UvA-DARE (Digital Academic Repository) Functional defecation disorders in children Kuizenga-Wessel, S. Link to publication

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1 UvA-DARE (Digital Academic Repository) Functional defecation disorders in children Kuizenga-Wessel, S. Link to publication Citation for published version (APA): Kuizenga-Wessel, S. (2017). Functional defecation disorders in children: Associated comorbidity and advances in management. General rights It is not permitted to download or to forward/distribute the text or part of it without the consent of the author(s) and/or copyright holder(s), other than for strictly personal, individual use, unless the work is under an open content license (like Creative Commons). Disclaimer/Complaints regulations If you believe that digital publication of certain material infringes any of your rights or (privacy) interests, please let the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the material inaccessible and/or remove it from the website. Please Ask the Library: or a letter to: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. You will be contacted as soon as possible. UvA-DARE is a service provided by the library of the University of Amsterdam ( Download date: 21 Apr 2019

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3 Chapt%r *(%vß SURGICAL MANAGEMENT FOR CHILDREN WITH FUNCTIONAL CONSTIPATION S. Kuizenga-Wessel, I.J.N. Koppen, L.W. Zwager, C. Di Lorenzo, J.R. de Jong, M.A. Benninga Neurogastroenterology & Motility [Accepted] 237

4 ABSTRACT Background Children with intractable functional constipation (FC) may eventually require surgery. However, guidelines regarding the surgical management of children with intractable FC are lacking. The aim of this study was to describe the surgical management of intractable FC in children. Methods A retrospective chart review was performed of children with FC (according to the Rome III criteria) who underwent ileostomy, colostomy or (sub)total colectomy at a tertiary hospital. Treatment success was defined as no longer fulfilling the Rome III-FC-criteria or having a functional ostomy. Additionally, a self-developed questionnaire was administered to parents by telephone to assess post-surgical satisfaction (yes-no question and rated on a scale of 1-10). Key Results Thirty-seven patients (68% female) were included; median age at first surgery was 12 years (range ). The initial surgical procedure consisted of ileostomy (n=21), colostomy (n=10), sigmoid resection (n=5) and subtotal colectomy (n=1). Success criteria were fulfilled by 85% of the patients. Post-surgical satisfaction of parents was 91% with a median post-operative satisfaction score of 8 (range 2-10), and 97% would opt for the same procedure(s) if necessary. Thirty patients (81%) experienced stoma-problems, with 12 patients (32%) requiring stoma-revisions. Other complications occurred in 16 patients (43%). Conclusions & Inferences Surgery can improve symptoms in children with intractable FC. Despite morbidity and complications, parental satisfaction is high. Prospective, highquality research is necessary in order to develop guidelines for the diagnostic work-up and surgical management in children with intractable FC. 238

5 INTRODUCTION Functional constipation (FC) is a common pediatric disorder with a worldwide prevalence ranging from 0.7% to 29.6% 1. Although the majority of patients with FC respond well to conventional (non)pharmacological treatment, a proportion of patients suffer from intractable FC. In tertiary care centers, 50% of children referred to a pediatric gastroenterologist recover within 5 years, with the majority of patients no longer taking laxatives 2,3. Yet, after 10 years of intensive medical treatment, 20% of the children still suffer from severe symptoms of constipation, such as infrequent hard and painful stools and severe abdominal pain 2. For these patients and their parents, intractable FC is a frustrating problem that significantly reduces quality of life and leads to school absenteeism 4 6. Surgery has been described as a successful therapeutic solution in this subset of patients in whom conservative treatment has failed In their recent systematic review on surgical management in children with FC, Siminas et al. concluded that surgical interventions are based on low-quality evidence. 11 Currently, the choice for surgery is generally based on severity of symptoms, lack of response to intensive (non)pharmacological treatment and results of diagnostic investigations. There is no consensus regarding the diagnostic-work up that is required for surgical decision-making 11,12. Surgery is a treatment of last resort and should only be considered after a thorough evaluation has ruled out treatable organic causes of constipation. Surgery is generally performed in a step-up approach, beginning with the least invasive procedure and proceeding to more invasive treatment options only after failure of the previous step. Since there are no guidelines for surgery in children with intractable FC, the choice of surgical intervention is challenging and the approach differs among centers 13. Surgical options include botulinum toxin injections into the anal sphincter complex, anal sphincter myectomy, sacral nerve stimulation (SNS), creation of an access for administration of antegrade enemas, segmental or total colonic resection, and temporary/permanent diverting ileostomy and colostomy 11,14 The rationale for diversion via an ostomy is to relieve symptoms and to decompress the colon, giving the diverted colonic segment time to recover. Several studies have shown that diversion of a dysmotile colonic segment can lead to improvement of colonic motility in that segment 7,9,15. Segmental and total colonic resections have also been described in this setting. Resection of the dilated and dysfunctional colonic segment can lead to improvement of symptoms and a better quality of life 10,16,17. Since there is not much evidence on the success and possible shortcomings of Surgical management

