Long-term outcome after free autogenous muscle transplantation for anal incontinence in children with anorectal malformations

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Long-term outcome after free autogenous muscle transplantation for anal incontinence in children with anorectal malformations
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  Long-term outcome after free autogenous muscletransplantation for anal incontinence in childrenwith anorectal malformations  Johan Danielson a, ⁎ , Urban Karlbom b , Wilhelm Graf  b , Tomas Wester  c a   Department of Pediatric Surgery, Akademiska Sjukhuset, Uppsala, Sweden  b  Department of Surgery, Akademiska Sjukhuset, Uppsala, Sweden c  Department of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden Received 9 November 2009; revised 4 June 2010; accepted 7 June 2010 Key words: ARM;Anal incontinence;Long-term follow-up;Free muscle transplant  AbstractPurpose:  Patients with high anorectal anomalies are often incontinent after reconstruction, particularlywith the older forms of surgical treatment, that is, anorectal pull-through or Stephen's operations. In1974, a new treatment for anal incontinence in children was introduced at the Akademiska Hospital: freeautogenous muscle transplantation (FAMT) to the perirectal area. All the patients receiving FAMT weretotally incontinent before the procedure and had no rectal sensitivity. The aim of this study was toevaluate the long-term functional outcome of this procedure. Methods:  Twenty-two patients (17 males) operated on with FAMT below the age of 15 years wereidentified through records. One of the patients had died, and 2 were not available for follow-up. Theremaining 19 were sent a validated bowel function questionnaire, and 15 (78.9%) of 19 patientsresponded (12 males). These 15 patients were compared with 15 patients with the same sex, age, and asimilar malformation from our patient database. Results:  At follow-up, after an average of 30 years postoperatively, 2 of 15 patients with FAMT had astoma compared with 3 of 15 in the control group. The Miller incontinence score had a mean of 6.2(median, 6; range, 0-15) in the FAMT group and 3.7 (median, 4; range, 0-12) in the control group. All patients in both groups could sense stool, and 11 of 13 patients in the FAMT group could distinguish between feces and flatus. Conclusions:  The patients with FAMT had a slightly inferior anorectal function compared with thecontrols. Considering they were all totally incontinent before FAMT, we conclude that FAMT has anacceptable effect 30 years postoperatively. Therefore, we find that FAMT could be an alternative for anorectal malformation patients who are totally incontinent.© 2010 Elsevier Inc. All rights reserved. Anorectal malformations (ARMs) are relatively commonanomalies with an incidence of 1:2500 to 1:5000 live births[1-3]. The clinical presentation is highly variable rangingfrom mild forms managed with minor surgical procedures tocomplex malformations that require multistaged surgery. ⁎ Corresponding author. Barnkirurgiska kliniken, Akademiska Sjukhu-set, S-75185 Uppsala, Sweden. Tel.: +46 18 611 00 00.  E-mail address:  johan.danielsson@lul.se (J. Danielson).www.elsevier.com/locate/jpedsurg0022-3468/$  –  see front matter © 2010 Elsevier Inc. All rights reserved.doi:10.1016/j.jpedsurg.2010.06.009Journal of Pediatric Surgery (2010)  45 , 2036 – 2040  Anorectal malformations are somewhat more common inmales than in females, with 56% of the cases presenting inmales [4].The understanding of the anatomy, pathophysiology,management, and consequences of ARMs has evolvedtremendously since the 1950s. However, many patientsoperated for a more complex ARM, especially with older techniques, have a high incidence of anal incontinence [5,6].Therefore, many different surgical procedures have beensuggested to improve continence after previous surgery.One of the operative techniques described was the freeautogenous muscle transplantation (FAMT), which was first described by Hakelius [7] in 1975. The procedure is performed in 2stages. In the first step, a musclegraft (usuallythe palmaris longus muscle, but also sartorius and extensor digitorumbrevishavebeenused)isdenervated bydivisionof the nerves close to the muscle. This is done to decrease themetabolism in the graft and thus enabling the graft to surviveas a free transplant. Two weeks later, the muscle is taken as afree muscle graft and is transplanted in a U-shaped slingaround the rectum. During a period of 9 months, the graft isthen reinnervated from the puborectal nerves, and approxi-mately one third of the graft survives and contributes to theimprovement of anal continence [7]. The procedure wasevaluated 3 to 5 years postoperatively [8-10], and the resultswere encouraging. In 1984, Grotte et al [11] reported theresults 4 to 9 years postoperatively in their series, and theystated virtually normal continence in 10 of 21 patients andsocial satisfactory continence in 9 of 21 patients. Hakeliusand Olsen [12] reported results with an average follow-up of 11yearsand4months.Intheirstudy,60%ofthecaseshadanoutcome that was regarded as good and 16% as fair, 8% hadimproved, and 16% were considered as failures.The aim of this study was to investigate the long-termfunctional outcome of all pediatric patients operated withFAMT for anal incontinence caused by an ARM at theAkademiska Hospital in Uppsala, Sweden. 1. Materials and methods 1.1. Patients The patient files and operative registry at the Department of Pediatric Surgery, Akademiska Hospital, Uppsala,Sweden, were searched for patients diagnosed with ARMsfrom 1964 to 1993. We found 257 patients and reviewedtheir charts. Among the 257 patients, we identified 22 patients that were operated on with FAMT. One of the patients had died in 2001, and 2 of the patients were lost tofollow-up because they were foreign citizens and could not  be traced. The 19 remaining patients were included in thisstudy and received a questionnaire. Fifteen (79%) of the 19 patients responded to the questionnaire. All of the patientshad been operated for a high ARM in infancy. They weretotally fecally incontinent and had no rectal sensitivity after their primary surgery according to their charts.To identify a suitable control group, we searched our register of ARM patients for patients with similar age, samesex, and a similar malformation (ie, high ARM) as the studygroup. Fifteen patients with the best matching characteristicswere selected. The selection of these cases was blinded to theauthors in such a way that an independent person was askedto chose the most appropriate patients from a database list only containing age, sex, and type of malformation.The characteristics of the patients in the FAMT group andthe control group are presented in Table 1. 1.2. Patient assessments Patients responded to a validated bowel questionnaire [13].This questionnaire consist of 49 questions relating to fecalincontinence andgeneralbowelfunctionsymptoms.Fromthisquestionnaire, Miller's incontinence score can be calculated.This score is based on the type and frequency of incontinenceepisodes, where 0 represents total continence and 18 maximalincontinence [14]. The questionnairealsogives information of type of incontinence (classified as soiling, urge, nonurge, or combination incontinence), medication, anal sensibility, andwhether the anal continence affects social function. Table 1  Patient characteristics of the FAMT group and controlgroup listing the discrepancies between the groups, age at FAMT procedure, and length of follow-upParameter FAMTgroupControlsAge at follow-up (y)Mean 40.9 39.6Median 40 40Range 34-47 32-49Age at FAMT procedure (y)Mean 11.2 N/AMedian 11Range 8-13.5Time elapsed after FAMT procedure (y)Mean 29,7 N/AMedian 30Range 23-35SexFemale 3 3Male 12 12Presence and type of fistula No fistula 5 2Rectourethral fistula 5 8Rectovesical fistula 5 2Rectovaginal fistula 0 3Type of primary procedureStephens procedure 3 8Abdominoperineal pull-through 12 7 2037Long-term outcome after FAMT for anal incontinence  The patients also responded to questions about their occupation/studies and whether they thought their analincontinence had affected their choice of occupation/studies.Patients also responded to the Swedish version of the 36-item Short-Form Health Survey (SF-36) questionnaire,which is validated for the Swedish population [15]. Theresults are expressed as scores where a higher score implies ahigher or better functioning level. 1.3. Ethical considerations The study was approved by the local ethics committee of Uppsala University. 2. Results 2.1. Anal continence Two patients (13.3%) in the FAMT group had a permanent colostomy caused by incontinence. The remaining 13 patientshadameanMillersincontinencescoreof6.2(median,6;range,2-15),meaningthattheaverageFAMTpatientwasincontinent to gas daily and to loose stools at least once a week.Of the 13 FAMT patients not having a colostomy, 7 patients (53,8%) had soiling-type incontinence, 5 patients(38,5%) hade urge-type incontinence, and 1 patient (7,7%)had a combination. None were totally continent.Three patients (20%) in thecontrol grouphad a permanent colostomycausedbyincontinence.Theremaining12patientshad a mean Millers incontinence score of 3.7 (median, 4;range, 0-12), meaning that the average control patient wasincontinent to gas daily and occasionally to loose stool.Of the 12 control group patients not having acolostomy, 2 patients (16.7%) had soiling-type inconti-nence, 1 patient (8.3%) hade urge-type incontinence, and 3 patients (25%) had a combination. Six patients (50%) weretotally continent. 