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Many surgical techniques to repair anorectal malfor- mations (ARM) in females have been described in- cluding an endorectal dissection [1–3], an anterior perineal approach [4], and many different types of anoplasties [5]. The majority of pediatric surgeons now use the posterior sagittal approach to repair these malformations. The debate recently has been centered more on the possibility of performing these opera- tions primarily without a protective colostomy.
21.1 The Posterior Sagittal Approach
The posterior sagittal approach is utilized as described in Chap. 20.
21.2 Repair of specific Defects in Girls
21.2.1 Rectoperineal Fistulas
From the therapeutic and prognostic points of view, this common defect is equivalent to the perineal fis- tula described in the male patients. The rectum is lo- cated within the sphincter mechanism, except for its lower portion, which is located anteriorly. The rectum and vagina are well separated (Fig. 21.1). This defect is repaired in the same way as described for male pa- tients with the exception of course that the dissec- tion is off the posterior vaginal wall rather than the
Contents
21.1 The Posterior Sagittal Approach . . . 303 21.2 Repair of specific Defects in Girls . . . 303 21.2.1 Rectoperineal Fistulas . . . 303
21.2.2 Rectovestibular Fistulas . . . 303
21.2.3 Imperforate Anus Without Fistula . . . 305 21.2.4 Postoperative Care . . . 305
References . . . 305
urethra. Complete mobilization of the rectum is vital until the surgeon sees the areolar tissue that demon- strates that the rectal and vaginal walls are completely separated, so that the anoplasty is performed with no tension. Failure to complete this separation can lead to retraction of the pull-through and to dehiscence of the perineal body.
21.2.2 Rectovestibular Fistulas
Patients with rectovestibular fistulas are frequently er- roneously diagnosed as having a rectovaginal fistula.
Rectovestibular fistula is by far the most common de- fect in females, It has an excellent functional progno- sis. The precise diagnosis is a clinical one requiring only a meticulous inspection of the newborn genita- lia. The clinician observes a normal urethral meatus, a normal vagina, and a third hole in the vestibule, which is the rectovestibular fistula (Fig. 21.2). About 5% of these patients also have two hemivaginas, and a vaginal septum is visible, which should be removed at the time of the pull-through.
This defect may be repaired without a protec- tive colostomy. This is a well-recognized trend in the management of ARM [6, 7], avoids the potential morbidity of a colostomy, and reduces the number of operations to one rather than three (colostomy, main repair, and colostomy closure). Many patients do very well with a single neonatal primary operation without a protective colostomy. However, a perineal infection followed by dehiscence of the anal anasto- moses and recurrence of the fistula provokes severe fibrosis, which may interfere with the sphincteric mechanism. In such a case, the patient may have lost the best opportunity for an optimal functional result, because secondary operations do not render the same good prognosis as successful primary operations [8].
Thus, a protective colostomy is still the safest way to avoid these complications. The decision related to the opening of a colostomy or operating primarily must be taken by individual surgeons, taking into consider- ation his or her experience and the clinical condition
21 Operative Management of Anomalies in the Female
Marc A. Levitt and Alberto Peña
Marc A. Levitt and Alberto Peña
304
Fig. 21.3 Repair of rectovestibular fistula. The rectum is com- pletely separated from the vagina ([11], with permission)
Fig. 21.2 Vestibular fistula ([11], with permission)
Fig. 21.4 Repair of the rectovestibular fistula. The perineal body is repaired ([11], with permission)
Fig. 21.5 Repair of the vestibular fistula.
Muscle complex sutures anchor the rectum
([11], with permission) Fig. 21.6 Repair of the vestibular fistula. Anoplasty ([11], with permission)
Fig. 21.1 Perineal fistula ([11], with permission)
305
21 Operative Management of Anomalies in the Female
of the patient. At our institution, patients who are born with this kind of malformation without serious associated defects are operated primarily as newborns without a colostomy.
The term “rectovaginal fistula” is often misused in patients who actually have a rectovestibular fistula or a cloaca. A real rectovaginal fistula occurs in less than 1% of all cases [9, 10].
