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

Publisher: MDPI Country of Publication: Switzerland NLM ID: 101238455 Publication Model: Electronic Cited Medium: Internet ISSN: 1660-4601 (Electronic) Linking ISSN: 16604601 NLM ISO Abbreviation: Int J Environ Res Public Health Subsets: MEDLINE

Original Publication: Basel : MDPI, c2004-

Mucocutaneous Lymph Node Syndrome/*pathology ; Pharynx/*pathology; Aspirin/therapeutic use ; Biomarkers ; Child, Preschool ; Diagnosis, Differential ; Edema ; Fever/drug therapy ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Inflammation/complications ; Male ; Mucocutaneous Lymph Node Syndrome/diagnosis ; Mucocutaneous Lymph Node Syndrome/drug therapy

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.

Am J Otolaryngol. 2003 May-Jun;24(3):143-8. (PMID: 12761699)
Pediatr Infect Dis J. 2010 Jun;29(6):483-8. (PMID: 20104198)
Ital J Pediatr. 2018 Aug 30;44(1):102. (PMID: 30157897)
J Infect. 2013 Jul;67(1):1-10. (PMID: 23603251)
Int J Pediatr Otorhinolaryngol. 2014 Oct;78(10):1774-8. (PMID: 25081603)
Expert Rev Anti Infect Ther. 2015;13(12):1557-67. (PMID: 26558951)
Circulation. 2017 Apr 25;135(17):e927-e999. (PMID: 28356445)
Braz J Otorhinolaryngol. 2016 Jul-Aug;82(4):484-6. (PMID: 26520438)
J Pediatr. 2013 Jun;162(6):1259-63, 1263.e1-2. (PMID: 23305955)
Cardiol Young. 2019 Jun;29(6):828-832. (PMID: 31169101)
Korean J Radiol. 2011 Nov-Dec;12(6):700-7. (PMID: 22043152)
Pediatr Int. 2016 Nov;58(11):1146-1152. (PMID: 27097838)
Pediatrics. 2002 May;109(5):E77-7. (PMID: 11986483)
Ital J Pediatr. 2018 Aug 30;44(1):103. (PMID: 30157893)
J Comput Assist Tomogr. 2012 Jan-Feb;36(1):138-42. (PMID: 22261784)
J Pediatr. 2019 Dec;215:118-122. (PMID: 31477383)
Emerg Radiol. 2012 Apr;19(2):159-63. (PMID: 22134843)
Eur J Pediatr. 2014 Mar;173(3):381-6. (PMID: 24146166)
Front Pediatr. 2019 Apr 05;7:124. (PMID: 31024869)
Case Rep Pediatr. 2014;2014:296456. (PMID: 25349761)
Pediatr Rev. 2013 Apr;34(4):151-62. (PMID: 23547061)
Int J Pediatr Otorhinolaryngol. 2000 Apr 15;52(2):123-9. (PMID: 10767459)

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

0 (Biomarkers)
0 (Immunoglobulins, Intravenous)
R16CO5Y76E (Aspirin)

Date Created: 20190908 Date Completed: 20200214 Latest Revision: 20200309

20240829

PMC6765912

10.3390/ijerph16183262

31491922