Chronische intestinale Pseudoobstruktion – Übersicht und Update 2008

Zusammenfassung

Bei der chronischen intestinalen Pseudoobstruktion handelt es sich um eine gravierende Störung der gastrointestinalen Motilität, die klinisch schwer vom Bild der mechanischen intestinalen Obstruktion zu differenzieren ist. Zugrunde liegen können Neuropathien des extrinsischen oder intrinsischen Nervensystems oder intestinale Myopathien. Fast ein Drittel der Patienten benötigt langfristig eine parenterale Heimernährung. Chirurgische Eingriffe bis hin zur Dünndarmtransplantation müssen erforderlichenfalls angewendet werden, um vital bedrohliche Verläufe abzuwenden. Wenngleich sich das Verständnis von Pathogenese und therapeutischen Ansätzen der seltenen Entität noch stark im Fluss befinden, erlaubt der aktuelle Wissenstand eine differenzierte Diagnostik, eine Klassifikation der primären und sekundären Ursachen und eine gezielte Therapie.

Abstract

The term chronic intestinal pseudo-obstruction describes a syndrome of severly altered gastrointestinal motility that clinically resembles mechanical intestinal obstruction. The syndrome comprises numerous underlying primary or secondary neuropathies of the intrinsic or entrinsic nervous system as well myopathies. Almost a third of the patients requires long-term total parenteral nutrition (TPN). However, emergency surgery and even small bowel transplantation as an ultimate option after failure of TPN may become necessary to evade a vital threat. Although our understanding of pathogenesis and therapeutical options is still evolving, current knowledge allows a differentiated diagnostic approach, classification of the primary and secondary causes and differentiated therapy.