The complexity of the rectovesticular fistula defect is frequently underestimated. Multiple 6-0 silk stitches are placed at the mucocutaneous junction of the fis- tula. The incision used to repair this defect is shorter than that used to repair the male rectourethral fistula.
The incision continues down to the fistula and around the fistula into the vestibule. Once the entire sphinc- ter mechanism has been divided, the posterior rectal wall is evident by its characteristic whitish appear- ance. The fascia that surrounds the rectum must be removed to be sure that the dissection is performed as close as possible to the rectal wall. The dissection continues creating the plane of dissection along the lateral walls of the rectum while applying traction on the multiple silk stitches. The last part is the most im- portant part of this dissection, which is the separation of the rectum from the vagina.
There is a long common wall, and two walls must be created out of one using a meticulous technique, trying to keep both walls of the rectum and vagina intact. The dissection continues cephalad until both walls of the rectum and vagina are fully separated (a location identified when the surgeon encounters are- olar tissue between rectum and vagina). At this point both walls are full thickness (Fig. 21.3). If the rectum and the vagina are not completely separated, a tense rectal anastomosis would be created, which, as for the rectoperineal fistula, would predispose the patient to dehiscence and retraction.
Once the dissection has been completed, the peri- neal body is repaired (Fig. 21.4). The anterior edge of the muscle complex is reapproximated as described previously. The muscle complex must be reconstructed posterior to the rectum, with the stitches including the posterior edge of the muscle complex and the pos- terior rectal wall to avoid rectal prolapse (Fig. 21.5).
The anoplasty is then performed (Fig. 21.6).
21.2.3 Imperforate Anus Without Fistula This defect in female patients carries the same thera- peutic and prognostic implications as described for male patients. The surgical technique is similar to that described for males, with the obvious difference being a separation of the rectum from the posterior vaginal wall rather than the posterior urethra.
21.2.4 Postoperative Care
The dilatation protocol is similar to that described for males in Chap. 20. The Foley catheter can be removed in the first 24 h.
References
1. Kiesewetter WB (1967) Imperforate anus, II: the rationale and technique of sacroabdominoperineal operation. J Pe- diatr Surg 2:106–117
2. Rehbein F (1967) Imperforate anus: experiences with ab- dominoperineal and abdomino sacroperineal pullthrough procedures. J Pediatr Surg 2:99–105
3. Soave F (1969) Surgery of the rectal anomalies with pres- ervation of the relationship between the colonic muscular sleeve and puborectal muscle. J Pediatr Surg 4:705–712 4. Mollard P, Soucy P, Luis D, Meunier P (1989) Preserva-
tion of infralevator structures in imperforate anus repair. J Pediatr Surg 24:1023–1026
5. Nixon HH (1988) Nixon anoplasty. In: Stephens FD, Smith ED (eds) Anorectal Malformations in Children:
Update 1988. Alan R. Liss, New York, pp 378–381 6. Goon HK (1990) Repair of anorectal anomalies in the
neonatal period. Pediatr Surg Int 5:246–249
7. Moore TC (1990) Advantages of performing the sagittal anoplasty operation for imperforate anus at birth. J Pedi- atr Surg 25:276–277
8. Peña A (1988) Posterior sagittal anorectoplasty: results in the management of 332 cases of anorectal malformations.
Pediatr Surg Int 3:94–104
9. Rosen, NG, Hong AR, Soffer SZ, Rodriguez G, Peña A (2002) Rectovaginal fistula: a common diagnostic error with significant consequences in girls with anorectal mal- formations. J Pediatr Surg 37:961–965
10. Bill AH, Hall DG, Johnson RJ (1975) Position of rectal fis- tula in relation to the hymen in 46 girls with imperforate anus. J Pediatr Surg 10:361–365
11. Peña A (1989) Female defects. In: Peña A (ed) Atlas of sur- gical management of anorectal malformations, Springer- Verlag, New York, pp 50–55
