Scientific paper

Outcome of the antegrade colonic enema procedure in children withchronic constipation

Evan R. Kokoska, M.D., Martin S. Keller, M.D., Thomas R. Weber, M.D.Department of Surgery, Division of Pediatric Surgery, Saint Louis University Health Sciences Center and Cardinal Glennon Children’s Hospital,

1465 S. Grand Blvd., St. Louis, MO 63104, USA

Manuscript received July 31, 2001; revised manuscript September 13, 2001

Presented at the 53rd Annual Meeting of the Southwestern Surgical Congress, Cancun, Mexico, April 29–May 2, 2001.

Abstract

Background: Chronic constipation and fecal incontinence in children related to pelvic trauma, congenital anomalies, or malignancy willeventually lead to significant social and psychologic stress. Maximal medical treatment (daily enemas and laxatives) can also be difficultto maintain in many children.Methods: At our children’s hospital, 11 children with chronic constipation or fecal incontinence or both underwent the antegrade colonicenema (ACE) procedure. The operation involved constructing a conduit into the cecum using either the appendix (n � 8) or a“pseudo-appendix” created from a cecal flap (n � 3). We report our surgical results.Results: Mean child age was 9.6 (5 to 18) years. With a mean follow-up of 14 (6 to 24) months, 10 of the children (91%) had significantimprovement and 7 children (64%) are completely clean with no soiling and controlled bowel movements after irrigation.Conclusions: Regular colonic lavage after the ACE procedure allows children with chronic constipation and fecal incontinence to regainnormal bowel habits and a markedly improved lifestyle. This procedure should be considered before colostomy in children and adults forthe treatment of fecal incontinence from a variety of causes. © 2002 Excerpta Medica, Inc. All rights reserved.

Keywords: Fecal incontinence; Chronic constipation; Children; Antegrade colonic enema; Congenital anorectal anomalies

Chronic constipation and fecal incontinence in children isrelated to a variety congenital or acquired disorders. Thirtypercent of children develop fecal incontinence after surgicalrepair of anorectal malformations such as imperforate anusor after surgical treatment of Hirschsprung’s disease [1].Chronic and intractable constipation related to pelvictrauma, spina bifida, or malignancy can eventually lead tofecal impaction and overflow incontinence (encopresis).Such severe constipation and fecal soiling can cause bothphysical and emotional disturbance.

Initial conservative management of fecal incontinenceinvolves a bowel management program that includes dailyrectal enemas, diet modification, suppositories, and laxa-tives. However, these programs become more unpleasantand difficult to maintain as children age. When conservativemanagement fails, children either face a lifetime of alteredlifestyle, utilizing maneuvers such as wearing pads for fecal

soiling or, as a last resort, a colostomy for fecal diversion.The concept of the antegrade colonic (or continence) enema(ACE) originated from Malone in 1990 [2]. It had beenpreviously demonstrated that antegrade colonic irrigationvia a rectal tube dramatically decreased fecal soiling inchildren with spina bifida [3]. Malone [2] expanded thistechnique by using the appendix as a conduit for the instil-lation of colonic irrigant. The theory of the ACE is thatchildren may become clean when managed with routine largevolume antegrade enemas that cause complete colonic evacu-ation. We currently report our surgical results of the ACEprocedure in children with chronic constipation and fecal in-continence refractory to maximal medical management.

Methods

The following study was approved by the InstitutionalReview Board at Saint Louis University Health SciencesCenter.

* Corresponding author. Tel.: �1-314-577-5629; fax: �1-314-268-6454.E-mail address: webermz@slu.edu

The American Journal of Surgery 182 (2001) 625–629

0002-9610/01/$ – see front matter © 2002 Excerpta Medica, Inc. All rights reserved.PII: S0002-9610(01)00816-9

The surgical technique is as follows. Children undergoroutine bowel preparation and receive preoperative intrave-nous antibiotics which target enteric organisms. A trans-verse celiotomy is performed in the right lower quadrantcentered on McBurney’s point (Fig. 1A). When available,the appendix is mobilized with care to preserve its bloodsupply. The tip of the appendix is removed and an appro-priately sized red rubber catheter (8 to 12 Fr) is placedwithin the appendiceal lumen directed toward the cecum.The base to the appendix is then imbricated into the cecumwith circumferential, interrupted 3-0 silk (Fig. 1B and C) tocreate an antirefluxing valve. If the child has previouslyundergone appendectomy or if the appendix has been em-ployed for another procedure such as a urinary catheteriza-tion stoma, a conduit is created from a cecal flap (pseudo-appendix). The cecal flap is oriented such that themesenteric vascular supply can be preserved (Fig. 2). Whenpossible, the pseudo-appendix is also plicated into the ce-cum to form an antireflux or nipple valve.