2.2. Need for protection of underwear  Of the 13 FAMT patients not having a colostomy, 6(46.1%) used protection in their underwear only during theday and 3 (23.1%) used it both day and night.In the control group, 3 patients (25%) used protection intheir underwear only during the day and 4 patients (33.3%)used it both day and night. 2.3. Medication and diet Seven patients (46.6%) in the FAMT group usedloperamide, and none used bulking agents on a regular  basis. Five (33.3%) of the patients in the FAMT group usedenemas on a regular basis. Nine patients (60%) kept a diet  because of their bowel function.Two patients (13.3%) in the control group usedloperamide, and 3 patients (20%) used bulking agents on aregular basis. Two patients (13.3%) in the control group usedenemas on a regular basis. Five patients (33.3%) were on adiet because of their bowel function. 2.4. Rectal sensibility Thirteen (100%) of the FAMT patients without colostomyclaimed rectal perception and could feel when defecationwas pending. Eleven patients (84.6%) could distinguish between stool and flatulence.In the control group, all patients without a colostomy-claimed rectal perception and could feel when defecationwas pending. All patients could distinguish between stooland flatulence. 2.5. Psychologic and social effects In the FAMT group, 11 patients (73.3%) reported that their bowel function had a negative effect on their well- being, 10 patients (66.7%) reported that their bowel functionhad a negative effect on their social life, and 2 patients Table 2  Clinical outcome characteristics of FAMT patientsand controls listing parameters regarding continence and socialand psychological effects of ARMParameter FAMTgroupControlsMiller incontinence scoreMean 6,2 3,7Median 6 4Range 2-15 0-12Permanent colostomy 2 (13.3%) 3 (20%)Use of protection in underwear  None 4 (30.8%) 5 (41.7%)During daytime 6 (46.1%) 3 (25%)During both day and night 3 (23.1%) 4 (33.3%)Medication on a regular basisLoperamide 7 (46.6%) 2 (13.3%)Bulking agent 0 (0%) 3 (13.3%)Enemas 5 (33.3%) 2 (13.3%)Dietary regimen 9 (60%) 5 (13.3%)Rectal sensibilitySense of rectal filling 13 (100%) 12 (100%)Distinction between stool/gas 11 (84.6%) 12 (100%)Social and psychologiceffects of bowel function Negative effect on well-being 11 (73.3%) 7 (46.7%) Negative effect on social life 10 (66.7%) 6 (40%)Does not dare to go on vacation 2 (13.3%) 0 (0%)ARM effect on choice of occupationYes 6 (40%) 1 (6.7%) No 9 (60%) 14 (93.3%) 2038 J. Danielson et al.  (13.3%) reported that their bowel function resulted in that they did not dare to go on vacation.In the control group, 7 patients (46.7%) reported that their  bowel function had a negative effect on their well-being, 6 patients (40%) reported that their bowel function had anegative effect on their social life, and no patient (0%)reported that their bowel function resulted in that they did not dare to go on vacation. 2.6. Influence on the choice of occupation Most patients (60%) in the FAMT group stated that their choice of occupation had not been influenced by their  bowel function, whereas 6 patients (40%) stated that their occupational choice had been influenced. Two of the patients were retired, and this was partly because of their  bowel function. Four had avoided occupations with heavylabor and situations where they could not get to a toilet ona very short notice.In the control group, only 1 patient stated that theoccupational choice had been influenced by his bowelfunction/ARM.The results from Sections 3.1 to 3.5 are summarized inTable 2. 2.7. Quality of life The SF-36 quality of life scores of the FAMT groupand control group did not differ significantly from age- andsex-matched reference values for the Swedish population(Fig. 1). 