POLSKIPRZEGLĄD CHIRURGICZNY 10.2478/v10035-007-0007-92007, 79, 1, 36–41

SURGERY FOR INFLAMMATORY BOWEL DISEASE IN CHILDRENAND ADOLESCENTS

WIKTOR MEISSNER1, WALDEMAR SZABŁOŃSKI1, PIOTR KROKOWICZ2,IWONA IGNYŚ3, JACEK SZMEJA1, MICHAŁ DREWS1

Chair and Department of General, Gastroenterological and Endocrine Surgery,K. Marcinkowski Medical University in Poznań1

Kierownik: prof. dr hab. M. DrewsChair and Department of General and Endocrine Surgery,

K. Marcinkowski Medical University in Poznań2

Kierownik: dr hab. P. KrokowiczChair and Department of Pediatric Gastroenterology and Metabolic Diseases,

K. Marcinkowski Medical University in Poznań3

Kierownik: prof. dr hab. W. Cichy

Recent decades have seen a constant rise in the incidence of IBD in both adults and children. Despite consi-derable progress in the pharmacological treatment of this disease, surgery has become the more frequentlyused treatment modality in younger patients. In the presence of massive haemorrhage, free perforation,fulminate colitis or acute obstruction, only surgical intervention has a chance of saving the patient’s life.The aim of the study was to present the results of surgical treatment of IBD in children and adole-scents who were operated on in a department which copes with “adult surgery” in its everyday practice.Materials and methods. 235 patients were operated on for IBD in the years 1998-2005. There were18 (7,66%) children in this group, 10 girls and 8 boys. 12 patients were diagnosed with ulcerative colitis(66.7 %) and (6) patients were diagnosed with Crohn’s disease (33.3%). The age of the patients rangedfrom 12 to 17 years (mean 15.6). Among the 18 children, 10 (55.6%) were operated on for elective re-asons and 8 (44.4%) of the interventions were emergencies (three perforations, two obstructions, oneacute haemorrhage and one fulminate colitis). In all cases of ulcerative colitis, a two-step restorativeproctocolectomy with J pouch anal anastomosis was performed. Patients with Crohn’s disease weretreated by limited (sparing)[it seems that either limited or sparing works here, pick one] bowel resectionand/or strictureplasty.Results. There were no postoperative deaths in the study group. Postoperative complications wereobserved in 6 (33.3%) patients, the complications were ileus in 3 patients (1 patient demanded relaparo-tomy), pneumonia in 2 patients and wound suppuration with subsequent dehiscence in 1 patient. In onepatient treated preoperatively with large doses of Imuran, the postoperative histology revealed a mali-gnant lymphoma. Hospital stays ranged from 8 to 19 days (mean 12 days).Conclusions. Surgery for IBD in children and adolescents has become a widely accepted method, andit is often the only treatment modality that offers a chance of a cure. Restorative proctocolectomy shouldbe considered earlier in many cases of younger patients with ulcerative colitis, prior to conservativetreatment, as imunosupression and steroid therapy in particular produce undesired side effects. A con-sulting surgeon should be involved in the treatment of younger patients with IBD at a much earlierstage of therapy than is currently practiced.Key words: ulcerative colitis, Crohn’s disease, surgery

Until recently, it was believed that inflam-matory bowel diseases (IBD), which include ul-cerative colitis (UC) and Crohn’s disease (CD),

affect children less frequently than adults.However, it is currently recognized that in 20-30% of patients, the onset of the disease oc-

37Surgery for inflammatory bowel disease in children and adolescents

curs in childhood or early adolescence beforesexual maturity has been reached (1, 2). Addi-tionally, a constant rise in the incidence of IBDhas been recorded both in adults and in chil-dren (3). At the same time as surgeons are be-coming more experienced in the treatment ofIBD in adults, surgical intervention is moreoften performed as the method of choice in pae-diatric patients. In cases of massivehaemorrhage, bowel perforation, fulminatecolitis, toxic megacolon or acute bowel obstruc-tion, surgery is the only treatment which of-fers a chance of saving the child’s life (4).

The aim of this study was to present theresults of surgical treatment for IBD in chil-dren in our surgical department, which rou-tinely treats adult patients.

