Visceral Neuropathies Responsible for Chronic Intestinal Pseudo-Obstruction Syndrome in Pediatric Practice

Navarro, J; Sonsino, E; Boige, Nathalie; Nabarra, B; Ferkadji, L.; Mashako, L; Cézard, Jean Pierre

doi:10.1097/00005176-199008000-00006

Cited by 72 publications

(38 citation statements)

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“…The true extent of mucosal nerve depletion in ICCC probably exceeds that indicated in our results because autonomic nerves, much like somatic peripheral nerves, possess a remarkable capacity to extend collateral sprouts into a denervated territory [10]. Although ganglia number was not the focus of this study, a reduced population of neurons must be considered as one possible cause of the depletion of mucosal nerves in patients with ICCC [2,11].…”

Section: Discussionmentioning

confidence: 56%

Mucosal nerve deficiency in chronic childhood constipation: a postmigration defect?

Wendelschafer‐Crabb

Neppalli

Jessurun

et al. 2009

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 56%

Mucosal nerve deficiency in chronic childhood constipation: a postmigration defect?

Wendelschafer‐Crabb

Neppalli

Jessurun

et al. 2009

Journal of Pediatric Surgery

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“…10,17 O mesmo aconteceu com dois dos pacientes (casos 1 e 2), que foram admitidos no hospital com diagnóstico inicial de invaginação intestinal e obstrução intestinal neonatal, respectivamente, sendo confirmada a miopatia visceral, posteriormente, em ambos.…”

Section: Discussionunclassified

“…Apenas um paciente (caso 6), nesta casuística, tinha características de neuropatia visceral: hipertrofia das fibras nervosas e acentuada despopulação neuronal dos plexos nervosos intra-murais do intestino e da bexiga, correspondendo à classificação de neuropatia com hipoganglionismo, referida por Navarro et al, em 1990 10 . Nos demais pacientes foi registrada a presença de neurônios, assim como sua normalidade em número e tamanho.…”

Section: Casuísticaunclassified

Chronic intestinal pseudo-obstruction in children - report of seven cases

Almeida

Penna²

2000

J Pediatr (Rio J)

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ResumoA pseudo-obstrução intestinal crônica (POIC) é uma rara e grave enfermidade clínica, caracterizada por disfunção intestinal, na ausência de obstrução mecânica. A fisiopatologia da POIC permanece pouco conhecida. O diagnóstico é difícil e o tratamento, na maioria das vezes, paliativo.Objetivo: A proposta deste estudo é descrever as manifestações clínicas e alterações histológicas na biopsia intestinal de 7 crianças atendidas no serviço de gastroenterologia (GI).Métodos: Estudo retrospectivo de todos os pacientes atendidos no serviço de GI no período de 10 anos. Os prontuários de sete pacientes foram revisados e todos os dados clínicos relevantes registrados. O diagnóstico foi baseado nos achados clínicos, laboratoriais e histológicos.Conclusão: Embora seja uma síndrome rara, o diagnóstico diferencial deverá ser realizado nos pacientes que apresentem sinais e sintomas de obstrução intestinal do trato digestivo, sem causa mecânica associada. Estudos devem ser incentivados com relação ao diagnóstico precoce, utilizando técnicas menos invasivas, assim como, uma terapêutica específica (clínica e cirúrgica), a fim de melhorar a qualidade de vida e obter maior sobrevida para os pacientes afetados. J. pediatr. (Rio J.). 2000; 76(6):453-457:pseudo-obstrução intestinal crônica, infância, diagnóstico, tratamento. AbstractChronic intestinal pseudo-obstruction (CIP) is a rare and severe clinical entity characterized by impaired intestinal function in the absence of mechanical occlusion demonstrable by X-ray or surgery. The pathophysiology of CIP remains poorly known. The diagnostic approach in CIP is difficult and in most of the times the treatment is merely palliative.Objective: The purpose of this report is to describe the clinical characteristics and histological abnormalities on full-thickness intestinal biopsy in seven children from the Gastroenterology (GI) outpatient clinic.Methods: A retrospective study of all patients who attended the GI outpatient clinic in the last ten years. The medical records of seven patients were reviewed, and all the relevant clinical data recorded. Diagnosis was based on clinical, laboratorial, and histological findings.Conclusion: Although this is a rare syndrome, the differential diagnosis shall be performed whenever the patient presents signs and symptoms of obstruction along the digestive tract with no association of any mechanical cause. Studies shall be encouraged regarding the precocious and accurate diagnosis, using less invasive techniques and specific therapeutics (both clinical and surgical), in order to improve life quality and extended survival of affected patients. Descrita pela primeira vez em 1958 por Dudley 1 , a pseudo-obstrução intestinal crônica (POIC) é uma entidade clínica rara e heterogênea. Caracteriza-se por sinais e sintomas intermitentes de obstrução intestinal sem causa mecânica, decorrente de alteração neuromuscular do tubo digestivo, reversível ou irreversível, na qual o segmento acometido pode ser único ou múltiplo. A apresentação clínica é variável e dep...

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“…In maturational arrest, the myenteric plex us may contain a deficient number of neurons or may be almost completely absent [76,77]. These patients may also have mental develop mental delays or mental retardation.…”

Section: Myenteric Plexus Abnormalitiesmentioning

confidence: 99%

Chronic Intestinal Pseudo-Obstruction

Verne

Sninsky

1995

Dig Dis

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Chronic intestinal pseudo-obstruction denotes the clinical picture that results due to the failure of intestinal peristalsis to overcome the normal resistance to flow and is characterized by recurrent episodes of signs and symptoms of intestinal obstruction in the absence of any mechanical compromise of the intestinal lumen. The region(s) of the gut affected may be isolated or diffuse. It is not uncommon to find evidence of autonomic neuropathy and smooth muscle dysfunction with extraintestinal manifestations such as urinary symptoms from abnormal ureter or bladder function. Intestinal pseudo-obstruction can be caused by a variety of diseases, and for simplicity, certain authors have divided it into myopathic and neuropathic categories. Intestinal pseudo-obstruction may present at any age with a variable amount of abdominal pain, distension, nausea, diarrhea, or constipation and with laboratory abnormalities usually reflecting the degree of malabsorption and malnutrition present. The radiologic findings are varied but commonly include paralytic ileus or signs of apparent clinical obstruction with dilated loops of bowel. The number of pseudo-obstruction cases is dependent on how one defines the condition. It appears prudent to require radiographic abnormalities consistent with obstruction on a plain film of the abdomen for the diagnosis. More recently, studies have focused on the gastrointestinal manometric abnormalities of the stomach and small intestine in chronic intestinal pseudo-obstruction during fasting and fed states; however, sensitivity and specificity of these abnormalities are not well defined. Treatment is aimed at limiting symptoms and maintaining adequate nutrition. Prokinetic agents should be tried in an attempt to restore normal intestinal propulsion. However, their overall efficacy appears to be variable. It is still too premature to consider intestinal pacing or small bowel transplantation in this condition. Surgical approaches to chronic intestinal pseudo-obstruction should be limited to patients refractory to medical therapy, and even then, an approach focused on the patient’s primary presenting symptoms should be considered.

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Visceral Neuropathies Responsible for Chronic Intestinal Pseudo-Obstruction Syndrome in Pediatric Practice

Cited by 72 publications

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Mucosal nerve deficiency in chronic childhood constipation: a postmigration defect?

Mucosal nerve deficiency in chronic childhood constipation: a postmigration defect?

Chronic intestinal pseudo-obstruction in children - report of seven cases

Chronic Intestinal Pseudo-Obstruction

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