6 surgical management of children with FC, the aim of this study was to describe our experience with surgical management in children with intractable FC. METHODS Study subjects This study was performed at a tertiary center for children with defecation disorders (Emma Children s Hospital/Academic Medical Center, Amsterdam, the Netherlands). Children (age 0-18 years) who received surgical interventions for FC between January 2010 and June 2015 were identified retrospectively. Prior to undergoing surgery, all children met the criteria for FC as defined by the Rome III criteria 18 and suffered from intractable constipation, defined as failure to respond to intensive conventional therapy for 3 months as described in the ESPGHAN-NASPGHAN guidelines for FC 2. Patients with organic causes of constipation were excluded. Surgical interventions were categorized as: 1) ileostomy; 2) colostomy or; 3) (sub)total colectomy. Patients who had solely received anal botox injections, SNS, appendicocecostomy (also referred to as Malone ) or Chait cecostomy were excluded. Parents of all eligible patients were approached via telephone to answer a questionnaire. Informed consent was provided over the telephone. The study protocol was approved by the medical ethics committee of the Academic Medical Center. Retrospective chart review Clinical characteristics regarding the medical history before surgery were collected through chart reviews. These data included demographics, constipation-associated symptoms, duration of symptoms, treatment prior to surgery and the pre-operative diagnostic evaluation. Additionally, data regarding the operation and post-surgical follow-up were obtained, including the type of operation, occurrence of complications and subsequent reoperations. Complications were classified as per the Clavien-Dindo classification 19, which is based on the type of therapy that is required to treat the complication. Questionnaire Questionnaires developed by our team were administered to parents over the telephone by two authors (S.K.W or L.W.Z.) who were not involved in the initial evaluation and treatment of the child. If possible, parents were asked to answer the questions together with their children. Post-surgical health-related problems and satisfaction about the surgery were assessed together 240

7 with current bowel habits, constipation-associated symptoms according to the Rome III criteria, abdominal pain, school absences, and current medications. Treatment success was defined as no longer fulfilling the Rome III criteria for FC in patients after stoma closure or as having a functional ostomy in children with an ileostomy or colostomy, independent of pharmacological treatment. Parental satisfaction after surgical treatment was rated with a yes-no question, and on a scale of 1-10 (1 = very unsatisfied with the result, 10 = very satisfied with the result) (Attachement 1). The telephone questionnaire took approximately 10 minutes to administer. Statistical analysis Data were analyzed using IBM SPSS Statistics for Windows v 22.0 (IBM Corporation Armonk, New York). Results are shown as percentages of the total sample unless otherwise specified. Results are expressed as means or medians, depending on whether the data were normally distributed or not. RESULTS Patient characteristics Patient characteristics of all 37 included children (68% female) who underwent surgery for intractable FC are depicted in Table 1. At the first appointment in our center, the median age of the included patients was 9.1 (range ) years and they underwent the first invasive surgical procedure (i.e. ostomy or (sub)total colectomy) at a median age of 12.0 (range ) years. A total of 23 patients (62%) experienced severe abdominal pain and 15 patients (41%) suffered from fecal incontinence at the time of presentation. In addition, in 11 children (29.7%) SNS had been tried, 5 (14%) had received botulinum toxin injection in the anal sphincter and 9 (24%) had received an appendicostomy/ cecostomy previous to the surgical interventions described in this article. The choice of the surgical procedure was discussed for every individual patient during a multidisciplinary meeting with pediatric surgeons, pediatric gastroenterologists, specialized stoma nurses and a child psychologist. Surgical management 11 Preoperative testing The results of preoperative diagnostic investigations are shown in Table 2. In 32 (87%) children colonic transit time was measured using radiopaque markers (a capsule with 10 radio opaque markers was ingested on 6 consecutive days with an abdominal X-ray on day 7) 20 : 25 (78%) had proven slow transit constipation 241

