Surgical Management of Crohn Disease in Children: Guidelines From the Paediatric IBD Porto Group of ESPGHAN.

The incidence of Crohn disease (CD) has been increasing and surgery needs to be contemplated in a substantial number of cases. The relevant advent of biological treatment has changed but not eliminated the need for surgery in many patients. Despite previous publications on the indications for surgery in CD, there was a need for a comprehensive review of existing evidence on the role of elective surgery and options in pediatric patients affected with CD. We present an expert opinion and critical review of the literature to provide evidence-based guidance to manage these patients. Indications, surgical options, risk factors, and medications in pre- and perioperative period are reviewed in the light of available evidence. Risks and benefits of surgical options are addressed. An algorithm is proposed for the management of postsurgery monitoring, timing for follow-up endoscopy, and treatment options.

Upper Gastrointestinal Screening of Polyp Load in Children With Familial Adenomatous Polyposis: Is It Required?

Objectives and study: Patients with familial adenomatous polyposis (FAP) have a propensity to form not only large bowel polyps but also upper gastrointestinal (GI) polyps with malignant potential. International guidance suggests that upper GI screening need not begin until patients are at least into their twenties. It is our experience that patients develop upper GI polyps long before this point which have the potential for malignant change. Methods: A prospective record of all upper GI endoscopies in children (aged 9 to 17) with FAP was kept across a 12-year period by 1 surgeon in our center. For each scope performed, we recorded the location, histology, and treatment of upper GI polyps. Results: Twent-eight patients aged 9 to 17 underwent a total of 48 esophagogastroduodenoscopies across a 12-year period. Thirty-eight esophagogastroduodenoscopies (79%) identified at least 1 gastric or duodenal polyp in 22 (79%) patients; 10 (36%) patients had gastric adenomas. Eight (29%) patients showed very high numbers of polyps. All 21 patients who had duodenal polyps had adenomas. None had yet developed malignancy, but 1 patient required extensive polyp excision and one is awaiting endoscopic mucosal resection. Conclusion: Our results demonstrate that young people with FAP are at the risk of developing upper GI polyps long before current guidance suggests screening the upper GI tract. We advocate for screening of the upper GI tract to start along with colonoscopy and happen at the same sitting for pediatric and young adult patients with FAP.

Functional outcomes, quality of life, sexual function, and fertility of adult patients undergoing ileo-anal pouch anastomosis in childhood.

BACKGROUND: We conducted a questionnaire-based study of pouch function, quality of life, sexual function, and fertility among patients who had undergone ileal pouch anal anastomosis (IPAA) in childhood and who are now more than 18 years old. METHODS: A consecutive series of patients were asked to complete the following questionnaires: Pouch function score (PFS), short form 36 (SF-36), International index of erectile function (IIEF) (males), and Female sexual function index (FSFI) (females). Fertility in females was also assessed. Data are quoted as a median (SD). RESULTS: Of 144 patients who had IPAA, 101 were eligible, and 70 responded. Mean age at surgery was 14 years, and mean current age is 26 years. Most patients had either ulcerative colitis or familial adenomatous polyposis. The questionnaire was completed by 38/70 (54%). Median PFS score was 6 (5). SF-36 were lower than previous reports. Median FSFI was 30 (7.6), 84% of possible maximum. Median IIEF was 69, 92% of possible maximum. Successful conception was reported in 5/7 women who had tried. However, there were several miscarriages and two surgical emergencies during pregnancies. CONCLUSIONS: IPAA can be performed in childhood with similar pouch function to reported adult series. Quality of life appears poorer, but sexual function is maintained. There may be an association with adverse events in pregnancy. LEVEL OF EVIDENCE: IV.

Severity of complications following restorative proctocolectomy in children is related to staging not diagnosis.

