Imperforate Anus: Experiences with Abdomino-Perineal and Abdomino-Sacro-Perineal Pull-Through Procedures By
FRITZ REHBEIN
p
EDIATRIC SURGEONS have been dealing with the problem of imperforate anus for many years. Especially in your country, numerous papers have been presented in recent years concerning this problem. In the male, it is the patient with recto-urethral or recto-vesical fistula that turns out to be difficult to treat. Therefore, I want to restrict my remarks to these cases in the male, plus those females having an imperforate anus combined with a high recto-vaginal fistula. I would like to report a total number of 70 cases of both groups operated on in our hospital since 1959:55 males and 15 females. The procedure created by Rhoads et al. 1 in 1948 represents a major essential step forward in the treatment of these children in comparison to all types of operations used before. The essential part of this procedure is the pelvic dissection of the blind pouch of the rectum using the abdominal approach. The recto-urethral fistula is transected and closed and the rectum is pulled through to the perineum. Obviously this procedure is hazardous in many ways, though it has many advantages. If the dissection of the blind pouch is not carried out very carefully, there is a possibility of causing damage to the autonomic nerve fibers. The technical procedure turns out to be difficult because the opening of the fistula into the urethra in most cases is situated higher than the deepest part of the blind pouch [Fig. 1 (a) ]. Therefore, we have to dissect a long distance to reach this point. Rarely does one find a funnel-shaped opening [Fig. 1 (b) ]. Most male children with imperforate anus have fistulas, the so-called low forms as well, in which the distance between the floor of the blind pouch and the anal dimple in the perineum is not more than 1.5 cm. Division of the fistula using the perineal approach is difficult even in low forms, and there is danger of causing injury to the anal sphincter in performing the perineal repair. In order to avoid any damage to the innervation, we perform the pullthrough procedure by pulling the colon, that will be the new rectum, through the denuded muscular sleeve of the old blind pouch. We transect the rectum transversely, the blind pouch is pulled up by sutures and the mucous membrane is removed. Then a canal is prepared to that point, where the anal dimple is situated and the colon pulled through. In 1959 I published a paper on this procedure in male newborns in Germany. 2 One year later Romualdi 3 suggested the same procedure, especially for vestibular fistulae in girls. Since 1959 we have treated a total of 70 cases according to the method described. Professor Rehbein was guest lecturer at the October, 1966, meeting of the Surgical Section of the American Academy of Pediatrics, Chicago, Illinois. FRITZ REH~IN, M.D.: Director, Pediatric Surgical Clinic, Municipal Hospital, Bremen, Germany.
99 JOURNAL OF PEDIATRICSURGERY,VOL. 2, NO. 2 (APRIL), 1967
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Fig. 1.--(a) The opening of the fistula is usually situated higher than the deepest part of the blind pouch. (b) Seldom is a funnel-shaped opening found. As a rule we perform this operation in the newborn as long as there are no complications or additional malformations. Our desire is to divide the fistula as soon as possible and thus to remove the source of urinary tract infection. In spite of early repair a considerable number of patients still have a urinary tract infeetion postoperatively. If a newborn has some complication or an additional malformation, we only perform a eolostomy; the pull-through procedure is done a few months later. We have not gained the impression that this operation is easier to perform later and, therefore, we believe it should not be postponed for reason of age alone. In most eases the dissection of the mucous membranes in newborns is carried out more easily than in older babies. We must take care, of course, not to operate on a low imperforate anus without fistula by using the abdominal approach. This occurred only once, in a mongoloid child who later died. As a rule, one can decide without diffleulty by inspection and palpation of the perineum if there is a thick membrane or if one is dealing with an imperforate anus probably combined with fistula. By any technic, our diagnostic accuracy is not perfect. Stephens, 4 in 1953, suggested the sacro-perineal approach in order to accurately locate the tevator ani muscle and the puho-reetalis sling. In 1963 Kiesewetter and Turner a reported their results using this procedure in 15 patients, and pointed out the danger of missing the pubo-rectalis sling and of performing the pull-through procedure somewhere other than through the levator ani muscle whenever the abdominal perineal approaeh is used exclusively. Is there still a danger of missing the pubo-rectalis by our technic? As a matter of fact, Dr. Kiesewetter, during his visit to Bremen last year, demonstrated that we made the same mistake in one child with our method as well. Thus it became necessary to find a way of avoiding these failures. The following points of view seem to be very essential: (1) One has to differentiate between the low and high types. W e feel that the blind pouch penetrates' the levator muscle in the low types. The pathway for the pull-through procedure is therefore preformed, and one cannot miss the right pathway in these eases [Fig. 2 (a) ].