3. Discussion Since the introduction of the posterior sagittal anorecto- plasty procedure (PSARP) in the early 1980s, the main postoperative complication has been constipation andsecondary to this overflow-type incontinence. This affectsmore than half of the patients with high and intermediateARM operated on with PSARP [16] and can often be treatedconservatively with medication. Patients not responding toconservative treatment almost always become sociallycontinent with the use of antegrade continence enema[17,18]. However, a small number of patients operated onwith PSARP are still incontinent with antegrade continenceenema and might be candidates for further reconstructivesurgery. During the last decade, only one new procedure for the treatment of incontinence in ARM patients has beenintroduced, namely, dynamic graciloplasty. In short term, patients with ARM had a worse outcome compared with patients with anal incontinence for other reasons. Still, patients with ARM improved after the procedure [19]. Theuse of sacral nerve stimulation (SNS) on ARM patients has been suggested, and we have encountered several ARM patients who have had SNS procedures. However, to our knowledge, no systematic study has been published on SNSin ARM patients. When keeping this in mind, the plethora of different procedures suggested for improving incontinence inARM patients becomes interesting. Among these proceduresare Stephens' secondary pull-through and secondary repair of damaged or hypoplastic muscle complex [20], Kott-meier's levatorplasty [21], Puri's and Nixon's levatorplasty[22], gracilis muscle transplant  [23], gluteus maximus transplant  [24], and flap smooth muscle transplantation [25]. We know from the article of Grotte et al [11] from 1991that FAMT procedure was performed on 26 patients.However, 2 of these had Hirschsprung disease, and 2 wereolder than 15 years at the time of the FAMT procedure andwere not included in our study, and therefore, we areconfident that we have identified all pediatric patientsoperated with FAMT procedure in Uppsala.The prognosis of ARM is very closely related to the typeof malformation according to the Krickenbeck classification[26]. A big problem in this study was to extract relevant information to accurately classify the patients ARM. Wehave scrutinized the charts for clinical data and descriptionsof status and correlated these to the Krickenbeck classifica-tion. However, because the charts are 30 years old, theinterpretation of them is fraught with difficulties and risk of misinterpreting the type of malformation. We have mini-mized the bias in the interpretation of data by letting one person extract the data from charts.We found it interesting to compare the FAMT patientswith a control group that did not undergo secondaryreconstructive surgery to improve continence. Our selec-tion of a control group is, of course, debatable. The type of operation is not the same with a much higher incidence of Stephen's procedures, and the age matching is not perfect.In a retrospective study, it is, however, very difficult toavoid this selection bias, and considering the rarity of highARM, this control group was considered feasible. Whencomparing the FAMT and control groups, one shouldremember that the patients who were offered a FAMT Fig. 1  Short-Form Health Survey (36 items) scores (min 0, max100) of the FAMT group and control group compared with thematched Swedish population. 2039Long-term outcome after FAMT for anal incontinence   procedure had the worst functional outcome after the primary reconstruction of the ARM.It is a very interesting fact to see that the FAMT patients who, before their FAMT procedure, were reportedas having no rectal sensibility had a good rectal sensibilityat follow-up. Whether this can be attributed to the FAMToperation, to a definite improvement over time after ARMsurgery as suggested by Puri and Nixon [6], or to the fact that when a person ages, he/she learns to listen to his/her  body's signals better and adapts his/her life to symptoms isof course open for debate. It should also be kept in mindthat this is only self-reported data and not data gained fromobjective measurements.The large proportion of patients reporting that their ARM had a negative effect on their well-being and sociallife in both the FAMT group and in the control groupindicates that these patients are in need of further medicalattention. The lack of significant differences in the SF-36data was not unexpected because the SF-36 is a generalinstrument of measuring quality of life. Comments made by the patients on the returned forms also indicate that many feel that they cannot be helped further and their  problems are ignored by physicians. This strongly indicatesthe need for interdisciplinary medical support in adulthoodas suggested by Koroda [27].Our results show that at follow-up, the patients operatedon with FAMT have an inferior continence, slightlyinferior rectal sensibility, more frequent use of protectionin their underwear, and higher incidence of negative psychologic and social effects attributed to their ARMwhen compared with our control group. However, the patients operated on with FAMT were a highly selectedgroup of patients that were totally incontinent before theFAMT procedure. When this is taken into account, weconsider that the FAMT procedure seems to provide fair long-term results in children with severe incontinence after  primary ARM surgery. Therefore, we think that the FAMT procedure can be an option in ARM patients who aretotally incontinent. However, we strongly advocate further investigation of the effect of SNS and dynamic gracilo- plasty on ARM patients. References [1] Cho S, Moore SP, Fangman T. One hundred three consecutive patientswith anorectal malformations and their associated anomalies. ArchPediatr Adolesc Me 2001;155(5):587-91.