1. MedPix Medical Image Database COW - Case of the Week Case Contributor: Alex Galifianakis Affiliation: Uniformed Services University

2. MedPix No: 12061 - History Pt Demographics: Age = 10 y.o. Gender = girl 10 y.o. girl with known cystic fibrosis presents with acute onset of right lower quadrant pain, fever, and leukocytosis. Downloaded by (-1)

3. MedPix No: 12061 - EXAM & LABS +tnderness to palpation over McBurney's point.Increased WBC countFever to 102 F

4. Appendicitis Trans- abdominal ultrasound demonstrates a dilated tubular structure with thickened walls and surrounding free fluid. Initially thought too- large to represent the appendix, a CT was recommended for further evaluation. Downloaded by (-1)

5. Appendicitis Trans- abdominal ultrasound demonstrates a dilated tubular structure with thickened walls and surrounding free fluid. Initially thought too- large to represent the appendix, a CT was recommended for further evaluation. Downloaded by (-1)

6. Appendicitis CT confirms dilated edematous appendix with surrounding inflammatory fat stranding consistent with appendicitis. Downloaded by (-1)

7. Appendicitis CT confirms dilated edematous appendix with surrounding inflammatory fat stranding consistent with appendicitis. Downloaded by (-1)

8. Appendicitis CT confirms dilated edematous appendix with surrounding inflammatory fat stranding consistent with appendicitis. Downloaded by (-1)

9. Appendicitis CT confirms dilated edematous appendix with surrounding inflammatory fat stranding consistent with appendicitis. Downloaded by (-1)

10. Appendicitis CT confirms dilated edematous appendix with surrounding inflammatory fat stranding consistent with appendicitis. Downloaded by (-1)

11. Appendicitis CT confirms dilated edematous appendix with surrounding inflammatory fat stranding consistent with appendicitis. Downloaded by (-1)

12. FINDINGS Ultrasound demonstrates a dilated, blind-ended tubular structure with surrounding fluid.CT confirms a massively dilated appendix with surrounding inflammatory changes.

13. DIFFERENTIAL DIAGNOSIS What is your Differential Diagnosis? Prior to imaging, the most important diagnostic consideration is to rule out appendicitis. Additional entities which patients with Cystic Fibrosis are predisposed to include intussusception, Crohn Disease, fibrosing colonopahty and bowel wall edema secondary to hypoproteinemia.

14. Diagnosis: Acute Appendicitis in Cystic Fibrosis Patient Dx Confirmed by: Pathology from appendectomy

15. DISCUSSION Although the pulmonary manifestations of Cystic Fibrosis are prominent, significant morbidity secondary to gastrointestinal disease is also seen. Concerning bowel, CF patients are at a higher risk for the development of meconium ileus, meconium ileus equivelent, intussusception, GERD, Crohn Disease, fibrosing colonopahty and bowel wall edema (from hyproteinemia). - With respect to appendicitis, however, the incidence is actually lower in patients with CF. This is important to remember when imaging a patient with CF for appendicitis as the appendix may be dilated to 6mm without obstruction or inflammation.