hendren because I always value his opinion. I did that in 1982 with the original PSARP procedure.

Concerning the external sphincter, my experience in the management of cloacas is that the girls who suffer from urinary incontinence don’t suffer from the lack of urinary sphincter but rather because of lack of contractil- ity of the bladder. When the common channel is longer than 3 cm, 70% of my patients need intermittent catheter- ization. These girls have urinary retention and therefore, they only leak urine as an overflow phenomenon. That is why, fortunately, they can manage with intermittent catheterization. Therefore. I believe that the main mecha-

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nism of urinary retention is the bladder neck. These patients don’t show that they have a poor bladder neck. With this new mobilization, those patients who have a poor sacrum and long common channel (more than 3 cm) behave like those subjected to my previous approach, which means that they need intermittent catheterization because they cannot empty the bladder. I don’t believe that the external sphincter plays an important role in urinary control in general.

Four of my patients had a vesicostomy or vaginostomy. Before I placed them in the prone position, I mobilized the vaginostomy or the cystostomy or vesicostomy to avoid that tethering phenomenon that you described. To my surprise when I turned them again into the supine position, I did not find retracted the bladder or the vagina. It is a good precaution because at some point these

vaginostomies or vesicostomies may interfere with this total urogenital mobilization.

Concerning your last question for a pediatric surgeon faced with a cloaca for the first time, my suggestion is to ask for help. At some point I think all the professors from the pediatric surgical training programs in the United States should sit and discuss what our policy should be about the training in the treatment of defects such as this and to find a mechanism to train as many pediatric surgeons as possible. If this maneuver turns out to be as good as I think, perhaps we could consider the possibility of training all pediatric surgeons to do the low type of cloaca with common channels shorter than 2.5 cm; otherwise, I think these patients should be referred to somebody who is particularly specialized in these kinds of problems.