[2] Christensen K, Madsen CM, Hauge M, et al. An epidemiological studyof congential anorectal malformations: 15 Danish birth cohortsfollowed for 7 years. Paediatr Perinat Epidemiol 1990;4(3):269-75.[3] Loening-Baucke VA. Factors responsible for persistence of childhoodconstipation. J Pediatr Gastroenterol Nutr 1987;6:915-22.[4] Smith ED. Incidence, frequency of types, and etiology of anorectalmalformations. Birth Defects Orig Artic Ser 1988;24:231-46.[5] Rintala R, Mildh L, Lindahl H. Fecal continence and quality of life inadult patients with an operated or intermediate anorectal malformation.J Pediatr Surg 1994;29:777-80.[6] Puri P, Nixon HH. The results of treatment of anorectal anomalies:a thirteen to twenty year follow-up. J Pediatr Surg 1977;12:27-37.[7] Hakelius L. Free Autogenous muscle, transplantation in two cases of total anal incontinence. Acta Chir Scand 1975;141:69-75.[8] Hakelius L, Gierup J, Grotte G. Further experience with freeautogenous muscle transplantation in children for anal incontinence.Z Kinderchir 1980;31:141-7.[9] Mollard P, Valla V, de Beajujeau MJ. Incontinence anale apresimperforation: traitment per transplantation musculaire libre. Chir Pediatr 1979;20:205-8.[10] Holschneider AM, Hecker WC. Flapped and free muscle transplan-tation in the treatment if anal incontinence. Z Kinderchir 1981;32:244-58.[11] Grotte G, Hakelius L, Frykberg T, et al. Nine years of free autogenousmuscle transplantation for anal incontinence in children. Z Kinderchir 1984;39:80-2.[12] Hakelius L, Olsen L. Free autogenous muscle transplantation inchildren. Long-term results. Eur J Pediatr Surg 1991;1:353-7.[13] Österberg A, Graf W, Karlbom U, et al. Evaluation of a questionnairein the assessment of patients with faecal incontinence and constipation.Scand J Gastroenterol 1996;31:575-80.[14] Miller R, Bartolo DC, Locke-Edmunds JC, et al. Prospective study of conservative and operative treatment for faecal incontinence. Br J Surg1988;75:101-5.[15] SF-36 hälsoenkät, Swedish manual and interpretation guide, 2nd ed.2002.[16] Rintala R, Lindahl H, Marttinen E, et al. Constipation is a major functional complication after internal sphincter-saving posterior sagittal anorectoplasty for high and intermediate anorectal malforma-tions. J Pediatr Surg 1993;28(8):1054-8.[17] Peña A, Hong A. Advances in the management of anorectalmalformations. Am J of Surg 2000;180(5):370-6.[18] Bischoff A, Levitt MA, Bauer C, et al. Treatment of fecal incontinencewith a comprehensive bowel management program. J Pediatr Surg2009;44(6):1278-83 [discussion 1283-4].[19] Koch SM, Uludag O, Rongen MJ, et al. Dynamic graciloplasty in patients born with an anorectal malformation. Dis Colon Rectum2004;47:1711-9.[20] Stephens FD, Smith ED. Anorectal malformations in children: update.March of Dimes Foundation Birth Defects. Original Article Series1988, Volume 24, Number 4. Alan R. Liss, New York.[21] Kottmeier PK. A physiological approach to the problem of analincontinence through use of the levator ani as a sling. Surgery 1966;60:1262-6.[22] Puri P, Nixon HH. Levatorplasty: a secondary operation for fecalincontinence following primary operatio for anorectal agenesis. JPediatr Surg 1976;11:77-82.[23] Pickrell KL, Georziade N, Crawford H, et al. Construction of a rectalsphincter and restoration of anal continence by transplanting thegracilis muscle. Ann Surg 1952;135:853-62.[24] Hentz VR. Construction of a rectal sphincter using the srcin of thegluteus maximus muscle. Plast Reconstr Surg 1982;70:82-5.[25] Holschneider AM, Hecker WC. Smooth muscle reverse plasty. A newmethod to trat anorectal incontinence in infants with high anal andrectal atresia. Results after gracilis plasty and free muscle tyransplan-tation. Progr Pediatr Surg 1984;1:131-45.[26] Hasset S, Snell S, Hughes-Thomas A, et al. Ten-year outcome of children born with anorectal malformation, treated by posterior sagittal anorectoplasty, assessed according to the Krickenbeckclassification. J Pediatr Surg 2009;44(2):399-403.[27] Koroda T. Continuing problems in patients who undergo repair for anorectalmalformation.NipponGekaGakkaiZasshi2009;110(4):191-4. 2040 J. Danielson et al.
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