8 (>62 hours) and the remaining 7 (22%) had normal transit (<62 hours). However, two children diagnosed with normal colonic transit had continued to take their laxatives during the test. In 18 children anorectal manometry was performed, and all 18 children had a normal rectoanal inhibitory reflex (RAIR) and normal anal sphincter resting pressure. Ganglion cells were present in all 19 children in whom rectal biopsies were taken. In 10 children, no anorectal manometry or rectal biopsies were performed. Twelve patients underwent defecography: among them 7 had abnormal findings, including a rectocele in 3 patients, incomplete relaxation of the puborectalis muscle in 1 patient and 3 other patients failed to defecate during the test. High-resolution colonic manometry was performed in 6 patients, 5 of these children never had abdominal surgery before and 1 child had an ileostomy at the time of colonic manometry. Colonic manometry showed abnormal basal and post-prandial motor activity of the colon in all patients (manometric details of these patients have been described in detail elsewhere 21 ). Post-prandial high amplitude propagating contractions (HAPCs) were identified in one child after the meal (patient 34, Attachement 2). These HAPCs were observed to extend over the descending and sigmoid colon, terminating at the top of the rectum. After administration of intraluminal bisacodyl, HAPCs were observed in 5 out of 6 children. Defecation occurred after bisacodyl infusion in all 5 children. The remaining child had no HAPCs and did not defecate during the test (patient 25, Attachement 2). After colonic manometry, 5 patients without a history of abdominal surgery received an ileostomy, with 2 patients subsequently undergoing a subtotal colectomy with ileo-rectal anastomosis (patient 33, 35, Attachement 2). In one patient colonic motility testing was performed to evaluate the possibility of ileostomy closure, but results showed abnormal colonic motility, which resulted in a subtotal colectomy (patient 19, Attachement 2). Surgical interventions The initial surgical interventions are presented in Table 1 and Attachement 2. Twenty patients underwent a second surgical procedure and 7 patients required 3 surgical procedures. At the end of the study period, 11 patients (30%) had an ileostomy and 7 (19%) a colostomy. Stoma closure with anastomosis was performed in 19 patients (51%). Two senior and experienced pediatric surgeons in colorectal surgery were involved in all surgical procedures. 242

9 Table 1 Patient characteristics No. of patients, N 37 Sex Female, N (%) 25 (68) Median duration of FC symptoms, in months (range) Before first presentation Before surgery Median age at first appointment in our center, in years (range) 48 (3 180) 68 (14-195) 9.1 ( ) Median age at surgery 12.0 ( ) Prior pharmacologic treatment Osmotic laxatives Stimulant laxatives Enemas Rectal irrigation Initial surgical intervention Ileostomy Colostomy Subtotal colectomy Requiring 2 procedures Requiring 3 procedures 37 (100) 23 (62) 33 (89) 29 (78) 21 (57) 10 (27) 6 (16) 20 (54) 7 (19) No. of patients with anastomosis 6 months after initial surgery 13 (35) 1 year after initial surgery 17 (46) 2 year after initial surgery 19 (51) Co-morbidity History of gastroschisis 1 (3) Behavioral problemsa 6 (16) Developmental delay 1 (3) a Behavioral problems: n=2 ASD; n=1 ASD and ADHD; n=1 ADHD; n=1 PDD-NOS and PTSD; n=1 anxiety disorder ASD= autism spectrum disorder, ADHD= attention deficit hyperactivity disorder, PDD-NOS= pervasive developmental disorder not otherwise specified, PTSD= posttraumatic stress disorder, GI= gastrointestinal, UTI=urinary tract infections, GP= general practitioner Surgical management

