The use of stomas in the early management of Hirschsprung’s Disease: Findings of a National, Prospective Cohort Study

Background Primary pull-through without a stoma has become preferred practice in managing Hirschsprung’s disease (HD). The aims of this study were to establish stoma rate and identify factors associated with stoma formation in a population-based cohort in the UK and Ireland. Methods Live-born infants with HD were prospectively identified in all 28 specialist paediatric surgical units in the UK and Ireland between October 2010 to September 2012. Method of colonic decompression was recorded and multivariable logistic regression was used to identify factors associated with stoma formation. Results 305 infants with HD were identified. Rectal washouts were initially used in 86% (263) with a defunctioning stoma formed as the primary management in 13% (39). Ultimately, 36% (111) required a stoma prior to definitive surgery. Compared to infants managed with rectal washouts alone; infants managed with a stoma were more likely to have a right-sided transition zone, Down (or another) syndrome, and HD diagnosis established more than 28 days after presentation. Conclusions Although rectal washouts are commonly employed, a stoma prior to definitive surgery was required in 36% of infants in a national cohort. Delayed diagnosis, right-sided disease and presence of other anomalies are associated with stoma formation.</p

Hirschsprung's disease in the UK and Ireland: incidence and anomalies.

<h4>Objectives</h4> <p>To describe clinical characteristics and pre-operative management of a national cohort of infants with Hirschsprung’s disease (HD).</p> <h4>Design</h4> <p>Population based cohort study of all live-born infants with HD born in the UK and Ireland from October 2010 to September 2012.</p> <h4>Setting</h4> <p>All 28 paediatric surgical centres in the UK and Ireland.</p> <h4>Participants</h4> <p>305 infants presenting before 6-months of age with histologically-proven HD.</p> <h4>Main outcome measures</h4> <p>Incidence, clinical characteristics including gestational age, birth weight, gender, associated anomalies; age and clinical features at presentation; and use of rectal washouts or stoma.</p> <h4>Results</h4> <p>The incidence of HD in the UK and Ireland was 1.8 per 10,000 live births (95% confidence interval 1.5 to 1.9). Male:female ratio was 3.3:1. An associated anomaly was identified in 23% (69), with 15% (47) having a recognisable syndrome. The proportion of infants who presented and were diagnosed in the neonatal period was 91.5% (279) and 83.9% (256) respectively. 23.9% (73) and 44.2% (135) passed meconium within 24 and 48 hours of birth. 81% (246) first presented to a hospital without tertiary paediatric surgical services, necessitating inter-hospital transfer. Initial colonic decompression was by rectal washouts in 86.2% (263); and by defunctioning stoma in 12.8% (39). Subsequently, 27.4% (72) of infants failed management with rectal washouts and required a delayed stoma, resulting in 36.4% (111) of infants having a stoma.</p> <h4>Conclusions</h4> <p>In this population-based cohort, presentation outside the neonatal period was rare. Nearly half infants with HD passed meconium within 48 hours of birth, and over one third were managed with a stoma.</p

The use of stomas in the early management of Hirschsprung’s Disease: Findings of a National, Prospective Cohort Study

Background Primary pull-through without a stoma has become preferred practice in managing Hirschsprung’s disease (HD). The aims of this study were to establish stoma rate and identify factors associated with stoma formation in a population-based cohort in the UK and Ireland. Methods Live-born infants with HD were prospectively identified in all 28 specialist paediatric surgical units in the UK and Ireland between October 2010 to September 2012. Method of colonic decompression was recorded and multivariable logistic regression was used to identify factors associated with stoma formation. Results 305 infants with HD were identified. Rectal washouts were initially used in 86% (263) with a defunctioning stoma formed as the primary management in 13% (39). Ultimately, 36% (111) required a stoma prior to definitive surgery. Compared to infants managed with rectal washouts alone; infants managed with a stoma were more likely to have a right-sided transition zone, Down (or another) syndrome, and HD diagnosis established more than 28 days after presentation. Conclusions Although rectal washouts are commonly employed, a stoma prior to definitive surgery was required in 36% of infants in a national cohort. Delayed diagnosis, right-sided disease and presence of other anomalies are associated with stoma formation.</p

Hirschsprung's disease in the UK and Ireland: incidence and anomalies.

