Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


Related Searches

Pneumatosis intestinalis

INTRODUCTION

Pneumatosis intestinalis (PI) refers to the presence of gas within the wall of the small or large intestine. Since its first description in 1783 by Du Vernoi, PI has appeared in the literature under many names, including pneumatosis cystoides intestinalis, intramural gas, pneumatosis coli, pseudolipomatosis, intestinal emphysema, bullous emphysema of the intestine, and lymphopneumatosis [1,2]. For the sake of simplicity and to avoid confusion, we will use the term "pneumatosis intestinalis."

The pathogenesis of PI is poorly understood, and is probably multifactorial. PI is not itself a disease, but rather a clinical sign. In some cases, PI is an incidental finding, whereas in others, it portends a life threatening intra-abdominal condition. As a result of the diverse array of clinical settings in which PI is encountered, its implications are often misinterpreted.

EPIDEMIOLOGY

The incidence of pneumatosis intestinalis (PI) is difficult to ascertain because most patients are asymptomatic and never come to clinical attention [2]. PI can be seen in infants and adults. The majority of cases in infants are secondary to necrotizing enterocolitis, a disease associated with a high mortality rate. (See "Clinical features and diagnosis of necrotizing enterocolitis in newborns".)

PI in adults typically presents in the fifth to eighth decade and is idiopathic (15 percent) or secondary (85 percent) to a wide variety of gastrointestinal and non-gastrointestinal illnesses (table 1) [3,4]. The course of PI is variable and determined largely by the underlying disorder. Mortality rates are high when PI is associated with diseases that lead to bowel necrosis or perforation. In contrast, the clinical course is generally benign when PI is idiopathic, such as when it occurs in association with obstructive pulmonary disease.

PATHOGENESIS

Numerous hypotheses have been proposed to explain the pathogenesis of pneumatosis intestinalis (PI), including mechanical, bacterial, and biochemical causes. For any of these theories to gain acceptance, they must explain the association of PI with many different underlying conditions. Although the theories are distinctly different, they are not necessarily mutually exclusive. It is likely that multiple pathogenic mechanisms are involved in the formation of PI.

                 

