key: cord-0949949-6130abzm
authors: Gupta, Purbasha; Giri, Prabhas Prasun; Das, Dipankar; Pal, Priyankar
title: Pediatric inflammatory multisystem syndrome (PIMS) presenting with retropharyngeal phlegmon mimicking Kawasaki disease
date: 2021-01-13
journal: Clin Rheumatol
DOI: 10.1007/s10067-020-05538-x
sha: c01082554ee6b60d45e58fc0b6a12790ffa81fbf
doc_id: 949949
cord_uid: 6130abzm

nan

A 4-year-old boy presented with high fever for 6 days along with a painful neck swelling associated with bilateral nonpurulent conjunctivitis, facial puffiness, and extreme irritability. He had tachycardia, hypotension, S3 gallop, and generalized edema. The neck swelling was extremely painful causing neck stiffness. With a working diagnosis of sepsis and myocarditis empirical broad spectrum, antibiotics were started, but a possibility of KD and PIMS was also considered. Echocardiography showed grade 1 systolic dysfunction, and MRI neck (Fig.1 ) demonstrated a retropharyngeal fluid collection with soft tissue edema, likely to be a retropharyngeal phlegmon, as described in KD. COVID RT-PCR was negative but COVID IgG was positive.

He was treated with IVIg @2 g/kg followed by IV methyl prednisolone @5 mg/kg/day for 3 days. Fever subsided within 48 h of IVIg, and the inflammatory markers started normalizing (Table 1 ) on repeat testing after3 days of methylprednisolone. The child was discharged on low-dose aspirin and oral prednisolone 2 mg/kg/day to be tapered over 15 days, with advice for a follow-up echocardiography after 14 days. The child is doing well at present, and repeat echocardiography is within normal limit.

There has been several case reports of KD presenting with retropharyngeal edema/phlegmon [1, 2] . This fact that cervical lymphadenopathy in KD can be associated with deeper soft tissue inflammation leading to non-suppurative edema is being increasingly recognized and has been included as "Other Clinical Finding" in the AHA 2017 Scientific Statement on KD [3] .

With the onset of the COVID 19 pandemic, cases presenting with multisystem hyperinflammation (PIMS) resembling atypical KD are being frequently reported globally [4, 5] . Though cervical lymphadenopathy is present as an associated finding in some of these patients, the authors did not come across any description of retropharyngeal edema in the available literature. This child presented with high fever with painful torticollis and had other features of systemic inflammation with myocarditis resembling PIMS. Although there was no history of exposure to COVID, he tested positive for COVID IgG signifying an asymptomatic past infection. He responded very well to IVIg and IV methyl prednisolone with improvement of biochemical parameters and echocardiography findings.

Disclosures None.

Patient consent Obtained. Written consent has been attached. 

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Computed tomographic findings of Kawasaki disease with cervical lymphadenopathy

Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the

An outbreak of severe Kawasaki like disease at the Italian epicentre of the SARS-CoV-2 epidemic: an observational cohort study

Epidemiological and clinical profile of pediatric inflammatory multi system syndrome -temporally associated with SARSCOV-2 (PIMS-TS) in Indian children. Indian Pediatr Publisher's note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations