Long-term outcome of congenital intestinal pseudo-obstruction

Mousa, Hayat; Cocjin, Jose; Flores, AF; Hyman, P E; Lorenzo, C Di

doi:10.1016/s0016-5085(98)83292-7

Cited by 33 publications

(89 citation statements)

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“…CIPO in children most commonly occurs sporadically, is congenital and primary, and affects the intestine diffusely. At the present time, although mesenchymopathies have been implicated in a few reports, the majority of conditions are classified as neuropathies and myopathies 65,66,67,68,69 . For the majority this is adequately achieved through appropriate, often specialised, functional assessment and histopathology.…”

Section: Pathophysiologymentioning

confidence: 99%

Gastrointestinal and nutritional problems in neurologically impaired children

Quitadamo

Thapar

Staiano

et al. 2016

European Journal of Paediatric Neurology

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Fax: + 44 20 7813 8382Gut Motility disorders comprise a heterogeneous group of disorders that result from disruption of the functional integrity of the intrinsic neuromusculature of the gastrointestinal (GI) tract. This intrinsic neuromusculature includes the smooth muscle layers, the intrinsic nervous system of the GI tract (enteric nervous system or ENS) and the interstitial cells of Cajal. The contribution of each of these components to function can be compromised by alterations in structure (ranging from absence to derangement in numbers and/or anatomy) or of function (complete or partial failure in physiological function). This pathology can be primary or secondary to a number of insults e.g. infections, inflammation, toxins etc. In children disorders may be congenital being present from birth or acquired later in life. Finally involvement of the enteric neuromusculature can be patchy/segmental or affect the gastrointestinal tract diffusely.Neuro-gastroenterology is the study of the interface of all aspects of the gastrointestinal tract or digestive system with the different branches of the body's nervous system including the enteric, central and autonomic nervous systems. The combined terms 'Neurogastroenterology and motility' are designed to encompass the study of all the components of the enteric neuromuscualture and their modulating influences and represents one of the fastest growing areas in gastroenterology clinical practice and research. This chapter aims to address some of the most common neurogastroenterology and motility conditions seen in clinical practice ranging from those with defined pathology to those which fall under the umbrella of functional gastrointestinal disorders. The latter comprise some of the commonest but challenging disorders and the term 'functional' reflects the fact that in the majority of such conditions no organic cause can be found. It should also be noted that at the present time many functional disorders e.g. abdominal pain-related functional GI disorders, represent symptom complexes that can further be subdivided into more discrete entities e.g. irritable bowel syndrome, functional abdominal pain etc depending on the nature, location and associations of the symptoms. Clinicians will often rely on the presence or absence of 'red flags' e.g. associated weight loss, severe or significant symptoms etc to decide whether patients are likely to have organic or functional disorders. Exhaustive investigations in the latter are likely to prove fruitless, expensive and perpetuate 'illness behaviours' in the patients. Abdominal pain-related functional gastrointestinal disorders IntroductionAbdominal pain-related functional gastrointestinal disorders (FGID) constitute a spectrum of conditions characterised by the presence of recurrent abdominal pain in the absence of any defined pathology 1 . In the past these disorders fell under the single term 'Recurrent abdominal pain' (RAP) but have since been recategorised under an international initiative (Rome Foundation) to improve the dia...

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Section: Pathophysiologymentioning

confidence: 99%

Gastrointestinal and nutritional problems in neurologically impaired children

Quitadamo

Thapar

Staiano

et al. 2016

European Journal of Paediatric Neurology

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“…Children generally present the first manifestations of CIPO at birth or during the first years of age [50][51][52][53] . The pediatric expression of the disease is often characterized by a particularly severe course, with mortality rates extremely high within the first year of age, mainly due to surgical and parenteral nutrition complications [52,53] . Several predictors of poor outcome have been identified in children including myopathic forms, malrotation, short bowel syndrome, and urinary tract involvement [50,51] .…”

Section: Natural Historymentioning

confidence: 99%

“…Nevertheless, the clinical course of CIPO is almost invariably severe [1,2,[52][53][54] with progressive deterioration of bowel function and digestive symptoms. In order to control both the body weight and the abdominal pain most patients progressively limit oral nutrition and end up on long-term parenteral nutrition.…”

Section: Natural Historymentioning

confidence: 99%

“…The main causes of death are TPN-related complications, surgery-related complications, and post-transplantation complication, together with septic shock of GI origin. A variety of clinical, histological and manometric parameters have been found to be predictive of a poor clinical outcome in adult patients, including myopathy and decreased contractile activity [2,50,52,53,[55][56][57][58][59][60] . MNGIE has a particularly poor prognosis with slowly progressive evolution and death around 40 years of age [11] .…”