10 Table 2 Preoperative testing Study N (%) Abnormal/positive results, N (%) Findings Colonic transit time (CTT) 32 (87) 25 (78) 25, delayed CTT (>62 hours) 7, normal CTT (<62 hours) a Anorectal manometry 18 (49) 0 (0) 18, RAIR present and normal resting pressure Rectal biopsy 19 (51) 0 (0) 19, ganglion cells present Barium enema 19 (51) 13 (68) 10, dolichocolon 8, megarectum or sigmoid Defecography 12 (32) 7 (58) Functional findings: 5, normal 3, failed to defecate 1, incomplete relaxation of the puborectalis muscle Anatomic findings: 3, rectocele MRI lumbar spine 10 (27) 0 (0) 10, normal Colonic manometry 6 (19) 6 (86) 6, abnormal colonic motility b a 2 patients with normal CTT used laxatives during the measurement b High-resolution colonic manometry recordings are analyzed for the presence of different colonic motor patterns, including HAPCs [as described previously 21 ] Complications Surgical complications were graded according to the Clavien-Dindo classification 19 and are shown in Table 3. Grade III complications occurred most often (46 times, 27 patients); the majority of these complications were caused by stoma problems. Grade I and Grade II complications occurred respectively twice (2 patients) and 37 times (20 patients). Stoma problems occurred in 30 patients (81%), with revisions of the stoma in 12 patients (32%). 244

11 Table 3 Surgical complications according to Clavien-Dindo Classification Grade of surgical complication a N Grade I Pain at stoma Leakage of stoma High output stoma Re-feeding syndrome Ileus Prolapse of stoma Grade II Diversion colitis Infection Grade III-a Grade III-b ACNES Ileus Infection Retraction of stoma Stoma stenosis Relocation of stoma Stoma leakage Prolapse of stoma Parastomal wound problems Necrotic tissue removal Stomal perforation Parastomal abscess Volvulus Anastomotic leakage Grade IV Grade V Complications were counted again if they re-occurred after (surgical) treatment. a Grade I: Any deviation from the normal postoperative course without the need for pharmacological treatment or surgical, endoscopic and radiological interventions. Allowed therapeutic regimens are: drugs as antiemetics, antipyretics, analgetics, diuretics and electrolytes and physiotherapy. This grade also includes wound infections opened at the bedside. Grade II: Requiring pharmacological treatment with drugs other than such allowed for grade I complications. Blood transfusions and total parenteral nutrition are also included. Grade III: Requiring surgical, endoscopic or radiological intervention, a) intervention not under general anesthesia, b) intervention under general anesthesia. Grade IV: Life-threatening complication (including CNS complications) requiring IC/ICU-management. Grade V: Death of a patient 19. n/a: not applicable, complications did not occur n/a n/a n/a Surgical management

12 Table 4 Results from parental questionnaire - outcomes after surgery N (%) Ileostomy / Colostomy Anastomosis No. of patients Length of follow-up (mo), median (range) 22 (1-42) 20.5 (4-37) 22 (1-42) Medication use None 8 (24) 5 (29) 3 (19) Laxatives a 12 (36) 7 (41) 5 (31) High-dose laxative solutions b 4 (12) 1 (6) 3 (19) Rectal irrigation 2 (9) 1 (6) 1 (6) CHAIT 1 (3) 0 1 (6) Laxatives a + rectal irrigation 1 (3) 0 1 (6) Laxatives a + high-dose laxative solutions b 4 (12) 3 (18) 1 (6) Rectal irrigation + 1 (3) 0 1 (6) high-dose laxative solutions b Bowel movements/week < 2 times/week 7 (21) 3-8 times/week 7 (21) > 9 times/week 2 (6) Stoma 17 (52) Fecal incontinence Yes 3 (9) No 12 (36) Stoma 17 (52) School absence in last month, due to FC Yes 19 (58) 12 (70) 7 (44) No 12 (36) 4 (24) 8 (50) Too young 2 (6) 1 (6) 1 (6) Median duration in days/month (range) 4 (0-20) 5 (0-20) 4 (0-20) a Laxatives included lactulose, polyethylene glycol, bisacodyl, and magnesium hydroxide (regular dosages used in the maintenance treatment of FC 2 b High-dose laxative solutions included polyethylene glycol 3350 (PEG 3350) with electrolytes or sodium picosulfate (solutions that are commonly used for peri-procedural bowel cleanouts) 246

