Visceral myopathy of the colon mimicking Hirschsprung's disease

Leon, Samuel H.; Schuffler, Michael D.

doi:10.1007/bf01299818

Cited by 4 publications

(2 citation statements)

References 10 publications

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“…99]. Full thickness rectal biopsies may have some add ed efficacy as reported in 1 case of a visceral myopathy mimicking Hirschsprung's disease [100]. 174 Veme/Sninsky Chronic Intestinal Pseudo-Obstruction …”

Section: Antroduodenalmentioning

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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Section: Antroduodenalmentioning

confidence: 99%

Chronic Intestinal Pseudo-Obstruction

Verne

Sninsky

1995

Dig Dis

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“…Careful studies of full-thickness samples of the GJ wall have revealed a variety of pathologies to underlie the syndrome of ClIP. The vast majority of ClIPs have identifiable abnormalities within intestinal smooth muscle layers or intrinsic neurons (12)(13)(14)(15); moreover, both systems may be affected by the same disease (16).…”

mentioning

confidence: 99%

Antroduodenojejunal Manometry in the Diagnosis of Chronic Idiopathic Intestinal Pseudoobstruction in Children

Cucchiara,

Annese,

Minella

et al. 1994

J. pediatr. gastroenterol. nutr.

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We analyzed the antroduodenojejunal (ADJ) manometric patterns in a group of 19 consecutive children (mean age, 53 months; range, 5 months to 14 years) referred for suspected chronic idiopathic intestinal pseu‐doobstruction. Diagnosis was based on typical symptoms, absence of extraintestinal diseases, and structural lesions of the gut at endoscopy and radiology. Surgical full‐thickness intestinal biopsies were evaluated in nine patients. Manometry of the stomach and small bowel was performed in the fasting and fed state with a multilumen perfused probe. All patients showed severe abnormalities of ADJ motor activity that were not seen in the eight controls (mean age, 38.2 months; range, 1–9 years). In 12 patients, manometric patterns suggesting neuropathic disease were detected with fasting and/or fed sustained and incoordinated duodenojejunal phasic waves, aberrant propagation and/or configuration of phase III of the inter‐digestive motility complex, inability of a meal to convert a fasting into a fed pattern, and prolonged groups of fasting and fed nonpropagated phasic waves. In seven of these patients, histology revealed marked changes of the intrinsic neurons. In four cases, manometry disclosed features suggestive of a myogenic disease, including severe fasting and fed infrequent low‐amplitude contractions, sometimes with some degree of propagation; in two of these cases, histology showed morphological abnormalities of smooth muscle cells of the gut wall. In three patients, manometry revealed signs suggestive of mechanical obstruction of the gut, such as repetitive post‐feeding clusters and simultaneous repeated broad‐based waves; in these patients, more detailed x‐ray studies showed organic obstructive causes (ileal lymphoma, Hirschsprung's disease, and intestinal malrotation). We conclude that, in children with symptoms suggesting chronic intestinal pseudoobstruction, manometry of the stomach and small bowel reveals severe gut dysmotility and can help establish the diagnosis and distinguish the underlying disease process.

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Pathophysiologie, Diagnostik und Therapie

Liehr¹,

Riecken²

1997

Gastrointestinale Funktionsstörungen

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Visceral myopathy of the colon mimicking Hirschsprung's disease

Cited by 4 publications

References 10 publications

Chronic Intestinal Pseudo-Obstruction

Chronic Intestinal Pseudo-Obstruction

Antroduodenojejunal Manometry in the Diagnosis of Chronic Idiopathic Intestinal Pseudoobstruction in Children

Pathophysiologie, Diagnostik und Therapie

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