MATERIAL AND METHODS

The group of 235 patients were operated onfor IBD between 1998 and 2005 at the Depart-ment of General, Gastroenterological and En-docrine Surgery in Poznań, Poland. There were18 children in this group (7.66%), 10 girls(55.6%) and 8 boys (44.4%). Of the children, 12suffered from ulcerative colitis (66.7%) and 6from Crohn’s disease (33.3%). The age of thepaediatric patients ranged from 12 to 17 years(mean 15,6).

Common presenting symptoms includedabdominal pains in 18 patients (100%), diar-rhoea (more than 4 stools per day) in 14 pa-tients (77.6%) and fresh blood in stools in 11patients (61.1%). Menstruation abnormalitieswere observed in all female patients. The timebetween the onset of the disease and the op-eration ranged from 3 months to 6 years (mean2.3 years). 10 patients were on steroids beforethe operation and 3 received Imuran (Azathio-prine). Symptoms of iatrogenic Cushing syn-drome were present in 6 of the 10 patients onsteroid therapy. Preoperative serum proteinlevels ranged from 3,8 g/dL to 6,7 g/dL (mean5,1 g/dL). The patients’ absence from schoolbecause of symptomatic disease requiring hos-pitalization ranged from 3 to 25 months (mean5 months)

RESULTS

In the group of paediatric patients, 10 op-erations were performed for elective indicationsand 8 for urgent or emergent reasons (3 ileus

cases, 2 perforation cases, 1 severe bleedingcase, 1 case of toxic dilatation of the colon and1 giant retroperitoneal abscess with severe sys-temic septic complications).

In all UC patients, the first stage of surgicaltreatment consisted of a restorative procto-colectomy with ileal “J” pouch-anal anastomo-sis and a temporary loop ileostomy. This tech-nique is currently advocated by both Ameri-can and European centres renowned for theirexpertise in the surgical treatment of IBD (5,6, 7).

In three CD patients, right hemicolectomiesincluding the distal portion of the terminal il-eum were performed. In two patients, smallsegmental bowel resections were carried out.In one child, the resection of 20 cm of thestrictured ileum was performed together witha Heineke- Mikulicz strictureplasty of a 4 cmlong narrowing of another portion of the ileumand a by-pass anastomosis of a nonresectableinflammatory tumour.

In five patients with CD, intestinal anasto-moses were constructed with linear staplers,creating wide side-to-side anastomoses. Thechoice of this type of anastomosis was basedon our own encouraging experience in adultpatients and convincing positive data on thistechnique from the literature (8, 9, 10).

No postoperative deaths were recorded inthe study group. Postoperative complicationswere seen in 6 patients (33.3%). Postoperativeileus occurred in 3 patients (16.7%), which inone case demanded relaparotomy. Pneumoniawas observed in two patients (11.1%) andwound suppuration with subsequent dehis-cence was detected in one patient (5.6%).

In one female patient treated preoperativelywith large doses of Azathioprine (200 mg perday), histological evaluation of the resectedcolon revealed lymphoma.

The patients’ hospital stay ranged from 8to 19 days (mean 12 days).

In all patients subjected to restorativeproctocolectomy (n=12), the loop ileostomy wasreversed in the second stage of surgical treat-ment. The follow-up period after ileostomy clo-sure range[d] from 6 months to 6 years. Func-tional results after the restoration of intesti-nal continuity were satisfactory. The patientshad 2 to 7 bowel openings per day (mean 3.2)and only two patients reported night soiling ofthe underwear (16.7%). Clinical signs ofpouchitis occurred in two patients (16.7%) and

38 W. Meissner et al.

were successfully treated with metronidazoleand antibiotics. In one patient operated on forCD, the active disease relapsed, calling forreoperation (7.14%).

In February 2006, a 17 year old male patientwas readmitted to the ward who had been op-erated on 17 months earlier for UC (a restor-ative proctocolectomy had been performed).Four months after ileostomy closure, the boypresented with severe symptoms of pouchitisrefractory to all conservative measures. Thepatient was operated on; the pouch was excisedand a terminal ileostomy was performed. His-tological examination of the pouch revealedprofound inflammatory changes typical of CD.