13 Treatment success Out of 37 parents, 33 completed the questionnaire (Table 4.) Seventeen of the 33 children (52%) had a colostomy or ileostomy at time of survey. Intestinal continuity was successfully reestablished in the remaining 16/33 patients (48%) after closure of the ostomy. In total, 28 out of 33 patients (85%) fulfilled our criteria for success. In children with an ostomy in situ, 14/17 (82%) children fulfilled the criteria for success. The remaining 3 children had a variable stoma output, despite laxatives. Twelve out of 17 patients (71%) required additional medical treatment. Fourteen out of 16 (88%) children in whom closure of their stoma was performed fulfilled our success criteria. Additional medical treatment (e.g. laxatives, rectal irrigation) was required in the majority of these patients (12/14 patients, 86%). One of the children still fulfilling the Rome III criteria for FC used laxatives, whereas the other child used daily rectal irrigation. According to the retrospective chart review, complaints of abdominal pain were absent or only present to a slight degree in 23 patients (70%). Ten patients (30%) still experienced abdominal pain, with 6/10 patients having diminished complaints of abdominal pain and 4/10 patients still experiencing the same abdominal pain as they did prior to surgery. Parental satisfaction The majority (91%) of parents was satisfied with the results of the surgical intervention. The median post-operative satisfaction score for the total group was 8 (range 2-10), for parents of patients with an ostomy was 7 (range 2-10) and for parents of patients in whom continuity was reestablished was 8 (5-10). All except one parent would make the same decision in choosing a surgical approach for their child. This was the parent of a child with persistent constipation despite current treatment with laxatives and rectal irrigation after surgery (Table 4, patient 35). DISCUSSION Surgical management This study shows that surgery can be an effective treatment option for children with intractable FC. Although the majority of patients required multiple surgical procedures, intestinal continuity was successfully reestablished in more than half of our patients. Treatment success was reported in 85% of all patients, including 88% of children who underwent closure of their stoma and 82% of children who still had an ostomy

14 Intractable FC is a difficult and frustrating problem for healthcare-providers, parents and children. When intensive (non)pharmacological management (including bowel regimens with oral and/or rectal enemas) is ineffective, alternative and more invasive treatment modalities need to be sought. Surgical interventions should only be considered in severe cases when maximal medical therapies have failed and symptoms significantly affect the child s quality of life. Invasive surgery for FC is preferably performed in specialized centers and requires close collaboration between pediatric gastroenterologists, pediatric surgeons, specialized stoma nurses and child psychologists. Before surgical interventions are considered in these patients, families should be counseled by this type of a multi-disciplinary team. Surgical interventions, especially ileostomies and colostomies, can be both physically and psychologically challenging, and therefore thorough education and psychological assessment should be performed prior to surgery 22. The possible benefits of these major surgical interventions should be balanced against the risk of complications and stoma problems. Several surgical interventions have been described to address intractable constipation, including antegrade enema s, pull-through type procedures 16, J-pouches 17,23, partial or total colonic resections, and temporary or permanent diverting ileostomy or colostomy. In accordance with previous reports 7,11, the majority of the included patients (84%) had an ileostomy or colostomy as initial surgical procedure. In 5 patients an initial sigmoid resection was performed to remove a dilated sigmoid colon. In addition, one initial subtotal colectomy was performed in a teenager that refused an ostomy. The choice for an initial ileostomy was based on the results of colonic dysmotility, based on abnormal transit studies or colonic manometry, or in children who had tried transanal rectal irrigation without success. A colostomy was initially performed if diagnostic investigations showed abnormalities of only the descending colon or if successful rectal irrigation had been successful but was no longer possible due to psychosocial problems. Unfortunately, due to the lack of guidelines regarding the diagnostic work-up and management of children with intractable FC, it remains difficult to decide which surgical intervention should be performed in each case. We present the experience of our center, but without recommended guidelines the choice for surgery will likely differ among centers. The successful outcomes reported in our patients are in line with previous reports in the medical and surgical literature. Nine out of 10 studies included in the analyses of the recently published systematic review by Siminas et al., reported favorable results after colon resections and anastomosis. Outcomes 248