Section: Natural Historymentioning

confidence: 99%

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Chronic intestinal pseudo-obstruction

Antonucci¹,

Fronzoni²,

Cogliandro³

et al. 2008

WJG

190

238

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“…En lo referente a los pacientes con afectación desde la infancia, 32 de los 44 pacientes seguidos en el hospital Great Ormond de Londres necesitaron NP prolongada, 19 menos de seis meses, 13 más de 6 meses y 10 de forma indefinida (3). De los 105 de la serie multicéntrica de Faure, 78 requirieron NP prolongada (4), y 53 de los 85 de un centro de referencia para trastornos de la motilidad en niños (5).…”

Section: Sr Directorunclassified

Pseudo-obstrucción intestinal crónica primaria debida a miopatía visceral

Villares

2006

Rev. esp. enferm. dig.

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La pseudo-obstrucción intestinal crónica (POIC) es un sín-drome poco frecuente caracterizado por episodios repetidos o continuos de vómitos y signos sugestivos de obstrucción intestinal en ausencia de obstrucción anatómica. La afectación del intestino delgado es la norma pero pueden verse afectados también el esófago, el estómago y el colon.La manometría antroduodenal y el estudio histopatológico de todo el espesor de la pared intestinal pueden ayudar al diagnósti-co, aunque en la mayoría de ocasiones no modifica el tratamiento.Muñoz Yagüe y cols. presentan un interesante caso de una mujer de 37 años con POIC y una afectación posterior de la musculatura estriada (1). Los autores señalan que el tratamiento se dirige a mejorar los síntomas y a mantener una adecuada nutrición. Afirman que los casos más graves, refractarios al tratamiento médico, pueden necesitar tratamiento endoscópico o quirúrgico, incluida la colectomía y el trasplante intestinal.La pérdida de peso y la malnutrición se ven frecuentemente en estadios avanzados de la enfermedad. Estas formas graves requieren, con frecuencia, el uso de nutrición enteral o nutrición parenteral por periodos prolongados. Antes de utilizar la nutrición parenteral (NP) debería intentarse antes el uso de nutrición enteral (NE). Algunos pacientes requerirán NP a medida que progrese la enfermedad, aunque puedan beneficiarse de un tratamiento exitoso previo durante algún tiempo con una NE por sonda. Episodios repetidos de distensión abdominal, náuseas y vómitos junto con pérdida de peso a pesar de la NE son una indicación para considerar la NP prolongada. En la serie de Mann (2) de 20 pacientes adultos con POIC, sólo 8 mantuvieron su estado nutricional sin necesidad de NE o NP; mientras que ocho de ellos eran dependientes de NP a domicilio (NPD), constituyendo el 16% de todos los pacientes con NPD en su hospital. En lo referente a los pacientes con afectación desde la infancia, 32 de los 44 pacientes seguidos en el hospital Great Ormond de Londres necesitaron NP prolongada, 19 menos de seis meses, 13 más de 6 meses y 10 de forma indefinida (3). De los 105 de la serie multicéntrica de Faure, 78 requirieron NP prolongada (4), y 53 de los 85 de un centro de referencia para trastornos de la motilidad en niños (5).Los pacientes con POIC constituyen entre el 5 y el 10% de los pacientes con NPD incluidos en el registro anual de NPD del grupo de Nutrición Artificial Domiciliaria y Domiciliaria de la Sociedad Española de Nutrición Parenteral y Enteral (SEN-PE) (6). En nuestra propia experiencia, de los 32 enfermos adultos con NPD seguidos desde 1992, dos padecieron una POIC; una de ellas una forma miopática que debutó con estreñimiento pertinaz y dificultad para la ganancia de peso desde los 8 años. En la actualidad lleva 13 años con NPD, ha sufrido 4 bacteriemias asociadas a catéter y ha precisado recambio de catéter en 3 ocasiones -una oclusión del mismo y dos retiradas accidentales-. Durante este tiempo ha llevado una vida prácti-camente normal y ha tenido dos gestaciones sin inci...

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Long-term outcome of congenital intestinal pseudo-obstruction

Cited by 33 publications

References 0 publications

Gastrointestinal and nutritional problems in neurologically impaired children

Gastrointestinal and nutritional problems in neurologically impaired children

Chronic intestinal pseudo-obstruction

Pseudo-obstrucción intestinal crónica primaria debida a miopatía visceral

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