Chronic intestinal pseudo-obstruction

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Classification according to ICD-10
K59.8 Other specified functional bowel disorders
ICD-10 online (WHO version 2019)

The chronic intestinal pseudo-obstruction (CIPO) is a rare motility disorder of the digestive tract with recurring events an apparent mechanical installation , but without any detectable organic, systemic or metabolic disorders, without tangible closure.

It is a chronic form of pseudo-obstruction as opposed to acute forms, also known as Ogilvie syndrome , when the large intestine is primarily affected.

The name was coined by HA Dudley, IS Sinclair and I. F, McLaren in 1958.

distribution

The frequency is not known. CIPO mostly occurs in children, sometimes at or shortly after birth. The male sex is affected 2 to 4 times more often. In Japan , a frequency of 1 in 100,000 was found with no difference between the sexes.

root cause

The cause is not known. Pathophysiologically, there is a lack of or uncoordinated contractility of the smooth muscles with a disorder of the intestinal peristalsis .

One can muscle-related (myopathic) and nerve-related (neuropathic), autosomal dominant , autosomal - recessive and X-linked (with mutations in FLNA - gene on the X chromosome locus q28, the same gene, which is also in congenital short bowel syndrome is concerned) inherited , also differentiate between primary and secondary forms in corresponding diseases.

CIPO can also occur after infection , surgery , radiation, and with drugs and pollutants.

CIPO can be counted among the ACTG2-Related Disorders with mutations in the ACTG2 gene on chromosome 2 locus p13.1:

CIPO can occur with other diseases:

rarely in lupus erythematosus or systemic sclerosis

Clinical manifestations

Clinical criteria are:

  • Pronounced, chronic signs of obstruction: abdominal pain (80%), distension, feeling of fullness, nausea and vomiting (80%)
  • Diarrhea, untreatable constipation (40%)
  • intestinal malabsorption with weight loss and / or failure to thrive
  • Fever (37%)

diagnosis

For diagnosis, a manometry of the intestine, measurement of the passage time through fractionated small intestine passage and histology from intestinal biopsies are required. A mechanical lock must be excluded.

therapy

Unnecessary laparatomies with the risk of subsequent adhesions should be avoided. In addition to biopsies , palliative measures are possible, and an intestinal transplant can also be considered.

forecast

CIPO is a serious, seriously debilitating disease that is often not recognized or recognized late.

literature

  • J. Jäkel, JW Heise, N. Gassler, CG Dietrich: More attention to chronic intestinal pseudo-obstruction (CIPO): A case report with CIPO as the initial manifestation of atypical seronegative scleroderma. In: Journal of Gastroenterology. Volume 50, number 10, October 2012, pp. 1100-1103, doi: 10.1055 / s-0032-1312921 , PMID 23059804 .
  • D. Ang, EK Teo, TL Ang, KH Lim, P. Madhukumar, AY Chung, Y. Wang, KM Fock: Unexplained small-bowel obstruction in a patient with presumptive achalasia: need for early recognition of chronic intestinal pseudo-obstruction ( CIPO). In: Digestive diseases and sciences. Volume 55, Number 9, September 2010, pp. 2691-2692, doi: 10.1007 / s10620-009-1048-1 , PMID 19949864 .
  • A. Amiot, D. Cazals-Hatem, F. Joly, A. Lavergne-Slove, M. Peuchmaur, Y. Bouhnik, P. Bedossa, B. Messing: The role of immunohistochemistry in idiopathic chronic intestinal pseudoobstruction (CIPO): a case-control study. In: The American journal of surgical pathology. Volume 33, Number 5, May 2009, pp. 749-758, doi: 10.1097 / PAS.0b013e31819b381a , PMID 19252433 .
  • A. Maier, V. Mannartz, H. Wasmuth, C. Trautwein, UP Neumann, J. Weis, J. Grosse, M. Fuest, MJ Hilz, JB Schulz, C. Haubrich: GAD Antibodies as Key Link Between Chronic Intestinal Pseudoobstruction , Autonomic Neuropathy, and Limb Stiffness in a Nondiabetic Patient: A CARE-Compliant Case Report and Review of the Literature. In: Medicine. Volume 94, number 31, August 2015, p. E1265, doi: 10.1097 / MD.0000000000001265 , PMID 26252289 , PMC 4616576 (free full text) (review).
  • DH Sutton, SP Harrell, JM Where: Diagnosis and management of adult patients with chronic intestinal pseudoobstruction. In: Nutrition in clinical practice: official publication of the American Society for Parenteral and Enteral Nutrition. Volume 21, Number 1, February 2006, pp. 16-22, doi: 10.1177 / 011542650602100116 , PMID 16439766 (review).

Individual evidence

  1. ↑ a b c d e f g Pseudo-obstruction, chronic intestinal. In: Orphanet (Rare Disease Database).
  2. ↑ a b c d e emedicine
  3. ^ HA Dudley, IS Sinclair, IF McLaren et al .: Intestinal pseudo-obstruction. In: Journal of the Royal College of Surgeons of Edinburgh. Volume 3, Number 3, March 1958, pp. 206-217, PMID 13514744 .
  4. ↑ H. Iida, H. Ohkubo, M. Inamori, A. Nakajima, H. Sato: Epidemiology and clinical experience of chronic intestinal pseudo-obstruction in Japan: a nationwide epidemiologic survey. In: Journal of epidemiology. Volume 23, number 4, 2013, pp. 288-294, doi: 10.2188 / jea.je20120173 , PMID 23831693 , PMC 3709546 (free full text).
  5. ^ Neuronal Intestinal Dysplasia, Type B.  In: Online Mendelian Inheritance in Man . (English)
  6. ↑ Visceral Neuropathy, Familial, Autosomal Dominant.  In: Online Mendelian Inheritance in Man . (English)
  7. ↑ Visceral Neuropathy, Familial, Autosomal Recessive.  In: Online Mendelian Inheritance in Man . (English)
  8. ↑ Intestinal pseudoobstruction, neuronal.  In: Online Mendelian Inheritance in Man . (English)
  9. ↑ GeneReviews
  10. ↑ Rare Diseases ACTG2 related disorders
  11. ↑ H. Ohkubo, A. Fuyuki, J. Arimoto, T. Higurashi, T. Nonaka, Y. Inoh, H. Iida, M. Inamori, T. Kaneda, A. Nakajima: Efficacy of percutaneous endoscopic gastro-jejunostomy (PEG -J) decompression therapy for patients with chronic intestinal pseudo-obstruction (CIPO). In: Neurogastroenterology and motility: the official journal of the European Gastrointestinal Motility Society. Volume 29, number 12, December 2017, p., Doi: 10.1111 / nmo.13127 , PMID 28631871 .

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