15 were successful in 84% of patients and reoperations were only required in 11%. On the other hand, a study by Christison-Lagay et al. described poor results, with a successful outcome achieved only in 22% 7. The systematic review by Siminas et al. showed that treatment success of colonic diversion ranged between %. However, complication rates were significant, with a morbidity rate up to 40% 11. The high rate of abdominal cutaneous nerve entrapment syndrome (ACNES) could possibly be explained by the usage of a Pfannenstiel incision in patients undergoing a subtotal colectomy. This incision has been associated with chronic abdominal pain because of nerve entrapment 24,25. Although we reported a higher rate of stoma related complications in our study (81%), our results are similar to a study by Formijne Jonkers et al 26. They included 100 adult patients who underwent intestinal stoma formation for different indications (e.g. colorectal malignancies, complicated diverticulitis, inflammatory bowel disease, anastomotic leakage) and reported that 82% of adult patients had one or more stoma-related complications. Described differences in treatment success and complications could also be partially caused by differences in definitions of treatment success and outcome measures. Siminas et al. reported that the diagnostic work-up varied significantly in studies, ranging from no work-up to a full set 11. A comprehensive preoperative evaluation is important to exclude underlying pathology. Our results show that differences in the preoperative diagnostic work-up also exist in our center. In 10 patients, no rectal biopsies or anorectal manometry testing was performed. Although in these children Hirschsprung s disease was very unlikely because constipation symptoms started at puberty, one could argue that these tests should be performed in every case before surgical interventions are considered. Since colonic manometry has only recently become available in our center, colonic motility testing was performed only in a minority of patients. Although the precise role of colonic manometry in surgical decision-making is currently not well defined 11, colonic manometry is currently being used to guide surgery by identifying dysmotile colonic segments that can be resected or bypassed via a stoma 7,9,1 and to guide decision making regarding re-anastomosis after a period of diversion 8. Since patient selection is of key importance for the success of surgical treatment, guidelines regarding preoperative testing are needed. Surgical management 11 It should be emphasized that the aim of this study was not to determine what the best treatment strategies for intractable FC are. We aimed to evaluate and present our current practice and outcomes of surgical treatment in children 249

16 with FC. The limitations of the study are the small sample size, the retrospective study design, and a variable number of study results that were not available for each patient. We could not gather more detailed information about bowel habits prior to the surgical intervention other than what was reported in the medical charts. Although our sample size was small, it was larger than previously published studies on this topic 11. Furthermore, we used a self-developed non-validated questionnaire, not taking into account validated measurements before and after treatment. There is a crucial need for guidelines to guide physicians in the process of surgical-decision making in children with intractable FC. Therefore, prospective studies on the surgical management of these challenging patients, and on the impact of these interventions, are needed, preferably using validated quality of life questionnaires and symptom-based outcome measures (bowel diary and fecal incontinence scores). In addition, standardized definitions and outcomes of treatment success are necessary in order to compare the results of clinical trials. In conclusion, surgical interventions can lead to improvement of FC symptoms in children with intractable FC. Despite considerable morbidity and complications, parental satisfaction is high. Prospective, high-quality research is necessary in order to develop guidelines for the diagnostic work-up and surgical management in children with intractable FC. Since this concerns a specific small subset of patients, a multicenter study approach would be preferred. 250

