Long-term outcomes in total colonic aganglionosis: a 32-year experience

Escobar, Mauricio A.; Grosfeld, Jay L.; West, Karen W.; Scherer, L.R.; Rouse, Thomas M.; Engum, Scott A.; Rescorla, Frederick J.

doi:10.1016/j.jpedsurg.2005.03.043

Cited by 88 publications

(88 citation statements)

References 39 publications

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“…48 It has long been recognized as presenting particular problems in diagnosis and management. [48][49][50] Many different surgical techniques have been utilized for TCA, [49][50][51][52] with outcomes mostly related to the type of surgical technique performed. Many surgeons now accept a modification of the Duhamel procedure as the best option in TCA in terms of long-term function.…”

Section: Total Colonic Agangli Onosis (Tca)mentioning

confidence: 99%

“…Many surgeons now accept a modification of the Duhamel procedure as the best option in TCA in terms of long-term function. 51,52 Genetic counseling and prevention Genetic factors are known to be implicated in HSCR pathogenesis. However, the pattern of inheritance remains unclear, and variable Mendelian inheritance with autosomal dominant, recessive, and multigenic patterns being reported.…”

Section: Total Colonic Agangli Onosis (Tca)mentioning

confidence: 99%

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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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Section: Total Colonic Agangli Onosis (Tca)mentioning

confidence: 99%

Section: Total Colonic Agangli Onosis (Tca)mentioning

confidence: 99%

Hirschsprung disease: current perspectives

Moore¹

2016

OAS

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“…Extended HD [28,29] The diagnosis of the long form of HD is often difficult and delayed [30] . The clinical picture may be very variable: (1) intestinal (ileal) neonatal occlusion, sometimes incomplete with or without spontaneous evacuation of meconium, and clinical signs, such as X-ray, could help to diagnose meconium ileus; (2) neonatal enterocolitis [31,32] could be related to extended HD in the absence of prematurity or severe cardiopathy; (3) acute presentation with appendicular or cecal perforation [33] , and (4) severe constipation in neonates, particularly due to bad tolerance with a major effect on growth.…”

Section: Later Diagnosismentioning

confidence: 99%

Diagnosis of Constipation in Children

Sauvat¹

2007

Ann Nestlé [Engl]

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Constipation is a frequent reason for consulting a pediatrician. As a symptom, constipation can be due to various etiologies in terms of frequency, nature and seriousness. Constipation may be of medical or surgical origin or idiopathic. Surgical etiologies are mainly represented by Hirschsprung’s disease. It is the main differential diagnosis even if it is 50 times less frequent than idiopathic or functional constipation. A thorough history and physical examination, including anal examination, are the best way to reach a diagnosis. No other examination is required in most of the cases and all investigations have to be managed according to clinical considerations.

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“…Maladie de Hirschsprung étendue [28,29] Le diagnostic de la forme longue de la maladie de Hirschsprung est souvent difficile et tardif [30] . Le tableau clinique peut être très variable: (1) occlusion intestinale néonatale (iléale) parfois incomplète avec ou sans évacuation spontanée de méconium, et des signes cliniques, tels qu'une radiographie, pourraient aider à diagnostiquer un iléus méconial; (2) entérocolite néonatale [31,32] , qui pourrait être liée à la maladie de Hirschsprung étendue en l'absence de prématurité ou de cardiopathie sévère; (3) présentation aiguë avec perforation appendiculaire ou caecale [33] , et (4) constipation sévère Une entérocolite [34] peut être considérée comme une septicémie d'origine intestinale, secondaire à une prolifération microbienne endoluminale induite par une stase associée à une ulcération de la paroi intestinale et à une ischémie par distension.…”

Section: Présentation Cliniqueunclassified

Diagnostic de la constipation chez l’enfant

Sauvat¹

2007

Ann Nestlé [Fr]

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La constipation est un motif fréquent de consultation d’un pédiatre. A titre de symptôme, une constipation peut être due à des etiologies diverses en termes de fréquence, de nature et de gravité. La constipation peut être d’origine médicale ou chirurgicale, ou idiopathique. Les étiologies chirurgicales sont principalement représentées par la maladie de Hirschsprung. Il s’agit du principal diagnostic différentiel même s’il est 50 fois moins fréquent que la constipation idiopathique ou fonctionnelle. Une anamnèse détaillée et un examen clinique, incluant un examen anal, sont les meilleurs moyens de parvenir à un diagnostic. Aucun autre examen n’est nécessaire dans la plupart des cas et toutes les explorations doivent être gérées en fonction des considérations cliniques.

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Long-term outcomes in total colonic aganglionosis: a 32-year experience

Cited by 88 publications

References 39 publications

Hirschsprung disease: current perspectives

Hirschsprung disease: current perspectives

Diagnosis of Constipation in Children

Diagnostic de la constipation chez l’enfant

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