Abstract
A 6 year old female with symptoms of small bowel obstruction underwent an exploratory laparotomy which revealed widespread evidence of inflammatory fibrotic adhesions involving the jejunal mesentery. In view of persistent growth failure, chronic anaemia, elevated acute phase reactants and imaging evidence of a diffuse progressive inflammatory process, the child was treated with corticosteroids and methotrexate with complete response. The literature on juvenile idiopathic sclerosing mesenteritis has been reviewed.
Highlights
Idiopathic sclerosing mesenteritis (ISM) is an uncommon disease involving the small bowel mesentery and characterised by chronic inflammatory changes progressing to fibrosis
The first reported series featured 34 cases of “retractile mesenteritis and mesenteric sclerosis"; published in 1924 [1,2,3]. It has been described variously as fibrosing mesenteritis, retractile mesenteritis, liposclerotic mesenteritis, xanthogranulomatous mesenteritis, mesenteric Weber Christian disease and systemic nodular panniculitis [4]. These names possibly reflect the underlying pathology involved with mesenteric lipodystrophy, mesenteric panniculitis and retractile mesenteritis being the common histologic variants
We describe a case of ISM in a young girl who was treated successfully with corticosteroids and methotrexate, and briefly review the pediatric literature on this subject
Summary
Idiopathic sclerosing mesenteritis (ISM) is an uncommon disease involving the small bowel mesentery and characterised by chronic inflammatory changes progressing to fibrosis. No evidence of mono-clonality was seen on flow cytometry for immuno-phenotyping of lymph node tissue Three months later, she moved interstate and presented to the gastroenterology clinic at our hospital with continued weight loss and an exacerbation of abdominal pain and vomiting of fecal matter. A diagnosis of idiopathic sclerosing mesenteritis was made and the child was treated with 3 daily pulses of methyl prednisolone therapy repeated monthly for 3 months She made a remarkable and rapid clinical recovery with marked reduction in fatigue, abdominal pain and resolution of fever within 2 weeks. At 6 months follow - up from the onset of treatment, she had gained 3 kgs, was completely asymptomatic and her laboratory investigations remained normal Her repeat CT scan this time had revealed resolution of the lymphadenopathy with normal mesentery (Figure 1b).
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