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Fig. 2.--(a) Low type of imperforate anus. The blind pouch penetrates the levatot muscle. (b-d) Three varieties of high imperforate anus or rectal atresia. (2) In the high cases the blind pouch is located at a point as high as the levator muscle or even higher. These cases are pretty similar to the atresia of the rectum. Here there is a great danger of missing the sling [Fig. 2 (b-d) ]. How Can W e Make a Differentiation Between These Cases? In our experience this anatomical difference does not have to be determined before starting the operation. X-rays in head-down position preoperatively are not necessary. The operation may be done without determining the pubo-coccygeal line by a lateral x-ray, as was suggested by Stephens. 4 The decision is made during the operation at the moment of the transection of the upper rectum. Therefore, it is necessary to start with an abdominal approach in every case. If the blind pouch is located directly above the pubo-rectalis sling, it may be difficult to
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determine which type it is. In this situation palpation of the levator may help. After removal of the mucous membrane of the blind pouch, the skin of the anal dimple is excised or incised. Now one can palpate the levator with both index fingers, one above and one below, and try to find the sling [Fig. 3 (a) ]. Often it is represented by a small dimple. It is not always situated at the lowest point of the pouch, but more often points toward the urethra. Under the guidance of the index finger a canal is formed using a clamp directly behind the urethra [Fig. 3 (b) ]. This canal is carefully widened and prepared for the pull-through procedure. Combination with Sacral Approach. If after careful palpation we are not sure which type we are dealing with, we use in addition a sacral approach. The peritoneal cavity is closed temporarily, the child is turned over on the abdomen, and the region of the levator muscle is exposed from behind across the sacrococcygeal articulation. Directly behind the urethra, in which a catheter has been inserted, the puborectalis sling has to be identified [Fig. 4 (a) ]. From here we prepare a canal running to the perineum [Fig. 4 (b) ]. A polyethylene tube is inserted through a hole made in the bottom of the blind pouch into the peritoneal cavity through the muscular sleeve [Fig. 4 (c) ]. This tube serves as a guide for dilatation of the canal. The main difference between this procedure and the procedure of Stephens 6 is the fact that we use the additional approach only for localization of the puborectalis sling and not for the mobilization of the blind pouch or even the ligation of the fistula from here. Because of the danger of injury to the innervation, dissection of the blind pouch is not only unnecessary after the removal of the mucous membrane, but is contraindicated. All High Cases Above the Levator Should Be Operated on in the AbdominoSacro-Perineal Manner. In June 1965 we tried for the first time a combined abdomino-sacro-perineal pull-through procedure in a child who also had an esophageal atresia without fistula. 7 Unfortunately, this child died of peritonitis after a perforation caused by bouginage. Since then we have performed this operation on an additional 5 newborns. The relatively high number of 5 combined pull-through procedures in one year is just chance. There is no doubt at all that our material includes some cases which, according to our present knowledge, would have been better operated upon with such a combined procedure. Because of the danger of missing the sling, Kiesewetter and Nixon 8,9 suggest the combined procedure, even for the low cases, as they found that the blind pouch never does pass the levator ani muscle but that the pouch pushes the muscle downward instead. If further investigations confirm these findings, then there is perhaps some danger even in the low case of missing the sling. According to our present results, I am not convinced this will be necessary, and therefore we will use the combined operation only in high or doubtful cases. I~ESULTS
Since 1959 we have operated on the total number of 70 cases, 55 males and
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Fig. 3.--(a) After removal of the mucous membrane of the blind pouch, the skin of the anal dimple is excised or incised. With bidigital palpation the pubo-reetalis sling may be found. (b) Under guidance of the right index finger a canal is formed using a clamp. 15 females. Two other surgeons participated with me in this number. The technic has been the same through the years except for the additional use of the sacral approach. Thirty patients have been operated on in the immediate postnatal period. Of these children we lost 5, 1 to 6 days postoperatively, from peritonitis, mesenteric-vein thrombosis, complications of esophageal atresia, congenital heart disease and pneumonia. Of the 40 patients operated on later, 27 had a colostomy initially, 4 had a perineal approach and a colostomy and 9 were females with only a perineal procedure. Nowadays we would perform a colostomy first in such high-risk patients as those who died. We lost 3 other children to make a total number of 8 deaths out of 70 patients who have been operated on. No child who was operated upon in two stages died of complications following the, pull-through procedure. W e checked 45 of the 62 surviving children, all of whom are older than 2 years, or in whom the operation has been performed more than 2 years ago. In 18 cases we found good results, in 13 cases adequate results. "Good" means that these children are able to hold formed stools. Some of them have been able even to hold liquid stools, but most of them are not continent when they have diarrhea. In the "adequate" group, the children had incontinence periodically but not regularly. Thus they were handicapped to a slight degree but not severely. In many cases the children have been able to tell when they had to move their bowels. This indicates not only that the normal rectal mucous mem-
