Typhlitis, also known as ileocaecal syndrome or neutropenic colitis, is an acute inflammation of the caecum, appendix and occasionally terminal ileum. It is primarily described in children with leukemia and a severe neutropenia. Histologically the changes are of oedema and ulceration of the entire bowel wall, with transmural necrosis and perforation possible. It is thought to be due to a leukemia or lymphoma-tous infiltrate, ischaemia, focal pseudo membranous colitis and/or infection. The most common organisms found include cytomegalovirus virus, Pseudomonas, Candida, Klebsiella, E Coli, B Fragilis and Enterobacter. Whilst being most common in childhood leukemia, it is also seen in aplastic anaemia, lymphoma and during immunosuppressive therapy. Children present with abdominal pain (which may be localized to the right iliac fossa), watery diarrhoea, a palpable mass in the right iliac fossa, fever in neutropenia or with bloody stools.
The caecum and ascending colon are the most common sites of involvement, the appendix and the terminal ileum may be secondarily involved. The plain abdominal radiograph may show distention of nearby small bowel loops, possibly a fluid-filled mass-like density in the right iliac fossa, representing the caecum, thumb printing of the ascending colon and thickening of the caecal wall to greater than 4 mm. Typhlitis may be associated with pneu-matosis coli which is a common finding in markedly immunosuppressed children. Ultrasound examination can be either by a curvilinear or a linear probe. The circumferential thickening of the caecal wall, and possibly the terminal ileum, will be seen. There may be adjacent oedema. Some centers advocate the use of CT where the main findings will again be of
wall thickening of the caecum, decreased bowel wall attenuation due to oedema, streaking and stranding of the adjacent fat and thickening of the fascial planes, as well as fluid around the colon associated with intramural pneumatosis.
Contrast studies should not be attempted due to the risk of perforation. In the absence of perforation surgery is not indicated.
The differential diagnosis includes appendicitis with an appendicular abscess, inflammatory bowel disease or leukaemic deposit (in which case the wall thickening is likely to be eccentric) (Alexander et al. 1988) (Fig. 6.14).
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