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22.1 Introduction
Persistent cloaca was, in the past, considered an un- usual defect, with a high incidence of “rectovaginal fistula” reported in the literature [1]. In retrospect, it seems that cloaca represents a much more common defect in female patients than previously thought, and rectovaginal fistula is an almost nonexistent defect that is present in fewer than 1% of all cases, except in a few specific geographical areas [2]. Most patients suffering from a persistent cloaca were erroneously thought to have a rectovaginal fistula. Many of those patients underwent surgery with repair of the rectal component only and were left with the urogenital si- nus untouched [2], requiring a complete redo opera- tion.
This group of defects represents the extreme in the spectrum of complexity of female malformations. A cloaca is defined as a defect in which the rectum, va- gina, and urinary tract meet and form a confluence exiting the perineum via a single common channel (Figs. 22.1–22.3). The diagnosis of persistent cloaca is a clinical one. This defect should be suspected in a female born with imperforate anus and small-look- ing genitalia. Careful separation of the labia discloses a single perineal orifice.
Contents
22.1 Introduction . . . 307
22.2 Surgical Treatment of Cloacas . . . 307 22.2.1 Cloacas With a Common Channel
Shorter than 3 cm . . . 309 22.2.2 Cloacas with a Common Channel
Longer than 3 cm . . . 310 22.2.3 Vaginal Switch Maneuver . . . 311 22.2.4 Vaginal Augmentation
and/or Replacement . . . 312 22.2.5 Two Hemivaginas Attached
to the Bladder Neck . . . 313 22.3 Postoperative Care . . . 314
References . . . 314
The length of the common channel varies from 1 to 7 cm. Common channels longer than 3 cm are usu- ally associated with complex defects (Fig. 22.3). The mobilization of the vagina is difficult and often some form of vaginal replacement is needed during the de- finitive repair. A common channel of less than 3 cm usually means that the defect can be repaired with a posterior sagittal approach only without opening the abdomen (Fig. 22.1).
The rectum sometimes opens high into the dome of the vagina. Therefore, a laparotomy or laparoscopy must be part of the procedure to mobilize the bowel.
The vagina is frequently abnormally distended and full of secretions (hydrocolpos; Fig. 22.3). This dis- tended vagina can compress the trigone and interfere with the drainage of the ureters, leading to megaure- ters. The dilated vagina can also become infected, pyo- colpos, which can lead to perforation and peritonitis.
On the other hand, such a large vagina may represent a technical advantage for the repair, because having more vaginal tissue will facilitate its reconstruction.
A frequent finding in cloacal malformations is the presence of different degrees of vaginal and uterine septation or duplication (Fig. 22.3). The rectum usu- ally opens in between the two hemivaginas. These pa- tients may also suffer from cervical atresia and during puberty are unable to drain menstrual blood through the vagina; the menstrual blood accumulates in the peritoneal cavity and sometimes requires emergency surgery [3].
Low cloacal malformations (less than 3 cm;
Figs. 22.1 and 22.2) are usually associated with a well- developed sacrum, a normal-appearing perineum, and adequate muscles and nerves. Therefore, a good functional prognosis is expected.
22.2 Surgical Treatment of Cloacas
Prior to undertaking the repair of cloacal malforma- tions, the surgeon should perform endoscopy to de- termine the length of the common channel. There are two well-characterized groups of patients with cloaca
22 Treatment of Cloacas
Marc A. Levitt and Alberto Peña
Fig. 22.4 Cloaca repair. A Inci- sion. B Rectum and common channel are exposed. C Rectal opening ([6], with permission) Fig. 22.1 Spectrum of cloacae.
A Most common channel. B Long common channel ([6], with per- mission)
Fig. 22.2 Spectrum of cloacae. A High rectal implantation into the vagina. B Short common channel ([6], with permission)
Fig. 22.3 A Associated hydrocol- pos. B Double vagina and double uterus ([6], with permission)
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[4], and each represent different technical challenges that must be recognized preoperatively.
The first is represented by patients who are born with a common channel shorter than 3 cm. Fortu- nately, these patients represent the majority (over 60%) of all cloacas. The great majority of these pa- tients can be repaired using only a posterior sagittal approach, without a laparotomy, and the operation is a reproducible one that can be performed by most general pediatric surgeons.
