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Ho et al.

Abdominal Imaging • Review
Pneumatosis Intestinalis in
the Adult

Pneumatosis Intestinalis in the Adult:
Benign to Life-Threatening Causes
Lisa M. Ho1 OBJECTIVE. The frequency of detection of pneumatosis intestinalis (PI) appears to be in-
Erik K. Paulson creasing. This increase may be the result of increased CT use. New medications and surgical pro-
William M. Thompson cedures have been reported to be associated with an increase in the incidence of PI. The purpose of
this review is to provide an update on the imaging features and clinical conditions associated with PI.
Ho LM, Paulson EK, Thompson WM CONCLUSION. This article illustrates the imaging findings of PI due to benign and life-
threatening causes, with emphasis placed on describing newly associated conditions and also
the imaging appearance on CT.

neumatosis intestinalis (PI) is de- In this article, we divided PI into two cat-

P fined as the presence of gas in the
bowel wall [1–4]. This imaging
finding is associated with numer-
egories: benign causes and life-threatening
causes (Appendix 1). It is important to un-
derstand that PI is a sign not a disease, and
ous conditions, ranging from benign to life it must be interpreted relative to the pa-
threatening [1–5]. The overall incidence of tient’s overall clinical condition. Therefore,
PI in the general population has been re- clinical symptoms and laboratory data pro-
ported to be 0.03% based on an autopsy se- vide the most important clues in determin-
ries [4]. Although PI can be seen on abdom- ing whether PI is due to benign or life-
inal radiographs, CT is the most sensitive threatening causes.
imaging test for identification of PI [6]. The
CT detection of PI appears to be increasing, Pathogenesis
likely as a consequence of increased use of Although the cause of PI appears to be
CT technology [7]. Increased imaging detec- multifactorial, the exact cause is not known.
tion of PI could also be due to an increased Two main theories have been proposed in
incidence of PI. Relatively new surgical pro- the medical literature. A mechanical theory
cedures and medications associated with PI hypothesizes that gas dissects into the bowel
may be contributing to an increase in inci- wall from either the intestinal lumen or the
dence of PI. The aim of this article is to de- lungs via the mediastinum [1] due to some
scribe the imaging appearance and clinical mechanism causing increased pressure (i.e.,
findings of PI in the adult population. bowel obstruction or emphysema). A bacte-
rial theory proposes that gas-forming bacilli
Classification System enter the submucosa through mucosal rents
Keywords: colon, CT, gastrointestinal radiology, ischemia,
small bowel
In 1754, Duvernoy wrote the first report of or increased mucosal permeability and pro-
PI, which appeared in the French literature duce gas within the bowel wall [1].
DOI:10.2214/AJR.06.1309 [8]. Since then, numerous case reports and re- Studies have shown that gas collections in
views have appeared in the world literature. In the bowel wall can have a hydrogen content
Received October 5, 2006; accepted after revision
1998, Pear [5] undertook the most recent of up to 50%. Hydrogen is a product of bac-
December 29, 2006.
comprehensive review in the U.S. radiology terial metabolism and is not produced by hu-
1All authors:
Department of Radiology, Duke University literature. His classification scheme was man cells [1]. The major argument against
Medical Center, Box 3808 DUMC, Durham, NC 27710. based on the current evidence and theories re- the bacterial theory is that long-standing
Address correspondence to L. M. Ho ( garding the cause and clinical significance of pneumoperitoneum can occur with PI and
AJR 2007; 188:1604–1613
PI. In his review, PI was classified pathogen- rarely is it associated with peritonitis [8]. A
ically into four categories: bowel necrosis, combination of both theories is also plausi-
mucosal disruption, increased mucosal per- ble. Bacterial overgrowth in the gastrointes-
© American Roentgen Ray Society meability, and pulmonary disease. tinal tract from a variety of causes can lead