After mobilization and construction of a conduit, a V-shaped skin incision is made with the apex inferior to anddirected toward McBurney’s point (see Fig. 1A). The cath-eter and conduit are brought through the abdominal wall andsutured to skin with interrupted 4-0 Monocryl (poligleca-prone 25; Ethicon, Inc., Somerville, New Jersey). The re-maining skin is closed in a V to Y fashion for creation of aburied, tubular cutaneous stomal anastamosis (Fig. 3). Thecatheter is placed to gravity drainage and the stoma site isdressed with triple antibiotic ointment and loosely coveredwith gauze. A nasogastric tube is place in the operatingroom.

After return of bowel function, the nasogastric tube isremoved and colonic irrigation is initiated. We prefer toleave the conduit catheter in place for 1 month and thenbegin intermittent catheterization for antegrade enema in-

stillation. Children and parents are instructed to allow forirrigation and evacuation before the child goes to bed so asto avoid soiling at night. The child is placed on the toiletwhere irrigations, given over 10 to 15 minutes, can beadministered with either a Toomey syringe or by gravitywith an enema bag. Complete evacuation usually requires20 to 30 minutes. After catheterization, the stoma is washedwith soapy water and covered with a bandage. Daily cath-eterization of the stoma is recommended even when enemasare administered less frequently.

Results

Between 1995 and 2000, 11 children underwent the ACEprocedure at Cardinal Glennon Children’s Hospital in St.

Fig. 1. Creation of orthotopic appendicostomy. A. A transverse celiotomyis performed in the right lower quadrant centered on McBurney’s point.The appendix is mobilized with care to preserve its blood supply. B. Thetip of the appendix is removed and a red rubber catheter (8 to 12 Fr) isplaced within the appendiceal lumen directed toward the cecum. C. Thebase to the appendix is then imbricated into the cecum with circumferen-tial, interrupted 3-0 silk to create an antirefluxing valve.

Fig. 2. Creation of cecal flap (pseudo-appendix). A. The cecal flap isoriented such that the mesenteric vascular supply can be preserved. B. Thececal flap is tubularized around a red rubber catheter using running 3-0Vicryl. C. The cecal defect is then closed with interrupted 3-0 Vicryl.

Fig. 3. Skin leveling of the cutaneous-enterostomy. A V-shaped skinincision is made with the apex inferior to and directed toward McBurney’spoint (see Fig. 1A). A. The catheter and conduit are brought through theabdominal wall. B. The inferior aspect of conduit is spatulated withscissors. C and D. Starting at the apex , the conduit is then circumferen-tially sutured to skin with interrupted 4-0 Monocryl. E. The remaining skinis closed in a V to Y fashion for creation of a buried, tubular cutaneousstomal anastamosis.

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Louis, Missouri. All children were either noncompliantwith, or refractory to maximal nonoperative managementincluding rectal enemas, stool softeners, laxatives, suppos-itories, diet manipulation, and, in a few cases, psychiatricconsultation with “biofeedback” therapy attempts. All chil-dren underwent extensive preoperative counseling with re-gard to management of the ACE stoma and maintenance ofthe enema protocol. Mean child age was 9.6 (5 to 18) yearsand 64% (7 of 11) of the children were female. Preexistingconditions included imperforate anus (n � 6), spina bifida(n � 2), Hirschsprung’s disease (n � 1), resection of apresacral tumor (n � 1), and pelvic trauma (n � 1).

The ACE procedure involved constructing a conduit us-ing either the appendix (n � 8) or a pseudo-appendixcreated from a cecal flap (n � 3). After return of bowelfunction, colonic irrigation with either saline or Golytely(polyethylene-glycol electrolyte solution; Braintree Labora-tories, Inc., Braintree, Massachusetts) was initiated with thevolume (200 cc to 2000 cc) and frequency (daily to threetimes daily) titrated to effect. With a mean follow-up of 14(6 to 48) months, 10 of the children (91%) had less frequentfecal soiling and 7 children (64%) are completely clean withno soiling and controlled bowel movements after irrigation.Five children have been weaned to every other day irriga-tions with the development of fecal continence betweenirrigations. Close follow-up of serum electrolytes haveshown no abnormalities.

