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Diagnosis Please |
1 From the Department of Radiology, Mayo Clinic, Rochester, Minn. Received August 27, 2001; revision requested October 17; revision received February 5, 2002; accepted February 28. Address correspondence to B.J.H., Department of Diagnostic Radiology, Saint Mary’s Hospital, 1216 Second St SW, MB-M664E, Rochester, MN 55902 (e-mail: huppert.bonnie@mayo.edu).
Index terms: Celiac disease, 792.7611 • Diagnosis Please • Mesentery, neoplasms, 792.31 • Lymphatic system, abnormalities, 99.7611 • Lymphatic system, CT, 99.12912 • Lymphatic system, US, 99.1298
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HISTORY |
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 TOP HISTORY IMAGING FINDINGSDISCUSSIONREFERENCES |
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The patient was thin, but the results of a physical examination were otherwise unremarkable. Pertinent laboratory tests revealed signs of hyposplenism, with presence of target cells and Howell-Jolly bodies on a peripheral smear. Her level of serum albumin was low at 20 g/L (normal range, 34-48 g/L). Serum gliadin immunoglobulin A antibody titers were positive, which suggested recent exposure to gluten. Repeat small-bowel endoscopy with jejunal biopsies revealed a malabsorption pattern with villous atrophy and chronic inflammation, findings that are consistent with changes of celiac disease. Computed tomographic (CT) images of the abdomen and pelvis were obtained (Fig 1). Transverse ultrasonography (US) of the abdomen was subsequently performed (Fig 2).
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IMAGING FINDINGS |
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DISCUSSION |
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Extraintestinal manifestations are common, including iron-deficiency anemia (1–4). Idiopathic osteopenia and exocrine pancreatic dysfunction may be present. Recurrent oral aphthous ulcers and dental enamel defects have been reported in 10%–40% of adult patients. Neurologic symptoms are well documented and include peripheral neuropathy, ataxia, dementia, and seizures. Dermatitis herpetiformis, a pruritic bullous skin rash, is a common skin manifestation of gluten intolerance and may be the only symptom.
Patients with celiac disease have an increased risk of developing malignant neoplasms with a reported incidence as high as 14% (5,6). Approximately 50% of these are lymphomas (5,7). The most common lymphoma is enteropathy-associated T-cell lymphoma of the small intestine, which accounts for 85%–90% (5,7). Prior to the discovery that T-cell lymphocytes represented the malignant cell type involved, enteropathy-associated T-cell lymphoma was classified as reticulum cell sarcoma or malignant histiocytosis. Patients with enteropathy-associated T-cell lymphoma can present with refractory symptoms, despite adherence to a gluten-free diet. Extraintestinal lymphomas, the majority of which are Hodgkin lymphomas, have also been reported.
Nonlymphomatous malignancies of the gastrointestinal tract account for 24% of all malignancies reported. Adenocarcinoma of the small intestine is the second most common malignancy associated with celiac disease and accounts for 7% of the total, while squamous cell carcinoma of the esophagus accounts for 4% (5,7). Malignancies of the oropharynx, ovaries, testicles, thyroid, breast, and lungs also have a reported association (2,5,7,8).
A diagnosis of celiac disease is based on the combination of clinical presentation, a jejunal biopsy with typical histopathologic features of the disease, and a positive clinical response to a gluten-free diet (1,2). Recently, serologic testing for the presence of antibodies to gliadin and endomysium has proved useful in screening, monitoring compliance, and diagnostic confirmation (1,2,4,9). There is now greater clinical awareness of the prevalence of celiac disease. Celiac disease was previously classified as a pediatric condition and thought to be uncommon in adults; however, some estimates now suggest an incidence of 1 per 200–500 people in the general adult population, with some regional variations (1,3,4).
CMLNS is a documented but uncommon and poorly understood complication of celiac disease (10–14). To our knowledge, reported cases of CMLNS have occurred only in patients with confirmed celiac disease or in patients whose clinical features were highly suggestive of celiac disease. In reported cases of CMLNS in which celiac disease was suspected but not confirmed with a clear response to a gluten-free diet, the syndrome manifested as advanced intestinal ulceration, which makes diagnosis of celiac disease difficult (14). These cases were also reported in the literature prior to the development and availability of confirmatory serologic testing.
Patients with CMLNS often present with refractory weight loss, fatigue, and diarrhea in the clinical setting of celiac disease. Villous atrophy is invariably present at small-bowel biopsy. Clinical signs of hyposplenism may be evident, with target cells and Howell-Jolly bodies present in the peripheral blood smear as a result of underlying splenic atrophy (12,14). Prior to the availability of modern cross-sectional imaging, diagnosis was made with a histopathologic examination at exploratory laparotomy or autopsy (10–12,14). Multiple cystic masses containing thin, milky fluid or thick, creamy material are typically present along the jejunoileal mesentery. Histopathologic examination reveals the mesenteric masses to be pseudocystic lymph nodes with a central cavity containing chylous fluid and a thin peripheral rim composed of fibrous material and scant elements of atrophic lymph node structures. Cavitary changes are confined to the mesenteric nodal chain, without evidence of malignancy or mycobacterial infection.