The second group is represented by patients with longer common channels. These patients usually need a laparotomy and a decision-making algorithm for the reconstruction that requires a large experience and special training in urology. These patients are therefore best cared for in centers with special exper- tise in the repair of these defects.
22.2.1 Cloacas With a Common Channel Shorter than 3 cm
The incision extends from the middle portion of the sacrum down to the single perineal orifice. The entire sphincter mechanism is divided in the midline. The first structure that the surgeon finds after the division of the sphincter mechanism is the rectum (Fig. 22.4).
Because of the complexity of these malformations, the surgeon must be prepared to find bizarre anatomic arrangements of the rectum and vagina.
The rectum is opened precisely in the midline (Fig. 22.4) and silk stitches are placed along the edges of the posterior rectal wall. The incision is extended distally through the posterior wall of the common channel. The entire common channel is exposed, which allows measurement of the common channel under direct vision. The rectum is then separated from the vagina (Fig. 22.5) in the same way as described for the repair of rectovestibular fistula. The rectum and vagina share the same type of common wall that has already been described.
Once the rectum has been completely separated from the vagina, a total urogenital mobilization is performed [5]. In the past, the vagina was separated from the urinary tract, which was a technically chal- lenging maneuver associated with a significant mor- bidity. Total urogenital mobilization consists of the mobilization of both the vagina and urethra as a unit. After the rectum has been separated, multiple silk stitches are placed, taking the edges of the vagina and the common channel, in order to apply uniform traction on the urogenital sinus for its mobilization.
Another series of fine stitches is placed across the
urogenital sinus approximately 5 mm proximal to the clitoris (Fig. 22.6). The urogenital sinus is transected between the last row of silk stitches and the clitoris, and is dissected, taking advantage of the fact that there is a natural plane between it and the pubis. Very rapidly, and in a bloodless field, one can reach the upper edge of the pubis. There, a fibrous, avascular structure is identified that gives support to the vagina and bladder and is called the “suspensory ligament of the urethra and bladder.” While applying traction to the multiple stitches, these suspensory ligaments are divided, which provides significant mobilization (2–3 cm) of the urogenital sinus. In addition, one can then dissect the lateral and dorsal walls of the vagina to gain a further 5–10 mm (Fig. 22.7).
This dissection is enough to repair about 60% of all cloacas and is a reproducible maneuver. It has the additional advantage of preserving an excellent blood supply to both the urethra and the vagina, and placing the urethral opening in a visible location to facilitate intermittent catheterization if necessary (Fig. 22.8). It also provides a smooth urethra that can be catheter- ized easily.
What used to be the common channel is divided in the midline creating two lateral flaps that are sutured to the skin, creating the new labia. The vaginal edges are mobilized to reach the skin to create the introitus.
The limits of the sphincter are then determined elec- trically and the perineal body is reconstructed, bring- ing together the anterior limit of the sphincter. The rectum is placed within the limits of the sphincter as described previously.
Fig. 22.5 Total urogenital mobilization. The rectum is sepa- rated from the vagina ([7], with permission)
22.2.2 Cloacas with a Common Channel Longer than 3 cm
When the endoscopy shows that the patient has a long common channel, the surgeon must be prepared to face a very significant technical challenge. In the presence of a long common channel, patients should receive a total body preparation, as it is likely that they will require a laparotomy. The rectum is sepa- rated from the vagina and urethra. The presence of a very long common channel (more than 5 cm) means that there is no way that the total urogenital mobiliza- tion will be enough to repair that malformation, and it is therefore advisable to leave the common channel in place using it for the urethra for intermittent cath- eterization.
In that situation, it is suggested that the surgeon tries to separate the vagina from the urinary tract from within the abdomen, rescuing it from the back of the common channel, and then closing the posterior aspect of the common channel, which will become the catheterizable urethra. The bladder is opened in the midline and feeding tubes are placed into the ure- ters to protect them. In these types of malformations, there is a significant common wall between the vagina
Fig. 22.8 Total urogenital mobilization. Urethra and vagina sutured into their new positions ([8], with permission) Fig. 22.6 Total urogenital mobilization. Stitches placed on the edges of the sinus and across, near the clitoris ([8], with per- mission)
Fig. 22.7 Total urogenital mobilization. Urogenital sinus fully mobilized ([8], with permission)
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and the bladder. The ureters run through that com- mon wall and must therefore be consistently identi- fied and palpated during the separation of the vagina from the urinary tract. The surgeon must be familiar with the different techniques of ureteral reimplanta- tions because these patients frequently require that operation during the same procedure.
