BLOG: An 86 year old male with abdominal bloating

Cecilia C. Kelly, MD, is in her third year of Gastroenterology and Hepatology fellowship at Thomas Jefferson University Hospital. She completed her undergraduate degree at Fordham University and her medical training at Sidney Kimmel Medical College. She looks forward to practicing in Philadelphia after fellowship graduation.

Case:

An 86 year old white male with a history of polymyalgia rheumatica, coronary artery disease and umbilical hernia repair presents for evaluation of abdominal bloating. His symptoms initially developed 10 months ago, at which point he was hospitalized at an outside facility for small bowel obstruction and underwent small bowel resection and resection of a mesenteric mass.

Cecilia C. Kelly

He again complains of mild abdominal bloating. He reports normal bowel movements and denies abdominal pain. His review of systems is negative for night sweats, fever, weight change, rash and lumps or bumps.

An esophagogastroduodenoscopy (EGD) and colonoscopy a year ago prior to the onset of bloating revealed only diverticulitis.

Laboratory data:

Complete blood count (CBC), comprehensive metabolic panel, calcium, lipase, amylase are within normal limits. Erythrocyte sedimentation rate (ESR) is elevated at 18 (ULN 9). C-reactive protein (CRP) is 2.0 (ULN 2.9).

An upper GI series with small bowel follow through is normal.

Computed Tomography of the abdomen and pelvis reveals 7.6cm x 3.8cm calcified mesenteric mass (Figure 1) and a 9mm hypervascular nodule along the medial border of the proximal jejunum. Mildly distended, fluid-filled small bowel loops are present in the left lower abdomen without a clear transition point. There is small perihepatic and pelvic ascites, and mild mistiness of the mesentery to the right of midline (Figure 2).

Figure 1. 7.6cm x 3.8cm calcified mesenteric mass

Figure 2. Mild mistiness of the mesentery to the right of midline

Question: What is the diagnosis?

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Answer: Sclerosing mesenteritis.

Gastrointestinal pathologist review of prior resection tissue reveals sclerosing mesenteritis of mesenteric nodules (Figure 3A, B, C) with immunohistochemical staining positive for smooth muscle actin (Figure 3D) and Vimectin and negative for epithelial markers and Ckit.

Figure 3A

Figure 3A

Figure 3B

Figure 3C

Figure 3D

Sclerosing mesenteritis is a rare spectrum of disease characterized by three abnormalities of the mesenteric adipose tissue: necrosis, inflammation, and fibrosis. It most commonly affects the small bowel mesentery. It was first described in 1924 as “retractile mesenteritis” and has had different names, each highlighting one of the three aspects of mesenteric change: “mesenteric lipodystrophy” for the necrosis, “mesenteric panniculitis” for the inflammation, and “retractile mesenteritis” or “sclerosing mesenteritis” for the fibrosis. The natural history of the disorder includes mesenteric adipose necrosis, which progresses to chronic inflammation and then thickening and fibrosis.

The condition is thought to be a benign, self-limited disorder, which may spontaneously regress. However, some cases can be severely debilitating. Patients may be asymptomatic, or present with pain, bloating, nausea/vomiting, fatigue, weight loss or fever. Patients can develop complications including chylous ascites, small bowel obstruction, or acute abdomen. There is a male predominance with a male to female ratio of two to one. It is reported most commonly in the 6th decade, and incidence increases with age.

The etiology is unclear. It may be an idiopathic reaction to prior ischemia, infection, drugs or trauma. It may have an autoimmune component as some series have shown favorable response to immunosuppression. In some cases, underlying malignancy has been found, and here it may be thought of as a paraneoplastic syndrome. It may be part of a larger spectrum of fibrosclerotic disorders, as it has been reported with retroperitoneal fibrosis, Sjögren’s syndrome, and sclerosing pancreatitis.

Diagnosis can be suggested by imaging with finding of soft tissue mesenteric mass and the classically described “misty mesentery”. Biopsy is required for histopathologic diagnosis.

Management is not standardized and should be symptom-guided. Asymptomatic patients can be followed clinically. Patients with acute abdomen or obstruction should undergo appropriate surgical management. Non-surgical symptoms can be managed with medical therapy, most often corticosteroids plus tamoxifen. Alternate agents (azathioprine, colchicine, thalidomide) have also been used.

Key Points

Sclerosing mesenteritis is a rare condition characterized by necrosis, inflammation, and fibrosis of the adipose tissue of the mesentery.
The small bowel mesentery is most commonly affected.
Males are more commonly affected than females.
Incidence increases with age.
Findings of mesenteric mass with “misty mesentery” on computed tomography of the abdomen and pelvis can suggest the diagnosis, but confirmation should be made with biopsy.
Presentation can range from asymptomatic incidental finding to acute abdomen, and treatment should be symptom-guided.

References:

Akram S, et al. Clin Gastroenterol Hepatol. 2007;5:589-596.

Bala A, et al. Can J Gastroenterol. 2001;15:533.