Objectives To describe clinical characteristics and pre-operative management of a national cohort of infants with Hirschsprung’s disease (HD). Design Population based cohort study of all live-born infants with HD born in the UK and Ireland from October 2010 to September 2012. Setting All 28 paediatric surgical centres in the UK and Ireland. Participants 305 infants presenting before 6-months of age with histologically-proven HD. Main outcome measures Incidence, clinical characteristics including gestational age, birth weight, gender, associated anomalies; age and clinical features at presentation; and use of rectal washouts or stoma. Results The incidence of HD in the UK and Ireland was 1.8 per 10,000 live births (95% confidence interval 1.5 to 1.9). Male:female ratio was 3.3:1. An associated anomaly was identified in 23% (69), with 15% (47) having a recognisable syndrome. The proportion of infants who presented and were diagnosed in the neonatal period was 91.5% (279) and 83.9% (256) respectively. 23.9% (73) and 44.2% (135) passed meconium within 24 and 48 hours of birth. 81% (246) first presented to a hospital without tertiary paediatric surgical services, necessitating inter-hospital transfer. Initial colonic decompression was by rectal washouts in 86.2% (263); and by defunctioning stoma in 12.8% (39). Subsequently, 27.4% (72) of infants failed management with rectal washouts and required a delayed stoma, resulting in 36.4% (111) of infants having a stoma. Conclusions In this population-based cohort, presentation outside the neonatal period was rare. Nearly half infants with HD passed meconium within 48 hours of birth, and over one third were managed with a stoma.</p

Hirschsprung's disease in the UK and Ireland: incidence and anomalies.

Objectives To describe clinical characteristics and pre-operative management of a national cohort of infants with Hirschsprung’s disease (HD). Design Population based cohort study of all live-born infants with HD born in the UK and Ireland from October 2010 to September 2012. Setting All 28 paediatric surgical centres in the UK and Ireland. Participants 305 infants presenting before 6-months of age with histologically-proven HD. Main outcome measures Incidence, clinical characteristics including gestational age, birth weight, gender, associated anomalies; age and clinical features at presentation; and use of rectal washouts or stoma. Results The incidence of HD in the UK and Ireland was 1.8 per 10,000 live births (95% confidence interval 1.5 to 1.9). Male:female ratio was 3.3:1. An associated anomaly was identified in 23% (69), with 15% (47) having a recognisable syndrome. The proportion of infants who presented and were diagnosed in the neonatal period was 91.5% (279) and 83.9% (256) respectively. 23.9% (73) and 44.2% (135) passed meconium within 24 and 48 hours of birth. 81% (246) first presented to a hospital without tertiary paediatric surgical services, necessitating inter-hospital transfer. Initial colonic decompression was by rectal washouts in 86.2% (263); and by defunctioning stoma in 12.8% (39). Subsequently, 27.4% (72) of infants failed management with rectal washouts and required a delayed stoma, resulting in 36.4% (111) of infants having a stoma. Conclusions In this population-based cohort, presentation outside the neonatal period was rare. Nearly half infants with HD passed meconium within 48 hours of birth, and over one third were managed with a stoma.</p

Outcomes at five to eight years of age for children with Hirschsprung’s disease

Objective This study describes core outcomes of Hirschsprung’s disease (HD) in a UK-wide cohort of primary school-aged children. Design A prospective cohort study conducted from 1 October 2010 to 30 September 2012. Outcomes data were collected from parents and clinicians when children were 5–8 years of age, and combined with data collected at birth, and 28 days and 1 year post diagnosis. Setting All 28 UK and Irish paediatric surgical centres. Participants Children with histologically proven HD diagnosed at <6 months of age. Main outcome measures NETS1HD core outcomes. Results Data were returned for 239 (78%) of 305 children. Twelve children (5%) died prior to 5 years of age. Of the 227 surviving children, 30 (13%) had a stoma and 21 (9%) were incontinent of urine. Of the 197 children without a stoma, 155 (79%) maintained bowel movements without enemas/washouts, while 124 (63%) reported faecal incontinence. Of the 214 surviving children who had undergone a pull-through operation, 95 (44%) underwent ≥1 unplanned reoperation. 89 unplanned reoperations (27%) were major/complex. Of the 83 children with returned PedsQL scores, 37 (49%) had quality of life scores, and 31 (42%) had psychological well-being scores, that were ≥1 SD lower than the reference population mean for children without HD. Conclusion This study gives a realistic picture of population outcomes of HD in primary school-aged children in the UK/Ireland. The high rates of faecal incontinence, unplanned procedures and low quality of life scores are sobering. Ensuring clinicians address the bladder, bowel and psychological problems experienced by children should be a priority