Clin J Gastroenterol (2009) 2:257–261 DOI 10.1007/s12328-009-0086-8

CASE REPORT

Sclerosing mesenteritis, a rare mesenteric disorder, in a patient with ulcerative colitis Muhammad Zakir Hossain Khan Æ Yoshiaki Takeuchi Æ Chitose Ohishi Æ Takashi Hashizume Æ Takehiko Gokan Æ Michio Imawari

Received: 27 January 2009 / Accepted: 27 March 2009 / Published online: 2 May 2009 Ó Springer 2009

Abstract A 60-year-old female who had been ill with ulcerative colitis for more than ten years presented with upper abdominal pain. A flare-up of ulcerative colitis was unlikely, because she did not report rectal bleeding, altered bowel habit, and changes of stool form. A poorly defined mass with mild tenderness was palpable in the upper abdomen, with increased levels of serum pancreatic enzymes, leading us to suspect pancreatic disease. Although CT scan revealed no abnormalities in the pancreas, a well-defined, heterogeneous soft tissue mass was found in the small bowel mesentery. Although several different diagnoses were considered, characteristic features on CT strongly supported diagnosis of sclerosing mesenteritis. The symptoms resolved quickly without specific treatment. The mesenteric lesion has never changed and no unfavorable events have yet occurred.

bowel mesentery [1]. It occasionally involves the large bowel mesentery, the peripancreatic and omental fat, and the retroperitoneal/pelvic fat [2]. Numerous terms have been used to describe this condition, including retractile mesenteritis (predominant fibrosis) [3], mesenteric panniculitis (marked chronic inflammation) [4], and mesenteric lipodystrophy (predominant fatty degeneration and necrosis) [5], on the basis of their predominant histology. Although it may be possible to differentiate these entities on the basis of their histological characteristics, accumulation of case series studies suggests that all the terms may represent a spectrum of a single entity, and SM may be the most preferred term [6]. In this report, we present a case of SM in a patient with ulcerative colitis and emphasize the importance of CT in diagnosis and follow-up of this rare condition. We also discuss different diagnoses by reviewing some literatures.

Keywords Sclerosing mesenteritis  Ulcerative colitis  Abdominal CT

Case report

Introduction Sclerosing mesenteritis (SM) is an inflammatory and fibrotic disorder of unknown etiology that primarily affects the small

M. Z. H. Khan  Y. Takeuchi (&)  C. Ohishi  M. Imawari Department of Gastroenterology, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawaku, Tokyo 142-8666, Japan e-mail: [email protected] T. Hashizume  T. Gokan Department of Radiology, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawaku, Tokyo 142-8666, Japan

A 60-year-old female visited our hospital on November 2005 presenting with upper abdominal pain. She had been followed-up since 1991 because of left-sided ulcerative colitis, for which salazosulfapyridine had been given orally as maintenance therapy, and disease activity had been calm during the past four years. Oral prednisolone was also used on flare-ups of ulcerative colitis; an estimated total dosage of the corticosteroid was approximately 1200 mg. She had taken trichlormethiazide and imidapril hydrochloride for hypertension for more than ten years. At the time of presentation, she never complained about altered bowel habit or changes of stool form. No clinical evidence of bleeding from the upper gastrointestinal tract or rectal bleeding was noted. She had no history of previous abdominal surgery, trauma, or malignancy. She was non-alcoholic.

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Table 1 Laboratory data Blood cell count WBC

7,100/ll

RBC

498 9 104/ll

Hb

13.7 g/dl

Ht

28.5%

Plt

29.2 9 104/ll

ESR

23 mm/h

Urinalysis Protein

(-)

Sugar

(-)

Occult blood

(-)

