Abstract
BackgroundApproximately 50–70% of patients with Kawasaki disease (KD) could present with cervical lymphadenopathy associated with deep neck inflammation, which may result in Grisel’s syndrome (GS). Given the possibility of neurological impairment owing to GS, it is important to understand the disease profile in KD. Therefore, we carried out this study to investigate this possible complication of KD, with the aim of improving pediatricians’ recognition and awareness.MethodsPatients with KD complicated by GS in our hospital were retrospectively recruited for our study. The profiles of patients with GS (n = 10) were compared to those patients without GS (n = 1254). All the available literature describing these complications of KD was reviewed.ResultsThe incidence of GS in KD was 0.6% in our population. Compared to patients without GS, KD patients with GS were older, presented with a significantly lower male:female ratio, and a higher incidence of cervical lymphadenopathy, a higher level of neutrophil count, and erythrocyte sedimentation rate. Ten articles reporting 14 KD patients with GS were reviewed. Of the total 24 patients, GS affected 7 males and 17 females, aged from 3.5 to 9 years old. Encouragingly, no delayed diagnosis and treatment of KD was found, and all patients received conservative therapy for GS, without intravenous immunoglobulin resistance, coronary artery lesions, and neurological impairment.ConclusionsGS is a rare complication of KD with an incidence of 0.6%, predominantly affecting older, female children. The overall outcome of this disorder in KD was satisfactory with conservative therapy. Pediatricians, especially pediatric surgeons, should recognize and be aware of this possible complication of KD to avoid misdiagnosis and overtreatment.
Highlights
50–70% of patients with Kawasaki disease (KD) could present with cervical lymphadenopathy associated with deep neck inflammation, which may result in Grisel’s syndrome (GS)
Of the 1582 KD patients recruited in our study, ten cases of GS were identified as a complication of KD, with the incidence of 0.6% (10/1582)
In patients with KD presenting cervical lymphadenopathy, its constituent ratio referred to 1.5% (10/657)
Summary
50–70% of patients with Kawasaki disease (KD) could present with cervical lymphadenopathy associated with deep neck inflammation, which may result in Grisel’s syndrome (GS). Given the possibility of neurological impairment owing to GS, it is important to understand the disease profile in KD. Involvement of the pulmonary, gastrointestinal, Grisel’s syndrome (GS), a rare, nontraumatic, atlantoaxial subluxation resulting from any inflammatory condition of the upper neck and otolaryngologic procedures, is rarely reported as a possible complication of KD [8,9,10]. The presence of cervical lymphadenopathy associated with deep neck inflammation, occurring in 50–70% of patients with KD, could lead to parapharyngeal and retropharyngeal edema and nonsuppurative phlegmon [11], and in turn possibly predispose patients to GS.
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