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DOI: 10.1148/radiol.2282011952
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(Radiology 2003;228:452-455.)
© RSNA, 2003

Diagnosis Please

Case 61: Ileocecal Sarcoidosis1

Alain Nchimi, MD, Nadine Francotte, MD, Léon Rausin, MD and Jamil Khamis, MD

1 From the Departments of Medical Imaging (A.N., L.R., J.K.) and Pediatrics (N.F.), Clinique de l’Espérance, 447 rue Saint Nicolas, 4420 Liège, Belgium. Received November 29, 2001; revision requested February 12, 2002; revision received March 18; accepted April 2. Address correspondence to A.N. (e-mail: ahainel@yahoo.com).

Index terms: Colon, diseases, 752.22 • Diagnosis Please • Intestines, diseases, 742.22 • Sarcoidosis, 742.22, 752.22


   HISTORY
TOP
 HISTORY
IMAGING FINDINGS
 DISCUSSION
 REFERENCES
 
A 7-year-old boy with no relevant past medical history was admitted for cough and exertional dyspnea. The patient stated that the symptoms started 3 months before and were progressively worsening. He also reported a 2-kg weight loss and intermittent diarrhea during this time. The findings of a physical examination were positive for swelling of the mandibular angle on both sides, tenderness of the right lower quadrant, and decreased breath sounds at the bases of the lungs. When the patient was admitted, his rectal temperature was 97.7°F (36.5°C). Laboratory findings revealed a normal level of serum electrolytes and a normal blood cell count. Serum inflammation markers tested (C-reactive protein and fibrinogen) were not at elevated levels. Posteroanterior chest radiography (Fig 1) and abdominal computed tomography (CT) with 500 mL of orally administered 5% ioxithalamate meglumin (Telebrix Gastro; Guerbet, Aulnay-sous-bois, France) and 100 mL of intravenously administered 300mg/mL iohexol (Omnipaque; Nycomed, Cork, Ireland) (Fig 2) were performed at admission. Gallium 67 (67Ga) whole-body scanning (Fig 3) was performed 24 hours after injection, on the day after admission.



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  		Figure 1. Posteroanterior chest radiograph obtained at admission. Diffuse, bilateral perivascular opacities in the middle and lower zones of the lungs are associated with focal areas of consolidation (curved arrow), peripheral nodules (straight arrows), and bilateral hilar enlargement (arrowheads).

 


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  		Figure 2a. Transverse CT images of the lower abdomen obtained at admission, after administration of oral and intravenous contrast material. (a) Marked circumferential thickening of the terminal ileum (arrowheads) is seen, along with preserved wall stratification. (b) Image obtained at a lower level than a shows a markedly thickened cecal wall (arrowheads).

 


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  		Figure 2b. Transverse CT images of the lower abdomen obtained at admission, after administration of oral and intravenous contrast material. (a) Marked circumferential thickening of the terminal ileum (arrowheads) is seen, along with preserved wall stratification. (b) Image obtained at a lower level than a shows a markedly thickened cecal wall (arrowheads).

 


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  		Figure 3. 67Ga whole-body scan obtained 24 hours after injection, on the day after admission, shows marked uptake in lungs (*), nose (straight arrow), and lacrimal and parotid glands (upper and lower curved arrows, respectively) and moderate uptake in the hemipelvis (arrowheads). The midline pelvic activity represents the bladder (B).

 

   IMAGING FINDINGS
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 HISTORY
 IMAGING FINDINGS
DISCUSSION
 REFERENCES
 
Chest radiography (Fig 1) revealed bilateral diffuse parenchymal air-space and interstitial opacities, as well as peripheral nodules. These findings were predominant in the middle and lower zones of the lungs. There was associated bilateral hilar lymph node enlargement, and lung volumes were normal. Abdominal CT (Fig 2) showed marked symmetric wall thickening of the terminal ileum and cecum, with preserved wall stratification (layers visible). On the day after the patient was admitted and 24 hours after injection, 67Ga whole-body scintigraphy (Fig 3) revealed marked bilateral and symmetric uptake in the lungs and the parotid and lacrimal glands and moderate uptake in the right hemisphere of the pelvis. The appearance of the skeleton, mediastinum, spleen, and liver was normal.


