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DOI: 10.1055/s-2002-20211
Die chronische intestinale Pseudoobstruktion: Pathogenese, Diagnostik und Therapie
Chronic intestinal pseudoobstruction: Pathogenesis, diagnosis and therapyPublication History
23.1.2001
9.7.2001
Publication Date:
20 February 2002 (online)
Zusammenfassung
Die chronische intestinale Pseudoobstruktion (CIPO) ist ein seltenes Krankheitsbild, bei dem eine schwere intestinale Motilitätsstörung zur Behinderung des Chymustransports bis zum Vollbild eines mechanischen Ileus führt, ohne dass ein Passagehindernis vorliegt. Eine CIPO kann als primäre Erkrankung oder sekundär auftreten aufgrund muskulärer Erkrankungen, neurologischer, metabolischer und endokriner Störungen, aber auch postinfektiös, postoperativ, nach abdomineller Bestrahlung, durch Medikamente oder Noxen. Beim Vollbild ist die typische Anamnese mit frustranen Laparotomien unter dem Verdacht eines mechanischen Ileus diagnostisch wegweisend. Bei Verdacht auf eine CIPO muss mithilfe radiologischer und endoskopischer Verfahren sorgfältig nach einem mechanischen Hindernis gesucht werden. Aussagekräftige histologische Untersuchungen sind i. d. R. nur an transmuralen Darmwandbiopsien möglich. Mittels Dünndarmmanometrie kann die Diagnose einer CIPO auch im Intervall gesichert und zwischen Neuropathie und Myopathie unterschieden werden. Primäre Therapieziele sind: 1. Die Aufrechterhaltung eines adäquaten Ernährungszustandes durch orale und/oder enterale Ernährung; bei schweren Verlaufsformen kommt eine heimparenterale Ernährung und insbesondere bei Kindern als Ultima Ratio die Dünndarmtransplantation infrage. 2. Die Wiederherstellung einer intestinalen Propulsion durch Prokinetika. 3. Die Behandlung von Komplikationen wie bakterielle Fehlbesiedlung und ausgeprägte Schmerzen durch Antibiotika bzw. gezielte chirurgische Eingriffe. Unnötige Operationen sollten unbedingt vermieden werden, da sie das klinische Bild durch Bridenbildung wesentlich komplizieren können.
Abstract
Chronic intestinal pseudoobstruction (CIPO) is a rare disease in which a severe intestinal motility disorder impairs transit of chyme so that patients suffer from symptoms of a mechanical ileus without mechanical obstruction. CIPO may be a primary or secondary disorder due to muscular, neurologic, metabolic or endocrine disorders, but may also occur postinfectiously, postoperatively, following abdominal radiation or be caused by drugs or noxae. In severe cases, the typical history of (repeated) symptoms of mechanical obstruction leading to unsuccessful laparotomies will give key clues for diagnosis. If CIPO is suspected, mechanical obstruction must be searched for carefully by radiologic and endoscopic examinations. Histologic diagnosis usually demands full thickness biopsies of the intestinal wall. Small intestinal manometry allows diagnosis of CIPO even during oligosymptomatic intervals as well as differentiation among neuropathic and myopathic forms of the disease. The main therapeutic goals consist in: 1. Maintenance of an adequate nutritional state by oral and/or enteral nutrition; in severe cases home-parenteral nutrition may be required and particularly in children intestinal transplantation may be the ultima ratio. 2. Reconstitution of intestinal propulsion by prokinetic drugs. 3. Therapy of complications such as bacterial overgrowth and severe pain by antibiotics and specific surgical procedures. Unnecessary laparotomies should be strictly avoided because they may lead to adhesions and markedly complicate the clinical course.
