Grisel’s syndrome as an early manifestation of Kawasaki disease preceding Kawasaki disease shock syndrome: a case report

BACKGROUND: Kawasaki disease shock syndrome (KDSS) is a severe and uncommon complication of Kawasaki disease (KD). Grisel's syndrome (GS), a rare non-traumatic atlantoaxial subluxation, has been seldom described in KD. To our knowledge, this is the first documented case in which GS was the initial manifestation of KD and preceded the development of KDSS. CASE PRESENTATION: A 4-year-old boy presented with fever, cervical lymphadenopathy, and restricted neck mobility. Cervical computed tomography revealed atlantoaxial subluxation. He was initially diagnosed with cervical lymphadenitis and treated with antibiotics, but his symptoms persisted and rapidly progressed to shock. He was subsequently diagnosed with GS and KDSS. Management with fluid resuscitation, intravenous immunoglobulin, corticosteroids, and aspirin stabilized his hemodynamic status and improved KD-related symptoms. With systemic inflammation controlled and cervical immobilization applied, GS subsequently resolved. At 6 months of follow-up, echocardiography showed no coronary artery abnormalities, and no recurrence of GS or neurological sequelae was observed. CONCLUSIONS: Clinicians should recognize GS as a potential early manifestation of KD and maintain vigilance for progression to KDSS. Timely recognition and management could prevent severe cardiac and neurological complications in patients with KD complicated by GS and KDSS.