DOI: 10.1148/rg.253045145
RadioGraphics 2005;25:719-730
© RSNA, 2005
Unusual Nonneoplastic Peritoneal and Subperitoneal Conditions: CT Findings1
Perry J. Pickhardt, MD and
Sanjeev Bhalla, MD
1 From the Department of Radiology, University of Wisconsin Medical School, Madison (P.J.P.); the Department of Radiology, Uniformed Services University of the Health Sciences, Bethesda, Md (P.J.P.); and the Mallinckrodt Institute of Radiology, St Louis, Mo (S.B.). Recipient of a Certificate of Merit award for an education exhibit at the 2003 RSNA Scientific Assembly. Received July 9, 2004; revision requested August 18 and received September 23; accepted September 27. P.P. is a medical consultant for Viatronix, Inc; S.B. has no financial relationships to disclose.
Address correspondence to P.J.P., Department of Radiology, University of Wisconsin Hospital and Clinics, E3/311 Clinical Science Center, 600 Highland Ave, Madison, WI 53792 (e-mail: ppickhardt{at}mail.radiology.wisc.edu).
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Abstract
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Peritoneal disease can manifest at computed tomography (CT) as fluid accumulation within the peritoneal cavity (ascites) or soft-tissue infiltration of the various peritoneal ligaments and mesenteries. Beyond the commonly encountered cases of typical ascites and peritonitis, there is a wide spectrum of uncommon nonneoplastic conditions that may involve the peritoneal and subperitoneal spaces. For example, systemic or organ-based diseases that occasionally involve the peritoneum include eosinophilic gastroenteritis, amyloidosis, extramedullary hematopoiesis, Erdheim-Chester disease, sarcoidosis, and mesenteric cavitary lymph node syndrome. Tumorlike conditions that may affect the peritoneum include aggressive fibromatosis (desmoid), inflammatory pseudotumor, retractile mesenteritis, and Castleman disease. Atypical peritoneal infections include tuberculosis, actinomycosis, echinococcosis, Whipple disease, and mesenteric adenitis. Conditions involving the subperitoneal fat include epiploic appendagitis, mesenteric panniculitis, and segmental omental infarction, all of which have characteristic CT findings. CT is an excellent imaging modality for detection and characterization of peritoneal involvement from these unusual diseases.
© RSNA, 2005
Abbreviations: FAPS = familial adenomatous polyposis syndrome, IUD = intrauterine device
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Introduction
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Peritoneal disease, including both neoplastic and nonneoplastic conditions, can manifest at computed tomography (CT) as either fluid accumulation within the peritoneal cavity (ascites) or as soft-tissue masses or infiltration of the various peritoneal ligaments and mesenteries. Beyond the common causes of ascites and peritonitis, a wide spectrum of uncommon nonneoplastic conditions exist that may involve the peritoneal and subperitoneal spaces. This article will review the salient clinical and CT features of these unusual diseases. Although several entities allow an imaging-specific diagnosis at CT, most of the diseases have a nonspecific CT appearance that requires careful correlation with the clinical history. In some cases, the combination of certain clinical and CT features may suggest a specific diagnosis. Categories to be covered include systemic processes, tumorlike conditions, and atypical infections (Table). Several unique conditions involving the subperitoneal fat will also be covered. Overall, CT is an excellent imaging modality for detecting and characterizing peritoneal involvement from these unusual diseases, many of which can mimic malignancy.
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Anatomic Considerations
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The visceral and parietal peritoneum enclose the large potential space referred to as the "peritoneal cavity" (Fig 1). Pathologic processes that gain access to the peritoneal cavity can disseminate throughout this space via the relatively unrestricted movement of fluid and cells. Often overlooked, however, is the potential for disease extension within the subperitoneal space, which lies deep to the surface lining of the visceral and parietal peritoneum, the omentum, and the various peritoneal ligaments and mesenteries (1). Furthermore, this subperitoneal space is in continuity with the retroperitoneum, which allows bidirectional spread of disease processes. This concept helps explain the dual involvement that is sometimes encountered with these traditionally separate and distinct compartments.
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Figure 1. Peritoneal and subperitoneal spaces. Sagittal diagram shows the relationship of the peritoneal cavity (purple) to the peritoneal lining and subperitoneal space (yellow). Note the continuity between the intraperitoneal and extraperitoneal components of the subperitoneal space.