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Aug 2014. | This topic last updated: Nov 25, 2013.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2014 UpToDate, Inc.
References
Top
  1. Sachse RE, Burke GW 3rd, Jonas M, et al. Benign pneumatosis intestinalis with subcutaneous emphysema in a liver transplant recipient. Am J Gastroenterol 1990; 85:876.
  2. Heng Y, Schuffler MD, Haggitt RC, Rohrmann CA. Pneumatosis intestinalis: a review. Am J Gastroenterol 1995; 90:1747.
  3. KOSS LG. Abdominal gas cysts (pneumatosis cystoides intestinorum hominis); an analysis with a report of a case and a critical review of the literature. AMA Arch Pathol 1952; 53:523.
  4. Knechtle SJ, Davidoff AM, Rice RP. Pneumatosis intestinalis. Surgical management and clinical outcome. Ann Surg 1990; 212:160.
  5. Pieterse AS, Leong AS, Rowland R. The mucosal changes and pathogenesis of pneumatosis cystoides intestinalis. Hum Pathol 1985; 16:683.
  6. Pear BL. Pneumatosis intestinalis: a review. Radiology 1998; 207:13.
  7. KAY-BUTLER JJ. Interstitial emphysema of the caecum. Gut 1962; 3:267.
  8. KEYTING WS, MCCARVER RR, KOVARIK JL, DAYWITT AL. Pneumatosis intestinalis: a new concept. Radiology 1961; 76:733.
  9. Davila AD, Willenbucher RF. Other diseases of the colon and rectum. In: Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 6th ed, Feldman M, Scharschmidt B, Sleisenger M (Eds), WB Saunders Company, Philadelphia 1998. p.1979.
  10. Read NW, Al-Janabi MN, Cann PA. Is raised breath hydrogen related to the pathogenesis of pneumatosis coli? Gut 1984; 25:839.
  11. Florin TH, Hills BA. Does counterperfusion supersaturation cause gas cysts in pneumatosis cystoides coli, and can breathing heliox reduce them? Lancet 1995; 345:1220.
  12. Yale CE, Balish E, Wu JP. The bacterial etiology of pneumatosis cystoides intestinalis. Arch Surg 1974; 109:89.
  13. Ellis BW. Symptomatic treatment of primary pneumatosis coli with metronidazole. Br Med J 1980; 280:763.
  14. van der Linden W, Marsell R. Pneumatosis cystoides coli associated with high H2 excretion. Treatment with an elemental diet. Scand J Gastroenterol 1979; 14:173.
  15. Mehta SN, Friedman G, Fried GM, Mayrand S. Pneumatosis cystoides intestinalis: laparoscopic features. Am J Gastroenterol 1996; 91:2610.
  16. Hoover EL, Cole GD, Mitchell LS, et al. Avoiding laparotomy in nonsurgical pneumoperitoneum. Am J Surg 1992; 164:99.
  17. Sartor RB, Murphy ME, Rydzak E. Miscellaneous inflammatory and structural disorders of the colon. In: Textbook of Gastroenterology, 3rd ed, Yamada T, Alpers D, Laine L, et al (Eds), Lippincott Williams & Wilkins, Philadelphia 1999. Vol 1877.
  18. Christl SU, Gibson GR, Murgatroyd PR, et al. Impaired hydrogen metabolism in pneumatosis cystoides intestinalis. Gastroenterology 1993; 104:392.
  19. Levitt MD, Olsson S. Pneumatosis cystoides intestinalis and high breath H2 excretion: insights into the role of H2 in this condition. Gastroenterology 1995; 108:1560.
  20. Hisamoto A, Mizushima T, Sato K, et al. Pneumatosis cystoides intestinalis after alpha-glucosidase inhibitor treatment in a patient with interstitial pneumonitis. Intern Med 2006; 45:73.
  21. Hayakawa T, Yoneshima M, Abe T, Nomura G. Pneumatosis cystoides intestinalis after treatment with an alpha-glucosidase inhibitor. Diabetes Care 1999; 22:366.
  22. Yanaru R, Hizawa K, Nakamura S, et al. Regression of pneumatosis cystoides intestinalis after discontinuing of alpha-glucosidase inhibitor administration. J Clin Gastroenterol 2002; 35:204.
  23. Azami Y. Paralytic ileus accompanied by pneumatosis cystoides intestinalis after acarbose treatment in an elderly diabetic patient with a history of heavy intake of maltitol. Intern Med 2000; 39:826.
  24. Cordum NR, Dixon A, Campbell DR. Gastroduodenal pneumatosis: endoscopic and histological findings. Am J Gastroenterol 1997; 92:692.
  25. Jamart J. Pneumatosis cystoides intestinalis. A statistical study of 919 cases. Acta Hepatogastroenterol (Stuttg) 1979; 26:419.
  26. Galandiuk S, Fazio VW. Pneumatosis cystoides intestinalis. A review of the literature. Dis Colon Rectum 1986; 29:358.
