平 成4年6月19,20日(金,土)

京 都 市 国 際 交 流 会 館 ・都 ホ テ ル

Defecation Patterns after Posterior Sagittal Anorectopasty

―Imperforateanus,SeoulExperience―

Woo-Ki Kim

Seoul National University, Seoul, KOREA

Imperforate anus, common name representing more wide spectrum ofanorectal malformation of the newborn is one of the major congenitallesions in pediatric surgery. Since the establishment of pediatric surgeryin Seoul National University (1978), more than 500 cases of anorectalanomalies were treated until 1990.

To evaluate the life after corrective treatment, defecation patternswere studied using clinical Kelly scores in 90 high type imperforate anuswho were repaired during 1978-1985, period. These 90 patients were operatedby the author, using only two surgical repair methods (26 Rehbein, 64Pena). Not only the continence scores but also other aspect of socialadaptation as well as index of quality of life were compared to controlgroup.

Although precise anatomical repair under direct view (Pena) will givebetter defecation pattern compared to blind type of abdomino-perinealrepair, their long term adaptation is not significantly superior. Thisstudy showed the function is not always net result of anatomic repair.Thus, the importance of initial corrective operation is again emphasized.

Index(Key) words: Imperforate anus, anorectal malformations, Posteriorsagittal anorectoplasty, Kelly score, incontinence

Footnote: This study was supported by 1989 Seoul National UniversityHospital Research Grant.

Professor Iwafuchi, members and guests, it is great honor to give a special

presentation at the annual meeting of 17th Japanese College of Surgeonsmeeting at the old city of Kyoto. My personal gratitute to Professor Ozawaand members of the organizing committee for this wonderful opportunity.

We all knew the famous remark made by Potts(1):"In general, atresia ofthe rectum is more poorly handled than any other congenital anomaly of thenewborn." Unfortunately, over 30 years later, situation now is not that

greatly different. The purpose of this study is to analyze the result ofthe correction of high type imperforate anus who is followed more than 6

years.(Table 1). To get the reaction of the medical profession, we askedabout 60 doctors and 40 nurses work at the Childrens Hospital.(Table 2.) Of

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course number one response was Pediatric surgery, Shonigeka . Next popularresponse was incontinence. I assume they knew where the problem was . Otherresponses were mostly not happy one. Stool, sphincter, enema, poor familyand mother. We, pediatric surgeons, all agree that these imperforate anusor anorectal malformation is one of the most difficult, unattractive ,unsolved problem. It is well known that term "imperforate anus" is used for

Table 1. Purpose of this study:Evaluation of the Results of 87 high type (1978-85)

Table 2.Response of 100 doctors and nurses at the SNU CH:

What comes in your mind when you heard "imperforate anus":

Table 3.Anorectal malformations: very wide spectrum

Table 4.Seoul National University CH experience

May 1978 - Dec 1990 ( 12.5 years)

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a long time. But it is too simplified and descriptive. Ano-rectalmalformation is more proper term to use. But for convenience, it will beused as interchangably in this presentation. (Table 3.)

MATERIALS AND METHODS

MATERIALS:Since the establishment of the first pediatric surgical units in universityin Korea, fairly large volume of patients were collected during earlier

year. It was handled by single surgeon. Table 4. is our top ten list. Asyou can see, again imperforate anus/or anorectal malformation is our number2 on top ten list. This does not mean we have an epidemic of these disease.It simply means we have more patient compare to other hospital in Korea. Itis kind of reward of having the first department of pediatric surgery atthe university hospital in Korea. Also our neonatal emergency or indexcases were analyzed to see the frequency. Again, anorectal malformation arethe most common neonatal surgical problem. (table 5) Table 6. shows statusof anorectal malformation at our hospital. Eight hundred operations weredone for 539 patients till end of 1990. High type is about 30 % of total.For high type, Rehbein type abdominoperineal repair(2) was used at thebeginning. Then we have been using posterior sagittal approach of deVriesand Pena(3) since 1982. Our general plan of management of imperforate anusis shown on Table 7. Most of intermediate types were treated as high typeanomaly. For the girl, miniature Pena or posterior sagittal approach are

Table 5. 929 Neonatal Surgical Emergencies (May 1978-1990)from 11,733 operations (74 newborn/938 op./year)

Table 6.801 operations for anorectal malformation (May 1978-1990):

539 patients (Seoul Nat'l Univ Child Hosp)

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used more frequently in these days. This, in other term, is an "anatomicrepair" under direct vision.