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Systemic Diseases
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A vast, heterogeneous array of systemic and multiorgan diseases exists that can occasionally manifest with peritoneal involvement. Nonspecific CT features generally include soft-tissue infiltration or masses involving the peritoneal ligaments and mesenteries, with or without associated ascites. As such, distinguishing these benign conditions from peritoneal carcinomatosis can be difficult.
Eosinophilic gastroenteritis is a rare condition of unknown etiology characterized by eosinophilic infiltration of the gastrointestinal tract and is often associated with peripheral eosinophilia and an allergic diathesis (2). The disease can be categorized according to the predominant layer of gastrointestinal tract involvement: mucosal, intramural, or subserosal. Of these, subserosal disease is the least frequent, accounting for about 10% of cases. Subserosal involvement, also referred to as "eosinophilic peritonitis," will typically manifest with (eosinophilic) ascites at imaging, sometimes associated with soft-tissue infiltration of the omentum and/or mesentery (Fig 2) (3). The CT findings are nonspecific but typically raise concern for peritoneal carcinomatosis.
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Figure 2a. Eosinophilic peritonitis in a symptomatic 55-year-old man with unexplained intractable ascites. Axial contrast-enhanced CT images show soft-tissue infiltration predominantly involving the omentum (arrowheads) with lesser involvement of other peritoneal ligaments, mesenteries, and the parietal peritoneum. Eosinophilic ascites is also present (* in b). No mucosal abnormality was seen at panendoscopy, but peripheral eosinophilia was present. The diagnosis was confirmed with omental biopsy.
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Figure 2b. Eosinophilic peritonitis in a symptomatic 55-year-old man with unexplained intractable ascites. Axial contrast-enhanced CT images show soft-tissue infiltration predominantly involving the omentum (arrowheads) with lesser involvement of other peritoneal ligaments, mesenteries, and the parietal peritoneum. Eosinophilic ascites is also present (* in b). No mucosal abnormality was seen at panendoscopy, but peripheral eosinophilia was present. The diagnosis was confirmed with omental biopsy.
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Systemic amyloidosis is caused by deposition of extracellular protein in an abnormal fibrillar form and may represent a primary process or occur secondary to a variety of underlying chronic diseases (4). The gastrointestinal tract is frequently involved, which sometimes leads to demonstrable bowel wall thickening at imaging. Rarely, multifocal (Fig 3) or diffuse (Fig 4) mesenteric and/or omental infiltration can occur, typically associated with areas of coarse dystrophic calcification (5). The CT appearance of peritoneal amyloidosis is largely nonspecific and can closely mimic that of carcinomatosis. However, its typically indolent clinical course is in stark contrast to that of malignancy.
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Figure 3a. Primary systemic amyloidosis in an asymptomatic 73-year-old man. He had received chemotherapy and radiation therapy for symptomatic thoracic disease but had not had significant abdominal symptoms. Axial contrast-enhanced CT images (a obtained at a higher level than b) show multifocal mesenteric masses containing areas of dystrophic calcification. The size of the soft-tissue lesions had increased only slightly compared with that on previously obtained CT images over a period of 5 years.
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Figure 3b. Primary systemic amyloidosis in an asymptomatic 73-year-old man. He had received chemotherapy and radiation therapy for symptomatic thoracic disease but had not had significant abdominal symptoms. Axial contrast-enhanced CT images (a obtained at a higher level than b) show multifocal mesenteric masses containing areas of dystrophic calcification. The size of the soft-tissue lesions had increased only slightly compared with that on previously obtained CT images over a period of 5 years.
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Figure 4. Advanced systemic amyloidosis in a 59-year-old man. Axial contrast-enhanced CT image shows extensive peritoneal soft-tissue infiltration and multifocal coarse calcifications.
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Extramedullary hematopoiesis is a compensatory response seen mainly in the setting of myelofibrosis or a hemoglobinopathy (such as thalassemia). The liver, spleen, and thoracic paraspinal region are the sites most often involved (6). Mesenteric involvement is rare but can simulate malignancy at CT (Fig 5). However, these soft hematopoietic masses generally do not cause bowel obstruction or other symptoms. CT findings of medullary expansion or additional sites of extramedullary hematopoiesis would help suggest the diagnosis, but correlation with the clinical history is most helpful.
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Figure 5a. Extramedullary hematopoiesis in an 81-year-old woman with myelofibrosis. Axial contrast-enhanced CT images (a obtained at a higher level than b) show bulky but soft mesenteric masses. Note the lack of significant mass effect on the bowel loops. The diagnosis was confirmed with CT-guided biopsy.