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5. Literaturverzeichnis
- 1 Faure C, Goulet O, Ategbo S. et al . Chronic intestinal pseudoobstruction syndrome: Clinical analysis, outcome, and prognosis in 105 children. French-Speaking Group of Pediatric Gastroenterology. Dig Dis Sci. 1999; 44 953-959
- 2 Patel R, Christensen J. Chronic intestinal pseudo-obstruction: Diagnosis and treatment. Gastroenterologist. 1995; 3 345-356
- 3 Auricchio A, Brancolini V, Casari G. et al . The locus for a novel syndromic form of neuronal intestinal pseudoobstruction maps to Xq28. Am J Hum Genet. 1996; 58 743-748
- 4 FitzPatrick D R, Strain L, Thomas A E. et al . Neurogenic chronic idiopathic intestinal pseudo-obstruction, patent ductus arteriosus, and thrombocytopenia segregating as an X linked recessive disorder. J Med Genet. 1997; 34 666-669
- 5 Malagelada J R, Distrutti E. Management of gastrointestinal motility disorders. A practical guide to drug selection and appropriate ancillary measures. Drugs. 1996; 52 494-506
- 6 Mitros F A, Schuffler M D, Teja K, Anuras S. Pathologic features of familial visceral myopathy. Hum Pathol. 1982; 13 825-833
- 7 Shimotake T, Iwai N, Yanagihara J, Tokiwa K, Fushiki S. Biliary tract complications in patients with hypoganglionosis and chronic idiopathic intestinal pseudoobstruction syndrome. J Pediatr Surg. 1993; 28 189-192
- 8 Forchielli M L, Young M C, Flores A F, Richardson D, Lo C W. Immune deficiencies in chronic intestinal pseudo-obstruction. Acta Paediatr. 1997; 86 1077-1081
- 9 Husebye E. The patterns of small bowel motility: Physiology and implications in organic disease and functional disorders. Neurogastroenterol Motil. 1999; 11 141-161
- 10 DiMagno E P, Hendricks J C, Go V L, Dozois R R. Relationships among canine fasting pancreatic and biliary secretions, pancreatic duct pressure, and duodenal phase III motor activity - Boldyreff (n??) revisited. Dig Dis Sci. 1979; 24 689-693
- 11 Keller J, Groeger G, Cherian L, Günther B, Layer P. Circadian coupling between pancreatic secretion and intestinal motility in humans. Am J Physiol. 2001; in press;
- 12 Layer P, Chan A T, Go V L, DiMagno E P. Human pancreatic secretion during phase II antral motility of the interdigestive cycle. Am J Physiol. 1988; 254 G249-G253
- 13 von der nnn O, Layer P, Wollny C. et al . Somatostatin 28 and coupling of human interdigestive intestinal motility and pancreatic secretion. Gastroenterology. 1992; 103 974-981
- 14 Layer P, Chan A T, Go V L, Zinsmeister A R, DiMagno E P. Cholinergic regulation of phase II interdigestive pancreatic secretion in humans. Pancreas. 1993; 8 181-188
- 15 Layer P H, Chan A T, Go V L, Zinsmeister A R, DiMagno E P. Adrenergic modulation of interdigestive pancreatic secretion in humans. Gastroenterology. 1992; 103 990-993
- 16 Vantrappen G, Janssens J, Hellemans J, Ghoos Y. The interdigestive motor complex of normal subjects and patients with bacterial overgrowth of the small intestine. J Clin Invest. 1977; 59 1158-1166
- 17 Husebye E, Skar V, Hoverstad T, Iversen T, Melby K. Abnormal intestinal motor patterns explain enteric colonization with gram-negative bacilli in late radiation enteropathy. Gastroenterology. 1995; 109 1078-1089
- 18 Khurana R K, Schuster M M. Autonomic dysfunction in chronic intestinal pseudo-obstruction. Clin Auton Res. 1998; 8 335-340
- 19 Camilleri M, Balm R K, Low P A. Autonomic dysfunction in patients with chronic intestinal pseudo-obstruction. Clin Auton Res. 1993; 3 95-100
