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To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/0002-8703(95)90147-7 Byline: Girish S. Shirali (a), E. O'Brian Smith (b), Tal Geva (a)(c) Abstract: Clinical and morphometric features such as ventricular septal defect (VSD) size and location may determine outcome in infants with an isolated VSD. However, no currently available data allow quantitative estimation of the probability of spontaneous closure or surgery in individual patients. To identify independent predictors of outcome and to quantitate the probability of spontaneous closure and surgery in patients with isolated VSD, we studied 156 consecutive infants who had a diagnosis of an isolated VSD between January 1, 1988, and December 31, 1990, and who were subsequently monitored for 28.5 [+ or -] 15 months. Of the 149 patients with membranous (n = 100) and muscular (n = 49) defects who were studied, 46 (31%) patients had spontaneous closure, and an additional 37 (25%) patients underwent surgical repair. Univariate analysis identified defect cross-sectional area indexed to body surface area, location in the muscular septum, presence of Down syndrome, and in membranous defects the presence of aneurysmal tissue as potential predictors of spontaneous closure or surgery. Multiple logistic regression analysis with these candidate variables identified indexed defect cross-sectional area as an independent predictor of spontaneous closure and surgery (p < 0.001). An inverse nonlinear relationship was seen between indexed VSD area and the probability of spontaneous closure (probability = (1 + e[-1.74 + 4.57CSA]).sup.-1 and a positive nonlinear relationship between indexed VSD area and the probability of surgery (probability = (1 + e[3.39 - 2.31CSA]).sup.-1). Muscular defects were more likely to close spontaneously than membranous defects (odds ratio 2.6, 95% CL = 1.01 - 6.8, p = 0.04). Aneurysmal tissue formation and presence of Down syndrome did not predict outcome after controlling for defect size. Thus echocardiographic measurement of VSD cross-sectional area enables quantitation of probability of spontaneous closure and surgery in individual infants with an isolated VSD. Author Affiliation: (a) Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children's Hospital and the Departments of Pediatrics and Pathology, Baylor College of Medicine, Houston, Tex., USA (b) United States Department of Agriculture/Agricultural Research Service Children's Nutrition Research Center, Texas Children's Hospital and the Departments of Pediatrics and Pathology, Baylor College of Medicine, Houston, Tex., USA (c) Department of Pathology, Texas Children's Hospital and the Departments of Pediatrics and Pathology, Baylor College of Medicine, Houston, Tex., USA Article History: Received 14 December 1994; Accepted 8 May 1995

Byline: Maria Menezes (1), Prem Puri (1) Keywords: Hirschsprung's disease; Enterocolitis; Risk factors; Bowel function Abstract: Enterocolitis remains the most serious complication of Hirschsprung's disease (HD). The purpose of this study was to evaluate the risk factors in the development of enterocolitis and the long-term outcome in these patients. The hospital records of 259 consecutive patients with HD during 1975--2003 were examined. The data was analysed for age at presentation, associated anomalies, level of aganglionosis, clinical features, number of episodes of enterocolitis, type of pullthrough, necessity for post-pullthrough sphincterectomy. Follow up was carried out by personal interviews and interviews over the telephone with patients/parents. Enterocolitis was diagnosed on the basis of clinical features of diarrhoea, pyrexia, abdominal distention and vomiting. Of the 259 patients with HD, 74 patients (28.5%) were found to have enterocolitis. Out of 39 patients with Down's syndrome and HD, 19 (48%) had enterocolitis. Fifteen (20%) patients had other associated anomalies. Fifty-six patients (75.6%) were male and 18 (24.3%) were female. In 30 patients enterocolitis was the presenting feature in the neonatal period, 22 of which presented in the first 2 weeks of life. Fifty-six patients (75.6%) had rectosigmoid disease and 18 (24.3%) had long segment disease or total colonic aganglionosis. Eighteen (24.3%) had only preoperative enterocolitis and 31(41.8%) had only postoperative enterocolitis. Twenty-five (33.7%) had both pre- and post-operative enterocolitis. Twenty (27%) patients had more than 2 episodes of enterocolitis. Various pullthrough procedures were performed. Twenty-six patients (35.1%) required internal sphinctermyectomy to treat the enterocolitis. At the time of follow-up, 16 patients were lost to follow-up. Of the remaining 58 patients, 3 patients died, 2 due to enterocolitis and 1 due to sepsis. Six patients remained with a stoma. Twenty-two patients were continent and were stooling normally, but 14 of these were on laxatives for several years after pullthrough procedure. The mean age at achieving full continence was 4.95 years in the 22 patients with normal bowel function. Nineteen patients are still on laxatives and 8 patients are soiling. Eight patients continue to have multiple recurrent episodes of enterocolitis at follow-up. Down's syndrome is an important risk factor in the development of enterocolitis. The majority of patients with enterocolitis complicating HD continue to have disturbances of bowel function many years after surgery for HD. Author Affiliation: (1) Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, Ireland Article History: Registration Date: 11/01/2006 Accepted Date: 09/01/2006 Online Date: 04/02/2006

Byline: Antonino Morabito (1), Anupam Lall (1), Sobbia Gull (2), Amar Mohee (3), Adrian Bianchi (1) Keywords: Hirschsprung's disease; Aganglionosis; Enterocolitis; Continence; Down's syndrome Abstract: Hirschsprung's disease (HD) in Down's syndrome (DS) patients is stated to have a worse outcome than HD alone. In our study we reviewed the immediate and long-term outcomes of these children and questioned whether DS should influence the operative management. Data were collected on all children with HD (including total colonic aganglionosis), between January 1990 and December 2000. They were divided into two groups based on the presence or absence of Trisomy 21 and compared retrospectively. In this time period we treated 173 children with HD 17 of these had DS. Both the groups were comparable in their mean gestational age, birth weight and presentation except that the DS group had a significantly higher overall incidence of pre and/or postoperative enterocolitis. A tota1 of 164 children underwent a Swenson pull-through and 9 had a Soave's procedure. Follow-up ranged from 1 to 10 years. Continence assessed using the Wingspread scoring system in children over the age of 4 years showed no significant difference. Although children with both HD and DS are predisposed to complications and required a more cautious management, long-term outcome in terms of continence was not significantly worse than in HD alone. Thus the co-existence of DS should not influence the decision to offer these children and their parents the choice of definitive repair. Author Affiliation: (1) Department of Paediatric Surgery, Royal Manchester Children's Hospital, Hospital Road, M27 4HA, Manchester, UK (2) St George's Hospital, Blackshaw Road, London, UK (3) University of Manchester Medical School, Manchester, UK Article History: Registration Date: 28/11/2005 Accepted Date: 20/05/2005 Online Date: 14/12/2005