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Figure 5b. Extramedullary hematopoiesis in an 81-year-old woman with myelofibrosis. Axial contrast-enhanced CT images (a obtained at a higher level than b) show bulky but soft mesenteric masses. Note the lack of significant mass effect on the bowel loops. The diagnosis was confirmed with CT-guided biopsy.
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Erdheim-Chester disease is a rare disease of histiocytic infiltration (lipoid granulomatosis) that characteristically results in osteosclerosis of the long bones (7). Extraskeletal manifestations are seen in about 50% of cases, but peritoneal involvement is exceedingly rare (Fig 6). Given the nonspecific CT features, only a known history of the disease would suggest this specific diagnosis.
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Figure 6. Peritoneal involvement in a 41-year-old man with known Erdheim-Chester disease and a long history of intermittent abdominal pain. Axial contrast-enhanced CT image shows soft-tissue infiltration predominantly involving the omentum (arrowheads).
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Peritoneal sarcoidosis is a relatively common systemic disorder characterized by noncaseating granuloma formation. Extrathoracic manifestations are protean, but true peritoneal involvement is rare (8). Ascites in the setting of sarcoidosis is more often secondary to hepatic or cardiac disease rather than true peritoneal involvement. However, when primary peritoneal involvement does occur, it can simulate tuberculous peritonitis at both CT (Fig 7) and pathologic evaluation. The presence of typical thoracic sarcoidosis at CT, with or without hepatosplenic disease or abdominal lymphadenopathy, would be necessary to suggest this specific diagnosis in the absence of known disease.
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Figure 7. Peritoneal sarcoidosis in a 33-year-old woman with increased abdominal distention, nausea, and vomiting. Axial contrast-enhanced CT image shows ascites as well as mild but diffuse soft-tissue thickening involving the mesentery, omentum, and parietal peritoneum.
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Mesenteric cavitary lymph node syndrome is a rare manifestation of celiac disease. The characteristic CT feature is that of enlarged mesenteric nodes containing fat-fluid levels and allows an imaging-specific diagnosis (Fig 8). The specific cause of cavitation remains uncertain, but the finding has been associated with a poor outcome (9). Additional CT findings of celiac disease may be apparent, such as nonobstructive dilatation of the proximal small bowel.
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Figure 8a. Mesenteric cavitary lymph node syndrome in an 83-year-old woman with a 3-month history of diarrhea and weight loss. Axial contrast-enhanced CT images (a obtained at a higher level than b) show enlarged mesenteric lymph nodes with characteristic fat-fluid levels (arrowheads). (Case courtesy of Denise Reddy, MD, Loyola University Medical Center, Maywood, Ill.)
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Figure 8b. Mesenteric cavitary lymph node syndrome in an 83-year-old woman with a 3-month history of diarrhea and weight loss. Axial contrast-enhanced CT images (a obtained at a higher level than b) show enlarged mesenteric lymph nodes with characteristic fat-fluid levels (arrowheads). (Case courtesy of Denise Reddy, MD, Loyola University Medical Center, Maywood, Ill.)
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Tumorlike Conditions
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Certain nonneoplastic conditions may primarily manifest at CT with focal soft-tissue masses that mimic peritoneal malignancy. These conditions include aggressive fibromatosis, inflammatory pseudotumor, retractile mesenteritis, and Castleman disease.
Aggressive fibromatosis, or intraabdominal desmoid tumor, represents a benign proliferative process that has a tendency to locally recur (10). Mesenteric involvement is more often seen in cases related to typical familial adenomatous polyposis syndrome (FAPS) (Gardner syndrome). At CT, the margins of these lesions may appear irregular (Fig 9) or smooth (Fig 10). The specific diagnosis of a mesenteric desmoid tumor would be strongly suggested by a known diagnosis of FAPS or its associated CT findings, such as colonic polyps or masses, previous total colectomy, and polyps or masses involving the duodenum, stomach, or periampullary region.
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Figure 9. Intraabdominal desmoid (fibromatosis) in a 36-year-old man with an abdominal mass that was increasing in size (and no history of FAPS). Axial contrast-enhanced CT image shows a solitary mesenteric mass with an irregular border (arrow).
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Figure 10. Intraabdominal desmoid (fibromatosis) in a 29-year-old woman with a history of early satiety (and no history of FAPS). Axial contrast-enhanced CT image shows a solitary mesenteric mass with a smooth border.
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Inflammatory pseudotumor, also known as "myofibroblastic tumor," is a rare reactive lesion that most often manifests before adulthood and occasionally involves the peritoneal cavity (11). Similar to mesenteric fibromatosis, these lesions may appear at CT as infiltrative (Fig 11) or well-defined (Fig 12) peritoneal-based masses. Pathologic distinction from inflammatory fibrosarcoma is often difficult. Other than the typically young age at presentation, the clinical and CT features are nonspecific.
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Figure 11. Inflammatory pseudotumor in a teenage boy. Axial contrast-enhanced CT image shows the infiltrative soft-tissue appearance of peritoneal inflammatory pseudotumor (myofibroblastic tumor) (arrows).
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Retractile mesenteritis represents the chronic form of sclerosing mesenteritis, where fibrosis predominates over inflammatory infiltration of the mesenteric fat (see the discussion of mesenteric panniculitis later in this article) (12). At CT, the fibrotic spiculated mesenteric mass may contain dystrophic calcification (Fig 13). The imaging features overlap with those of metastatic carcinoid in addition to several other entities.
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Figure 13. Retractile mesenteritis in a 54-year-old man with a long history of abdominal pain. Axial contrast-enhanced CT image shows a densely calcified, spiculated mesenteric mass (*). The CT appearance resembles that of metastatic carcinoid.
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Castleman disease, also known as "angiofollicular lymph node hyperplasia," manifests with lymphadenopathy at CT (13). The hyaline-vascular form is more common, typically involves the mediastinum, and appears hypervascular at CT. Although the plasma cell type is less frequent, it more often involves the abdomen (Fig 14). The plasma cell form also carries an increased risk of lymphoma (Fig 15). Striking contrast enhancement of enlarged nodal masses at CT represents the only suggestive feature of this entity.
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Figure 14. Castleman disease in a 31-year-old man with a history of fever. Axial contrast-enhanced CT image shows lymphadenopathy limited to the gastrosplenic region (arrow). This finding proved to represent Castleman disease.
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Atypical Infections
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There are a variety of uncommon organisms that can result in peritoneal abnormalities at CT. Depending on the specific infection, such findings may include soft-tissue infiltration, ascites, cystic lesions, rim calcification, or low-attenuation lymphadenopathy.
Tuberculous peritonitis usually represents direct extension from gastrointestinal tract or nodal disease. Manifestations at CT include a "wet type," characterized by ascites; a "fibrotic type," characterized by large omental and mesenteric masses; and a "dry type," characterized by diffuse, fibrous peritoneal thickening (Fig 16) (14). Low-attenuation caseating lymphadenopathy may be seen with any form (Fig 17) and helps narrow the differential diagnosis. Typical findings of thoracic involvement can be highly suggestive but are absent in approximately 50% of cases of abdominal disease.
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Figure 16. Tuberculous peritonitis in a 47-year-old man without evidence of thoracic disease. Axial contrast-enhanced CT image shows the "dry" form of peritoneal tuberculosis, which consists of diffuse fibrous thickening of the peritoneal lining without ascites.
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Peritoneal actinomycosis manifests as an aggressive, infiltrative soft-tissue process at CT (Fig 18) (15). A tendency for violating normal anatomic boundaries has been documented. A history of long-term intrauterine device (IUD) use is associated with pelvic involvement by actinomycosis and may be the only suggestive feature.
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Figure 18. Actinomycosis associated with an IUD in a 49-year-old woman. Axial contrast-enhanced CT image shows a complex solid (arrowheads) and cystic adnexal process, which proved to be actinomycosis. Arrow = IUD.
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Peritoneal echinococcosis is almost always secondary to hepatic hydatid disease, related to seeding from either spontaneous rupture or prior surgery (16). The resulting single or multiple cysts can be located anywhere within the peritoneal cavity but otherwise have a similar CT appearance to the liver lesions. A calcific rim is a suggestive feature (Fig 19).
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Figure 19. Peritoneal echinococcosis in a patient who previously had hepatic hydatid disease. Axial contrast-enhanced CT image shows a complex cystic peritoneal lesion with thick eccentric calcification (arrowheads).
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Whipple disease is a rare multisystem infection caused by a gram-positive bacillus (17). Small bowel involvement can lead to low-attenuation mesenteric lymphadenopathy, which may even have a fatty appearance due to infiltration of lipid-laden macrophages (Fig 20). The CT combination of small bowel wall thickening and low-attenuation mesenteric adenopathy is most suggestive of either mycobacterial infection or Whipple disease; associated central nervous system or articular disease would favor the latter.