- 20 Camilleri M, Carbone L D, Schuffler M D. Familial enteric neuropathy with pseudoobstruction. Dig Dis Sci. 1991; 36 1168-1171
- 21 Verne G N, Sninsky C A. Chronic intestinal pseudo-obstruction. Dig Dis. 1995; 13 163-181
- 22 Vanderwinden J M, Rumessen J J. Interstitial cells of Cajal in human gut and gastrointestinal disease. Microsc Res Tech. 1999; 47 344-360
- 23 Yamataka A, Ohshiro K, Kobayashi H. et al . Abnormal distribution of intestinal pacemaker (C-KIT-positive) cells in an infant with chronic idiopathic intestinal pseudoobstruction. J Pediatr Surg. 1998; 33 859-862
- 24 Wang Z Q, Watanabe Y, Toki A. et al . Involvement of endogenous nitric oxide and c-kit-expressing cells in chronic intestinal pseudo-obstruction. J Pediatr Surg. 2000; 35 539-544
- 25 Sarna S K. Cyclic motor activity; migrating motor complex: 1985 (nnn??). Gastroenterology. 1985; 89 894-913
- 26 Matulis S R, McJunkin B, Chang H H. Familial visceral neuropathy as part of a diffuse neuronal syndrome: Four fatal cases in one sibship. Am J Gastroenterol. 1994; 89 792-796
- 27 Fell J M, Smith V V, Milla P J. Infantile chronic idiopathic intestinal pseudo-obstruction: The role of small intestinal manometry as a diagnostic tool and prognostic indicator. Gut. 1996; 39 306-311
- 28 Coulie B, Camilleri M. Intestinal pseudo-obstruction. Annu Rev Med. 1999; 50 37-55
- 29 Koppelman R N, Stollman N H, Baigorri F, Rogers A I. Acute small bowel pseudo-obstruction due to AL amyloidosis: A case report and literature review. Am J Gastroenterol. 2000; 95 294-296
- 30 Sigurdsson L, Reyes J, Kocoshis S A. et al . Intestinal transplantation in children with chronic intestinal pseudo-obstruction [see comments]. Gut. 1999; 45 570-574
- 31 Cucchiara S, Borrelli O, Salvia G. et al . A normal gastrointestinal motility excludes chronic intestinal pseudoobstruction in children. Dig Dis Sci. 2000; 45 258-264
- 32 Mann S D, Debinski H S, Kamm M A. Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults. Gut. 1997; 41 675-681
- 33 Stanghellini V, Camilleri M, Malagelada J R. Chronic idiopathic intestinal pseudo-obstruction: Clinical and intestinal manometric findings. Gut. 1987; 28 5-12
- 34 Faber J, Fich A, Steinberg A. et al . Familial intestinal pseudoobstruction dominated by a progressive neurologic disease at a young age. Gastroenterology. 1987; 92 786-790
- 35 Chakravarti A, Blanton S, Kendall B J, McCallum R W. Cosegregation of familial intestinal pseudoobstruction and presence of digital arches in a large multigenerational pedigree. Dig Dis Sci. 1996; 41 1429-1433
- 36 Byrne W J, Cipel L, Ament M E, Gyepes M T. Chronic idiopathic intestinal pseudo-obstruction syndrome. Radiologic signs in children with emphasis on differentiation from mechanical obstruction. Diagn Imaging. 1981; 50 294-304
- 37 Luks F I, Chung M A, Brandt M L. et al . Pneumatosis and pneumoperitoneum in chronic idiopathic intestinal pseudoobstruction. J Pediatr Surg. 1991; 26 1384-1386
- 38 Barnett J L, McDonnell W M, Appelman H D, Dobbins W O. Familial visceral neuropathy with neuronal intranuclear inclusions: Diagnosis by rectal biopsy. Gastroenterology. 1992; 102 684-691
- 39 Borczuk A, Mannion C, Dickson D, Alt E. Intestinal pseudo-obstruction and ischemia secondary to both beta 2-microglobulin and serum A amyloid deposition. Mod Pathol. 1995; 8 577-582
- 40 Goulet O, Jobert-Giraud A, Michel J L. et al . Chronic intestinal pseudo-obstruction syndrome in pediatric patients. Eur J Pediatr Surg. 1999; 9 83-89
- 41 Hanks J B, Meyers W C, Andersen D K. et al . Chronic primary intestinal pseudo-obstruction. Surgery. 1981; 89 175-182
