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Case Reports
. 2019 Sep 5;16(18):3262.
doi: 10.3390/ijerph16183262.

A Case of Incomplete and Atypical Kawasaki Disease Presenting with Retropharyngeal Involvement

Chiara Isidori  1 , Lisa Sebastiani  2 , Susanna Esposito  3
Affiliations
Case Reports

A Case of Incomplete and Atypical Kawasaki Disease Presenting with Retropharyngeal Involvement

Chiara Isidori et al. Int J Environ Res Public Health. .

Abstract

Background: Kawasaki disease (KD) is a childhood acute febrile vasculitis of unknown aetiology. The diagnosis is based on clinical criteria, including unilateral cervical lymphadenopathy, which is the only presenting symptom associated with fever in 12% of cases. A prompt differential diagnosis distinguishing KD from infective lymphadenitis is therefore necessary to avoid incorrect and delayed diagnosis and the risk of cardiovascular sequelae. Case presentation: We describe the case of a 4 years old boy presenting with febrile right cervical lymphadenopathy, in which the unresponsiveness to broad-spectrum antibiotics, the following onset of other characteristic clinical features and the evidence on the magnetic resonance imaging (MRI) of retropharyngeal inflammation led to the diagnosis of incomplete and atypical KD. On day 8 of hospitalisation (i.e., 13 days after the onset of symptoms), one dose of intravenous immunoglobulins (IVIG; 2 g/kg) was administered with rapid defervescence, and acetylsalicylic acid (4 mg/kg/day) was started and continued at home for a total of 8 weeks. Laboratory examinations revealed a reduction in the white blood cell count and the levels of inflammatory markers, thrombocytosis, and persistently negative echocardiography. Clinically, we observed a gradual reduction of the right-side neck swelling. Fifteen days after discharge, the MRI of the neck showed a regression of the laterocervical lymphadenopathy and a resolution of the infiltration of the parapharyngeal and retropharyngeal spaces. Conclusion: Head and neck manifestations can be early presentations of KD, which is frequently misdiagnosed as suppurative lymphadenitis or retropharyngeal infection. A growing awareness of the several possible presentations of KD is therefore necessary. Computed tomography (CT) or MRI can be utilised to facilitate the diagnosis.

Keywords: Kawasaki disease; abscess-like lesions; lymphadenopathy; neck swelling; retropharyngeal involvement.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Body temperature and antibiotic therapy during the first 7 days of hospitalisation.
Figure 2
Figure 2
Magnetic resonance imaging (MRI) of the neck. (a): Right anterolateral multiple polycyclic lymph nodes with ill-defined contours and tendency to confluence. Intense enhancement of the adjacent sternocleidomastoid. Displacement of the vascular bundle of the neck and internal jugular vein compression. (b): Enhancement of parapharyngeal and retropharyngeal tissues without fluid collection and with preserved respiratory space. Multiple bilateral enlarged lymph nodes in parapharyngeal and retropharyngeal space. Minimum contrast enhancement of the atlantoaxial joint.
Figure 3
Figure 3
Body temperature from the time of the administration of intravenous immunoglobulin (IVIG) treatment.
See this image and copyright information in PMC

References

    1. McCrindle B.W., Rowley A.H., Newburger J.W., Burns J.C., Bolger A.F., Gewitz M., Baker A.L., Jackson M.A., Takahashi M., Shah P.B., et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals from the American Heart Association. Circulation. 2017;135:e927–e999. doi: 10.1161/CIR.0000000000000484. - DOI - PubMed
    1. Holman R.C., Belay E.D., Christensen K.Y., Folkema A.M., Steiner C.A., Schonberger L.B. Hospitalizations for Kawasaki syndrome among children in the United States, 1997–2007. Pediatr. Infect. Dis. 2010;29:483–488. doi: 10.1097/INF.0b013e3181cf8705. - DOI - PubMed
    1. Ghimire L.V., Chou F.S., Mahotra N.B., Sharma S.P. An update on the epidemiology, length of stay, and cost of Kawasaki disease hospitalisation in the United States. Cardiol. Young. 2019;29:828–832. doi: 10.1017/S1047951119000982. - DOI - PubMed
    1. Inagaki K., Blackshear C., Hobbs C.V. Deep Neck Space Involvement of Kawasaki Disease in the US: A Population-Based Study. J. Pediatr. 2019 in press. - PubMed
    1. Marchesi A., de Jacobis I.T., Rigante D., Rimini A., Malorni W., Corsello G., Bossi G., Buonuomo S., Cardinale F., Cortis E., et al. Kawasaki disease: Guidelines of the Italian Society of Pediatrics, part I-definition, epidemiology, etiopathogenesis, clinical expression and management of the acute phase. Ital. J. Pediatr. 2018;44:102. doi: 10.1186/s13052-018-0536-3. - DOI - PMC - PubMed

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