With the advent of cross-sectional imaging, typical imaging findings of CMLNS have been reported. At CT examination, multiple cystic mesenteric masses are identified that have central low attenuation, indicating fluid, and occasionally fatty material (11–14). Masses are variable in size, ranging from 2 to 7 cm in diameter. Splenic atrophy is common. At US, the mesenteric masses are cystic in appearance (10,11). Imaging is remarkable for the absence of mediastinal, retroperitoneal, or inguinal adenopathy.
The pathogenesis of CMLNS is poorly understood. The mesenteric lymphatics that drain the small bowel are called lacteals and serve a unique function, allowing the absorption of emulsified fat into the lymph. This specialized lymph is termed chyle. It is known that patients with celiac disease may have benign hypertrophy of mesenteric lymph nodes, which relapses and remits both over time and with treatment (13,15). In some patients, however, it is thought that these benign nodes may go on to cavitate, resulting in atrophy of the lymphoid tissue (10,12,14). Interestingly, only the mesenteric nodal chain supplying the small intestine is involved. It is postulated that this may be related to chronic, excessive antigenic exposure of the immune system via damaged intestinal mucosa, resulting in depletion of cellular lymph node elements and splenic atrophy (10,12,14). Cavitary nodal changes may also reflect disturbance of the usual mesenteric lymphatic drainage. Whatever the reason, nodal cavitation may occur and progress, even with institution of a gluten-free diet (10,12).
The importance of diagnosing CMLNS is multifold. Clearly, it must be distinguished from other clinical entities, including lymphoma. Unlike CMLNS, adenopathy in untreated lymphoma is usually of soft-tissue attenuation, and it is rarely confined solely to the mesenteric nodal chains. Splenic atrophy is atypical, but splenomegaly is common. Metastatic nodal involvement is also unlikely in the absence of preexisting malignancy, although patients with celiac disease do have an increased risk of developing malignant neoplasms.
Mycobacterial infections, especially tuberculosis, can manifest as low-attenuation abdominal adenopathy; however, with the mesenteric cyst, aspirates negative for mycobacterial growth, and absent clinical signs and symptoms of infection, this is an unlikely explanation.
Low-attenuation adenopathy can be seen in patients with Whipple disease, with nodes of fluid or fat attenuation. These may be present in the mesentery and retroperitoneum and have been described as echogenic on US images (16,17). Unlike the patient in our study, these patients are typically male and have arthralgias, generalized lymphadenopathy, thick small-bowel folds, and polyserositis. In the setting of known celiac disease, the presence of multiple chylous, cystic mesenteric masses is most compatible with the diagnosis of CMLNS.
Diagnosis of CMLNS is also important as a prognostic indicator. CMLNS has been associated with an alarmingly poor prognosis, and some reports estimate 50% mortality (12,14, 15). Many patients die of complications of cachexia and intestinal hemorrhage. Others are prone to overwhelming sepsis, often due to infections commonly associated with clinical hyposplenism, such as pneumococcal infection. Medical treatments have included immediate institution of a strict gluten-free diet, correction of electrolyte abnormalities, use of steroids, and pneumococcal vaccination—all with variable clinical efficacy. Reports suggest the cysts may recur after surgical aspiration (10,11). Cyst recurrence has not been documented after surgical resection of the affected mesentery and bowel; however, the clinical effect of surgery is not clear and has been used mainly for diagnosis or treatment of refractory intestinal ulceration and hemorrhage. Some authors have suggested the diagnosis of CMLNS can be made with imaging and imaging-guided aspiration of cystic mesenteric masses (11,12). Thus, patients may be spared the discomfort, morbidity, expense, and uncertain clinical benefit of exploratory laparotomy. It is also possible that imaging-guided aspiration of some of the larger cysts may produce temporary relief of symptoms (10,11).
CMLNS is an uncommon complication of celiac disease, and the diagnosis is suggested by the combination of clinical history and imaging findings. Differentiation from other disease entities is important. With greater clinical awareness of the prevalence of celiac disease, knowledge of its imaging findings and potential complications has become increasingly important.
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FOOTNOTES |
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There were two individuals who submitted the highest number of most likely diagnoses for cases 46–60. The names of these people will be announced in an upcoming issue of the journal.
This article has been cited by other articles:
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  P. T. Johnson, K. M. Horton, and E. K. Fishman Nonvascular Mesenteric Disease: Utility of Multidetector CT with 3D Volume Rendering RadioGraphics, May 1, 2009; 29(3): 721 - 740. [Abstract] [Full Text] [PDF]
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 M. K. Demir, D. P. Solis, and L. H. Luyando Diagnosis Please Comment Radiology, January 1, 2007; 242(1): 319 - 319. [Full Text] [PDF]
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 B. J. Huppert, M. A. Farrell, A. Kawashima, and J. A. Murray Diagnosis of Cavitating Mesenteric Lymph Node Syndrome in Celiac Disease Using MRI Am. J. Roentgenol., November 1, 2004; 183(5): 1375 - 1377. [Full Text] [PDF]
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L. Mendez-Uriburu, J. Ahualli, J. Mendez-Uriburu, M. Mendez-Uriburu, L. Fajre, F. Mendez-Uriburu, and R. Carabajal CT Appearances of Intraabdominal and Intrapelvic Fatty Lesions Am. J. Roentgenol., October 1, 2004; 183(4): 933 - 943. [Full Text] [PDF]
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