- 42 Huang Y C, Lee H C, Huang F Y. et al . Neonatal-onset chronic intestinal pseudo-obstruction syndrome. Clin Pediatr (Phila). 1995; 34 241-247
- 43 Bruyn G A, Bots G T, Van Wijhe M, Van Kersen F. Chronic intestinal pseudoobstruction as a possible sequel to encephalitis. Am J Gastroenterol. 1986; 81 50-54
- 44 McDonald G B, Schuffler M D, Kadin M E, Tytgat G N. Intestinal pseudoobstruction caused by diffuse lymphoid infiltration of the small intestine. Gastroenterology. 1985; 89 882-889
- 45 Arista-Nasr J, Gonzalez-Romo M, Keirns C, Larriva-Sahd J. Diffuse lymphoplasmacytic infiltration of the small intestine with damage to nerve plexus. A cause of intestinal pseudo-obstruction. Arch Pathol Lab Med. 1993; 117 812-819
- 46 Hutson J M, Chow C W, Borg J. Intractable constipation with a decrease in substance P-immunoreactive fibres: Is it a variant of intestinal neuronal dysplasia?. J Pediatr Surg. 1996; 31 580-583
- 47 Fogel S P, DeTar M W, Shimada H, Chandrasoma P T. Sporadic visceral myopathy with inclusion bodies. A light-microscopic and ultrastructural study. Am J Surg Pathol. 1993; 17 473-481
- 48 Arslan M, Bayraktar Y, Oksuzoglu G. et al . Four cases with chronic intestinal pseudo-obstruction due to hollow visceral myopathy. Hepatogastroenterology. 1999; 46 349-352
- 49 Ghoos Y F, Maes B D, Geypens B J. et al . Measurement of gastric emptying rate of solids by means of a carbon-labeled octanoic acid breath test. Gastroenterology. 1993; 104 1640-1647
- 50 Miller M A, Parkman H P, Urbain J L. et al . Comparison of scintigraphy and lactulose breath hydrogen test for assessment of orocecal transit: Lactulose accelerates small bowel transit. Dig Dis Sci. 1997; 42 10-18
- 51 Cucchiara S, Annese V, Minella R. et al . Antroduodenojejunal manometry in the diagnosis of chronic idiopathic intestinal pseudoobstruction in children. J Pediatr Gastroenterol Nutr. 1994; 18 294-305
- 52 Di Lorenzo C, Reddy S N, Flores A F, Hyman P E. Is electrogastrography a substitute for manometric studies in children with functional gastrointestinal disorders?. Dig Dis Sci. 1997; 42 2310-2316
- 53 Faure C, Wolff V P, Navarro J. Effect of meal and intravenous erythromycin on manometric and electrogastrographic measurements of gastric motor and electrical activity. Dig Dis Sci. 2000; 45 525-528
- 54 Keller J, Ahmadi-Simab K, Ohe M, Rabe B, Layer P. Intestinal manometry: Differentiation of neuropathic motor disorders and additional mechanical obstruction. Gastroenterology. 1998; 114 A776 (Abstract)
- 55 Heneyke S, Smith V V, Spitz L, Milla P J. Chronic intestinal pseudo-obstruction: Treatment and long-term follow-up of 44 patients. Arch Dis Child. 1999; 81 21-27
- 56 Di Lorenzo C, Flores A F, Buie T, Hyman P E. Intestinal motility and jejunal feeding in children with chronic intestinal pseudo-obstruction. Gastroenterology. 1995; 108 1379-1385
- 57 Watanabe Y, Ito T, Ando H, Seo T, Nimura Y. Manometric evaluation of gastrointestinal motility in children with chronic intestinal pseudo-obstruction syndrome. J Pediatr Surg. 1996; 31 233-238
- 58 Hyman P E, Di Lorenzo C, McAdams L. et al . Predicting the clinical response to cisapride in children with chronic intestinal pseudo-obstruction [see comments]. Am J Gastroenterol. 1993; 88 832-836
- 59 Wood J D. Neuropathy in the brain-in-the-gut [In Process Citation]. Eur J Gastroenterol Hepatol. 2000; 12 597-600
- 60 Mok M Y, Wong R W, Lau C S. Intestinal pseudo-obstruction in systemic lupus erythematosus: An uncommon but important clinical manifestation. Lupus. 2000; 9 11-18