Schlüsselwörter

Pseudoobstruktion - Motilität - Dünndarm - Manometrie

Key words

pseudoobstruction - motility - small bowel - manometry

Literatur

• 1 Dudley H A, Sinclair I S, Mc Laren IF. et al . Intestinal pseudo-obstruction.  J R Coll Surg Edinb. 1958;  3 206-217
• 2 Stanghellini V, Corinaldesi R, Barbara L. Pseudo-obstruction syndromes.  Baillieres Clin Gastroenterol. 1988;  2 225-254
• 3 Stanghellini V, Cogliandro R F, Giorgio de R. et al . Chronic intestinal pseudo-obstruction: manifestations, natural history and management.  Neurogastroenterol Motil. 2007;  19 440-452
• 4 De Giorgio R, Sarnelli G, Corinaldesi R. et al . Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction.  Gut. 2004;  53 1549-1552
• 5 Vantrappen G, Janssens J, Hellemans J. et al . The interdigestive motor complex of normal subjects and patients with bacterial overgrowth of the small intestine.  J Clin Invest. 1977;  59 1158-1166
• 6 Goyal R K, Hirano I. The enteric nervous system.  N Engl J Med. 1996;  334 1106-1115
• 7 Wood J. Neural and humoral regulation of gastrointestinal motility. Schuster MM, Crowell MD, Koch KL Gastrointestinal mobility in health and disease London; BC Decker 2002: 19-42
• 8 Giaroni C, De Ponti F, Cosentino M. et al . Plasticity in the enteric nervous system.  Gastroenterology. 1999;  117 1438-1458
• 9 Boeckxstaens G E, Rumessen J J, Wit de L. et al . Abnormal distribution of the interstitial cells of cajal in an adult patient with pseudo-obstruction and megaduodenum.  Am J Gastroenterol. 2002;  97 2120-2126
• 10 Vanderwinden J M, Liu H, De Laet M H. et al . Study of the interstitial cells of Cajal in infantile hypertrophic pyloric stenosis.  Gastroenterology. 1996;  111 279-288
• 11 Pardi D S, Miller S M, Miller D L. et al . Paraneoplastic dysmotility: loss of interstitial cells of Cajal.  Am J Gastroenterol. 2002;  97 1828-1833
• 12 Kenny S E, Vanderwinden J M, Rintala R J. et al . Delayed maturation of the interstitial cells of Cajal: a new diagnosis for transient neonatal pseudoobstruction. Report of two cases.  J Pediatr Surg. 1998;  33 94-98
• 13 Connor F L, Di Lorenzo C. Chronic intestinal pseudo-obstruction: assessment and management.  Gastroenterology. 2006;  130 S29-S36
• 14 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
• 15 Di Lorenzo C. Pseudo-obstruction: current approaches.  Gastroenterology. 1999;  116 980-987
• 16 Schappi M G, Smith V V, Milla P J. et al . Eosinophilic myenteric ganglionitis is associated with functional intestinal obstruction.  Gut. 2003;  52 752-755
• 17 Tornblom H, Lang B, Clover L. et al . Autoantibodies in patients with gut motility disorders and enteric neuropathy.  Scand J Gastroenterol. 2007;  42 1289-1293
• 18 Jean W C, Dalmau J, Ho A. et al . Analysis of the IgG subclass distribution and inflammatory infiltrates in patients with anti-Hu-associated paraneoplastic encephalomyelitis.  Neurology. 1994;  44 140-147
• 19 Altermatt H J, Rodriguez M, Scheithauer B W. et al . Paraneoplastic anti-Purkinje and type I anti-neuronal nuclear autoantibodies bind selectively to central, peripheral, and autonomic nervous system cells.  Lab Invest. 1991;  65 412-420
• 20 De Giorgio R, Guerrini S, Barbara G. et al . Inflammatory neuropathies of the enteric nervous system.  Gastroenterology. 2004;  126 1872-1883
• 21 Darnell R B, Posner J B. Paraneoplastic syndromes involving the nervous system.  N Engl J Med. 2003;  349 1543-1554
• 22 Lennon V A, Sas D F, Busk M F. et al . Enteric neuronal autoantibodies in pseudoobstruction with small-cell lung carcinoma.  Gastroenterology. 1991;  100 137-142
• 23 Sodhi N, Camilleri M, Camoriano J K. et al . Autonomic function and motility in intestinal pseudoobstruction caused by paraneoplastic syndrome.  Dig Dis Sci. 1989;  34 1937-1942
• 24 Howe S, Eaker E Y, Sallustio J E. et al . Antimyenteric neuronal antibodies in scleroderma.  J Clin Invest. 1994;  94 761-770
• 25 Sonsino E, Mouy R, Foucaud P. et al . Intestinal pseudoobstruction related to cytomegalovirus infection of myenteric plexus.  N Engl J Med. 1984;  311 196-197
• 26 Meneghelli U G. Chagas’ disease: a model of denervation in the study of digestive tract motility.  Braz J Med Biol Res. 1985;  18 255-264
• 27 Debinski H S, Kamm M A, Talbot I C. et al . DNA viruses in the pathogenesis of sporadic chronic idiopathic intestinal pseudo-obstruction.  Gut. 1997;  41 100-106
• 28 Basilisco G, Gebbia C, Peracchi M. et al . Cerebellar degeneration and hearing loss in a patient with idiopathic myenteric ganglionitis.  Eur J Gastroenterol Hepatol. 2005;  17 449-452
• 29 De Giorgio R, Bassotti G, Stanghellini V. et al . Clinical, morpho-functional and immunological features of idiopathic myenteric ganglionitis.  Gastroenterology. 1996;  110 A655
• 30 Collins S M. The immunomodulation of enteric neuromuscular function: implications for motility and inflammatory disorders.  Gastroenterology. 1996;  111 1683-1699
• 31 Camilleri M, Phillips S F. Acute and chronic intestinal pseudo-obstruction.  Adv Intern Med. 1991;  36 287-306