Biochemistry and serology T-P

7.9 g/dl

Alb

4.4 g/dl

T-Bil ALT

0.6 mg/dl 21 IU/l

AST

16 IU/l

c-GTP

15 IU/l

Amylase

151 IU/l

Lipase

213 U/l

T-Cho

192 mg/dl

TG

299 mg/dl

Glu

95 mg/dl

BUN

9.5 mg/dl

Cre

0.7 mg/dl

Na

141.6 mEq/l

K

3.9 mEq/l

Cl

103.1 mEq/l

S-IL2R

484 U/ml

CEA

1.2 ng/ml

CA19-9 CRP

2.8 U/ml 0.4 mg/dl

She complained general malaise but was not febrile. On physical examination, a poorly defined and deeply seated mass with mild tenderness was palpable in the upper abdomen. Peritoneal irritation signs were absent. Because it was unlikely the pain was due to flare-up of ulcerative colitis, laboratory tests and CT scan were performed. As shown in Table 1, increased levels of serum pancreatic enzymes were noted; amylase and lipase were 151 IU/l (normal range 30–130) and 213 U/l (10–50), respectively. Other routine tests including complete blood counts, blood chemistry, and serology were almost normal except for an increase of erythrocyte sedimentation rate (23 mm/h). Abdominal CT using contrast media revealed no pancreatic abnormalities, however, as in Fig. 1 there was a well-defined, heterogeneous soft tissue mass with higher attenuation of the retroperitoneal fat in the root of the mesentery. The mass was surrounded by a

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Fig. 1 Abdominal CT (2006.1) revealed a heterogeneous mass in the root of the mesentery. The arrow indicates a tumoral pseudocapsule; the hyper-attenuated stripe surrounds the mass

hyper-attenuated stripe of thickness less than 3 mm, consistent with so called tumoral pseudocapsule (arrow). The mesenteric vessels were involved in the mass but the calibres of the vessels were not reduced. A halo of uninvolved fat around the vessels was found, giving rise to a ‘‘fat ring’’ sign (arrow in Fig. 2). A few lymph nodes in the mesentery were observed but they were less than 5 mm in diameter. Although pancreatitis was suspected, symptoms quickly disappeared and no specific treatment for pancreatitis, for example fluid supplementation and protease inhibitors, was needed. Colonoscopy demonstrated that ulcerative colitis was in an inactive stage. Examinations for different diagnoses were subsequently performed. Abdominal MRI did not provide further information about the mass lesion. Although it appeared from abdominal CT that the horizontal portion of the duodenum was involved, esophagogastroduodenoscopy, which could reach to a portion involved, revealed swelled folds with no difficulty of inflation. In addition, the mucosal surface of the observed area was completely normal, leading us to consider that the mass was not originating from the epithelium. Gallium scintigraphy and positron emission tomography demonstrated abnormal accumulations in the upper abdomen, which were interpreted as a non-specific effect. According to the characteristic findings on CT, we finally concluded diagnosis of SM although histological examination was not performed. With the informed consent, we have followed-up her by CT regularly. Up to December 2008 the mesenteric lesion had not changed (Fig. 3) and no unfavorable events have occurred.

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Fig. 2 A halo of uninvolved fat around the vessels is called the ‘‘fat ring’’ sign (arrow)

Fig. 3 The mesenteric mass has not significantly changed during the three-year follow-up period (2008.10)

Discussion Sclerosing mesenteritis is a rare mesenteric disorder; its prevalence is reported to be 0.6% in over 7000 abdominal CT examinations [7]. Such rarity has limited the ability to study the nature of this condition. According to case series studies, SM is diagnosed in middle-aged or

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older adults and seems to be twice as common in men as in women [1, 8]. The presenting clinical features are non-specific and protean, because symptoms are associated with the organs and/or sites of mesenteric tissue involved; direct mechanical effect of the mesenteric mass encasing bowels, blood vessels, and lymphatics may result in bowel obstruction, ischemia, and chylous ascites, respectively [8, 9]. The most common symptoms are abdominal pain, altered bowel habits, and weight loss. However, many cases may be asymptomatic; an autopsy series reported a prevalence of 1% [10]. Also, one case series showed that most patients were incidentally identified during abdominal CT [7]. No specific findings in laboratory tests have also been reported; erythrocyte sedimentation rate and an increase of C-reactive protein may be common. In this case, upper abdominal pain with increased levels of serum pancreatic enzymes appeared to be the initial symptom of SM. Abdominal CT in 1991 demonstrated normal findings, thus, onset of SM could have been insidious. We speculate that pancreatic involvement of inflammatory mass or pancreatic ischemia could be responsible for the clinical manifestation. Patients with inflammatory bowel diseases occasionally exhibit elevation of serum pancreatic enzymes which is mostly accompanied by their flare-ups [11]; the patient, however, presented no clinical signs of flare-up. Therefore, it is less likely that pancreatic abnormality was associated with ulcerative colitis. The pathophysiology of SM remains unknown. Several causative factors have been suggested including previous abdominal surgery or trauma, autoimmunity, paraneoplastic syndrome, and infection [4, 7, 12]. We found none of these factors in this case. A possible etiological factor was ulcerative colitis; further studies are required to address the association of ulcerative colitis and SM, however. Diagnosis of SM is established by histological examination of a patient presenting radiologic findings consistent with SM [1]. However diagnostic value of histological examination remained to be established. In addition, the mostly benign and asymptomatic nature of SM may not justify biopsy in all cases [13]. Daskalogiannaki et al. reported that only four out of 49 patients had a biopsy proven diagnosis [7]. Akram et al. recently mentioned that few patients would require exploratory surgery unless indicated by intractable complications of SM, or if there was high clinical suspicion of an alternative diagnosis [8]. Thus, most of cases of SM may be diagnosed by noninvasive radiologic modalities, as we did in this case. CT is the most reliable and feasible modality for detecting SM. However, CT findings of SM depend on the extent of inflammation and fibrosis [7, 10]. Therefore,