- 61 Perlemuter G, Chaussade S, Wechsler B. et al . Chronic intestinal pseudo-obstruction in systemic lupus erythematosus. Gut. 1998; 43 117-122
- 62 Lennon V A, Sas D F, Busk M F. et al . Enteric neuronal autoantibodies in pseudoobstruction with small-cell lung carcinoma [see comments]. Gastroenterology. 1991; 100 137-142
- 63 Gerl A, Storck M, Schalhorn A. et al . Paraneoplastic chronic intestinal pseudoobstruction as a rare complication of bronchial carcinoid. Gut. 1992; 33 1000-1003
- 64 Scolapio J S, Ukleja A, Bouras E P, Romano M. Nutritional management of chronic intestinal pseudo-obstruction. J Clin Gastroenterol. 1999; 28 306-312
- 65 Layer P, Go V L, DiMagno E P. Fate of pancreatic enzymes during small intestinal aboral transit in humans. Am J Physiol. 1986; 251 G475-G48
- 66 Levitt M D, Hirsh P, Fetzer C A, Sheahan M, Levine A S. H2 excretion after ingestion of complex carbohydrates. Gastroenterology. 1987; 92 383-389
- 67 Stephen A M, Haddad A C, Phillips S F. Passage of carbohydrate into the colon. Direct measurements in humans. Gastroenterology. 1983; 85 589-595
- 68 Cavicchi M, Beau P, Crenn P, Degott C, Messing B. Prevalence of liver disease and contributing factors in patients receiving home parenteral nutrition for permanent intestinal failure. Ann Intern Med. 2000; 132 525-532
- 69 Carlsson G, Hakansson A, Rubensson A, Finkel Y. Home parenteral nutrition (HPN) in children in Sweden. Pediatr Nurs. 1997; 23 272-274
- 70 Moukarzel A A, Haddad I, Ament M E. et al . 230 patient years of experience with home long-term parenteral nutrition in childhood: Natural history and life of central venous catheters. J Pediatr Surg. 1994; 29 1323-1327
- 71 Chasco Y A, Guibelalde d C, Moreno V J. et al . Home parenteral nutrition in the management of patients with chronic idiopathic intestinal pseudo-obstruction. Nutr Hosp. 1995; 10 264-267
- 72 Vargas J H, Sachs P, Ament M E. Chronic intestinal pseudo-obstruction syndrome in pediatrics. Results of a national survey by members of the North American Society of Pediatric Gastroenterology and Nutrition. J Pediatr Gastroenterol Nutr. 1988; 7 323-332
- 73 Di Lorenzo C, Reddy S N, Villanueva-Meyer J. et al . Cisapride in children with chronic intestinal pseudoobstruction. An acute, double-blind, crossover, placebo-controlled trial. Gastroenterology. 1991; 101 1564-1570
- 74 Camilleri M, Brown M L, Malagelada J R. Impaired transit of chyme in chronic intestinal pseudoobstruction. Correction by cisapride. Gastroenterology. 1986; 91 619-626
- 75 Cohen N P, Booth I W, Parashar K, Corkery J J. Successful management of idiopathic intestinal pseudo-obstruction with cisapride. J Pediatr Surg. 1988; 23 229-230
- 76 Fraser A G, Arthur J F, Hamilton I. Intestinal pseudoobstruction secondary to amyloidosis responsive to cisapride. Dig Dis Sci. 1991; 36 532-535
- 77 Feighner S D, Tan C P, McKee K K. et al . Receptor for motilin identified in the human gastrointestinal system. Science. 1999; 284 2184-2188
- 78 Storr M, Allescher H D. Motilitätsmodifizierende Pharmaka. Internist. 2000; 41 1318-1330
- 79 Minami T, Nishibayashi H, Shinomura Y, Matsuzawa Y. Effects of erythromycin in chronic idiopathic intestinal pseudo-obstruction. J Gastroenterol. 1996; 31 855-859
- 80 Vassallo M J, Camilleri M, Sullivan S N, Thomforde G M. Effects of erythromycin on gut transit in pseudo-obstruction due to hereditary coproporphyria [see comments]. J Clin Gastroenterol. 1992; 14 255-259
- 81 Perlemuter G, Cacoub P, Chaussade S. et al . Octreotide treatment of chronic intestinal pseudoobstruction secondary to connective tissue diseases. Arthritis Rheum. 1999; 42 1545-1549
