Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study.

BACKGROUND & AIMS: Chronic idiopathic intestinal pseudo-obstruction (CIIP) is a rare disease characterized by episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders. Intestinal motor abnormalities have long been identified in CIIP patients. Little is known of the natural history of the disease in adults. This study evaluated the clinical course of CIIP over time. METHODS: Fifty-nine consecutive CIIP patients without underlying collagen, vascular diseases, or mitochondrial cytopathies were evaluated between 1985 and 2001. Family history, onset of digestive symptoms, previous surgeries, episodes suggestive of subacute intestinal obstruction, digestive symptoms, body mass index, and feeding habits were recorded. Small bowel manometry was performed by a perfusion technique, and abnormal motor patterns were visually identified. Full-thickness biopsies were available in 11 cases and were processed for immunohistochemical analysis of myogenic and neurogenic components of the gut wall. RESULTS: Patients were prospectively followed up for a median of 4.6 years (range, 1-13 years). Diagnosis was often made several years after symptom onset (median, 8 years). Thus, the majority of patients (88%) underwent useless and potentially dangerous surgeries (mean, 2.96 per patient). Manometry invariably showed abnormal motor patterns. Pathologic findings included neuropathies in all investigated cases and abnormalities of interstitial cells of Cajal in 5 of 11 cases. Long-term outcome was generally poor despite surgical and medical therapies; 4 patients died of disease-related complications, 4 underwent small bowel transplantation, almost one third required long-term home parenteral nutrition, and two thirds had some sort of nutritional limitations. CONCLUSIONS: CIIP is a severe, often unrecognized disease characterized by disabling and potentially life-threatening complications over time

Chronic intestinal pseudo-obstruction

none7Chronic intestinal pseudo-obstruction (CIPO) is a severe functional digestive syndrome characterised by a derangement of gut propulsive motility which resembles mechanical obstruction. It may be associated with disabling and potentially life-threatening complications. CIPO can be secondary to a variety of diseases, but it is more frequently idiopathic. Most cases are sporadic, but familial forms have also been described. Based on histological features CIPO can be classified into three major entities: neuropathies, mesenchymopathies, and myopathies depending on the predominant involvement of enteric neurones, interstitial cells of Cajal (ICC) or smooth muscle cells, respectively. Mitochondriopathies may be responsible for a syndromic form of CIPO, i.e. mitochondrial neurogastrointestinal encephalomyopathy. Management of CIPO involves nutritional, pharmacological and surgical therapies, but the long-term outcome turns out to be poor in the vast majority of cases. The main pathogenetic and clinical features of the syndrome, together with current management recommendations are reviewed in this chapter.noneCOGLIANDRO RF; DE GIORGIO R; BARBARA G; COGLIANDRO L; CONCORDIA A; CORINALDESI R; STANGHELLINI V.Cogliandro, Rf; DE GIORGIO, Roberto; Barbara, G; Cogliandro, L; Concordia, A; Corinaldesi, R; Stanghellini, V

Chronic intestinal pseudo-obstruction: clinical features, diagnosis, and therapy

CIPO is the very " tip of the iceberg" of functional gastrointestinal disorders, being a rare and frequently misdiagnosed condition characterized by an overall poor outcome. Diagnosis should be based on clinical features, natural history and radiologic findings. There is no cure for CIPO and management strategies include a wide array of nutritional, pharmacologic, and surgical options which are directed to minimize malnutrition, promote gut motility and reduce complications of stasis (ie, bacterial overgrowth). Pain may become so severe to necessitate major analgesic drugs. Underlying causes of secondary CIPO should be thoroughly investigated and, if detected, treated accordingly. Surgery should be indicated only in a highly selected, well characterized subset of patients, while isolated intestinal or multivisceral transplantation is a rescue therapy only in those patients with intestinal failure unsuitable for or unable to continue with TPN/HPN. Future perspectives in CIPO will be directed toward an accurate genomic/proteomic phenotying of these rare, challenging patients. Unveiling causative mechanisms of neuro-ICC-muscular abnormalities will pave the way for targeted therapeutic options for patients with CIPO

Chronic intestinal pseudo-obstruction: manifestations, natural history and management.

Chronic intestinal pseudo-obstruction (CIPO) is a rare pathological condition characterized by a marked derangement of gut propulsive motility mimicking mechanical obstruction, in the absence of any lesion occluding the gut lumen. This disease is often associated with a disabling and potentially life-threatening complications and is still too often unrecognized even in referral centres. As a result, patients receive neither appropriate care nor recognition of their severe health condition. Medical and surgical therapies are often unsatisfactory and long-term outcome turns out to be poor in the vast majority of cases. This article focuses on the main clinical features, the management and long-term outcome of patients affected by CIPO, with particular emphasis on those aspects which remain a matter of debate

Chronic intestinal pseudo-obstruction: manifestations, natural history and management.

