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 a...
| Main Authors: | , , , , |
|---|---|
| Format: | Journal article |
| Language: | English |
| Published: |
BMJ Publishing Group
2017
|
| _version_ | 1797073116329934848 |
|---|---|
| author | Bradnock, T Knight, M Kenny, S Nair, M Walker, G |
| author_facet | Bradnock, T Knight, M Kenny, S Nair, M Walker, G |
| author_sort | Bradnock, T |
| collection | OXFORD |
| description | <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> |
| first_indexed | 2024-03-06T23:17:24Z |
| format | Journal article |
| id | oxford-uuid:6798027b-2348-4102-b3ab-f9a269730930 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-06T23:17:24Z |
| publishDate | 2017 |
| publisher | BMJ Publishing Group |
| record_format | dspace |
| spelling | oxford-uuid:6798027b-2348-4102-b3ab-f9a2697309302022-03-26T18:39:22ZHirschsprung's disease in the UK and Ireland: incidence and anomalies.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:6798027b-2348-4102-b3ab-f9a269730930EnglishSymplectic Elements at OxfordBMJ Publishing Group2017Bradnock, TKnight, MKenny, SNair, MWalker, G <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> |
| spellingShingle | Bradnock, T Knight, M Kenny, S Nair, M Walker, G Hirschsprung's disease in the UK and Ireland: incidence and anomalies. |
| title | Hirschsprung's disease in the UK and Ireland: incidence and anomalies. |
| title_full | Hirschsprung's disease in the UK and Ireland: incidence and anomalies. |
| title_fullStr | Hirschsprung's disease in the UK and Ireland: incidence and anomalies. |
| title_full_unstemmed | Hirschsprung's disease in the UK and Ireland: incidence and anomalies. |
| title_short | Hirschsprung's disease in the UK and Ireland: incidence and anomalies. |
| title_sort | hirschsprung s disease in the uk and ireland incidence and anomalies |
| work_keys_str_mv | AT bradnockt hirschsprungsdiseaseintheukandirelandincidenceandanomalies AT knightm hirschsprungsdiseaseintheukandirelandincidenceandanomalies AT kennys hirschsprungsdiseaseintheukandirelandincidenceandanomalies AT nairm hirschsprungsdiseaseintheukandirelandincidenceandanomalies AT walkerg hirschsprungsdiseaseintheukandirelandincidenceandanomalies |