Manometrical Evaluation in Visceral Neuropathies in Children

Boige, Nathalie; Faure, C; Cargill, G.; Mashako, L; Cordeiro-Ferreira, Gonçalo; Viarme, F; Cézard, Jean Pierre; Navarro, J

doi:10.1097/00005176-199407000-00011

Cited by 32 publications

(12 citation statements)

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“…Medieal management remains diffieult and prognosis poor. Histological studies are essential to classify the syndrome, even if it was suggested that manometrie data ean differentiate between myopathie and neuropathie forms (8,12,19,41). It has not been shown that the histologiealtype influenees management and long-term outeome, but it ean point to the need for more extensive examinations, espeeially with regard to myenterie plexus hyperplasia whieh ean aeeompany a MEN syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Chronic Intestinal Pseudo-Obstruction Syndrome in Pediatric Patients

Goulet¹,

Jobert-Giraud²,

Michel³

et al. 1999

Eur J Pediatr Surg

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The aim of this study was to report the presentation and outcome of 22 consecutive children (13 female) who presented with a syndrome of chronic intestinal pseudo-obstruction with or without urinary tract involvement. We analyse the main clinical and histopathological features and discuss therapeutic management. Ten patients had signs of intestinal obstruction at birth, in which 6 presented antenatally with megacystis on ultrasound. Six children presented with constipation and/or obstruction between 1 and 6 months of age and in 6 other patients diagnosis was made between the ages of 1 and 12 years. There was a family history in 4 patients. Investigations showed diffusely dilated gut on x-ray with slow transit on small bowel follow through. Absent or abnormal motor migrating complex with low amplitude contractions were demonstrated on duodeno-jejunal manometry in 12/13. Megacystis occurred in 15/21 and megaureter in 2/21. Full thickness biopsies (n = 22) revealed involvement of muscle layers in 8, and abnormal myenteric plexus on histochemistry in 13. In 1, the biopsies were inconclusive. Recurrent urinary tract infections occurred in all with structural urinary tract abnormality and most had bacterial overgrowth. Severe recurrent episodes of obstruction which required parenteral nutrition (PN) occurred in all patients. Drugs were unhelpful and decompression ileostomies or colostomies were performed in 20/22. Five children died from sepsis (n = 3) or sudden death. Eleven patients remain partially or totally dependent on PN despite decompression ileostomy in 10/11. Six patients underwent colectomy and ileorectal pull-through, 2 of which remain on long-term PN, while the others are totally orally fed. Despite careful histological study pointing to 2 main forms, myopathy and neuropathy, the etiology of primary intestinal pseudoobstruction syndromes remains unknown. It may present antenatally while most of the time the gut and the urinary tract are diffusely involved. The condition has a high morbidity with a percentage requiring long-term PN. Although the mortality rate is high (23%), careful treatment of urinary tract infections and bacterial overgrowth, decompression surgery and judicious use of PN allows survival to adult life.

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Section: Discussionmentioning

confidence: 99%

“…The main aims of therapy were as follows: 1) To stimulate intestinal motility by mechanical methods (abdominal musculation and massage, catheterization and lavage) and prokinetic drugs such as oral cisapride (5)(6)(7)(8)(9)(10) mg/kg/day) or IV ceruleine (1-3 Ilg/kg/day). 2) A strict diet, poor in fiber and rich in lipids.…”

Section: Medieal Treatmentmentioning

confidence: 99%

Chronic Intestinal Pseudo-Obstruction Syndrome in Pediatric Patients

Goulet¹,

Jobert-Giraud²,

Michel³

et al. 1999

Eur J Pediatr Surg

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“…They can be summarized as follows: uncoordinated bursts of powerful contractions with variable duration are suggestive of an underlying intrinsic neuropathy [1][2][3][4][45][46][47][48] conversely, normally coordinated motor patterns with low amplitude have been reported in patients with a myogenic disorder [1][2][3][4][45][46][47][48] . Nonetheless, low amplitude contractions may merely reflect the inability of the manometric technique to record non-occlusive contractions, such as in the case of dilated bowel loops [1][2][3][4][45][46][47][48] .…”

Section: Manometrymentioning

confidence: 99%

“…Unlike what is observed in pseudo-obstruction, the manometric pattern of mechanical occlusion is characterized by giant contractions (prolonged contractions lasting at least 10 s and can be either propagated or non propagated) or clustered contractions (3-10 regular contractions, occurring 1 per 5 s preceded and followed by ≥ 1 min of absent motor activity lasting at least 20 min and can be either propagated or non propagated) [1][2][3][4][45][46][47][48][49] .…”

Section: Manometrymentioning

confidence: 99%

Chronic intestinal pseudo-obstruction

Antonucci¹,

Fronzoni²,

Cogliandro³

et al. 2008

WJG

190

238

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“…Therefore, the motor innervation of the duodenum is likely to differ from the other regions of the gastrointestinal tract, especially from the distal colon. Moreover, abnormal duodenal motility has been reported, but the mechanism underlying this dysfunction is unclear (Boige et al, 1994).…”

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confidence: 99%

Characterization of circular muscle motor neurons of the duodenum and distal colon in the Australian brush‐tailed possum

Konomi¹,

Meedeniya²,

Simula³

et al. 2001

J of Comparative Neurology

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The motor innervation of the duodenum and distal colon remains uncharacterized within the same species. Our aim was to compare the projections and neurochemical properties of duodenal and distal colon circular muscle motor neurons. Circular muscle motor neurons were retrogradely traced by using a neural tracer in vitro, processed for choline acetyltransferase (ChAT) and nitric oxide synthase (NOS) immunoreactivity and then visualized by using indirect immunofluorescence. A mean of 372 +/- 64 and 156 +/- 23 neurons (mean +/- SEM) were tracer-labeled within the duodenum and colon, respectively. The ChAT+/NOS- neurons comprised 57.6 +/- 6.6% and 39.6 +/- 4.4% of all labeled cells in the duodenum and colon, respectively, and projected mainly in the oral direction. Of all labeled cells, the ChAT-/NOS+ neurons comprised 8.5 +/- 2.3% in the duodenum and 46.6 +/- 5.0% in the distal colon and projected mainly in the anal direction. Of the remainder, 20.6 +/- 5.0% and 8.2 +/- 2.4% were ChAT+/NOS+ and 13.2 +/- 0.9% and 5.6 +/- 1.4% were ChAT-/NOS- in the duodenum and distal colon, respectively. Within both regions, the distribution of the ChAT+/NOS- and ChAT-/NOS+ neurons are consistent with the ascending excitatory and descending inhibitory reflexes. The proportion of ChAT-/NOS+ neurons is greater within the colon in comparison with the duodenum. A considerable proportion of duodenal motor neurons were ChAT+/NOS+ and ChAT-/NOS-. These two classes may underlie nonperistaltic motor patterns, which predominate within the duodenum. These findings demonstrate regional differences in the innervation of intestinal circular muscle.

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Manometrical Evaluation in Visceral Neuropathies in Children

Cited by 32 publications

References 0 publications

Chronic Intestinal Pseudo-Obstruction Syndrome in Pediatric Patients

Chronic Intestinal Pseudo-Obstruction Syndrome in Pediatric Patients

Chronic intestinal pseudo-obstruction

Characterization of circular muscle motor neurons of the duodenum and distal colon in the Australian brush‐tailed possum

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