Once in the abdomen, the patency of the Mullerian structures are investigated by passing a no. 3 feeding tube through the fimbriae of the Fallopian tubes and injecting saline through them to be sure that they are patent. If one of the systems is not patent, excision of the atretic Mullerian structure without damage to the blood supply of the ovary is recommended. When both Mullerian structures are atretic, they should be left in place, and the patient followed closely with fur- ther decisions made when she reaches puberty.
With the abdomen open, the surgeon has to a make decisions based on the specific anatomic find- ings. In the presence of a single mid-sized vagina, the surgeon must separate the vagina from the uri- nary tract, being sure to preserve the blood supply of
this structure, which comes from the uterine vessels.
When the vagina is found to be too short, the patient requires some form of vaginal replacement that can be performed using tissue from the rectum, colon, or small bowel.
22.2.3 Vaginal Switch Maneuver
There is one specific group of patients who are born with hydrocolpos and two hemivaginas. The hemiva- ginas are very large and the two hemiuteri are very separated, the distance between one hemiuterus and the other is longer than the vertical length of both the hemivaginas. In those cases, it is ideal to perform a maneuver called a “vaginal switch” (Figs. 22.9 and 22.10). One of the hemiuteri and the ipsilateral Fal- lopian tube is resected (Fig. 22.9), with particular care given to preserving the blood supply of the ovary.
The blood supply of the hemivagina of that side is sacrificed and the blood supply of the contralateral hemivagina is preserved. Most of the time it provides
Fig. 22.9 Bilateral hydrocolpos and very high vagina. Ideal anat- omy to be repaired with vaginal switch maneuver. L Left, R right ([9], with permission)
Fig. 22.10 Vaginal switch ma- neuver, where one vagina is brought to introitus, with exci- sion of ipsilateral hemiuterus and tube and vaginal septum.
([9], with permission)
good blood supply for both hemivaginas. The vaginal septum is resected, and both hemivaginas are tubu- larized into a single vagina, taking advantage of the long lateral dimension of both hemivaginas together.
Then, what used to be dome of the hemivagina, where the hemiuterus was resected, is turned down to the perineum (Fig. 22.10). This is an excellent maneuver that can be performed only when the anatomic char- acteristics fulfill these unique requirements.
22.2.4 Vaginal Augmentation and/or Replacement
The vagina can be augmented or totally replaced with bowel tissue when it is very small and is located very high, or in cases of absent vagina. The choices are: rec- tum, colon, or small bowel. Vaginal replacement with rectum is only feasible in patients who have a megar- ectum that is large enough to be able to divide it lon- gitudinally into a portion with its own blood supply that will represent a new vagina and to leave another half with enough circumference to reconstruct an adequate-sized rectum (Fig. 22.11). The blood sup- ply of the rectum will be provided transmurally from branches of the inferior mesenteric vessels.
The colon is an ideal substitute to replace the va- gina (Fig. 22.12). However, sometimes, the location of the colostomy interferes with this type of reconstruc- tion. When available, the sigmoid colon is preferable.
One must take the most mobile portion of the colon in order to use a piece that has a long mesentery.
When the patient has internal genitalia or a little cuff of vagina or cervix, the upper part of the bowel used for replacement must be sutured to the vaginal cuff.
When the patient has no internal genitalia (no vagina and no uterus), the vagina is created and is left with
Fig. 22.11 A Vaginal replacement with rectum. Planning the neovagina. B Neovagina separated from rectum ([10], with permission)
Fig. 22.12 Vaginal replacement with colon ([10], with permis- sion)
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its upper portion blind, and is used only for sexual purposes, not for reproduction.
When the colon is not available, then the most mo- bile portion of the small bowel is utilized for vaginal reconstruction. The mesentery of the small bowel is longer in an area located approximately 15 cm proxi- mal to the ileocecal valve. This is the best portion of small bowel to be used for vaginal replacement. A portion of this ileum is isolated and pulled down, pre- serving its blood supply (Fig. 22.13).