   DISCUSSION
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 HISTORY
 IMAGING FINDINGS
 DISCUSSION
REFERENCES
 
Abdominal CT and chest radiographic findings explained the long-standing symptoms, but when taken alone, neither was specific for a disease. Their association suggested a systemic infiltrative disease that involved both the lungs and the gastrointestinal tract in a nonimmunocompromised child. Lymphoma, chronic inflammatory bowel disease, tuberculosis, and sarcoidosis had to be considered.

The ileum trails the stomach as the second most frequent site of involvement in gastrointestinal tract mucosa-associated lymphoid tissue lymphomas (1), which may also affect the lungs, mediastinum, and hilar lymph nodes. These gastrointestinal tract tumors manifest as eccentric mural masses on cross-sectional (2) or endoscopic images (3), however, which contradicts the present case. Moreover, associated air-space opacities are rare.

Lung and airway diseases are uncommon in patients with chronic inflammatory bowel disease. Reported air-space opacities occurred as part of the granulomatous disease (4) or as a complication of the immunosuppressive treatment. Active chronic inflammatory bowel disease is virtually always associated with an elevated level of at least one of the serum inflammation markers (5). Serum inflammation markers were at normal levels in this patient.

Lower and middle zone predominance of lung opacities associated with hilar lymph node enlargement can be seen in patients with primary tuberculosis (6) and sarcoidosis (7,8). The ileocecal area is the most common site of gastrointestinal tract involvement of tuberculosis (9). Cases of gastrointestinal sarcoidosis in the ileocecal area have been reported, although they are far less common than tuberculosis (10,11). Since sarcoidosis and tuberculosis were not distinguishable with chest and abdominal findings alone, 67Ga scintigraphy was used and demonstrated bilateral uptake in the parotid gland, lacrimal gland, and nose, which resulted in the panda sign (12). Facial uptake of 67Ga correlates to the known high frequency of parotid glands and orbital involvement in sarcoidosis (13). Indeed, this pattern of uptake seems to represent the imaging findings of Heerfordt syndrome and uveo-parotid fever, both of which are clinical syndromes related to sarcoidosis with associated parotiditis and uveitis, the former with facial nerve palsy and the latter with fever (14). In our patient, however, the eyes demonstrated no symptoms, and the patient’s temperature was normal.

The panda sign is not specific for sarcoidosis and has been associated with other diseases, such as treated lymphomas, systemic lupus erythematosus, and Sjögren syndrome. Sulavik et al (15), however, showed that association of this sign with lung infiltrates yielded 100% specificity for the diagnosis of sarcoidosis. The most likely diagnosis, therefore, is systemic sarcoidosis.

Specific tests performed to confirm the diagnosis further showed moderately elevated levels of serum angiotensin-convertase enzyme (62.9 U/L; normal value range, 8–52 U/L) and urinary calcium (0.25 mmol/kg/d; normal value range in children, 0.05–0.1 mmol/kg/d). Transbronchial lung biopsies and bronchioloalveolar lavage revealed nonspecific lymphocytic infiltrates. A diagnosis of noncaseating granulomatous disease was made with surgical mesenteric lymph node biopsy.

Sarcoidosis is a relatively common disease of unknown cause. The prevalence of this disease has sex, racial, geographic, and age variations. It is more common in females, blacks, and people who live in a temperate climate during their 3rd and 4th decades, and it may develop in old or young patients. The estimated prevalence of this disease is between one in 100,000 and 64 in 100,000 children in the United States (16). Sarcoidosis behaves the same both in children and in adults. In biopsy specimens, the presence of this disease is suggested by typical noncaseating granulomas containing multinucleated giant cells, with no identification of infectious or inflammatory agents. Microscopic thoracic involvement is ubiquitous and determines the prognosis of the disease. Typical imaging findings include diffuse opacities of sarcoid micronodules with subpleural and peribronchovascular distribution with or without hilar or mediastinal lymph node enlargement (7,8). CT is superior to chest radiography in the detection both of enlarged lymph nodes and of micronodular infiltrates. Nodular and macronodular patterns are common, and they may be associated with ground-glass opacities or air-space consolidation. Occasionally, nodules may cavitate (17).