Chronic intestinal pseudo-obstruction (CIPO) is a rare pathological condition characterized by a marked derangement of gut propulsive motility mimicking mechanical obstruction, in the absence of any lesion occluding the gut lumen. This disease is often associated with a disabling and potentially life-threatening complications and is still too often unrecognized even in referral centres. As a result, patients receive neither appropriate care nor recognition of their severe health condition. Medical and surgical therapies are often unsatisfactory and long-term outcome turns out to be poor in the vast majority of cases. This article focuses on the main clinical features, the management and long-term outcome of patients affected by CIPO, with particular emphasis on those aspects which remain a matter of debate

Natural history of intestinal failure induced by chronic idiopathic intestinal pseudo-obstruction

Chronic intestinal pseudo-obstruction is a severe, often unrecognized disease characterized by disabling and potentially life-threatening complications over time. The diagnosis is based on the evidence of typical clinical manifestations, radiological evidence of distended bowel loops with air-fluid levels, and the exclusion of any organic obstruction of the gut lumen. The radiological sign of intestinal occlusion allows the distinction from enteric dysmotility, which is characterized by better outcomes. Manometry can play a supportive role in defining the diagnosis, and differences in the manometric pattern of chronic intestinal pseudo-obstruction and enteric dysmotility have been shown. The disease is often unrecognized, and the diagnosis, therefore, delayed by several years. Thus, the majority of patients undergo useless and potentially dangerous surgeries. Long-term outcomes are generally poor despite surgical and medical therapies characterized by disabling and potentially life-threatening complications over time. A substantial percentage of patients requires parenteral nutrition. Failure of this nutritional support represents an indication for small bowel transplantation

Natural history of intestinal failure induced by chronic idiopathic intestinal pseudo-obstruction

Chronic intestinal pseudo-obstruction is a severe, often unrecognized disease characterized by disabling and potentially life-threatening complications over time. The diagnosis is based on the evidence of typical clinical manifestations, radiological evidence of distended bowel loops with air-fluid levels, and the exclusion of any organic obstruction of the gut lumen. The radiological sign of intestinal occlusion allows the distinction from enteric dysmotility, which is characterized by better outcomes. Manometry can play a supportive role in defining the diagnosis, and differences in the manometric pattern of chronic intestinal pseudo-obstruction and enteric dysmotility have been shown. The disease is often unrecognized, and the diagnosis, therefore, delayed by several years. Thus, the majority of patients undergo useless and potentially dangerous surgeries. Long-term outcomes are generally poor despite surgical and medical therapies characterized by disabling and potentially life-threatening complications over time. A substantial percentage of patients requires parenteral nutrition. Failure of this nutritional support represents an indication for small bowel transplantation

Patient-reported outcomes and gut dysmotility in functional gastrointestinal disorders

Background Unlike chronic idiopathic intestinal pseudo-obstruction (CIIP), severe digestive syndromes that are not characterized by episodes resembling mechanical obstruction remain poorly characterized. The present study compared clinical features, small bowel motility, and quality of life (QoL) in patients with CIIP or severe functional gastrointestinal disorders (SFGID), compared to irritable bowel syndrome (IBS). Methods We enrolled 215 consecutive patients: 70 CIIP, 110 malnourished SFGID [body mass index (BMI) 17.8±1.8kgm -2] and 35 non-malnourished SFGID (BMI 22.8±3.6kgm -2). Key Results Abnormal motor patterns that fulfilled diagnostic criteria for small bowel dysmotility were virtually absent in IBS patients, but were recorded in69 CIIP patients (98.6%), 82 malnourished SFGID patients (74.5%;), and 23 SFGID patients without malnutrition (65.7%) (P<0.0001). CIIP patients presented more frequently abnormal activity fronts, lack of response to feeding, and hypomotility than malnourished and non-malnourished SFGID patients (61.4%vs 42.7% and 31.4%, P<0.05 only vs non-malnourished SFGID; 8.6%vs 0.9% and 2.9%; 21.4%vs 0.9% and 0%, P<0.05). Quality of life mean scores were all significantly lower in CIIP patients and malnourished SFGID patients than in IBS. Bodily pain, general health, and vitality scores were lower in CIIP also compared to non-malnourished SFGID. Conclusions & Inferences Chronic idiopathic intestinal pseudo-obstruction and SFGIDs are frequently associated with small bowel dysmotility and marked derangements of QoL which are significantly more severe than in IBS and result particularly in being severe in patients with recurrent sub occlusive episodes or inability to maintain a normal body weight

Patient-reported outcomes and gut dysmotility in functional gastrointestinal disorders.

Background Unlike chronic idiopathic intestinal pseudo-obstruction (CIIP), severe digestive syndromes that are not characterized by episodes resembling mechanical obstruction remain poorly characterized. The present study compared clinical features, small bowel motility, and quality of life (QoL) in patients with CIIP or severe functional gastrointestinal disorders (SFGID), compared to irritable bowel syndrome (IBS). Methods We enrolled 215 consecutive patients: 70 CIIP, 110 malnourished SFGID [body mass index (BMI) 17.8\ub11.8kgm -2] and 35 non-malnourished SFGID (BMI 22.8\ub13.6kgm -2). Key Results Abnormal motor patterns that fulfilled diagnostic criteria for small bowel dysmotility were virtually absent in IBS patients, but were recorded in69 CIIP patients (98.6%), 82 malnourished SFGID patients (74.5%;), and 23 SFGID patients without malnutrition (65.7%) (P<0.0001). CIIP patients presented more frequently abnormal activity fronts, lack of response to feeding, and hypomotility than malnourished and non-malnourished SFGID patients (61.4%vs 42.7% and 31.4%, P<0.05 only vs non-malnourished SFGID; 8.6%vs 0.9% and 2.9%; 21.4%vs 0.9% and 0%, P<0.05). Quality of life mean scores were all significantly lower in CIIP patients and malnourished SFGID patients than in IBS. Bodily pain, general health, and vitality scores were lower in CIIP also compared to non-malnourished SFGID. Conclusions & Inferences Chronic idiopathic intestinal pseudo-obstruction and SFGIDs are frequently associated with small bowel dysmotility and marked derangements of QoL which are significantly more severe than in IBS and result particularly in being severe in patients with recurrent sub occlusive episodes or inability to maintain a normal body weight