  27. Höer J, Truong S, Virnich N, et al. Pneumatosis cystoides intestinalis: confirmation of diagnosis by endoscopic puncture a review of pathogenesis, associated disease and therapy and a new theory of cyst formation. Endoscopy 1998; 30:793.
  28. Chaput U, Ducrotté P, Denis P, Nouveau J. Pneumatosis cystoides intestinalis: an unusual cause of distal constipation. Gastroenterol Clin Biol 2010; 34:502.
  29. Tobias R, Coleman S, Helman CA. Pneumatosis coli simulating hepatomegaly. Am J Gastroenterol 1985; 80:146.
  30. Gelman SF, Brandt LJ. Pneumatosis intestinalis and AIDS: a case report and review of the literature. Am J Gastroenterol 1998; 93:646.
  31. Kreiss C, Forohar F, Smithline AE, Brandt LJ. Pneumatosis intestinalis complicating C. difficile pseudomembranous colitis. Am J Gastroenterol 1999; 94:2560.
  32. Ihara E, Harada N, Motomura S, Chijiiwa Y. A new approach to Pneumatosis cystoides intestinalis by target air-enema CT. Am J Gastroenterol 1998; 93:1163.
  33. John A, Dickey K, Fenwick J, et al. Pneumatosis intestinalis in patients with Crohn's disease. Dig Dis Sci 1992; 37:813.
  34. Olson DE, Kim YW, Ying J, Donnelly LF. CT predictors for differentiating benign and clinically worrisome pneumatosis intestinalis in children beyond the neonatal period. Radiology 2009; 253:513.
  35. Kernagis LY, Levine MS, Jacobs JE. Pneumatosis intestinalis in patients with ischemia: correlation of CT findings with viability of the bowel. AJR Am J Roentgenol 2003; 180:733.
  36. Vernacchia FS, Jeffrey RB, Laing FC, Wing VW. Sonographic recognition of pneumatosis intestinalis. AJR Am J Roentgenol 1985; 145:51.
  37. Rabushka LS, Kuhlman JE. Pneumatosis intestinalis. Appearance on MR examination. Clin Imaging 1994; 18:258.
  38. Varano VJ, Bonanno CA. Colonoscopic findings in Pneumatosis cystoides intestinalis. Am J Gastroenterol 1973; 59:353.
  39. Wayne E, Ough M, Wu A, et al. Management algorithm for pneumatosis intestinalis and portal venous gas: treatment and outcome of 88 consecutive cases. J Gastrointest Surg 2010; 14:437.
  40. Holt S, Gilmour HM, Buist TA, et al. High flow oxygen therapy for pneumatosis coli. Gut 1979; 20:493.
  41. Grieve DA, Unsworth IP. Pneumatosis cystoides intestinalis: an experience with hyperbaric oxygen treatment. Aust N Z J Surg 1991; 61:423.
  42. van der Linden W. Letter: Reappearance of intestinal gas cysts after oxygen treatment. Lancet 1974; 2:1388.
  43. Tak PP, Van Duinen CM, Bun P, et al. Pneumatosis cystoides intestinalis in intestinal pseudoobstruction. Resolution after therapy with metronidazole. Dig Dis Sci 1992; 37:949.
  44. Shinagare AB, Howard SA, Krajewski KM, et al. Pneumatosis intestinalis and bowel perforation associated with molecular targeted therapy: an emerging problem and the role of radiologists in its management. AJR Am J Roentgenol 2012; 199:1259.
  45. Jauhonen P, Lehtola J, Karttunen T. Treatment of pneumatosis coli with metronidazole. Endoscopic follow-up of one case. Dis Colon Rectum 1987; 30:800.
  46. Johnston BT, McFarland RJ. Elemental diet in the treatment of pneumatosis coli. Scand J Gastroenterol 1995; 30:1224.
  47. Miralbés M, Hinojosa J, Alonso J, Berenguer J. Oxygen therapy in pneumatosis coli. What is the minimum oxygen requirement? Dis Colon Rectum 1983; 26:458.
  48. Shemen LJ, Stern H, Sidlofsky S, Myers ED. Treatment of pneumatosis cystoides intestinalis with high FIO2: report of two cases. Dis Colon Rectum 1979; 22:245.
  49. Wyatt AP. Prolonged symptomatic and radiological remission of colonic gas cysts after oxygen therapy. Br J Surg 1975; 62:837.
  50. Forgacs P, Wright PH, Wyatt AP. Treatment of intestinal gas cysts by oxygen breathing. Lancet 1973; 1:579.
  51. Simon NM, Nyman KE, Divertie MB, et al. Pneumatosis cystoides intestinalis. Treatment with oxygen via close-fitting mask. JAMA 1975; 231:1354.
  52. Masterson JS, Fratkin LB, Osler TR, Trapp WG. Treatment of pneumatosis cystoides intestinalis with hyperbaric oxygen. Ann Surg 1978; 187:245.
  53. Greenstein AJ, Nguyen SQ, Berlin A, et al. Pneumatosis intestinalis in adults: management, surgical indications, and risk factors for mortality. J Gastrointest Surg 2007; 11:1268.
  54. Johansson K, Lindström E. Treatment of obstructive pneumatosis coli with endoscopic sclerotherapy: report of a case. Dis Colon Rectum 1991; 34:94.