DISCUSSION

Ulcerative colitis and Crohn’s disease con-stitute a diverse group of clinical entities com-monly referred to as non-specific inflammatorybowel diseases (IBDs). IBDs are increasinglyaffecting children and adolescents, and theirpresenting symptoms and complications makethe choice of adequate treatment of paramountimportance in paediatric patients. One of themost relevant issues is the timing of surgicalintervention and the extent of surgery whichwill result in the best long-term results. Treat-ment of IBD in children demands attention todifferent factors than in adults. The evaluationof a child suffering from IBD should not be lim-ited to intestinal symptoms. Extraintestinalmanifestations are extremely important inqualifying a child for surgery. Growth retarda-tion, delayed pubescence and psychosocial prob-lems are extremely important in the paediatricpatient group. The differences between adultsand children imply the necessity of a varied ap-proach to both conservative and surgical treat-ment. Kirschner (10) points out a lack of pro-spective trials on the efficacy of the conventionaldoses of non-steroid[al] anti-inflammatory medi-cations, steroids and immunosuppresants, inparticular age groups. He stresses the need forsufficient nutrition therapy augmenting thechild’s growth and draws attention to the un-desired side-effects of prolonged conservativetreatment by medication, especially for steroidsand immunosupression.

Because of the different nature of these twodiseases, the surgical treatment approach mustalso be based on different principles. In the caseof CD, an operation is necessary in cases of se-

vere complications. Indications for surgery in-clude mechanical obstruction, septic complica-tions (abscess, inflammatory tumour, fistulaconnecting the intestine to the urinary tractor resulting in short bowel syndrome) and freeperforation. Additionally, an operation shouldbe considered in all patients with severe, per-sistent anaemia resulting from protracted in-testinal bleeding and in patients with grosslyimpaired intestinal absorption which producescachexia or growth retardation (12).

In children, important elective indicationsfor surgery include the failure of adequate con-servative measures, fast relapse of the diseaseafter steroid dose reduction, a lack of thera-peutic effect at maximal doses, undesired side-effects or growing steroid-dependency. Thisview is shared also by El-Baba, who evaluatedindications for surgery and long-term results.In his paper, he reported on 32 children ofwhom 22 were histologically diagnosed as CD.He listed the indications for surgery in the or-der of frequency of incidence: side-effects ofpharmacotherapy, growth retardation, me-chanical obstruction, septic complications andperforations. In 47% of the patients in thisgroup, surgical intervention led to considerablealleviation of symptoms in the year followingsurgery. In 22% of the patients, active diseaserecurred, however the relapses were controlledmuch better with conservative measures. Twopatients demanded relaparotomy andparenteral nutrition. The authors concludedthat surgery considerably improves the pa-tients’ quality of life, and best results shouldbe anticipated in patients with locally limiteddisease (6). The decision of whether to operatefor complicated cases of CD is still not an easytask, which was also confirmed in the study byBaldassano. The author reported that recur-rences occur in 17% of patients a year aftersurgery, 38% after three years and approxi-mately 60% after 5 years. He observed that inpatients with disease limited to the colon, thetime of remission ranged from 1 to 2 years.When the disease affected the ileo-caecal junc-tion, the disease recurred after a mean of 4,4years. Cases with multifocal localization of thedisease [reached a] mean of three years with-out postoperative recurrence. The conclusionfrom his study was that recurrence is more fre-quent in children with more advanced diseaseand with disease localized in the colon. Resec-tion of the affected portion of the bowel im-

39Surgery for inflammatory bowel disease in children and adolescents

proves the general condition and allows fornormal growth of the child (14).

In this study, the technique of strictureplastywas applied in only one patient. The potentialbenefits of this method have been presented byOliva among others. He reported strictureplasty(with or without bowel resection) in paediatricpatients with multifocal localization of CD, andfound considerably decreased intestinal symp-toms resulting in a body mass gain and a markedreduction of steroid doses (15).