One child who initially had good results (for 4 years) hasredeveloped encopresis and moderate soiling. A recent con-trast enema demonstrated fecal impaction with little evac-uation or peristalsis after colonic irrigation. Another childwith myelomeningocele developed ischemia and necrosis ofthe tubularized cecal flap requiring excision. In this case, thececal flap was oriented in the direction of the tenia with thebase toward the appendiceal stump. This is in contrast to ourcurrent approach as depicted in Fig. 2. The child recoveredquickly and awaits a second attempt. Finally, 2 children(18%) have developed stenosis at the cutaneous stoma re-quiring either surgical revision (n � 1) or dilation withHegar dilators (n � 1).

Comments

Chronic constipation and fecal incontinence remains asignificant problem in children with congenital anomalies(eg, spina bifida and anorectal malformations) and acquireddisorders (eg, pelvic trauma or tumors). The goals of stan-dard nonsurgical management are to achieve regular bowelhabits, control the stool consistency with diet and medica-tion, and promote regular colonic emptying with enemasand purgatives. Historically, a colostomy was necessarywhen nonoperative treatment failed.

In 1987, Shandling and Gilmore [3] described moderatesuccess in spina bifida patients with their bowel manage-ment program. They employed an enema continence cath-

eter that administered, in retrograde fashion, large volumesaline enemas. The results of Shandling and Gilmore sug-gested that large volume total colonic washout, throughcomplete bowel emptying, helps prevent fecal incontinenceand soiling. However, maintaining a daily regimen employ-ing retrograde enemas can be arduous in children withimmobility, lax perineal muscles, and a scarred and sensi-tive perineum. Furthermore, rectal enemas are more difficultto administer as children age and become more independent.

Mitrofanoff [4], in 1980, described implanting the ap-pendix, in a nonrefluxing manner, into the bladder for acontinent channel for intermittent urinary catheterization.Ten years later, Malone [2] applied the same principle forthe treatment of fecal incontinence. His technique involvedreimplanting the appendix in a nonrefluxing manner into thececum and exteriorizing the proximal portion (cecal cuff) asa continent stoma. Large volume enemas were then admin-istered through the continent appendico-cecostomy. Theearly results of the Malone ACE procedure were encourag-ing and suggested that soiling can be prevented in childrenwho had failed nonoperative management and who wouldotherwise have colostomy formation [2].

The success of the ACE procedure as demonstrated byMalone is reinforced by the current and previous reports. Inour experience, 10 children (91%) significantly benefitedfrom the ACE procedure and 7 children (64%) are com-pletely continent without soiling. Prior studies in childrenreport success (improved control of soiling) rates and com-pletely clean rates of 71% to 100% and 50% to 95%,respectively [1,5–8]. We observed that irrigations mayeventually be employed less frequently. Initially, childrenusually have massive and dilated transverse and descendingcolons and require large volume enemas and longer evacu-ation times. With daily antegrade irrigations, however, thesize of the colon will decrease and motility will improve,allowing lower enema volumes and less frequent irrigations.

The ACE procedure is not without complication. Greatcare must be taken to preserve the vascularity of the appen-dix or cecal conduit so as to prevent either necrosis orstomal stenosis. While the original Malone ACE procedureinvolved reimplantation of the transected appendix into thececum, our experience and that of other investigators [1,7,8]suggests that orthotopic appendicostomy with cecal imbri-cation may provide good continence while more reliablypreserving appendiceal blood supply. If the cecum is uti-lized for the conduit, the cecal flap should be based upon themesenteric blood supply as depicted in Fig. 2. Dependingupon patient girth and width, small bowel neoappendix, asdescribed by Monti et al [9], can be used in place of a longtubularized cecal flap. However, the ACE procedure em-ploying an orthotopic appendicostomy is associated with theleast complications. For this reason, surgeons should pre-serve the appendix when possible, especially in childrenwith spina bifida and anorectal anomalies. The appendixlikewise should never be used for initial biopsy in the

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assessment of the degree of aganglionosis in children withcongenital megacolon and Hirschprung’s disease.

There is a high incidence of morbidity (mucous dis-charge, stenosis) related to skin leveling of the appendix orcecal tube. The incidence of stenosis in the current report (2of 11; 18%) is similar to the literature (10% to 45%) [1,5,6,8,10] and is much higher in patients with reimplantion ofthe appendix or with a tubularized cecal flap [10]. An inlayof skin at the cutaneous stomal anastamosis, as depicted inFig. 3, is not only associated with less stenosis but alsoavoids problems with exposed mucosa (trauma from over-lying clothes, mucus drainage, and bleeding) and has anoverall better cosmetic effect [6,11]. Precise alignment ofthe cutaneous-enterostomy allows for easy, regular cathe-terization, which also promotes a patent ostomy. Stomalstenosis can usually be managed with either balloons orHegar dilators. Other reported complications of the ACEprocedure include intestinal obstruction [1,10,12], cecal tor-sion [5], and conduit perforation during catheterization[7,10].