Virtually every organ may be involved by the disease. Intraabdominal involvement is less common than involvement of the eyes, skin, heart, musculoskeletal system, and salivary glands. The liver, spleen, and lymph nodes are the most frequently involved abdominal organs. Mentions of gastrointestinal tract sarcoidosis and corresponding radiologic descriptions are few (10,11,18), with the stomach being the most common site of involvement in the gastrointestinal tract, followed by the colon. Barium studies may reveal plaque-like lesions, ulcers, fold thickening, focal nodularity, or segmental narrowing. CT may show segmental thickening. These findings are not specific, and their differential diagnosis includes chronic inflammatory bowel disease, infectious enteritis, tuberculosis, lymphoma, and carcinoma. Although sarcoidosis has few distinctive radiologic features for each site of involvement, knowledge of them in unknown cases involving many organs may suggest the diagnosis, as in the present case.

Other findings are reported to be helpful in the diagnosis of sarcoidosis, such as elevation of serum angiotensin convertase enzyme levels, calciuria and phosphaturia, and positive reaction to intradermal administration of a preparation of sarcoid tissue (Kveim-Siltzbach test). The prognosis of patients with this disease is related to the severity of lung involvement, and mortality occurs in less than 5% of patients (19). Except for fibrosis, all stages of the disease are reversible and respond favorably to steroid therapy or immunosuppressive agents.


   FOOTNOTES
 
Part 1 of this case appeared 4 months previously and may contain larger images.


   REFERENCES
TOP
 HISTORY
 IMAGING FINDINGS
 DISCUSSION
 REFERENCES
 

  1. Gurney KA, Cartwright RA, Gilman EA. Descriptive epidemiology of gastrointestinal non-Hodgkin’s lymphoma in a population-based registry. Br J Cancer 1999; 79:1929-1934.[CrossRef][Medline]
  2. Matsumoto Y, Matsumoto T, Nakamura S, et al. Primary ileal plasmacytoma arising in mixed low- and high-grade B-cell lymphoma of mucosa-associated lymphoid tissue type. Abdom Imaging 2000; 25:139-141.[CrossRef][Medline]
  3. Hasegawa N, Tsuboi Y, Kato K, et al. Endoscopic diagnosis of ileocecal mucosa-associated lymphoid tissue lymphoma. Gastrointest Endosc 1999; 50:115-117.[CrossRef][Medline]
  4. Mahadeva R, Walsh G, Flower CD, Shneerson JM. Clinical and radiological characteristics of lung disease in inflammatory bowel disease. Eur Respir J 2000; 15:41-48.[Abstract]
  5. Macfarlane PI, Miller V, Wells F, Richards B. Laboratory assessment of disease activity in childhood Crohn’s disease and ulcerative colitis. J Pediatr Gastroenterol Nutr 1986; 5:93-96.[Medline]
  6. Kim HY, Song KS, Goo JM, Lee JS, Lee KS, Lim TH. Thoracic sequelae and complications of tuberculosis. RadioGraphics 2001; 21:839-858.[Abstract/Free Full Text]
  7. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. Pulmonary sarcoidosis: correlation of CT and histopathologic findings. Radiology 1993; 189:105-109.[Abstract/Free Full Text]
  8. Miller BH, Rosado-de-Christenson ML, McAdams HP, Fishback NF. Thoracic sarcoidosis: radiologic-pathologic correlation. RadioGraphics 1995; 15:421-437.[Abstract]
  9. Marshall JB. Tuberculosis of the gastrointestinal tract and peritoneum. Am J Gastroenterol 1993; 88:989-999.[Medline]
  10. Sprague R, Harper P, McClain S, Trainer T, Beeken W. Disseminated gastrointestinal sarcoidosis: case report and review of the literature. Gastroenterology 1984; 87:421-425.[Medline]
  11. Bulger K, O’Riordan M, Purdy S, O’Brien M, Lennon J. Gastrointestinal sarcoidosis resembling Crohn’s disease. Am J Gastroenterol 1988; 83:1415-1417.[Medline]
  12. Kurdziel KA. The panda sign. Radiology 2000; 215:884-885.[Free Full Text]
  13. James DG, Sharma OP. Parotid gland sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2000; 17:27-32.[Medline]
  14. Fraser RS, Müller NL, Colman N, Paré PD. Sarcoidosis. In: Fraser RS, Paré PD, eds. Diagnosis of diseases of the chest. 4th ed. Philadelphia, Pa: Saunders, 1999; 1565.
  15. Sulavik SB, Spencer RP, Weed DA, Shapiro HR, Shiue ST, Castriotta RJ. Recognition of distinctive patterns of gallium-67 distribution in sarcoidosis. J Nucl Med 1990; 31:1909-1914.[Abstract/Free Full Text]
  16. Pattishall EN, Kendig EL, Jr. Sarcoidosis in children. Pediatr Pulmonol 1996; 22:195-203.[CrossRef][Medline]
  17. Rohatgi PK, Schwab LE. Primary acute pulmonary cavitation in sarcoidosis. AJR Am J Roentgenol 1980; 134:1199-203.[Abstract]
  18. Warshauer DM, Dumbleton SA, Molina PL, Yankaskas BC, Parker LA, Woosley JT. Abdominal CT findings in sarcoidosis: radiologic and clinical correlation. Radiology 1994; 192:93-98.[Abstract/Free Full Text]
  19. Marcille R, McCarthy M, Barton JW, Merten DF, Spock A. Long-term outcome of pediatric sarcoidosis with emphasis on pulmonary status. Chest 1992; 5:1444-1449.

