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home - Miscellaneous - Other - Sclerosing Mesenteritis Written by Dr Sebastian Zeki
Knowledge


Defines the pathophysiology of fluid and nutrient malabsorption,
including causes, e.g. anatomical and functional short bowel
syndrome, high output stomas, enterocutaneous fistulae and
pancreatic insufficiency

Knows how to investigate patients with malabsorption
Describes the clinical consequences of malabsorption, including
malnutrition, fluid and electrolyte disturbance and micronutrient
deficiency and anaemia and how to manage these

Describes all other causes of anaemia, including bone marrow
disorders and haemolysis

Describes the metabolism, absorption and bioavailability of iron, B12
and folate and clinical conditions and diets associated with their
deficiency

Skills
Identifies and appropriately investigates clinical features suggestive of
malabsorption

Manages fluid, electrolyte and micronutrient disturbances associated
with short bowel syndrome or high output stomas

Uses the appropriate investigations for the different types of anaemia
Behaviours
Takes a careful clinical approach to managing patients with
malabsorption and anaemia. Explains plan of management clearly to
patients and their relatives.

Also...

Knowledge


Knows the different causes of enteric dysmotility (myopathy and
neuropathy) and their presenting features

Have a knowledge of scleroderma amyloid and congenital motor
abnormalities of the gut that affect absorption

Knows the principles of investigation pain relief and prokinetic drug
treatment

Understands bacterial overgrowth and its treatment
Understands how emotional status can affect gut function
Knows how diabetic complications can affect the gut
Skills
Can determine when organic obstruction is occurring TO
Can understand the principles and interpret the results of
gastrointestinal motility investigations (including manometry transit
studies etc) and autonomic function tests
TO
Advises on appropriate prokinetic drugs and analgesics
Can detect and treat bacterial overgrowth
Advises on appropriate surgery including bypass procedures
Behaviours
Can relieve symptoms while not causing/risking harm with other
medications (e g opiates)

Works with the multidisciplinary NST psychiatrists/psychologists
surgeons and the pain management team

Can give careful explanation of the problems to the patient carers
friends and family

Sclerosing Mesenteritis

Sclerosing mesenteritisPrev: 1%M:F 2:1Median age 65 Mesenteric lipodystrophy Mesenteric panniculitis Sclerosing mesenteritis Spectrum: Abdominal surgery Abdominal traumaSurgical gloves 5% AutoimmunityAssociated with Riedel thyroiditis, PSCRetroperitoneal fibrosisOrbital pseudotumor.Autoimmune hemolytic anemiaMinimal change nephropathy, SLERelapsing polychondritis.Elevated serum IgG4 and/or autoimmune pancreatitis. Paraneoplastic syndromeMalignancy in up to 70 % - big variety of cancers Ischemia and infectionMesenteric ischaemia can cause a similar processAlso associated with a previous history of typhoid fever, dysentery, tuberculosis, syphilis, malaria, influenza, and rheumatic fever. Symptoms:75 % have abdominal pain.36 % have SBO.20 % have constipation or diarrhoea.Rectal bleeding occurs in 7 %.20 % have LOW. Physical examination:An abdominal mass in 50 %.Abdominal tenderness in 30%.Distension in about 10 to 15 %. Ascites (usually chylous) is rare. Nonspecific (hypoalbuminaemia/anaemia/CRPand ESR raised) 1.Soft tissue mass in the small bowel (often jejunal) mesentery- homogene-ous (more fibrotic lesions) or heterogeneous (more inflammatory). Mean size of 10cm 2. Fat ring sign (in 90 %) : Preservation of the densitometric values of fat nearest the mesenteric vessels.3. Tumoral pseudocapsule (60 %): hyperattenuated stripe partly surrounding the mass.4. Vascular displacement, encasement, or thrombosis is seen in over one-half of cases.5. Calcifications are present in about 20 % of lesions, probably resulting from fat necrosis.6. Cystic components is present, possibly from focal necrosis due to vascular obstruction.7. Lymphadenopathy (mesenteric or retroperitoneal) is present in 20 to 40 % of patients.8. “misty mesentery" has been used to describe the finding of increased attenuation of mesenteric fat with small lymph nodes but without evidence of a discrete mass.The increased attenuation is due to infiltration by inflammatory cells, fluid (edema, blood, lymph), tumor, or fibrosis.Thus, it is not specific for sclerosing mesenteritis, because hemorrhage, edema, or malignancy (particularly lymphoma) can present in an identical fashion. To distinguish from GIST and mesenteric fibromatosis, sclerosing mesenteritis does not stain for CD117/c-kit and beta catenin, respectively, but retains staining for smooth muscle actin. Treatment:Corticosteroids- useful especially if big inflamm-tory component.Hormonal therapy.Tamoxifen and progester-one combination may be useful.Cyclophosphamide- may be useful (trialled in 2 patients).Thalidomide- Improves CRP but not CT findings in small Aetiology Last 24 hours to two years. Laboratory findings Biopsy: CT Written by Dr Sebastian Zeki