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Figure 20. Whipple disease in a 38-year-old man with diarrhea and abdominal pain. Axial contrast-enhanced CT image shows mesenteric lymphadenopathy with a mixed soft-tissue and fatty appearance (arrowheads). Mild thickening of the small bowel wall was present at other levels.
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Mesenteric adenitis is a nonspecific diagnosis that may be considered at CT in a symptomatic patient with slightly prominent right-sided mesenteric lymph nodes (defined as three or more nodes measuring 5 mm or greater) but without an identifiable inflammatory process (Fig 21) (18). The nodes rarely exceed 10 mm in short-axis measurement. An underlying terminal ileitis is thought to represent the cause in at least some cases of mesenteric adenitis.
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Figure 21. Mesenteric adenitis in a 22-year-old man with acute right lower quadrant pain. Axial unenhanced CT image shows multiple ileocecal nodes greater than 5 mm in diameter adjacent to a normal, air-filled appendix.
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Fat-based Conditions
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There are several interesting conditions involving subperitoneal fat that typically manifest with abdominal pain but are generally self-limited in nature. Recognizing their characteristic findings at CT is important for excluding other inflammatory processes that are more threatening and might require more invasive treatment.
Epiploic appendagitis results from torsion or thrombosis of one of the many fatty appendages projecting from the colonic serosa (19). These appendages are generally indistinguishable from the adjacent intraabdominal fat at CT, but intervening ischemia or infarction renders them easily detectable. As such, epiploic appendagitis appears as hazy infiltration of the ovoid appendage at CT, with associated thickening of its visceral peritoneal lining and stranding into the surrounding fat (Fig 22). The CT findings are sufficient for specific diagnosis. Rarely, dystrophic calcification from a previously infarcted appendage may be evident (Fig 23).
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Figure 22. Epiploic appendagitis in a 28-year-old woman with acute left lower quadrant pain. Axial contrast-enhanced CT image shows soft-tissue infiltration in and around a fatty appendage (arrow) extending off the sigmoid colon.
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Figure 23. Chronic (old) epiploic appendagitis in an asymptomatic 52-year-old man undergoing screening virtual colonoscopy. Axial unenhanced CT image shows an appendage with dystrophic calcification (arrow), which is evidence of prior appendagitis.
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Mesenteric panniculitis is an inflammatory condition of unknown etiology but one that is being recognized with increasing frequency at CT (20). It represents the acute form of sclerosing mesenteritis. The predilection for segmental involvement of the jejunal mesentery is striking. CT findings include hazy mesenteric infiltration, often with discrete margins and relative sparing of the perivascular fat (Figs 24, 25). CT diagnosis generally requires exclusion of other causes of a "misty mesentery." Progression to retractile mesenteritis is difficult to predict but is fortunately rare.
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Figure 24. Mesenteric panniculitis in a 28-year-old man with vague abdominal symptoms. Axial contrast-enhanced CT image shows mesenteric soft-tissue infiltration that is characteristically localized to a jejunal segment, spares the perivascular fat, and has a pseudo-capsule appearance.
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Segmental omental infarction is a rare entity likely related to adhesions, torsion, or tenuous vascular supply to the omentum (21). Involvement of the right omental segment is the rule. CT findings range from subtle, focal hazy soft-tissue infiltration of the omentum (Fig 26) to more extensive, masslike fullness that can mimic pathologic infiltration from more ominous causes (Fig 27).
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Figure 26. Segmental omental infarction in a 40-year-old man with acute right-sided abdominal pain. Axial contrast-enhanced CT image shows localized soft-tissue infiltration (arrowhead), which represents a subtle example of right-sided omental infarction.
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Figure 27. Segmental omental infarction in a 42-year-old man with acute right lower quadrant pain. Axial contrast-enhanced CT image shows a localized masslike process (arrowhead), which represents a more advanced example of right-sided omental infarction.
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Conclusions
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A wide variety of unusual pathologic entities can involve the peritoneal and subperitoneal spaces. The differential diagnostic considerations can often be significantly narrowed when both the clinical and CT data are considered together. In certain cases, the CT findings alone allow a specific diagnosis.
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Footnotes
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The opinions and assertions contained herein are the private views of the authors and are not to be construed as official nor as reflecting the views of the Departments of the Navy or Defense.
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References
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Abstract
Introduction