17 REFERENCES 1. Mugie SM, Benninga MA, Di Lorenzo C. Epidemiology of constipation in children and adults: a systematic review. Best Pract Res Clin Gastroenterol. 2011;25(1): Tabbers MM, DiLorenzo C, Berger MY, et al. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr. 2014;58(2): Staiano A, Andreotti MR, Greco L, et al. Long-term follow-up of children with chronic idiopathic constipation. Dig Dis Sci. 1994;39(3): Bongers MEJ, Benninga MA, Maurice-Stam H, et al. Health-related quality of life in young adults with symptoms of constipation continuing from childhood into adulthood. Health Qual Life Outcomes. 2009;7: Silverman AH, Berlin KS, Di Lorenzo C, et al. Measuring Health-Related Quality of Life With the Parental Opinions of Pediatric Constipation Questionnaire. J Pediatr Psychol children: colonic manometry helps guide clinical decisions. J Pediatr Gastroenterol Nutr. 2001;33(5): Lee SL, DuBois JJ, Montes-Garces RG, et al. Surgical management of chronic unremitting constipation and fecal incontinence associated with megarectum: A preliminary report. J Pediatr Surg. 2002;37(1): Siminas S, Losty PD. Current Surgical Management of Pediatric Idiopathic Constipation: A Systematic Review of Published Studies. Ann Surg Reshef A, Alves-Ferreira P, Zutshi M, et al. Colectomy for slow transit constipation: effective for patients with coexistent obstructed defecation. Int J Colorectal Dis. 2013;28(6): Koppen IJN, Kuizenga-Wessel S, Lu PL, et al. Surgical decision-making in the management of children with intractable functional constipation: What are we doing and are we doing it right? J Pediatr Surg doi: /j.jpedsurg [Epub ahead of print] 14. van der Wilt AA, van Wunnik BPW, Sturkenboom R, et al. Sacral neuromodulation in children and adolescents with chronic constipation refractory to conservative treatment. Int J Colorectal Dis Martin MJ, Steele SR, Noel JM, et al. Total colonic manometry as a guide for surgical Surgical management 6. Kaugars AS, Silverman A, Kinservik M, et al. Families perspectives on the effect of constipation and fecal incontinence on quality of life. J Pediatr Gastroenterol Nutr. 2010;51(6): Christison-Lagay ER, Rodriguez L, Kurtz M, et al. Antegrade colonic enemas and intestinal diversion are highly effective in the management of children with intractable constipation. J Pediatr Surg. 2010;45(1):213 9; discussion Wood RJ, Yacob D, Levitt MA. Surgical options for the management of severe functional constipation in children. Curr Opin Pediatr Villarreal J, Sood M, Zangen T, et al. Colonic diversion for intractable constipation in management of functional colonic obstruction: Preliminary results. J Pediatr Surg. 2001;36(12): Levitt MA, Martin CA, Falcone RA, et al. Transanal rectosigmoid resection for severe intractable idiopathic constipation. J Pediatr Surg. 2009;44(6): ; discussion Godbole PP, Pinfield A, Stringer MD. Idiopathic

18 megarectum in children. Eur J Pediatr Surg Off J Austrian Assoc Pediatr Surg. [et al] = Zeitschrift für Kinderchirurgie. 2001;11(1): Rasquin A, Di Lorenzo C, Forbes D, et al. Childhood functional gastrointestinal disorders: child/adolescent. Gastroenterology. 2006;130(5): Clavien PA, Barkun J, de Oliveira ML, et al. The Clavien-Dindo classification of surgical complications: five-year experience. Ann Surg. 2009;250(2): Bouchoucha M, Devroede G, Arhan P, et al. What is the meaning of colorectal transit time measurement? Dis Colon Rectum. 1992;35(8): Wessel S, Koppen IJN, Wiklendt L, et al. Characterizing colonic motility in children with chronic intractable constipation: a look beyond high-amplitude propagating sequences. Neurogastroenterol Motil Bray L, Sanders C. Preparing children and young people for stoma surgery. Paediatr Nurs. 2006;18(4): Sugarman I. Treatment of severe childhood constipation with restorative proctocolectomy: the surgeon s view. Arch Dis Child. 2010;95(11): Loos MJ, Scheltinga MR, Mulders LG, et al. The Pfannenstiel incision as a source of chronic pain. Obstet Gynecol Apr;111(4): Luijendijk RW, Jeekel J, Storm RK, et al. The low transverse Pfannenstiel incision and the prevalence of incisional hernia and nerve entrapment. Ann Surg. 1997;225(4): Formijne Jonkers HA, Draaisma WA, Roskott AM, et al. Early complications after stoma formation: a prospective cohort study in 100 patients with 1-year follow-up. Int J Colorectal Dis. 2012;27(8):

