Prevalence of Hirschsprung’s disease in premature infants: a systematic review

Duess, Johannes W.; Hofmann, Alejandro D.; Puri, Prem

doi:10.1007/s00383-014-3540-8

Cited by 50 publications

(31 citation statements)

References 32 publications

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“…23 The prevalence of HSCR in premature infants in a systemic review extending from 2000 to 2013 was 4% to 19.4% (overall prevalence, 14%). 27 Preterm birth may have many causes, for example, preeclampsia or congenital malformations. Maternal preeclampsia was not overrepresented among the cases' mothers in this study.…”

Section: Figurementioning

confidence: 99%

Maternal Risk Factors and Perinatal Characteristics for Hirschsprung Disease

Granström¹,

Svenningsson²,

Hagel

et al. 2016

Pediatrics

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Section: Figurementioning

confidence: 99%

Maternal Risk Factors and Perinatal Characteristics for Hirschsprung Disease

Granström¹,

Svenningsson²,

Hagel

et al. 2016

Pediatrics

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“…In our earlier study, although 12.2% of babies weighed under 2.5 kg at birth, a scored gestational age of 37 weeks could only be established in 1.9%, illustrating its low prevalence in this group. More recent reports 9,10 indicate a rising prevalence in premature babies.…”

Section: Introductionmentioning

confidence: 97%

Hirschsprung disease: current perspectives

Moore¹

2016

OAS

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Hirschsprung disease is a complex congenital condition of the intestine, which is recognized as being of genetic origin and results from a disturbance of the normal development of the enteric nervous system. As a result, aganglionosis of the distal bowel occurs. It is the most common cause of a low intestinal obstruction in the neonate as well as older children. Occurring as an isolated condition in 70% of cases, it may be associated with other associated congenital abnormalities as well as a number of syndromic phenotypes. A number of distinct genetic sites have been identified in these syndromic phenotypes, which identify potential underlying genetic associations of the disease and indicate the probable gene-gene interaction in its pathogenesis. This review looks at the prevalence, congenital associations, and possible genetic factors influencing the development of Hirschsprung disease. Diagnostic dilemmas, surgical management, potential postsurgical complications, and outcomes are also explored.

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“…[18][19][20] To date, there are very few prospective, population-based observational studies of HD anywhere in the world 1 7 and none that provide representative data in a cohort of children born during a short time period. The aims of this study were to describe the incidence, clinical characteristics and management prior to definitive surgery in a national cohort of infants with HD in the UK and Ireland.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2017

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ObjectivesTo describe clinical characteristics and preoperative management of a national cohort of infants with Hirschsprung's disease (HD).DesignPopulation-based cohort study of all live-born infants with HD born in the UK and Ireland from October 2010 to September 2012.SettingAll 28 paediatric surgical centres in the UK and Ireland.Participants305 infants presenting before 6 months of age with histologically proven HD.Main outcome measuresIncidence, clinical characteristics including gestational age, birth weight, gender, associated anomalies; age and clinical features at presentation; and use of rectal washouts or stoma.ResultsThe incidence of HD in the UK and Ireland was 1.8 per 10 000 live births (95% CI 1.5 to 1.9). Male to 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 interhospital 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.ConclusionsIn this population-based cohort, presentation outside the neonatal period was rare. Nearly half of the infants with HD passed meconium within 48 hours of birth and over one third were managed with a stoma.

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Prevalence of Hirschsprung’s disease in premature infants: a systematic review

Abstract: In recent years, higher prevalence of HD has been reported in premature infants compared to previous years. Hirschsprung's disease should be considered in preterm infants presenting with features of intestinal obstruction.

Cited by 50 publications

References 32 publications

Maternal Risk Factors and Perinatal Characteristics for Hirschsprung Disease

Maternal Risk Factors and Perinatal Characteristics for Hirschsprung Disease

Hirschsprung disease: current perspectives

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

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