Atypical recurrent kawasaki disease with retropharyngeal involvement: A case study and literature review

Abstract

Kawasaki disease (KD) is a systemic inflammatory condition primarily affecting young children. We present an adolescent male with two episodes of complete KD between the age of 2 and 14 years. At age 14, he presented with findings suggestive of a retropharyngeal abscess. This was later determined to be a recurrence of KD, diagnosed after the development of coronary artery aneurysms. Our case reinforces the role of maintaining a high index of suspicion for KD, both in patients with prior KD episodes and in those with persistent fever who do not fulfill the diagnostic criteria for typical KD. This is particularly important for patients presenting with atypical symptoms not commonly associated with KD, such as inflammation of the retropharyngeal and parapharyngeal spaces.

Kawasaki shock syndrome in monozygotic twins

“…Deep tissue neck inflammation as was seen in the twins is a presenting clinical feature in 0.6% of KD cases. 5 While KD is the second most common vasculitis in children, KD complicated by hypotension or signs of poor perfusion (i.e. KSS) is rare.…”

“…Although one can find numerous case reports of deep tissue inflammation of the parapharyngeal and/or retropharyngeal spaces in recurrent KD (e.g. Cason et al 5 …”

“…1 Although one can find numerous case reports of deep tissue inflammation of the parapharyngeal and/or retropharyngeal spaces in recurrent KD (e.g. Cason et al 5 ), a formal relationship between the two is yet to be studied. Deep tissue neck inflammation as was seen in the twins is a presenting clinical feature in 0.6% of KD cases.…”

An Update on Reports of Atypical Presentations of Kawasaki Disease and the Recognition of IVIG Non-Responder Children

“…All results have been tabulated and analyzed in Table 1 and Table 2 . The first one reports on the general data and canonical KD manifestations found in such case reports with atypical KD; the second one details the non-canonical manifestations which were observed in these same patients, challenging the proper diagnosis of KD [ 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73…”

“…The pathogenesis of gut pseudo-obstruction is not clear, although vasculitis occurring in the mesenteric artery might lead to ischemia and dysfunction of the myenteric plexus [ 35 ]. Antibiotic-resistant pneumonia can herald atypical KD, but a peculiar retropharyngeal involvement with abscess-like lesions may also be an infrequent manifestation of atypical KD [ 36 , 37 , 38 , 39 , 40 ]. Renal involvement includes interstitial nephritis, hemolytic uremic syndrome, immune complex-mediated nephropathy, and acute nephritic syndrome.…”