BACKGROUND: Restorative proctocolectomy (RPC) is performed using a variety of staged procedures for several diseases. Our aim was to assess whether the severity of complications, classified according to Clavien-Dindo, was related to the diagnosis or the procedure. METHODS: A consecutive series of children receiving an ileoanal pouch was prospectively recorded. Complications were scored by two blinded observers. Major complications were Clavien-Dindo ≥3b. Procedures were classified as: colectomy, proctectomy and pouch or proctocolectomy and pouch. Diagnoses were classified as: ulcerative colitis, familial adenomatous polyposis or other: idiopathic constipation, total colonic Hirschsprung's disease, juvenile polyposis, Crohn's colitis, fibrosing colonopathy or necrotising enterocolitis. RESULTS: 128 children underwent 191 procedures: 61 colectomies, 63 proctectomies and 67 proctocolectomies. 84 children had ulcerative colitis, 20 had FAP and 24 had other indications. Major complications were significantly more likely with proctocolectomy (16/67, 24%) than with either colectomy (4/61, 7%) or proctectomy (8/63, 13%), p = 0.01. There was no association between diagnosis and major complications: ulcerative colitis (18/133, 14%), FAP (5/20, 25%), other (5/38, 13%) p = 0.4. There was no increase in major complications following proctectomy if a major complication had occurred during prior colectomy. Overall, 15% of procedures experienced a major complication. 6/9 stoma related complications required operative intervention. CONCLUSIONS: The severity of complications after RPC in children is related to use of a two stage rather than three stage sequence of surgery, not the underlying diagnosis. TYPE OF STUDY: Case control study. LEVEL OF EVIDENCE: Level III.

Does painless rectal bleeding equate to a colonic polyp?

BACKGROUND: It is often stated that if a patient presents with 'painless rectal bleeding' then a rectal polyp is the probable diagnosis. The aim of this study is to review our experience of children undergoing endoscopy to assess if the above statement is correct. METHODS: The senior author keeps a prospective database of every child undergoing flexible sigmoidoscopy or colonoscopy. As part of this database, the symptoms and signs that the patient presents with, specifically abdominal pain, diarrhoea, mucous per rectum and rectal bleeding, are recorded. These results have been analysed specifically to assess whether the opening statement is correct. RESULTS: Between 2000 and 2014, a total of 401 children have undergone flexible sigmoidoscopy (21) or colonoscopy (380) to investigate rectal bleeding. Of these 401 patients, 42 (10.5%) had at least one polyp. Four polyps (9%) occurred in 159 patients with no rectal bleeding during the study period. The remaining 42 polyps (91%) were identified in patients with rectal bleeding. Of these 42 polyps, painless rectal bleeding was the only symptom in 24 (57%). However, 123 patients were endoscoped with painless rectal bleeding alone, giving a polyp rate of 19.5% for this symptom. The polyp pickup rate was increased to 28% if rectal bleeding and mucous per rectum were present; however, only 25 patients had this clinical history. CONCLUSION: We confirm that the most common symptom of rectal polyps is painless rectal bleeding. However, only one in five patients with this clinical history has a rectal polyp at endoscopy. The polyp pickup rate at endoscopy is greater (28%) when a history of both rectal bleeding and mucous per rectumispresent. This information can be used to counsel parents preoperatively.

Improving outcomes in pilonidal sinus disease.

BACKGROUND: Optimal surgical treatment for pilonidal sinus disease remains controversial. Studies in children are uncommon with overall high postoperative recurrence and infection rates (20%). Our unit adopted a technique for excision of the pilonidal sinus with primary midline closure using a full-thickness flap. We present our results of this technique in the pediatric age group. METHODS: Single-center retrospective review and telephone follow-up was performed, including all children undergoing pilonidal sinus excision from 2005 to 2014 using the same operative technique and preoperative/postoperative care. RESULTS: 19 children were identified. Median age was 15years, and 47% were male. All had previously infected sinuses, with 5 requiring abscess drainage. Median follow-up was 13months (3.5-67months). Histology confirmed pilonidal sinus in 18/19 cases (one developmental malformation excluded from subsequent analysis). Recurrence requiring further surgery occurred in 4/18 (22%) patients at a median of 9months (range 8-36), wound dehiscence in 8/18 (44%), and wound infection in 5/18 (28%) cases. Laser hair removal of the natal cleft was performed in 7/18 (39%) patients with no further recurrence. CONCLUSION: This is the first report of this technique in children which showed a higher incidence of complications than a comparable adult study. Our study underlines the importance of accurate long-term outcomes when introducing new techniques to evaluate operation-associated morbidity. Our preliminary results of laser hair removal are promising but need to be evaluated with a prospective study.