Congratulations to the 41 individuals who submitted the most likely diagnosis (sarcoidosis) for Diagnosis Please, Case 61. The names and locations of the individuals, as submitted, are as follows:
Antonio Botero, MD, Bogotá, Colombia
Eric L. Bressler, MD, Minnetonka, Minn
Michael P. Buetow, MD, Okemos, Mich
James W. Cole, MD, Cincinnati, Ohio
André Noël Dardenne, MD, Brussels, Belgium
Jon De Witte, Phoenix, Ariz
Mustafa Kemal Demir, MD, Ataköy, Istanbul, Turkey
T. Dhurairaj, Pennsauken, NJ
Dra Estela Di Nella, Mar del Plata, Argentina
María Jesús Díaz Candamio, MD, A Coruzña, Spain
Gabriel C. Fernández Pérez, Vigo, Spain
Ricardo B. Fonseca, MD, São Paulo, Brazil
Arie Franco, Livingston, NJ
Ann S. Fulcher, MD, Richmond, Va
Mark Goldshein, MD, Andover, Mass
Mario Laguna, West Allis, Wis
Margaret H. Lee, MD, Los Angeles, Calif
Peter Miltner, MD, Heidelberg, Germany
Karl F. R. Neufang, MD, Euskirchen, Germany
Mike O’Loughlin, MD, West Hartford, Conn
Sanford M. Ornstein, MD, Phoenix, Ariz
David M. Panicek, MD, New York, NY
Mario Pliego, MD, Bloomington, Minn
Rubem Pochaczevsky, MD, Bronx, NY
Lisa K. Quane, MD, Orange, Calif
Shawn P. Quillin, MD, Charlotte, NC
Danny Rappaport, Etobicoke, Ontario, Canada
Stephen Irwin Schabel, MD, Charleston, SC
Steven M. Schultz, MD, Fort Worth, Tex
Mustafa Secil, MD, Izmir, Turkey
Matt Shapiro, MD, Charlottesville, Va
Mladen Sostaric, Brezice, Slovenia
Joerg Stattaus, MD, Essen, Germany
Kouichi Sugiyama, Hamamatsu, Japan
Denis Tack, MD, Charleroi, Belgium
Douglas L. Teich, MD, Brookline, Mass
Hiroyuki Ueda, Kyoto, Japan
Christopher Vittore, MD, Rockford, Ill
David Warshauer, MD, Chapel Hill, NC
Stanko Yovichevich, MD, Sydney, Australia
Joe Yut, Olathe, Kan





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