Most children have good success with normal salineenemas. When necessary, Golytely irrigations many also beemployed. Our third choice for colonic irrigation is an equalvolume of glycerin and saline. No child in the current seriesdemonstrated serum electrolyte abnormalities with routinefollow-up. We avoid either phosphate or tap water enemas,which can be associated with hyperphosphatemia, hypocal-cemia, or pain [5] and hyponatremia [7], respectively.

Prior to operation, we extensively counsel both the chil-dren and their families in an attempt to assess their level ofcommitment. Patients are told that complete and predictablebowel control is usually not immediate. Previous reportshave suggested that patient compliance is critical to successof the ACE procedure because it is not uncommon forirrigations to be abandoned due to poor patient and familymotivation [7,8]. For this reason, the ACE procedure is bestsuited for older children with severe symptoms and with thestigmata of fecal soiling. Shankar et al [10] performed afunctional assessment (colonic transit time and anorectalmanometry) in children undergoing the ACE procedure forfecal soiling. They concluded that an absent anorectalsqueeze pressure is also a poor prognostic indicator andsuggested that wheelchair-bound children with spinal bifidamay not be good candidates for the ACE procedure.

Few studies have assessed surgical results of the ACEprocedure in the adult population. Gerharz et al [13] re-ported that 50% (8 of 16) of adult patients with fecalincontinence were completely dry and able to achieve a highdegree of independence after the ACE procedure. However,they also reported a high failure rate in patients with chronicconstipation without soiling. Hill et al [14], in a study of 6patients, concluded that adults with severe idiopathic con-stipation and an associated pelvic floor weakness had asignificant decrease in the incidence of fecal soiling afterappendicocecostomy. These reports both suggest that adults

with chronic constipation, regardless of the etiology, andfecal soiling may benifit from the ACE procedure.

In summary, regular colonic lavage after the ACE pro-cedure allows children with chronic constipation and fecalincontinence to regain normal bowel habits and an markedlyimproved lifestyle. Children may no longer require diapers,can better participate in social activities, and will developimproved self-confidence and self-esteem. Although theACE procedure in not a cure for fecal incontinence, it is amore acceptable way for children to maintain a bowel man-agement program. This procedure should be consideredbefore colostomy in children and should receive strongconsideration in adults for the treatment of fecal inconti-nence from a variety of causes.

References

[1] Levitt MA, Soffer SZ, Pena A. Continent appendicostomy in thebowel management of fecally incontinent children. J Pediatr Surg1997;32:1630–3.

[2] Malone PS, Ransley PG, Kiely EM. Preliminary report: the antegradecontinence enema. Lancet 1990:336:1217–18.

[3] Shandling B, Gilmour RF. The enema continence catheter in spinabifida: successful bowel management. J Pediatr Surg 1987;22:271–3.

[4] Mitrofanoff P. Cystostomie continente trans-appendiculare dans letraitement des vessies neurologiques. Chir Pediatr 1980;21:297–305.

[5] Griffiths DM, Malone PS. The Malone antegrade continence enema.J Pediatr Surg 1995;30:68–71.

[6] Hensle TW, Reiley EA, Chang DT. The Malone antegrade continenceenema procedure in the management of patients with spina bifida.J Am Coll Surg 1998;186:669–74.

[7] Meier DE, Foster ME, Guzzetta PC, Coln D. Antegrade continentenema management of chronic fecal incontinence in children. J Pe-diatr Surg 1998;33:1149–52.

[8] Squire R, Kiely EM, Carr B, et al. The clinical application of theMalone antegrade colonic enema. J Pediatr Surg 1993;28:1012–15.

[9] Monti PR, Lara RC, Dutra MA, et. al. New techniques for construc-tion of efferent conduits based on the Mitrofanoff principle. Urology1997;49:112–15.

[10] Shankar KR, Losty PD, Kenny SE, et. al. Functional results followingthe antegrade continence enema procedure. Br J Surg 1998;85:980–2.

[11] Tsang TM, Dudley NE. Surgical detail of the Malone antegradecontinence enema procedure. Pediatr Surg Int 1995;10:33–6.

[12] Lynch AC, Beasley SW, Robertson RW, Morreau PN. Comparison ofresults of laparoscopic and open antegrade continence enema proce-dures. Pediatr Surg Int 1999;15:343–6.