1604 AJR:188, June 2007

which is usually related to the vated serum lactic acid of > 2 mmol/L [3]. a hyper. mmol/L was associated with a greater than nal perforation in cases of PI due to life. less there are peritoneal signs from intesti. 2—69-year-old woman with guaiac- positive stool—benign cause of pneumatosis intestinalis (PI). 80% mortality rate [9]. Clinical Features threatening causes. dissec. B. most frequently used techniques for diagno- Some patients may have mild abdominal bonate level of < 20 mmol/L. Abdominal CT image in 56-year-old man shows bubbly PI (arrows) and free air (arrowheads) in this case of PI in patient on chemotherapy for colon cancer. underlying associated medical condition. bowel distention. amylasemia of > 200 IU/L. sis of PI. A recent study found that the combination tion of intraluminal hydrogen gas into the Physical examination is rarely abnormal un. Scout radiograph from air-contrast barium enema shows cystic PI (arrow) consistent with pneumatosis cystoides intestinalis. acidosis with a blood pH of < 7. A B to excessive hydrogen gas production. A. presence of intestinal ischemia may reveal Imaging Methods and Findings cially PI associated with pulmonary disease. Laboratory values in the In cases of PI due to benign causes. espe. and an ele. Abdominal radiography and CT are the the patients are usually asymptomatic [1–4]. B. 1—Examples of linear and bubbly pneumatosis intestinalis (PI). June 2007 1605 . Pneumatosis Intestinalis in the Adult Fig. and subsequently. A B Fig.3. A. discomfort. of PI and a serum lactic acid level of > 2 bowel wall. a serum bicar. Spot film images from air-contrast barium enema show polypoid filling defects (arrows) due to gas in bowel wall (arrowheads) from pneumatosis cystoides intestinalis. Abdominal CT image in 54-year-old woman shows extraluminal gas tracking along small bowel mesentery (black arrowhead) and linear PI (arrows) in this case of PI associated with jejunostomy tube (white arrowhead). CT has been shown to be more sen- AJR:188.

whereas biliary air is B more central (Fig. teric vessels [15. PI that is confined to a portion of the small or large bowel within a specific vascular distribution also increases the likelihood that ischemia is the cause of PI. In PI due to benign causes. and its radiographic or A CT appearance alone does not allow differ- entiation between them. 16. benign cause of On both radiographs and CT. especially in the colon [11] without any special (Fig. absent or intense mucosal enhancement. the bowel wall is usually normal. 23]. [23] and Weisner et al. multiplanar reformations with a spatial res. Ho et al.and 64-MDCT scanners are capable dressed the capability of CT of distinguish- hepatic portal and portomesenteric venous of generating isotropic data sets that allow ing early and nontransmural mesenteric is- gas [12. dilated bowel. 21]. sagittal. olution similar to or even greater than the transmural infarction [17. It using soft-tissue window can be a combination of both linear and bub- setting shows PI of bly bowel-wall gas. Patient improved detection of PI. Occasionally. The use of coronal re- formatted images with MDCT may improve detection of portomesenteric gas owing to the oblique vertical orientation of the mesen- sitive than radiography at detecting PI further improve the detection of PI and he. 3—69-year-old man wall in the coronal. Fig. 1). arte- rial or venous occlusion. 16]. CT has also been shown to be more patic portal and portomesenteric venous Several reports in the literature have ad- sensitive than radiography at detection of gas. 4). 17] (Fig. June 2007 . The circular form of PI is usually benign and most often seen with pneumatosis cys- toides intestinalis (PCI). the presence of which in. radiography in detecting PI. Intraperitoneal or retroperi- toneal free air can be seen with PI due to life-threatening or benign causes [6. and axial planes on chemotherapy for may allow a more confident diagnosis of PI head–neck cancer with mild abdominal pain— and portal venous gas. 5). The presence of additional findings such as bowel wall thickening. Both Ker- threatening causes. [10–14]. CT can be used to clarify ambiguous radiographic findings and also to search for potential causes [11]. 15. Viewing CT im- (arrows) is much better seen using lung window ages with lung windows may accentuate the setting. Linear or bubble- like PI can be due to both benign and life- threatening causes. The association of spontaneous pneumo- peritoneum with PI has been attributed to the rupture of serosal and subserosal cysts in the bowel wall [8]. Portal venous gas is differentiated from biliary gas by its characteristic tubular branching lucencies that extend to the pe- riphery of the liver. 18–20]. [22] found 1606 AJR:188. bowel contents shows PI of cecum and mixed with air or air trapped between mu- ascending colon cosal folds can mimic PI. 22. Because CT is more sensitive than therapy. Abdominal CT image (Fig. and he- patic portal or portomesenteric venous gas increases the possibility of PI due to a life- threatening cause [15. B. The ability to study the bowel nagis et al. Abdominal CT image pattern of gas in the bowel wall (Fig. chemia from full-thickness and irreversible creases the possibility of PI due to life. PI usually pneumatosis intestinalis appears as a low-density linear or bubbly (PI). ascites. A. Advances in CT may axial plane. 2). There also may be cir- cecum and ascending cular collections of gas in the bowel wall colon (arrows). 3).