METHOD:The purpose of this presentation is to show the result of high type repairwho is followed more than 6 years. All of them are attending school.The result of 90 high type imperforate anus handled by single surgeon, andfollowed more than 6 years will be analyzed. (Table 8) To have faircomparison, new cases of both type of repair methods are analyzed indetail. For the objective evaluation of defecation patterns, clinical

Kelly (4) score were used. In this study, Kelly's scoring system ofincontinence were used for three reasons. The first one is its originality.The second one is it's easy and simple to check. The last reason is its'close correlation with other scoring system. We have been using Kelly'sclinical score for evaluation since it is very easy to get score. All youhave to is ask if the patient had accident in defecation, stain underpantand do rectal examination.(Table 9) For the radiologic evaluation, using cine radiologic technique,radiologic score can be studied. (table 10) Only small portion of ourpatients were studied for the radiologic score. It is useful for the radiologist to have some guideline to deal with incontinent patient. Idon't know the longterm value of this x-ray scoring sytem in relation withactual daily life situation. We did a limited study on radiologic scoring

Table 7. Management plan for Imperforate anus: new casesat Seoul National University Childrens Hospital(SNUCH)

Table 8. Grouping of high imperf. anus by study period (SNUCH)

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Table 9. Kelly's Clinical Continence Scoring System

Table 10. Cine "defecogram": Radiographic analysis: Kelly (1969)

Table 11.

Qantitative Assessment of Fecal Continence: Templeton/Ditesheim(1985)

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system, and it showed very close correlation with clinical scoring systemas well as other scoring system also. Kelly himself advocated the use ofboth scoring system expressed as the Code of continence.

Kelly Code of continence:clinical assessment + functional/radiological assessment

Since then, many system of scoring has been devised by several authors.(2,5,6-8). To test the correlation with other system, Templeton's

quantitative continence score (Table 11) were compared to Kelly's. For faircomparison, result of 1981 and 1982 were studied. This close proximity ofrepair period can minimize the difference of the surgeons skill level.(table 12) Finally, to check the satisfaction level of parent/or patient,a scoring system was made by us(table 13). To emphasize the socialactivity, higher score were given to school, camp or overnight trip, sportand popularity among friend.To give similar feeling, 0-2 were regarded as

Table 12.To study correlationship between Kelly(clinical and radiologic) score andTempleton/Ditesheim score: following patients were selected.

Table 13.Subjective feeling of parents and patient: Satisfaction level1 point to each "No": happy (6-5 point), unhappy (0-2 points)

Table 14. Non-parametric Statistical analytic method (goodness of fit )

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RESULTS

1) CONTINENCE RESULT:Table 15 show you the result of clinical Kelly score on 3rd, 6th year postrepair. Also current, year 1992's Kelly score was on the slide. As a wholeneither Rehbein or Pena group showed significant improvement as the time

passed. But new patients of each group and redo Pena showed somesignificant improvement of mean value over the time factors. Althogh forthe new cases, there is statistically significant improvement of mean Kellyscore, there is no significant improvement on individual patient. The

proportion of each group did not change greatly. (table 16)

2) CORRELATION WITH OTHER SCORING SYSTEM (Templeton/Ditesheim):Both clinical and radiologic score of Kelly were compared toTempleton/Ditesheim scoring system for the patients of 1981-82 period. Theclose proximity of year in two group was to provide the fairness ofcomparison. (Table 17) These three scores are statistically correlated in

Table 15.Clinical Kelly score(mean) at 3rd, 6th year post-repair

Table 16.Assessment using Clinical Kelly score: good: 5-6, fair: 3-4, poor:0-2in new cases of high imperforate anus

unhappy and score 5-6 were regarded as happy.For the statistical analysis of various scoring system, following non-

parametric statistical analytic methods were used. (table 14)

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mean value.

3) PARENT/PATIENT SATISFACTION SCORES(Table 18):These Kelly's assessment were compared with our parent satisfaction score.

It was found that these two scoring system is very closely related in mostcases. But these parent scoring system was made only in last year, itsreal value have to be tested more by time factor.