22.2.5 Two Hemivaginas Attached to the Bladder Neck
In the highest type of cloaca one may find two little hemivaginas attached to the bladder neck or even to the trigone of the bladder. In these cases, the rectum also opens into the trigone. Separation of these struc- tures is performed abdominally. Unfortunately, when that separation is completed, the patient is frequently left with no bladder neck or a severely damaged blad-
Fig. 22.13 A Vaginal replacement with small bowel, using the portion with the longest mesentery. B Pulling the small bowel down. C Operation completed ([10], with permission)
der neck. At that point, the surgeon must have enough experience to make a decision as to whether to recon- struct the bladder neck or to close it. In the first situa- tion, most patients will need intermittent catheteriza- tion to empty the bladder and there is no guarantee that the bladder neck reconstruction will work. In the second situation (permanent closure of the bladder neck), a vesicostomy is created, and the patient will require a continent diversion-type of procedure at the age of urinary continence (3–4 years old). In this par- ticular type of malformation, the patient also needs a vaginal replacement.
22.3 Postoperative Care
The patients with cloacas usually keep the Foley cath- eter for 10–14 days. In our series, about 20% of pa- tients with a cloaca with common channel shorter than 3 cm require intermittent catheterization to empty the bladder. Patients with common channels longer than 3 cm require intermittent catheterization 70–80% of the time. Therefore, we leave the Foley catheter in place as long as the patient shows signs of swelling in the genitalia and until the urethral meatus is not readily visible. Once we are able to see the ure- thral orifice, the Foley catheter can be removed in the clinic, and the baby watched to see if she is capable of emptying the bladder. If she cannot pass urine, then we can teach the parent to pass the catheter intermit- tently.
In cases with very long common channels, we pre- fer to leave a suprapubic tube. One month after sur- gery, we can perform a suprapubic cystogram and start clamping the tube and measuring the residual urine, which is an indicator of the efficiency in the function of the bladder. The suprapubic tube remains in place until we have evidence of a good bladder function or the caregiver learns to catheterize the bladder when
indicated. The rectal dilatation protocol is the same as described in Chaps 20 and 21. Once the rectum is the appropriate size, the colostomy can be closed. An endoscopy should always be performed prior to co- lostomy closure to inspect the repair.
References
1. Stephens FD, Smith ED (1971) Incidence, frequency of types, etiology. In: Stephens FD, Smith ED, Paul NW (eds) Anorectal Malformations in Children. Year Book Medical, Chicago, pp 160–171
2. Rosen, NG, Hong AR, Soffer SZ, Rodriguez G, Peña A (2002) Recto-vaginal fistula: a common diagnostic error with significant consequences in girls with anorectal mal- formations. J Pediatr Surg 37:961–965
3. Levitt MA, Stein DM, Peña A (1998) Gynecological con- cerns in the treatment of teenagers with cloaca. J Pediatr Surg 33:188–193
4. Peña A, Levitt MA, Hong AR, Midulla PS (2004) Surgi- cal management of cloacal malformations; a review of 339 patients. J Pediatr Surg 39:470–479
5. Peña A (1997) Total urogenital mobilization – an easier way to repair cloacas. J Pediatr Surg 32:263–268
6. Peña A (1989) Female defects. In: Peña A (ed) Atlas of sur- gical management of anorectal malformations, Springer- Verlag, New York, pp 60–64
7. Peña A (1997) Total urogenital mobilization – an easier way to repair cloacas. J Pediatr Surg 32:263–264
8. Peña A, Levitt MA (2005) Imperforate anus and cloacal malformations. In: Ashcraft KW, Holcomb W, Murphy JP (eds) Pediatric Surgery 4th edn. Elsevier Saunders, Phila- delphia, p 509
9. Kiely EM, Peña A (1998) Anorectal malformations. In:
O’Neil YA, Rowe MI, Grosfeld JL et al. (eds) Pediatric Sur- gery. Mosby Yearbook, Elsevier, p 1442
10. Peña A, Levitt MA, Hong AR, Midulla P (2004) Surgical management of cloacal malformations: a review of 339 pa- tients. J Pediatr Surg 39:474–475