Pneumatosis Intestinalis in the Adult B A Fig. 18 mmol/L—life-threatening cause of pneumatosis intestinalis (PI). The overall survival rate was higher bowel disease. June 2007 1607 . small-bowel distention. C) but also hepatic portal venous gas (arrowheads. Supine digital abdominal radiograph shows free air (arrows). It can also ap- chemia. portomesen. and plasma bicarbonate (HCO3). abdominal [30]. Furthermore. causes of PI.1 mmol/L. This technique is more commonly tion with portomesenteric venous gas corre. cholecystitis. severe enteritis.24. out PI as a result of nonischemic conditions. B) not seen on radiograph. cholangitis. avoidance of ionizing radiation is preferred infarction. clinical conditions that have been associated Rabushka and Kuhlman [33] described two AJR:188. lactic acid. C that linear PI was seen more frequently than Although the discovery of hepatic portal with hepatic portal and portomesenteric bubbly PI in patients with transmural bowel or portomesenteric venous gas helps to dis. PI can also be seen on MRI. bowel wall [32]. 21. transmural intestinal infarction. B and C. Abdominal CT images show free air (long arrows) and small-bowel PI (short arrows. pancreatitis. applied to the pediatric patient in whom lated strongly with transmural bowel Mesenteric abscess formation. venous gas [15. gastrointestinal surgery or liver transplanta. tinguish between benign and life-threatening Sonography can also be used to detect PI ies found that the detection of PI in associa. At surgery. PI seen on sonography has been de- portomesenteric venous gas was frequently trauma. 7. and small-bowel PI (arrowheads). 8. 22. both research stud. and diverticulitis and after pear as a continuous echogenic ring in the in patients with nontransmural intestinal is. whereas PI without evidence of teric thrombophlebitis. 4—79-year-old woman after recent surgery for gastric cancer. Patient died 1 week later. 24–27]. [28. chemia compared with those patients with tion are some of the various nonischemic Rarely. chronic scribed as linear or focal echogenic areas seen in cases of nontransmural intestinal is. infarction. diffuse ischemia of small bowel was found. Patient developed abdominal pain and blood pH. sepsis. A. it may also occur with or with. inflammatory within the bowel wall [31]. 29].

it can and normal laboratory mimic polyps when viewed en face results—benign cause of (Fig. Spontaneous resolution and recurrent episodes have been described in the literature [4. Patient of gas in the bowel wall and its mesentery had free air on routine chest radiograph and no [11. 2B). In most cases. 2B). A and B) and fibrosis. They found cir- cumferential collections of air adherent to or within the bowel wall that became more ap- parent on gradient-echo images due to bloom- ing artifact associated with magnetic field in- homogeneities at air–tissue interfaces. A. 6 continues on next tation [37. June 2007 . 34]. PI has been reported in discharged. This observation may be the effect of in- creased use of cross-sectional imaging. Abdominal CT image shows gas in bile ducts in central part of liver (arrowheads). Patient was observed and tion with PI [35. We have encountered a num- page) ber of cases (Fig. Benign Causes of PI Appendix 1 lists benign causes of PI in the adult. Although steroid therapy is one 1608 AJR:188. 38]. On barium enema studies. the natural history of PI due to benign causes is not known because there is often no imaging follow-up. 36]. Cystic (arrows. 39–41]. 23-year-old woman after heart transplant admitted for mild rejection but no abdominal symptoms and normal laboratory results. It almost always occurs in abdominal symptoms the colon. but in profile the gas cysts can be pneumatosis intestinalis (PI). B. 5—Comparison of hepatic portal venous gas and biliary gas in two different patients. Digital abdominal (Fig. Ho et al. and chronic obstructive pul- diffuse linear PI of colon monary disease have a well-known associa- (arrowheads). The number of benign conditions associated with PI appears to be increasing. Abdominal CT image shows hepatic portal venous gas in periphery of liver (arrows). A B Fig. cases of PI seen with MR. 6) after lung transplantation A [18. 2). PCI is Fig. 6—51-year-old man one subset of PI that is invariably benign. radiograph (A) and Pulmonary causes of PI are usually benign abdominal CT images (B and C) show free air and range from congenital to acquired. clearly identified within the colon wall A–C. patients who have undergone organ transplan- (Fig. after lung transplant for PCI is characterized by circular collections cystic fibrosis. 34] (Fig. 60-year-old man after Whipple procedure for pancreatic cancer. 8. asthma.