The philosophy behind the surgical ap-proach to UC treatment is much different fromthat practiced in CD. In cases of emergent in-tervention, the procedure of choice isHartmann’s colectomy with an end ileostomyand rectal remnant reaching above the perito-neal reflection. In cases of elective operations,total restorative proctocolectomy removes theprimary site of inflammation and the poten-tial site of severe dysplasia and subsequentmalignancy. In the 1970s, attempts were madeto replace the traditional ileostomy with an il-eal pouch with a valve (Kock’s pouch). In 1987,Ein reported on his almost 10 years of experi-ence in performing this technically demandingand complication-plagued surgery in paediat-ric patients. 15 months after pouch formation,no major problems were recorded. When pouchemptying was analysed, difficulties were de-tected, probably related to valve insufficiency.Major inflammatory changes in the pouch wereobserved between the 15th month and 5th yearafter pouch formation, while after five yearsthe risk of any complications was almost none(5). However, except for papers by Kock andsome of his followers, there is no convincingevidence proving this relatively complicatedmethod to be superior to properly constructed(according to Brooke’s principles) end ileo-stomy (16). The necessity of pouch emptyingthrough catheterization poses a serious prob-lem, especially in the case of paediatric patients.According to the opinions of many patients,pouch catheterization and the associated nec-essary irrigations significantly decreased theirquality of life.

In the cases when the inflammatory changestypical of UC are less prevalent or absent inthe rectum, it is possible to complete a colec-tomy with ileo-rectal anastomosis. This proce-dure leads to good functional results, decreasesthe number of stools to a mean of 3 per dayand lowers the risk of impaired continence.

However, this is not the optimal technique tobe used in children because of the necessaryendoscopic surveillance of the left portion ofthe rectum. In that case, rectoscopy is manda-tory in order to prevent severe inflammation,dysplasia and subsequent neoplastic transfor-mation of the rectal stump.

Presently, the preferred surgical option fortreatment of UC is restorative proctocolectomy.Paediatric surgeons often restore intestinalcontinuity after colectomy using Soave’s pro-cedure. During this operation also known asthe “pull-through” procedure, the terminal il-eum is pulled through a rectal cuff that hasbeen subjected to mucosectomy to the level ofthe dentate line. The introduction of an ilealpouch anastomosed to the anus just above thedentate line with preservation of sphincterfunction was a breakthrough in colorectal sur-gery. Even though restorative proctocolectomyhas become a “gold standard” in adult surgery,the “pull-through” operation is still preferredby paediatric surgeons. The study published byRintala confirms the observation that a restor-ative proctocolectomy with IPAA may be usedin children with the same rate of success as inadults. Complications after restorativeproctocolectomy are mainly related to preop-erative therapy with immunosuppressants.The main problem directly associated with theunderlying inflammatory condition is inflam-mation of the pouch mucosa (pouchitis), whichmay lead in extreme situations to pouch fail-ure (6). Alexander published the results of hisobservations on a group of 151 paediatric pa-tients after restorative proctocolectomy, witha postoperative follow-up period ranging from2 to 15 years. He identified Crohn’s disease asthe main reason of failure of this operation,which was diagnosed in 15% of the patientswith pouchitis and pouch failure. He alsoproved that indeterminate colitis and intraop-eratively diagnosed terminal ileitis are not al-ways associated with CD and failure of restor-ative proctocolectomy (18). Nagar also advo-cates the need for earlier surgical interventionfor children suffering from UC (17). The op-eration results in a fast withdrawal of the symp-toms, allows for the patient to resume schoolactivities, improves quality of life and facilitatesthe termination of aggressive forms of phar-macotherapy (steroids and immunosuppres-sants) (7). Nagar stresses the low complicationrate and very good functional results includ-

40 W. Meissner et al.

ing intact sphincter function and less than 4stools per day

CONCLUSIONS

1. Surgical treatment of IBD in children hasbecome an accepted method of treatment,and is sometimes the only treatment whichcan cure the severe complications of thesediseases.

2. Restorative proctocolectomy with IPAA isthe treatment of choice in adults with UC.This operation should also be consideredmore often in children with UC prior to con-servative treatment, especially since steroidsand immunosuppressants produce undes-ired side-effects

3. A consulting surgeon should be involved inthe treatment of IBD in paediatric patientsat an earlier stage than is currently practiced.

