Clinical Features of Kawasaki Disease With Atlantoaxial Rotatory Fixation

Purpose: Atlantoaxial rotatory fixation (AARF) is a rare complication of acute Kawasaki disease (KD). Early diagnosis and intervention are important for AARF because delayed diagnoses may incur neurological sequelae. However, previous studies on AARF associated with KD are limited, and its clinical characteristics and course are unknown. This study aimed to examine the clinical features and treatment course of KD with AARF using a Japanese national inpatient database. Methods: Using the Diagnosis Procedure Combination database, we identified KD patients who received intravenous immunoglobulin (IVIG) treatment between July 2010 and March 2020. The clinical characteristics of KD patients with AARF and their risk factors were evaluated using multivariable logistic regression analysis. We also examined the relationship between AARF, the proportion of coronary artery abnormalities (CAAs), IVIG resistance, length of stay and medical costs. Results: We identified 71,913 patients with KD, 166 of whom had AARF. The AARF group had older age, heavier bodyweight and atypical KD. In multivariable analysis, AARF was associated with older age [odds ratio (OR): 1.24; 95% confidence interval (CI): 1.19-1.29], lower body mass index (OR: 0.89; 95% CI: 0.82-0.96) and atypical KD (OR: 1.95; 95% CI: 1.12-3.40). AARF was not associated with CAAs (OR: 0.73; 95% CI, 0.23-2.32) and IVIG resistance (OR: 1.05; 95% CI, 0.74-1.49). However, AARF was associated with higher medical costs (difference, US$1064; 95% CI: 346-1781) and longer hospital stay (difference, 3.1 days; 95% CI: 1.7-4.4). Conclusion: AARF in patients with acute KD should be considered if cervical symptoms present in older patients with atypical KD.