4) IN SUMMARY, based on these findings, anatomic repair under directvision (posterior sagittal approach) gave the best result at the moment.Initial operation is so important since time does not improve much. Kellyscore is very usuful, though not perfect. Parent satisfaction level weretested. It gave very close correlation to other objective scores.

DISCUSSION:

HISTORY: If one try to understand the evolution of the treatment of hightype imperforate anus, it can be divided in four stages. The first stageof passing stool ended in mid-19 century. How to pass stool is the main

Table 17. Comparison of Kelly score and Templeton/Ditesheim scoreDefecation score in patients 1981-82 group (11 Rehbein, 29 Pena)

Table 18.Correlation between subjective feeling and Kelly score in 1992,expressed as per cent of each group:

(by Spearman rank correlation coefficient:rs P<0.01)Kelly score were expressed as good, fair, and poor group on top raw.

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goal of surgeons which usually was followed by severe stricture of the newanus. Afterward, finding of anal or rectal end can be more easily doneonce human can enter the peritoneal cavity after 1880's. This is the stageof finding rectal end. In 20th century, as death from ano-rectal malformation became less likely, incontinence became major problem in

Table 19. Historic Event of Imperforate Anus:

Table 20. Controversies on Anatomy: Striated Muscles of Ano-rectum:

Table 21. Anatomy of ano-rectal malformation

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dealing with this anomaly (stage of incontinence). It still has to besolved. But even with anatomic/functional sequellae, the quality of lifeshould be improved. Some of the historic events showing the evolution ofthe treatment are shown in table 19. It seems very slow but pleaseremember that human abdomen was entered only after 1880. Thus, evolution ofimperforate anus is not greatly underdeveloped.Although Amussat of Paris is credited for the proctoplasty, hisachievement is to bring mucosa of rectum to the skin of perineum which

Table 22. History of classification of anorectal malformations

Table 23. Operative Management of High type

Table 24. Posterior sagittal anorectoplasty :

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prevent immediate stricture which was quite natural after conventionaloperation of perineal tunnelling with trochar or knife. Major difficulty inthat time is to find rectum. (6)As soon as surgeon begin to open the abdomen, surgeons begin to useabdominoperineal approach to find the rectal blind end. Many surgeon usedcolostomy to find the blind rectal end. Use of air as a contrast materialto demonstrate rectal end in newborn by an x-ray is another triumph for thesurgeon. At that time abdomino-perineal approach was used as a stagedprocedure. More precise anatomic repair became surgeon's aim since they seemany incontinent unhappy child. Surgeons attention is now focused onanatomy.

ANATOMYThere are controversies about the anatomy of the sphincter of rectum.(9).Although very nice pictures of sphincters are present in many textbook, no one is for sure. Fortunately, Stephens and Smith(6) came up withpubo-rectalis sling idea. Whole attentions were focused on pubo rectalissling. So how to find and preserve pubo-rectalis sling became surgeons

primary goal. But pubo-rectalis sling is another rather vague muscle. Tosomeone, it is very big muscle, to others it is still very dificult tofind. At the time when men landed moon, we are looking for rather obiousmuscles around the rectum of a baby since still many surgical repair methodare not done under direct vision of surgeons. Table 20. summarize the controversies regarding anatomy of striated sphincter of ano-rectum. It isreally amazing why do they have such controversies at this space age. Itis matter of gross morphology, not cellular one. But morphology can bedifferent if they study different specimen with different fixation techniques with different mind.The major difference is external sphincter. Can you separate it frompuborectalis sling? How big is external sphincter? Does it has threeseparate component? Which one is more important in maintenance of contience, upper portion of sphincter or pubo-rectalis sling? Whichportion should we use or preserve maximally in repair operation?In regarding pelvic diaphragmatic portion of levator ani, there are alsobig difference in opinion on sling portion. The questionable muscle massbetween levator ani and ext sphincter was named as "muscle complex" bydeVris and Pena group.(table 21) Alberto Pena(10) described his musclecomplex during his recent workshop . Muscle complex begins at levatormuscle and ends at anal skin. It is located mainly between levator muscleand external sphincter, formally known as pubococcygeus muscle. It is notseparable from surrounding muscles, is not circular in shape, mostly

paramedian verticallly placed fibers. It is varying in size, and angled,curved trajectory course.Okada (11) also described this vertical muscle inmuscle complex. To me, who dealt with many high type ano-rectalmalformation, muscle complex is more attractive name for this muscle ofvariable in size and shape.