Patient was observed and discharged. Patient had free air on routine chest radiograph and no abdominal symptoms and normal laboratory results—benign cause of pneumatosis intestinalis (PI). A. 7—27-year-old woman with history of scleroderma who presented with abdominal distention. Pneumatosis Intestinalis in the Adult B C Fig. 6 (continued)—51-year-old man after lung transplant for cystic fibrosis. Physical examination and laboratory results were normal—benign cause of pneumatosis intestinalis (PI). A B Fig. Digital abdominal radiograph (A) and abdominal CT images (B and C) show free air (arrows. AJR:188. Upright abdominal radiograph shows pneumoperitoneum (arrows). A–C. Supine abdominal radiograph shows PI (arrows) of small bowel. B. A and B) and diffuse linear PI of colon (arrowheads). June 2007 1609 .

Superior mesenteric arteriogram of same patient as A shows acute thrombosis (arrows) resulting in small-bowel ischemia and infarction. Systemic diseases and intestinal disor- PI in the lung transplant patient. is a common opportunistic infection in lung tive therapy that includes bowel rest and em- plantation period. B. 8—Patients with sudden onset of abdominal pain—life-threatening cause of pneumatosis intestinalis (PI). ders make up a large number of causes of PI. Patient died. Ho et al. Abdominal CT in 65-year-old woman shows acute thrombus (arrows) in superior mesenteric artery. June 2007 . colitis has also been implicated as a cause of gastrointestinal disease [18]. In our experi. cytomegalovirus (CMV) transplant recipients. A. which can manifest as piric antiviral medication. Supine abdominal radiograph in 60-year-old man shows PI of small bowel (arrows). these patients respond well to conserva. A B Fig. C possible cause of PI in the post–lung trans. CMV colitis ence. 1610 AJR:188. C.

Rice RP. Steroids have marrow transplantation [56–58]. Am J Gastroen- nal tract. 90:1747–1758 AJR:188. lactulose. Emergency colectomy was performed. A B These include collagen vascular disease mucosal structural integrity and allow Conclusion such as scleroderma [42. causes). Pneumatosis oid administration is the most common cause also be life threatening. Rohrmann CA. continuation of the medication [4. dissection of intraluminal air into the intesti. 50]. lated with a higher severity of disease. Acute graft. 46. detection of hepatic portal or portomesenteric quently reported in the early days of the HIV Life-Threatening Causes of PI venous gas increases the likelihood of trans- epidemic [20. 51]. may look very similar. and collagen vascular diseases 2. most always benign. Heng Y. 4) is the most mural bowel infarction. PI has been associated with medica. pre. The imaging appearance of both et al. gregates (Peyer patches) in the gastrointesti. 47]. Ann Surg 1990. also lead to PI [59]. and of whether PI is due to a benign or life-threat- not dictate a specific course of treatment. References Iatrogenic causes can also be encountered. John nal wall. Knechtle SJ. 7) and in. and laboratory test results is the best indicator ever. gan transplantation is often benign. Pneumatosis cystoides intestinalis and with jejunostomy tubes [3. 52–55]. but it can 3. Pneumatosis Intestinalis in the Adult Fig. A and B. Keene JG. physical examination. cecal ileus. (which may also produce PI due to benign intramural intestinal gas. Dis Colon Rec- trast barium enema [48]. There are many benign and life-threatening flammatory bowel disease [19. come. dix 1). Corticoster. Galandiuk S. this finding Mesenteric ischemia (Fig. Davidoff AM. bowel obstruction. The development of PI in cancer patients has with clinical history. Medication side effects can be an over. In cases of PI associated cal picture. the presence of PI in these patients did peutic agents [4. 9—19-year-old man with toxic megacolon due to Crohn’s disease—life- threatening cause of pneumatosis intestinalis (PI). 8). also been attributed to several chemothera. 1. with suspected bowel ischemia. As described. 43] (Fig. appears to have become less common. which can in turn lead to loss of sub. Fazio VW. versus-host disease as a life-threatening com. toxic megaco. especially after bone intestinalis: surgical management and clinical out- of medication-induced PI [4]. PCI is one subset of PI that is al- and therapy was based on the overall clini. 29:358–363 merous reports of patients with PI associated lon (Fig. plication of bone marrow transplantation can Pneumatosis intestinalis: a review. Haggitt RC. Occasionally. Schuffler MD. June 2007 1611 . How. [45] reported that CT evidence of PI in tions that cause bowel distention or diarrhea. but often the exact cause is never established. ening cause. correlation patients with Crohn’s disease usually corre. causes of PI. the additional The association of PI with AIDS was fre. thromboembolization is ications and treatments for patients with HIV. voglibose have also been reported to cause PI. 49. PI in the setting of or. PI resolved with dis. 9). However. Pneumatosis cystoides in- PI has been seen as a sequela of double-con. In most of these cases. J Emerg Med 1989. 4. 44. Sorbitol. Other life-threatening causes of PI include testinalis: a review of the literature. There have been nu. tum 1986. 7:645–650 looked cause of unexplained PI. common life-threatening cause of PI (Appen- sumably due to the effectiveness of new med. 45]. proven as a cause of the ischemia [6] (Fig. 212:160–165 been shown to cause atrophy of lymphoid ag. Supine (A) and upright (B) abdominal radiographs show diffuse PI of colon (arrows). terol 1995. Therefore.

