The burden of surgery and postoperative complications in children with inflammatory bowel disease.

BACKGROUND: Pediatric inflammatory bowel disease (IBD) may be associated with a higher burden of surgery and postoperative complications. This study aimed to measure the burden in pediatric IBD over a 20-year period in a large tertiary referral center. METHODS: A retrospective review was conducted of children diagnosed with IBD between 1996 and 2015, with a focus upon operative intervention (excluding endoscopy) and postoperative outcomes. RESULTS: Of 786 IBD patients, 121/581 (20.8%) with Crohn's disease (CD) and 22/205 (10.7%) with ulcerative colitis (UC) underwent surgery during the study period. When comparing 10-year epochs for CD, median time from diagnosis to intervention decreased from 34 months to 3 months (P < 0.0001). Postoperative complications occurred in 16/121 (13%) CD patients (bowel obstruction: 10, anastomotic stricture: 4, stomal issues: 4, anastomotic leak: 1). Within the UC cohort, the median time from diagnosis to intervention decreased from 62 months to 6 months (P = 0.0019). Postoperative complications occurred in 9/22 (41%) UC patients (bowel obstruction: 7, stomal issues: 3, anastomotic stricture: 1). Compared with CD, complications were more frequent in UC patients (P = 0.004). CONCLUSION: Surgery and postoperative complications are common in pediatric IBD. The timing of intervention has trended towards earlier operations in both CD and UC. LEVEL OF EVIDENCE: Treatment study-level III (retrospective comparative study).

Achalasia: Outcome in children.

BACKGROUND: Oesophageal achalasia is well-recognized but relatively rare in children, occasionally appearing as the "triple A" syndrome (with adrenal insufficiency and alacrima). Treatment modalities, as in adult practice, are not curative, often needing further interventions and spurring the search for better management. The outcome for syndromic variants is unknown. We sought to define the efficacy of treatments for children with achalasia with and without triple A syndrome. METHODS: We conducted a retrospective analysis of presentation and outcomes for 42 children with achalasia presenting over three decades to a major pediatric referral center. Long term impact of the diagnosis was assessed by questionnaire. RESULTS: We identified 42 children including six with triple A syndrome. The median overall age at diagnosis was 10.8 years and median follow-up 1593 days. Initial Heller myotomy in 17 required further interventions in 11 (65%), while initial treatment with botulinum toxin (n = 20) was ultimately followed by myotomy in 17 (85%). Ten out of 35 patients who underwent myotomy required a repeat myotomy (29%). Patients with triple A syndrome developed symptoms earlier, but had delayed diagnosis, were more underweight at diagnosis and at last follow up. Questionnaire results suggested a significant long term deleterious impact on the quality of life of children and their families. CONCLUSION: Many children with achalasia relapse after initial treatment, undergoing multiple, different procedures, despite which symptoms persist and impact on quality of life. Symptoms develop earlier in patients with triple A syndrome, but the diagnosis is delayed and this has substantial nutritional impact.

Loss of response to long-term infliximab therapy in children with Crohn's disease.

Secondary loss of response (LoR) often precludes further use of infliximab in children with Crohn's disease. Immunomodulators may reduce the incidence of LoR but their combination with infliximab presents safety concerns. We aimed to determine the long-term durability of infliximab response in paediatric Crohn's, effect of immunomodulators on LoR, and secondarily the effect of infliximab on growth. We retrospectively audited patients on maintenance infliximab at a single centre. Data included height and weight, Paediatric Crohn's Disease Activity Index (PCDAI), and immunomodulator use. 71 children (32% female, mean age 14.4 years) had been commenced on maintenance infliximab before July 2011. 89% had been on immunomodulators concurrently with infliximab. LoR occurred in 20 (28%), with a median time to LoR of 4.31 years. LoR was significantly increased in children who did not enter remission (PCDAI ≤ 10) after induction (p < 0.05). LoR occurred more frequently in the 72% who ceased immunomodulators, but this failed to reach statistical significance (p = 0.300). Height and weight SDS improved significantly on infliximab. Infliximab is a durable long-term therapy for paediatric Crohn's refractory to conventional therapy. A large-magnitude increase in the rate of loss of response after immunomodulator cessation was not observed.