19 Surgical management

20 Attachment 1 Parental Questionnaire General information Name... Subject number:... Date filling in questionnaire: d d m m m j j j j Questionnaire answered by:... father mother other:.. School absence 1 Has your child s constipation resulted in school absence in the past? Yes No 2 Has your child s constipation resulted in school absence in the last month? Yes No 3 If yes, how many days at school missed your child due to constipation: Last month:... days At the time of severe constipation:...days Current symptoms 4 What is your child s defecation frequency (times per week): Appearance of stools: Normal Firm & marble-like Large amount of soft feces 6 Consistency of stools: Normal Hard Soft Watery 7 Straining during defecation: Yes No Sometimes 8 Pain during defecation: Yes No Sometimes 254

21 9 History of large diameter stools which may obstruct the toilet: Yes No 10 Abdominal pain: Yes No 11 Fecal incontinence: Yes... times per week No 12 History of retentive posturing or excessive volitional stool retention: Yes No Sometimes Current treatment 13 Current treatment for constipation: Laxatives Enema Rectal irrigation Kleanprep nasogastric tube/peg Other 14 Score the following on a scale of 0-10: Severity of current symptoms: Severity of symptoms during the most severe episode in the past:.. Surgery 15 How did you experience the first days post-surgery? Easy As expected Harder than anticipated Very hard 16 Are you satisfied with the results of the surgical intervention(s)? Yes No Surgical management 17 If you could choose again, would you opt for this surgical intervention(s) again? 11 Yes No 18 Grade your satisfaction of the surgical intervention(s) on a scale 1 10 (1: I am very unsatisfied, 10: I am very satisfied) Grade:

22 Attachment 2 Surgical interventions Study patient Primary surgery Surgical conversion 1 Surgical conversion 2 Surgical conversion 3 Surgical conversion 4 Infections a Complications Current surgical status Current symptoms 1 SubT + IRA A ^ 2 Sig + CRA A BM <3/wk, AP 3 Sig + CRA + CHAIT A - 4 b Sig + CRA A BM <3/wk 5 Sig + CRA A FI 6 Sig + C R desc colon + C Yes - C AP 7 C Yes - C - 8 C C AP 9 C C AP 10 C C AP 11 C C ^ 12 C High-output stoma C - 13 C SubT + IRA A ^ 14 C Sig + CHAIT SubT + I I AP 15 C I SubT + I I AP 16 C R desc colon + C I ACNES I AP 17 I Yes - I AP 18 I I AP 19 I I AP 20 I Ileus I AP 21 I I AP 22 I Ileus I AP 23 I SubT + IRA Ileus A - 24 I SubT + IRA Re-feeding syndrome A ^ 25 I SubT + IRA Yes Diversion colitis, ACNES A AP 26 I SubT + IRA Yes - A BM <3/wk, AP 27 I SubT + IRA A AP, FI 28 I SubT + IRA Ileus A BM <3/wk, AP 29 b I SubT + IRA A BM <3/wk 30 I SubT + IRA ACNES A BM <3/wk, AP 31 I SubT + IRA A AP, FI 32 I SubT + IRA Ileus A BM <3/wk, AP 33 I SubT + IRA I Ileus I AP 34 I SubT + IRA I A - - High-output stoma A AP 35 b I SubT + IRA I A I Yes Anastomotic leakage, ACNES I - 36 I R rectosig colon + I A A - 37 I T + IRA A - 256

23 << A= stoma closure with anastomosis, AP=abdominal pain, BM= bowel movement, C= colostomy, CRA=colorectal anastomosis, Desc= descending, FI= fecal incontinence, IRA=ileo-rectal anastomosis, R= resection of, Rectosig= rectosigmoid, Sig= sigmoid resection, SubT= subtotal colectomy, I= ileostomy, T= total colectomy, wk=week, - = not applicable, ^=did not participate in telephone survey. a 6 abscess on rectal stump; 7 parastomal infiltrate; 17 parastomal abscess; 25 wound infection and intra-abdominal abscess; 26 intra-abdominal abscess; 35 peritonitis due to anastomotic leakage. b Parents were not satisfied with the results of surgical intervention; patient 35 would not undergo the same surgical procedures if necessary. Surgical management