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careful follow-up may be recommended unless single CT gives us informative suggestion of SM. In this case, the fat ring sign and the tumoral pseudocapsule, both of which appear to be hallmarks of SM [7], led us to conclude diagnosis, although consensus of their specificity has not been achieved. There are many differential diagnoses for mass lesions of the mesentery including malignant lymphoma, carcinoid tumor, peritoneal carcinomatosis, desmoid tumor, retroperitoneal fibrosis, and liposarcoma [2, 14]. Bulky homogeneously enhancing masses that often envelope mesenteric fat and vessels, termed the ‘‘sandwich sign’’ [15], large heterogenous masses with central areas of necrosis displacing small bowel loops [6], and small bowel wall thickening with aneurismal dilatation may all prompt consideration of lymphoma. Large retroperitoneal lymph nodes are also suggestive of lymphoma whereas lymphadenopathy in SM is usually less than 5 mm in size [9, 16]. Carcinoid tumor may present as an ill-defined, infiltrating soft tissue mass in the root of the mesentery with associated calcification and desmoplastic reaction [9]. Urinary 5-hydroxyindoleacetic acid levels may also be helpful [9]. Peritoneal carcinomatosis is usually associated with enhancing omental nodule and ascites [9]. Desmoid tumors are usually related to prior surgery or trauma and are often found in association with Gardner’s syndrome and colonic polyposis [2, 17]. Desmoid tumors tend to be multifocal and lack the dense sclerosis commonly found in SM. Other modalities that are helpful for diagnosis of SM may be MRI, radiographic examination, and endoscopy, although their diagnostic value is currently unknown. We performed esophagogastroduodenoscopy, which revealed no pathologic condition. This may be reasonable because SM is primarily a retroperitoneal disease and, therefore, mucosal lesion is secondary to the surrounding mesenteric lesion. However, our evaluation may not be enough to completely evaluate the mucosal lesion of the involved intestine, because neither enteroscopy nor capsule endoscopy were performed. Because of a lack of standard therapy, treatment decisions are guided by anecdotal experience and small case series. According to the recently proposed treatment algorithm [7], no treatment is necessary in asymptomatic and nonprogressive case. Symptomatic and progressive forms of the disease can be treated with tamoxifen, corticosteroid, azathioprine, or colchicine. In refractory cases, for example bowel obstruction, surgery may be indicated [8]. Particularly, colonic involvement have been suggested to present a more aggressive course and require surgery more often [1]. However, attempted surgical resection or debulking does not relieve symptoms or prevent disease progression, because only 10% patients improved after surgery alone [8]. Fortunately, we have not experienced

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any clinical problems associated SM during the past three years in this case although careful follow-up should be mandatory. In conclusion, we diagnosed SM in a patient with ulcerative colitis. The etiologic factor(s) in this case was not known. CT was a useful modality for diagnosis and for monitoring of disease progression. Although the clinical course has not been problematic, we need to follow-up carefully. Because our understanding of this and/or related conditions may be poor, accumulation of clinical data from more cases is essential. Initially, establishment of a consensus, widely acceptable term will be required to avoid confusion of nomenclature describing this mesenteric fibroinflammatory condition.

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Sclerosing mesenteritis, a rare mesenteric disorder, in a patient with ulcerative colitis.

A 60-year-old female who had been ill with ulcerative colitis for more than ten years presented with upper abdominal pain. A flare-up of ulcerative co...
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