CLASSIFICATION: As the knowledge of anorectal malformation expand,advances in the classification progressed as Table 22 shown.(6) The mostpopular Ladd and Gross classification. Oversimplication of Type 3 is theweak point. Type 1 (stenosis), Type 2 (anal membrane) and type 4 (rectalatresia) are very rare. Dr. Santulli tried to modify this. Partridge andGough used the"low/high"descriptive terminology. The most recent

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classification was made in 1984, known as Wingspred classification (12).

OPERATIVE MANAGEMENT OF HIGH TYPE: This is a time table how the repairprogressed up till now.(Table 23) It is about time to show how Dr. Penahandle his muscle complex. I had a personal learning experience from himat New Orleans 1981 AAP surgical section. After initial colostomy, thismuscle complex was exposed via posterior sagittal incision. After properclosure of fistula, tapering of dilated rectum was made. Then anatomicrepair involving this new rectum finished the surgery. (Table 24)

ASSESSMENT OF CONTINENCE: Since many surgeons report their result with

poor, fair and good remark, objective type of system is required to evaluatecorrectly. Stephens and Smith introduced what is known as Kelly score. Itis the most well-known continence scoring system. This scoring system istoo simple. But it is useful even for the retrograde study since one can easily observe if they have accident, smearing of undergarment, and power of rectal squeeze. (Table 25)Stephens/Smith also advocated use of other parameters, such as rectal

Table 25. Assessment of continence:

Table 26. Current Management plan:for high imperforate anus:

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sensation, stool consistency, appearance of anus and others (age,mentality,social/parenteral adquacy). Other parameters are very important. But verydifficult to count score ojectively. Kelly suggested combining clinical andradiologic scoring together to stregthen his too simple clinical score.Althogh there are numerous newly advocated objective scoring system, we are

quite satisfied to use of this original clinical score.

PERSONAL MANAGEMENT PLAN: Table 26. is current management plan based onthese experience. I personally feel daily enema given after repair is veryuseful for "training" and maintenance of cleanliness. Training means both

patients and parent. Reoperation used to be very rewarding since almosttotal repair after abdomino-perineal by non-pediatric surgeons gave betterresult for the reoperative cases. But reoperation after failed Pena is notrewarding. It's repair is not easy. All critical area has been touched by

pediatric or amateur pediatric surgeon. Reoperation criteria after failedPena should be more selective and strictive. Usually 2-3 years with dailyenema is required to make decision of reoperation.

References:1. Potts WJ: The surgeons and the child. Philadelphia, Saunders, 1959, p.2132. Rehbein F: Imperforate anus: Experiences with abdomino-perineal andabdomino-sacro-perineal pull through procedure. J. Ped. Surg 2:99-105,1967 3. deVries PA, Pena A: Posterior sagittal anorectoplasty. J. Ped Surg 17:638-643,19824. Kelly JH: The clinical and radiological assessment of anal continence in childhood. Aust NZ J Surg 42:62-63,19725. Stephens FD, Smith ED: Anorectal Malformations in Children. Chicago,Year Book Medical Publishing, 19716. Kiesewetter WB: Imperforate anus: II. The rationale and technique ofthe sacro-abdomino-pernieal operation. J. Ped Surg 2:106-110, 19677. Swenson 0, Donnellan WL: Preservation of the puborectalis sling inimperforate anus repair. Surg Clinic N. Am 47:173-193, 19678. Templeton JM, Ditesheim JA: High imperforate anus- Quantitative resultsof Long-term fecal continence. J Ped Surg 20:645-652,1985 9. deVries PA, Cox KL: Surgery of Ano-rectal anomalies, Surg Clinic N Am.65:1139-1169, 198510. Pena A: The surgical treatment of anorectal malformations, an intensiveworkshop & teaching course. Dec 12-14, 1991, Taipei, Taiwan11. Okada A, Kamata S et al: Anterior Sagittal Anorectoplasty for Recto-vestibular and Anovestibular•fistula. J Ped Surg 27:85-88,199212. Stephens FD: Wingspread Conference on ano-rectal malformations, Racine,Wisconsin. USA, 1984

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