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Ash RC. pneumonitis. foration in a bone marrow transplant patient. AJR 1989. AJR zyk VT. intestinalis with free air mimicking intestinal per. Patterson B. Duncan B. Mizushima T. Hansen RM. Pneumatosis Intestinalis in the Adult 53. 14:323–326 59. Chmielarc. Pneumatosis 58. Eicher ML. intestinalis after bone marrow transplantation. Hyams JS. Day DL. et al. Medication-in. Mustard R. Barton LL. Hulett RL. Goodman LR. Life-threatening causes • Bowel obstruction • Adynamic ileus Intestinal ischemia • Inflammatory bowel disease Mesenteric vascular disease • Ulcerative colitis • Crohn’s disease Intestinal obstruction (especially strangulation) • Leukemia • Perforated jejunal diverticulum Enteritis • Whipple’s disease Colitis • Intestinal parasites • Collagen vascular disease (especially scleroderma) Ingestion of corrosive agents • Diverticulitis Toxic megacolon Iatrogenic Trauma • Barium enema • Jejunoileal bypass Organ transplantation (especially bone marrow transplants) • Jejunostomy tubes Collagen vascular disease • Postsurgical anastomosis • Endoscopy Note—A number of causes and associations occur under both benign and life-threatening categories. June 2007 1613 . Gurney JW. 16:1666–1668 APPENDIX 1. cause of functional gastrointestinal complaints. Bates FT. Erdman SH. 84:30–33 56. Sato K. Transplant tosis cystoides intestinalis after alpha-glucosidase in bone-marrow transplantation patients: diagno. Santamaria Pneumatosis intestinalis associated with graft-ver- 55. Ramsay NK. Letourneau JG. Zander A. AJR:188. Hall RR. 1997. Kanojia M. Pediatrics Marrow Transplant 1994. Pneumatosis intestinalis sus-host disease of the intestinal tract. 151:85–87 duced pneumatosis intestinalis. 45:73–76 152:991–994 Gastroenterology 1983. et al. Pneumatosis 54. Proc 1984. 99:633–636 57. Intern Med 2006. Lipton J. Benign causes Medications • Corticosteroids Pulmonary • Chemotherapeutic agents • Asthma • Lactulose • Bronchitis • Sorbitol • Emphysema • Voglibose • Pulmonary fibrosis • Positive end-expiratory pressure (PEEP) Organ transplantation • Cystic fibrosis • Bone marrow • Kidney Systemic disease • Liver • Scleroderma • Cardiac • Systemic lupus • Lung • AIDS • Graft versus host Intestinal causes Primary pneumatosis • Pyloric stenosis • Idiopathic (up to 15% of cases and usually involves the colon) • Intestinal pseudoobstruction • Pneumatosis cystoides intestinalis • Enteritis • Peptic ulcers B. Pneuma. Bone 1988. Causes of Pneumatosis Intestinalis in the Adult: Benign and Life-Threatening Causes and Associations A. Sorbitol intolerance: an unappreciated inhibitor treatment in a patient with interstitial sis on routine chest radiographs. Hisamoto A. JJ. Anagnostou A.