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Author: Thomas M Stoehr, MD, Staff Physician, Department of Diagnostic Radiology, Oregon Health Sciences University

Coauthor(s): D Bradley Koslin, MD, Associate Professor, Department of Diagnostic Radiology, Director of Body Imaging, Oregon Health Sciences University

Editors: Eric P Weinberg, MD, Associate Professor, Department of Radiology, University of Rochester Medical Center, Strong Memorial Hospital; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Spencer B Gay, MD, Professor of Radiology, Director of Body Computed Tomography, Department of Radiology, University of Virginia Health Sciences Center; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Eugene C Lin, MD, Consulting Staff, Department of Radiology, Virginia Mason Medical Center

Author and Editor Disclosure

Synonyms and related keywords: neutropenic colitis, necrotizing enterocolitis, ileocecal syndrome, cecitis

Background

Typhlitis means inflammation of the cecum. In 1960, Bierman and Amronin first coined the term ileocecal syndrome to describe inflammation and/or necrosis of the cecum, appendix, and/or ileum in patients with leukemia. Typhlitis subsequently has been associated with aplastic anemia, lymphoma, AIDS, and immunosuppression following renal transplantation or during treatment of malignancy.

Pathophysiology

Pathologically, typhlitis represents inflammation and/or necrosis of the cecum, appendix, and/or ileum. The etiology of typhlitis is unknown, but its pathogenesis is multifactorial. Profound neutropenia, with total neutrophil counts of less than 1000/µL, appears to be a universal predisposing factor. Mucosal injury from cytotoxic drugs plays an important role in the typhlitis observed during chemotherapy.

Cecal distension in typhlitis may impair the blood supply, leading to mucosal ischemia and ulceration. Infection may be involved, especially cytomegalovirus. Bacterial invasion leads to transmural penetration and, ultimately, perforation. Mucosal and submucosal necrosis can result in intramural hemorrhage. Neoplastic infiltration may be involved in some patients.

Frequency

United States

Typhlitis was found in 10% of leukemic children who died while undergoing chemotherapy.

Mortality/Morbidity

The mortality rate averages 40-50%, which is usually attributable to cecal perforation, bowel necrosis, and sepsis.

Sex

Prevalence is equal in males and females.

Age

Typhlitis occurs in both children and adults.

Anatomy

The maximum normal colonic wall thickness on CT is 3 mm. When the colon is distended with stool, fluid, or oral contrast, the normal colonic wall is nearly imperceptible. Pericolonic fat should demonstrate homogeneous fat attenuation.

Typhlitis is usually confined to the cecum, appendix, and terminal ileum; however, it can cause a pancolitis extending distally from the cecum.

Clinical Details

Typical presenting symptoms (of which time course and severity can vary considerably) include the following:

  • Watery or bloody diarrhea
  • Fever
  • Nausea
  • Vomiting
  • Abdominal pain (may be localized to right lower quadrant [RLQ])
  • Possible shock secondary to septicemia or colonic perforation

Physical examination findings include the following:

  • Abdominal distension
  • Absence of bowel sounds
  • Tympany
  • Palpation tenderness (usually most marked in RLQ)
  • Occasionally, a palpable mass
  • Diffuse direct and rebound tenderness (suggesting colonic perforation, peritonitis)

Preferred Examination

Abdominal CT with oral and intravenous contrast is the preferred examination.



Small-Bowel Obstruction

Other Problems to be Considered

Infectious colitis



Findings

Plain radiographs are nonspecific but may demonstrate a fluid-filled masslike density in the RLQ, distension of adjacent small bowel loops, and thumbprinting. Free intraperitoneal air and pneumatosis coli rarely are observed. Barium enema and colonoscopy are contraindicated in possible typhlitis because of perforation risk.

Degree of Confidence

Plain radiographs may suggest abnormality but are nonspecific. Confirm abnormal findings with CT as described below.



Findings

CT demonstrates circumferential and occasionally eccentric low-attenuation colonic wall thickening and cecal distension. High attenuation within the thickened colonic wall may represent hemorrhage. Inflammatory pericolonic stranding of mesenteric fat is common.

CT readily identifies complications, including pneumatosis coli, pneumoperitoneum, pericolonic fluid collections, and abscess. These complications may require urgent surgical management.

Degree of Confidence

CT findings consistent with typhlitis in a patient with an appropriate clinical scenario result in a high degree of confidence in the diagnosis of typhlitis.



Findings

Sonographic findings of typhlitis include absent or decreased bowel peristalsis in the RLQ, thickened hypoechoic bowel wall, and markedly thickened echogenic mucosa. Color-flow imaging reveals hypervascularity of the mucosa and bowel wall. The patient may complain of pain upon palpation with the transducer.

Degree of Confidence

The above findings, combined with an appropriate clinical history, indicate a high probability of typhlitis. CT may be indicated to exclude perforation or abscess (not visualized on ultrasound) and to establish a baseline to compare follow-up studies.

False Positives/Negatives

RLQ small bowel loops distended with air can produce a significant ring-down artifact on ultrasound, thus obscuring visualization of the right colon.



No radiologic intervention is indicated for typical cases of typhlitis.



Media file 1:  Typhlitis. Marked low-attenuation cecal wall thickening (large arrow) with moderate pericolonic inflammatory stranding (small arrows). Note thickening of transverse colon wall posteriorly.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT

Media file 2:  Typhlitis. Marked asymmetric cecal wall thickening (arrow) in this 64-year-old patient whose status is postchemotherapeutic for lymphoma.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT

Media file 3:  Typhlitis. CT of this 10-year-old patient with leukemia demonstrates fluid within the cecum, which has an asymmetrically thickened wall (arrows).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT

Media file 4:  Typhlitis. Mild asymmetric low-attenuation cecal wall thickening (arrow) in an 8-year-old patient with leukemia undergoing chemotherapy.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT

Media file 5:  Typhlitis. Marked circumferential cecal and ascending colon wall thickening (large arrows) with mild pericolonic inflammatory stranding (small arrows).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT



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Typhlitis excerpt

Article Last Updated: Nov 3, 2004