REFERENCES

1. Escher JC, Taminiau JA: Treatment of inflam-matory bowel siease in childhood. Scand J Gastro-enterol 2001; Suppl 234: 48-50.2. Spray C, Debelle GD, Murphy MS et al.: Currentdiagnosis, management and morbidity in paediatricinflammatory bowel disease. Acta Paediatr 2001;90(4): 400-05.3. Armitage E, Drummond HE, Wilson DC et al.:Increasing incidence of both juvenile – onset Crohn’sdisease and ulcerative colitis in Scotland. Eur J Ga-stroenerol Hepatol 2001; 13(12): 1439-47.4. Dodero P, Magillo P, Scarsi PL et al.: Total colec-tomy and straight ileo-anal Soave endorectal pull-through: personal experience with 42 cases. Eur JPaediatr Surg 2001; 11(5): 319-23.5. Fonkalsrud EW, Thakur A, Beanes S et al.: Ile-oanal pouch procedures in children. J Paediatr Surg2001; 36(11): 1689-92.6. Rintala RJ, Lindahl HG: Proctocolectomy andJ-pouch ileo-anal anastomosis in children. J Pa-ediatr Surg 2002; 37(1): 66-70.7. Wierzbicki T, Herman J, Drews M, Krokowicz P:Jakość życia i wyniki czynnościowe u chorych poodtwórczej proktokolektomii. Proktologia 2001;2(4): 350-57.8. Keighly MR: Stapled strictureplasty for Crohn’sdisease. Dis Colon Rectum 1991; 34: 945-47.9. Scott AD, Uff C, Philips RK: Suppression of ma-crophage function by suture materials and anasto-motic reccurence of Crohn’s disease. Br J Surg 1993;80: 387-91.

10. Herman J, Wierzbicki T, Krokowicz P i wsp.:Porównanie dwóch technik wykonywanej proktoko-lektomii odtwórczej u chorych leczonych z powoduwrzodziejącego zapalenia jelita grubego. Pol PrzeglChir 2000; 72(8): 708-17.11. Kirschner BS: Differences in the managementof inflammatory bowel disease in children and ado-lescents compared to adults. Neth J Med 1998; 53(6):13-18.12. Rampton D, Shanahan F: Nieswoiste zapaleniajelit. Wydawnictwo Via Media, Gdańsk 2002.13. El-Baba M, Lin CH, Klein M et al.: Outcomeafter surgical intervention in children with chronicinflammatory bowel disease. Am Surg 1996; 62(12):1014-17.14. Baldassano RN, Han PD, Jeshion WC et al.:Pediatric Crohn’s disease: risk factors for postope-rative recurrence. Am J Gastroenterol 2001; 96(7):2169-76.15. Oliva L, Wyllie R, Alexander F et al.: The re-sults of stictureplasty in pediatric patients withmultifocal Crohn’s disease. J Pediatr GastroenterolNutr 1994; 18: 306-10.16. Ein SH: A ten-year experience with the pedia-tric Kock pouch. J Pediatr Surg 1987; 22(8): 764-66.17. Nagar H, Rabau M: The importance of earlysurgery in children with ulcerative colitis. Isr MedAssoc J 2000; 2(8): 592-94.18. Alexander F, Sarigol S, DiFiore J et al.: Fate ofthe pouch in 151 pediatric patients after ileal pouchanal anastomosis. J Pediatr Surg 2003; 38(1): 78-82.

Received: 13.06.2006 r.Adress correspondence: 60-3355 Poznań, ul. Przybyszewskiego 49

COMMENTARY

Both Crohn disease and ulcerative colitis arethe inflammatory bowel diseases. To the causesof the both diseases are proposed: genetic, envi-ronmental, immunologic as well as infectiousetiologies.