[13] Gerharz EW, Vik V, Webb G, et al. The value of the MACE (Maloneantegrade colonic enema) procedure in adult patients. J Am Coll Surg1997;185:544–7.

[14] Hill J, Stott S, MacLennan I. Antegrade enemas for the treatment ofsevere idiopathic constipation. Br J Surg 1994;81:1490–1.

Discussion

Dr. Vantor: Anyone in this audience who takes care ofchildren born with either imperforate anus or Hirsch-sprung’s disease has to realize how difficult of a problemthis is in these young kids. I believe the operative strategiesreviewed today are fairly standard, although some who

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perform this procedure will place the exit side of the ap-pendix within the umbilicus. The idea is that this may beequally functional, but perhaps more cosmetically pleasing.I’ve done both and feel that I must admit that I’m not surewhich I prefer. The results, as one would expect, are reallyquite good with about 70% of their patients claiming to becompletely clean.

We also feel that if one can adequately evacuate thecolon, then most patients with these problems should beable to lead a fairly normal life without this awful fear andembarrassment of unexpected soiling.

I have three questions for the authors. First, have youtried placing the exit site within the umbilicus instead of theright lower quadrant?

Second, does the antirefluxing maneuvers that you doactually work or have you had problems with it not func-tioning?

Finally, if there is premature or accidental removal ofthat tube, prior to your 1-month time, who places it back in?Have you had any problems with that? Our one significantcomplication was from a perforation from an attemptedreplacement after a catheter got prematurely removed.

Dr. Evan R. Kokoska (St. Louis, MO): We haven’tattempted exteriorizing the stoma at the umbilicus. Thereare reports that do so, and I suspect in a child in whom thebody habitus and the mobilization of the appendix allowsone to bring it over to the umbilicus without undue tension,the differences probably wouldn’t be significant, but that’sour fear of trying to swing it over to the umbilicus, causingany kind of stretch or tension may compromise the conduitand may also lead to a higher stenosis rate.

The question about the antireflux manuevers we do is agood one and has been brought out recently by those whoare attempting this technique laparoscopically, suggestingthat just bringing the appendix up as an ostomy without anyfancy imbrications into the cecum may be just as beneficial,but these series have been small. We haven’t seen anyuntoward effects with imbricating the appendix into thececum. How much it benefits, though, I can’t give you ananswer in face of the recent data.

We haven’t had any occasions of premature removal ofthe catheter either. I suspect, and our protocol would sug-gest, that if this were to happen, the patient should come tothe emergency room and this should be performed by eithera surgery resident or an attending. Our recommendation ofleaving it in for a month is somewhat conservative. When

you look at the literature, most groups leave it for 2 weeks,but maybe we’re a little more anal than the rest.

Dr. Alan Thorson (Omaha, NE): There has been atechnique described using a Hickman or broviac catheter,implanted to irrigate through rather than doing an append-icostomy or your techniques. Do you have any experiencewith that technique and if so, is it successful?

Second, it wasn’t clear to me what your criteria forsuccess was.

Is it patients maintaining continence or did you take intoconsideration the frequency and volume of irrigation? Forinstance, the one patient who required 2,000 cc twice a daywould be questionably successful, at least in my concept.

Closing

Dr. Evan R. Kokoska: To answer your last questionfirst, that child did have the most minimal success. Ingeneral, when we say success, we mean a significant de-crease in the episodes of soiling. As you pointed out, thischild required an enormous amount of volume and fre-quency to achieve adequate emptying. I think one reason forthat is we got to this child a little bit late and at this point,the colon has been so massively dilated and stretched thatthe motility is of question, and this child does remain some-what of a problem. But in general, success would be con-sidered a decrease in the incidence of soiling and for thoseof you who have had any experience with the procedure, it’squite gratifying when it does go well.

We’ve had children tell us that this is perhaps the bestthing that’s happened to them, but it’s not for everybody.It’s best geared toward children who have such a stigmata offecal soiling that they’re afraid to go out into public, to goswimming, and to go to school. When they get to that point,I think this is when the procedure is indicated.

I’ve seen a report in the radiology literature with regardto percutaneous catheter technique in the cecum. We haveno experience with this, and my experience with cecostomytubes would suggest that this might not be a good long-termsolution because the cecostomy tubes, over time with leak-age and such, can be a nightmare. We look at this procedureas more of a long-term solution than as a quick fix and thatis what it is.

We don’t have enough follow-up to know when thesecan be removed, and the children may be free of inconti-nence with normal measures, and I don’t think anybody hasthat experience yet.

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