Nurse-assisted PEG in children.

The procedures reviewed in this article comprised 83 PEG insertions and 39 PEG changes in children aged six weeks to 19 years (median three years) with weights between 4.7 kg and 80 kg (median 13.5 kg). The complication rate was lower than previously reported paediatric series performed exclusively by medical practitioners. Four children required open conversion and one gastro-colic fistula was discovered five months later.

Investigating painless rectal bleeding--is there scope for improvement?

INTRODUCTION: Most patients with recurrent painless rectal bleeding warrant endoscopic examination of the lower gastrointestinal tract. This is often limited to rectum or distal colon. The purpose of this study was to compare the diagnostic yield of limited colorectal endoscopy with total colonoscopy in children with recurrent painless rectal bleeding. METHODS: Retrospective review of 314 patients aged 1 to 15 years who underwent colorectal endoscopy for recurrent rectal bleeding. Two hundred six underwent total colonoscopy, whereas 108 patients had endoscopy limited to the rectum in 22, sigmoid colon in 34, descending colon in 43, and transverse colon in 9. Diagnoses were recorded according to the endoscopic and histological findings. RESULTS: After total colonoscopy, a diagnosis was established in 70% of children compared with 35% of those undergoing limited endoscopy (P < .001). This difference was most noticeable in children with inflammatory bowel disease. Juvenile polyps were diagnosed with similar frequency in both groups, reflecting the rectosigmoid distribution of most polyps. CONCLUSIONS: For children presenting with painless rectal bleeding, a diagnosis can be established in the majority if total colonoscopy is performed. This is particularly true for inflammatory bowel disease. Unless a rectal polyp is suspected, full colonoscopy should be planned.

Endoscopic rectal sparing in children with untreated ulcerative colitis.

BACKGROUND: Ulcerative colitis (UC) typically is associated with a confluent proctitis, whereas rectal sparing may be seen in large bowel Crohn disease (CD). A few studies have reported rectal sparing in UC and suggested that this might indicate a more severe form of the disease. This study aimed to determine the prevalence and prognostic significance of rectal sparing in children with newly diagnosed, untreated UC. METHODS: The records of all children with untreated UC presenting to a regional pediatric gastroenterology service between January 1996 and December 2001 were retrospectively reviewed. Patients were divided into two groups according to the endoscopic appearance of the rectum: Group 1 (proctitis) and Group 2 (rectal sparing). Clinical features, intractability index (duration of active disease as a proportion of length of follow-up), response to treatment, relapse index (number of recurrences per year), and the need for surgery were compared. RESULTS: Thirty children with untreated UC were identified. Seven (23%) had rectal sparing at initial endoscopy, but disease distribution was otherwise similar in both groups. Presenting symptoms were similar in those with and without rectal sparing. In Group 1, 20 (87%) children achieved remission with initial medical treatment, compared with 3 (43%) in Group 2 (P < 0.05). The intractability index was higher in children with rectal sparing, but the difference was not statistically significant (P = 0.22). During a median follow-up period of 2 years, one (4%) child in Group 1 and two (29%) children in Group 2 experienced primary sclerosing cholangitis, and two (29%) children with rectal sparing required colectomy, compared with none in Group 1. CONCLUSIONS: Endoscopic rectal sparing was seen in 23% of children with newly diagnosed, untreated UC, but this feature did not correlate with presenting symptoms. However, the presence of rectal sparing may indicate more aggressive disease that is less responsive to medical treatment.