- 82 Peracchi M, Basilisco G, Bareggi B. et al . Plasma somatostatin levels in patients with chronic idiopathic intestinal pseudo-obstruction. Am J Gastroenterol. 1997; 92 1884-1886
- 83 Haruma K, Wiste J A, Camilleri M. Effect of octreotide on gastrointestinal pressure profiles in health and in functional and organic gastrointestinal disorders. Gut. 1994; 35 1064-1069
- 84 Verne G N, Eaker E Y, Hardy E, Sninsky C A. Effect of octreotide and erythromycin on idiopathic and scleroderma-associated intestinal pseudoobstruction. Dig Dis Sci. 1995; 40 1892-1901
- 85 Bouhnik Y, Alain S, Attar A. et al . Bacterial populations contaminating the upper gut in patients with small intestinal bacterial overgrowth syndrome. Am J Gastroenterol. 1999; 94 1327-1331
- 86 Layer P, Rosien U. Bakterielle Fehlbesiedlung. Layer P, Rosien U, Goebell H Praktische Gastroenterologie München, Wien, Baltimore; Urban & Schwarzenberg 1996: 225-226
- 87 Attar A, Flourie B, Rambaud J C. et al . Antibiotic efficacy in small intestinal bacterial overgrowth-related chronic diarrhea: A crossover, randomized trial. Gastroenterology. 1999; 117 794-797
- 88 Vanderhoof J A, Young R J, Murray N, Kaufman S S. Treatment strategies for small bowel bacterial overgrowth in short bowel syndrome. J Pediatr Gastroenterol Nutr. 1998; 27 155-160
- 89 Rolfe R D. The role of probiotic cultures in the control of gastrointestinal health. J Nutr. 2000; 130 396S-402S
- 90 Di Stefano M, Malservisi S, Veneto G, Ferrieri A, Corazza G R. Rifaximin versus chlortetracycline in the short-term treatment of small intestinal bacterial overgrowth. Aliment Pharmacol Ther. 2000; 14 551-556
- 91 Trespi E, Ferrieri A. Intestinal bacterial overgrowth during chronic pancreatitis. Curr Med Res Opin. 1999; 15 47-52
- 92 Ginies J L, Francois H, Joseph M G. et al . A curable cause of chronic idiopathic intestinal pseudo-obstruction in children: Idiopathic myositis of the small intestine. J Pediatr Gastroenterol Nutr. 1996; 23 426-429
- 93 Yokota T, Suda T, Tsukioka S. et al . The striking effect of hyperbaric oxygenation therapy in the management of chronic idiopathic intestinal pseudo-obstruction. Am J Gastroenterol. 2000; 95 285-288
- 94 Schuffler M D, Leon S H, Krishnamurthy S. Intestinal pseudoobstruction caused by a new form of visceral neuropathy: Palliation by radical small bowel resection. Gastroenterology. 1985; 89 1152-1156
- 95 Mughal M M, Irving M H. Treatment of end stage chronic intestinal pseudo-obstruction by subtotal enterectomy and home parenteral nutrition. Gut. 1988; 29 1613-1617
- 96 Murr M M, Sarr M G, Camilleri M. The surgeon’s role in the treatment of chronic intestinal pseudoobstruction. Am J Gastroenterol. 1995; 90 2147-2151
- 97 Hase T, Kodama M, Kishida A. et al . The application of radio-opaque markers prior to ileostomy in an infant with chronic intestinal pseudo-obstruction: Report of a case. Surg Today. 1998; 28 83-86
- 98 Reyes J, Bueno J, Kocoshis S. et al . Current status of intestinal transplantation in children. J Pediatr Surg. 1998; 33 243-254
- 99 Goulet O, Jan D, Lacaille F. et al . Intestinal transplantation in children: Preliminary experience in Paris. JPEN J Parenter Enteral Nutr. 1999; 23 S121-S125
- 100 Camilleri M. Study of human gastroduodenojejunal motility. Applied physiology in clinical practice [see comments]. Dig Dis Sci. 1993; 38 785-794
Anschrift für die Verfasser
Dr. J. Keller
Israelitisches Krankenhaus
Orchideenstieg 14
22297 Hamburg