Challenges in Diagnosing Multisystem Inflammatory Syndrome Related to SARS-CoV-2 in a Child With Severe Neurocognitive Impairment and Social Isolation.

On July 21, 2020, we evaluated a 14-year-old boy with Aicardi syndrome because of fever present for the last 5 days.

Because of the COVID-19 pandemic, the restrictive measures established by the Italian government, and family fears of potential consequences of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in a compromised child, the boy and his family respected the quarantine. For the same reason, they postponed the scheduled admission aimed at changing the percutaneous endoscopic gastrostomy (PEG) tube.

On admission, the child looked irritable with whole-body maculopapular rash (Figure, A) and a greenish and grainy PEG tube (Figure, B). Moreover, he presented dyspnea. Blood tests showed a rise in inflammation indices. After performing microbiological tests, the child was started on ceftriaxone. Owing to the persistence of high-grade fever, piperacillin/tazobactam was added 2 days later in the hypothesis of a possible Pseudomonas aeruginosa infection from the PEG tube insertion. Chest radiography (Figure, C) and chest-abdominal computed tomography (Figure, D) were also performed.

A, Maculopapular rash and diffuse erythroderma on the child's lower limb. B, The picture shows native PEG with the greenish and grainy feeding tube. It was placed in 2018 and had never undergone revision or replacement. C, Chest radiographic film shows severe rotoscoliosis with important deviation of the dorsal and lumbar spine toward the right chest cavity (blue arrow), resulting in marked loss of lung volumes and severe restrictive lung disease without parenchymal abnormalities. D, Coronal computed tomography image shows severe rotoscoliosis with important deviation of the dorsal and lumbar spine toward the right chest cavity and deviation of the lumbosacral spine toward the left (blue arrows). Coronal computed tomography image shows abdominal fluid (blue circles).

In the meantime, all microbiological examinations, including the nasopharyngeal swabs for respiratory viruses and SARS-CoV-2, resulted negative.

Although the child respected the quarantine and there was exclusion of alternative diagnoses, a suspicion of “pediatric multisystem inflammatory syndrome: temporally associated with SARS-CoV-2” (PIMS-TS) was raised. Hence, we performed SARS-CoV-2 serology, which turned positive for immunoglobulin G anti–SARS-CoV-2. Ultimately, the child fulfilled all the diagnostic criteria of PIMS-TS: clinical presentation (fever, rash, evidence of organ dysfunction), laboratory markers (raised inflammatory markers), exclusion of alternative diagnoses, and evidence of SARS-CoV-2 exposure (immunoglobulin G).[1,2] On July 27 (12th day of fever), the child received a bolus of methylprednisolone with resolution of fever.

Here, we describe a case of PIMS-TS in a child with severe comorbidities, highlighting the necessity of keeping high index of suspicion for this disease, especially in the context of the pandemic, and although the children with special needs do not have social interactions or evidence of SARS-COV-2 infection (neither in close relatives).

In this case, the confounding factor of the PEG tube suggested a bacterial infection, and the diagnosis was made after 12 days of fever. To date, the child did not require biological agents or cardiotropic therapies. Within the current state of knowledge, the conclusive answer whether the fast treatment may be associated with a better outcome is still missing.

Therefore, PIMS-TS should be definitively included in the differential diagnoses of any child with systemic inflammatory conditions.