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Fig. 4.--(a) Additional sacral approach. Localization of the pubo-rectalis sling. (b) A canal to the perineum is formed. (e) A polyethylene tube is inserted through a hole in the blind pouch into the peritoneal cavity through the muscular sleeve. branes are responsible for the sensitivity, but that the rectal wall and the sphincter muscle as well produce the sensation of the rectum being filled. Moreover, the mucous membranes of the colon which have been pulled through in newborns probably can gain normal sensitivity later. In all bad postoperative results we are not able to palpate any pubo-reetalis sling. In these eases the muscle is either too weak or never has been developed. Another reason may be that the sling has been missed during the pull-through procedure or the sling might have been damaged by a previous perineal operation. These results are estimated more or less individually, especially as we did not measure the intrareetal pressures. Function in some patients may improve with time. Swenson and Grana 1~ pointed out that the generally quoted percentage of 55 to 60 per cent adequate results seems to be a little exaggerated if one uses strict judgment. The permanence of a good function depends to a high degree on the intelligence and the energy of the mother and on the intelligence of the child as well. Whenever proper care is neglected, it is quite possible that fecal impaetion will occur, even in eases which have shown good results before. Relatively older patients (that means at the age of 7 to 8 years), especially girls, become anxious themselves to stay dean. That is another reason why the results still may improve. We saw 3 eases of retraction of the colon which had been pulled through. Two of them occurred in newborns. A fistula formed again and we had severe difficulties. Only in one of these eases, a girl, have we been successful in closing the fistula by a second pull-through procedure by our technique. Finally, I would like to mention the eases which have been incorrectly operated on by a perineal approach. Fortunately the number of the patients operated on in this way is gradually decreasing so that nowadays we rarely have to discuss the problems following this type of poor repair. Usually there is a stenosis of the anus, the fistula is open and there is a megareetum. With these findings one is forced to continue with bothersome therapy for a long time.
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One example may show the stages of treatment a timid 11 year old boy had to undergo. First dilatation of the anal stenosis and removal of the impacted feces was necessary. We then closed the fistula after performing a colostomy. A supra-pubic vesicostomy was also performed. After months of anal bouginage, a plastic procedure using the gracilis muscle was done. Finally the colostomy was closed. After these operative procedures the child did well and developed physically and mentally in a normal way. These procedures seem superior to attempting a pull-through operation in all cases previously incorrectly operated on from the perineum. CONCLUSIONS
Results of the pull-through procedure can be improved ff we accurately take care not to miss the pubo-rectalis sling. This should be possible without a sacral approach except with the very high types of imperforate anus. In the treatment of anal atresia it is our duty not to add surgical damage to the malformations already existing. By carefully avoiding such damage to the nervous tissues and by performing the pull-through procedure at the right site anatomically, we can achieve the best conditions for bowel function. We believe we meet these demands to a high degree by the procedures here described. SUMMARIO IN INTERLINGUA Le technica del transtraction pore esser meliorate si on succede a includer le ansa del musculo puborectal. Istos es possibile sin utilisar le accesso sacral, excepte in casos de ano imperforate del typo extrememente alte. In le tractamento de atresia anal, nostre prime deber es non adder trauma al jam existente malformationes. Per cautemente evitar tal trauma in le tissus nervose e per effectuar le operation transtractori al correcte sito anatomic, nos pote assecurar le melior conditiones possibile pro le functionamento intestinal. Iste demanda es satisfacite in alte grado per le hic-descrihite methodologia. REFERENCES liminary experience with the sacro1. Rhoads, J. E., Pipes, R. L., and Randall, perineal pull-through. Ann. Surg. J. P.: Simultaneous abdominal and 158:498, 1963. perineal approach of operations for 6. Stephens, F. D.: Congenital Malformaimperforate anus with atresia of rections of the Rectum, Anus and Genitotum and rectosigmoid. Ann. Surg. urinary Tract. Livingstone, Edinburgh, 127:552, 1948. 1963. 2. Rehbein, F:. Operation der Anal- und 7. Rehbein, F.: Zur Operation der hohen Rectumatresie mit Recto-UrethralRectumatresie mit Rectourethral-fisfistel. Chirurg 30:417, 1959. tel.-Abdomino-saero-perinealer Durch3. Romualdi, P.: Eine neue Operationszug. Z. Kinderchir. 2:503, 1965. technik fiir die Behandlung einiger 8. Nixon, H. H.: Surgical anatomy in imRectummissbildungen. Langenbeeks perforate anus. Z. Kinderehir. 3:652, Arch. Klin. Chir. 296:371, 1960. 1966. 4. Stephens, F. D.: Congenital imperforate 9. Kiesewetter, W. B., and Nixon, H. H.: rectum, recto-urethral and recto-vagiImperforate anus. I. Its surgical anatnal fistula. Aust. New Zeal. J. Surg. omy. J. Pediat, Surg. 2:60, 1967. 22:161, 1953. 1O. Swenson, D., and Grana, L.: Long-term 5. Kiesewetter, W. B., and Turner, C. R.: results of surgical treatment of imperContinence after surgery for imperforate anus. Dis. Colon Rectum 5:13, forate anus: a critical analysis and pre1962.