The mortality of CDH when complete case ascertainment is achieved is unaffected by new therapies. The survival rate is principally determined by the rate of antenatal termination and the incidence of associated anomalies. Reports of improved survival of CDH should be interpreted with caution, as variations in outcome are more likely to be explained by case selection artifact.

ObjectiveSymptoms and clinical course during inflammatory bowel disease (IBD) vary among individuals. Personalised care is therefore essential to effective management, delivered by a strong patient-centred multidisciplinary team, working within a well-designed service. This study aimed to fully rewrite the UK Standards for the healthcare of adults and children with IBD, and to develop an IBD Service Benchmarking Tool to support current and future personalised care models.DesignLed by IBD UK, a national multidisciplinary alliance of patients and nominated representatives from all major stakeholders in IBD care, Standards requirements were defined by survey data collated from 689 patients and 151 healthcare professionals. Standards were drafted and refined over three rounds of modified electronic-Delphi.ResultsConsensus was achieved for 59 Standards covering seven clinical domains; (1) design and delivery of the multidisciplinary IBD service; (2) prediagnostic referral pathways, protocols and timeframes; (3) holistic care of the newly diagnosed patient; (4) flare management to support patient empowerment, self-management and access to specialists where required; (5) surgery including appropriate expertise, preoperative information, psychological support and postoperative care; (6) inpatient medical care delivery (7) and ongoing long-term care in the outpatient department and primary care setting including shared care. Using these patient-centred Standards and informed by the IBD Quality Improvement Project (IBDQIP), this paper presents a national benchmarking framework.ConclusionsThe Standards and Benchmarking Tool provide a framework for healthcare providers and patients to rate the quality of their service. This will recognise excellent care, and promote quality improvement, audit and service development in IBD.

The Malone antegrade enema (MACE) and the caecostomy button (CB) are two methods of achieving colonic lavage in constipated children with faecal soiling. We reviewed our experience with the MACE and CB, aiming to compare results, complications, and outcomes. Between June 1998 and August 2002, 37 children (15 boys) underwent MACE and 12 children (9 boys) underwent CB for idiopathic constipation that had failed conventional treatment. Rectal biopsy was ganglionic in all cases. Mean age at surgery was 9.9 years for the MACE patients and 9.8 years for the CB patients. All children are under continuous review, and mean follow-up is 18 months. Statistical analysis of proportions used Fisher's exact test. Soiling stopped completely in 30 children with MACE and in 9 with CB. Occasional soiling is still present in two children with a CB and in one with MACE. One child with a CB had resumed regular bowel activity, and the CB was removed. MACE failed in 5 (14%) patients because of ineffective colonic lavage, and in one patient (3%) the appendix was replaced by a CB because of perforation of the appendicostomy. CB failed in one patient (8%) because of faecal leak around the button; the child was subsequently converted to MACE (P = >0.5). Complications requiring operative intervention were seen in 9 (24%) of the 37 patients who underwent MACE and none of the 12 patients who underwent CB (P = 0.09). The main complication requiring surgical intervention was stoma stenosis (11%). Complications not requiring operative intervention were seen in 7 (19%) patients after MACE and 11 (92%) of the 12 patients who underwent CB (P < 0.001). The MACE and CB procedures are reliable and effective with high success rates. The MACE has a higher incidence of complications requiring operative intervention. Conversely, complications not requiring operative intervention are more frequent with CB. CB is a safe and effective alternative to MACE in children with faecal soiling.

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