Inflammatory changes in ulcerative colitisare only in large bowel mucosa. These changes

in Crohn disease are in the full thickness ente-ric wall and can involve every part of the ali-mentary tract, but its main location is in theileo-cecal portion. It is believed that the diseaseonset in 15-20% of patients is in the childhood.The incidence of the inflammatory bowel dise-ase is increasing. The section of inflammatory

41Surgery for inflammatory bowel disease in children and adolescents

bowel disease has been founded in the PolishAssociation of Pediatric Gastroenterology, He-patology and Nutrition. The diagnostic andtherapeutic criteria including indications to thesurgery of the inflammatory bowel disease havebeen established in the Section forum. Theseindications depend mainly on the severity andduration of the disease. They are significantlydifferent for both entities.

The index assessing child’s nutrition sta-tus and Pediatric Crohn Disease Activity In-dex (PCDAI) are used for the evaluation of theseverity of the disease. PCDAI assess clinicalsymptoms, inflammatory parameters andchild’s nutrition degree. The basic manage-ment is constantly modified pharmacologicaltherapy. There is however believed that ca. 50% of children need surgical treatment in 3-5years after the onset of the disease. The indi-cations to the surgery can be divided into twogroups. The first group are emergencies likeileus, perforation, intraabdominal abscess, andrarely enteral bleeding; the second group areelective procedures: no relief after conservati-ve treatment of the acute disease crisis, intesti-nal stenoses and fistulas. Discussing electiveindications one should remember that surgi-cal treatment does not guarantee complete re-covery and most patients will need next opera-tions leading to the short bowel syndrome. Thesurgical treatment does not discharge the needof the simultaneous conservative treatment.Resections and anastomoses should be perfor-med based on surgical principles; in the caseof Bauhin valve resection, isoperistaltic smallbowel valve should be created. Special surgi-cal problem is the first recognition of the Crohndisease during the operation for the suspectedappendicitis. The management depends on thesubjective assessment and experience of thesurgeon and on the general condition of thechild. It seems that ileocecal resection is justi-fied when inflamatory changes of the cecumare obvious. Appendectomy as the only proce-dure threatens the production of the intestinalfistula or abscess and the disease recurrenceafter ileocecal resection is observed later afterlonger duration of the remission.

Ulcerative colitis is assessed by Trueloveand Witts scale complying clinical presenta-tion, inflammatory parameters and ischemiadegree. The disease is classified as mild, mo-

derate and severe forms. Pharmacologic tre-atment gives improvement in ca. 80% cases.The indication for emergency, life-preservingoperation is acute sepsis with paralytic ileuscalled as toxic megacolon. The emergency in-dication for the operation can be also the per-foration and rarely stenosis and fistula. Ho-wever, often recurrences leading to the growthfailure and the delayed maturation are theelective indications to the surgery. For the fa-vor of the surgery is the fact that surgical in-tervention in the ulcerative colitis, as oppo-sed to the Crohn disease is curative and fur-ther conservative treatment is unnecessary.One should also remember that after 10 yearof the disease the risk of neoplastic metapla-sia is increasing significantly. The surgicalintervention is complete colectomy. We perfor-med it based on the Soave procedure descri-bed for the Hirschsprung disease. The wholecolon to the level of the rectum is resected fromthe abdominal cavity aspect and the rest ofthe mucosa is removed from the perinealaspect. The small bowel is pulled through thecreated channel. In this way both internal andexternal sphincters are intact. We create J po-uch before the small bowel anastomosis, Ka-liciński-CZD – does not create the pouch butcreates invagination valve. The surgical re-sults are good as far as the stool frequencyand continence are concern. When the opera-tion is elective and performed after good pre-paration of the child, even temporary ileosto-my is not necessary.

The child with inflammatory bowel diseaseneeds also nutritional management and perma-nent psychological care besides pharmacologi-cal therapy both before and after operation.Therefore such a child should be treated by theexperienced team lead by pediatric gastroente-rologist with active presence of pediatric surge-on. This model is realized in large pediatriccenters in our country. Therefore, appreciatingthe author’s center, where “generally” and “eve-ryday” adult patients are treated, I would liketo stress finally that children with inflamma-tory bowel disease should be referred to the ter-tiary pediatric centers.

Prof. dr hab. Janusz BohosiewiczKierownik Katedry i Kliniki Chirurgii Dziecięcej

Górnośląskiego Centrum Zdrowia Dziecka i Matkiw Katowicach