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Purpose: Acute appendicitis is an uncommon diagnosis in patients with cystic fibrosis (CF). Small studies and case reports have shown that affected patients often have a delay in diagnosis and subsequent complications. Our goal was to investigate the delay in diagnosis for patients with CF who present with acute appendicitis, the factors that might contribute to this delay, and the sequellae of delayed operative management.
Methods: Using the Nationwide Inpatient Sample database, all patients in the United States with a diagnosis of acute appendicitis were examined during the study years 2005 through 2009. Patients with a diagnosis of CF were compared to patients without CF for demographic and procedural variables, as well as variables related to complication and cost. Patients with CF who did not undergo surgical management for acute appendicitis during hospital days 0 or 1 were compared against those who had prompt surgical management.
Results: During the study years 2005 through 2009, 1,350,995 patients nationwide were admitted to the hospital with a diagnosis of appendicitis, and of these patients, 526 had a diagnosis of CF. Patients with CF were less likely to have surgical management (78.1% vs. 94.8%, p <0.0005) and less likely to have appendectomy on hospital day 0 or 1 (56.8% s. 77.6%). The presence of abscess or peritonitis did not differ significantly between the two groups. For patients who underwent surgery, CF patients were more likely to undergo right colectomy or ileocecectomy (10.2% vs. 2.5%, p <0.0005), more likely to require conversion to an open procedure (8.5% vs. 4.4%, p <0.0005), and more likely to have an iatrogenic injury (3.7% vs. 0.5%, p<0.0005). Significantly fewer CF patients were attempted laparoscopically (52.8% vs. 58.7%, p = 0.015). The median length of hospital stay and total hospital charges were significantly higher for CF patients (4 days vs. 2 days; $23,228 vs. $19,251). Multivariate regression analysis demonstrated CF patients were more likely to be white, have public insurance, and have admission to a teaching hospital. Patients with CF who had surgery during hospital day 0 or 1 were less likely to require right colectomy or ileocecectomy (7.7% vs. 17.7%, p = 0.005). Variables associated with prompt surgical management included the non-teaching hospital setting (OR 0.446-0.964), non-urban location (0.207-0.989), and the absence of respiratory manifestations (OR 0.260-0.607).
Conclusions: The management of appendicitis in CF patients differs significantly with that of the general population. CF patients are more likely to have a delay in operative management and the resulting sequellae associated with a more advanced disease process. Comorbid respiratory manifestations of CF are associated with a delay in surgical management, possibly due to the use of antibiotics in these patients.
Appendicitis in Patient with Cystic Fibrosis

	Cystic Fibrosis	General Population
Appendectomy on Hospital Day 0 or 1*	55.9%	76.8%	OR 0.322-0.454
Iatrogenic Injury**	3.7%	0.5%	OR 4.862-13.652
Ileocecectomy or Right Colectomy**	10.2%	2.5%	OR 3.246-6.149
Attempted Laparoscopic Appendectomy**	52.8%	58.7%	OR 0.649-0.956
Conversion to Open Procedure***	13.9%	7.1%	OR 1.480-3.023
Median Hospital Length of Stay*	4 days	2 days	alpha = 0.05

(*) All patients included; (**) All surgical patients included; (***) All laparoscopic patients included