• 32 De Giorgio R, Stanghellini V, Barbara G. et al . Primary enteric neuropathies underlying gastrointestinal motor dysfunction.  Scand J Gastroenterol. 2000;  35 114-122
• 33 Tornblom H, Lindberg G, Nyberg B. et al . Full-thickness biopsy of the jejunum reveals inflammation and enteric neuropathy in irritable bowel syndrome.  Gastroenterology. 2002;  123 1972-1979
• 34 Stanghellini V, Cogliandro R F, De Giorgio R. et al . Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study.  Clin Gastroenterol Hepatol. 2005;  3 449-458
• 35 Cogliandro R, Stanghellini V, Cogliandro L. et al . Small bowel motility and natural history of chronic intestinal pseudo-obstruction.  Gastroenterology. 1998;  114 A735
• 36 Keller J, Layer P. Chronic intestinal pseudo-obstruction: pathogenesis, diagnosis and therapy.  Z Gastroenterol. 2002;  40 85-95
• 37 Stanghellini V, Camilleri M, Malagelada J R. Chronic idiopathic intestinal pseudo-obstruction: clinical and intestinal manometric findings.  Gut. 1987;  28 5-12
• 38 Camilleri M. Jejunal manometry in distal subacute mechanical obstruction: significance of prolonged simultaneous contractions.  Gut. 1989;  30 468-475
• 39 Hyman P E, Di Lorenzo C, McAdams L. et al . Predicting the clinical response to cisapride in children with chronic intestinal pseudo-obstruction.  Am J Gastroenterol. 1993;  88 832-836
• 40 Watanabe Y, Ito T, Ando H. et al . Manometric evaluation of gastrointestinal motility in children with chronic intestinal pseudo-obstruction syndrome.  J Pediatr Surg. 1996;  31 233-238
• 41 Di Lorenzo C, Flores A F, Buie T. et al . Intestinal motility and jejunal feeding in children with chronic intestinal pseudo-obstruction.  Gastroenterology. 1995;  108 1379-1385
• 42 Valdovinos M A, Camilleri M, Thomforde G M. et al . Reduced accuracy of 14C-D-xylose breath test for detecting bacterial overgrowth in gastrointestinal motility disorders.  Scand J Gastroenterol. 1993;  28 963-968
• 43 Ghirardo S, Sauter B, Levy G. et al . Primary intestinal autoimmune disease as a cause of chronic intestinal pseudo-obstruction.  Gut. 2005;  54 1206-1207
• 44 De Giorgio R, Camilleri M. Human enteric neuropathies: morphology and molecular pathology.  Neurogastroenterol Motil. 2004;  16 515-531
• 45 Oton E, Moreira V, Redondo C. et al . Chronic intestinal pseudo-obstruction due to lymphocytic leiomyositis: is there a place for immunomodulatory therapy?.  Gut. 2005;  54 1343-1344
• 46 Schuffler M D, Baird H W, Fleming C R. et al . Intestinal pseudo-obstruction as the presenting manifestation of small-cell carcinoma of the lung. A paraneoplastic neuropathy of the gastrointestinal tract.  Ann Intern Med. 1983;  98 129-134
• 47 Schobinger-Clement S, Gerber H A, Stallmach T. Autoaggressive inflammation of the myenteric plexus resulting in intestinal pseudoobstruction.  Am J Surg Pathol. 1999;  23 602-606
• 48 De Giorgio R, Barbara G, Stanghellini V. et al . Idiopathic myenteric ganglionitis underlying intractable vomiting in a young adult.  Eur J Gastroenterol Hepatol. 2000;  12 613-616
• 49 Smith V V, Gregson N, Foggensteiner L. et al . Acquired intestinal aganglionosis and circulating autoantibodies without neoplasia or other neural involvement.  Gastroenterology. 1997;  112 1366-1371
• 50 Lipton A B, Knauer C M. Pseudo-obstruction of the bowel. Therapeutic trial of metoclopramide.  Am J Dig Dis. 1977;  22 263-265
• 51 Camilleri M, Balm R K, Zinsmeister A R. Symptomatic improvement with one-year cisapride treatment in neuropathic chronic intestinal dysmotility.  Aliment Pharmacol Ther. 1996;  10 403-409
• 52 Chami T, Schuster M, Crowell M. et al . Effect of low dose erythromycin aon gastrointestinal motility and symptoms in chronic intestinal pseudoobstruction (abstract).  Gastroenterology. 1991;  100 41
• 53 Soudah H C, Hasler W L, Owyang C. Effect of octreotide on intestinal motility and bacterial overgrowth in scleroderma.  N Engl J Med. 1991;  325 1461-1467
• 54 Trevisani G T, Hyman N H, Church J M. Neostigmine: safe and effective treatment for acute colonic pseudo-obstruction.  Dis Colon Rectum. 2000;  43 599-603
• 55 Calvet X, Martinez J M, Martinez M. Repeated neostigmine dosage as palliative treatment for chronic colonic pseudo-obstruction in a patient with autonomic paraneoplastic neuropathy.  Am J Gastroenterol. 2003;  98 708-709
• 56 Coulie B, Camilleri M. Intestinal pseudo-obstruction.  Annu Rev Med. 1999;  50 37-55
• 57 Thompson A R, Pearson T, Ellul J. et al . Percutaneous endoscopic colostomy in patients with chronic intestinal pseudo-obstruction.  Gastrointest Endosc. 2004;  59 113-115
• 58 Bond G J, Reyes J D. Intestinal transplantation for total/near-total aganglionosis and intestinal pseudo-obstruction.  Semin Pediatr Surg. 2004;  13 286-292
• 59 Masetti M, Di Benedetto F, Cautero N. et al . Intestinal transplantation for chronic intestinal pseudo-obstruction in adult patients.  Am J Transplant. 2004;  4 826-829
• 60 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
• 61 Lin Z, Chen J D. Advances in gastrointestinal electrical stimulation.  Crit Rev Biomed Eng. 2002;  30 419-457