key: cord-0697415-z8zbr1ri
authors: Hamzavi, Seyedeh Sedigheh; Derakhshan, Dorna; Askari, Aida; Haghshenas, Hoda; Hajiani Ghotbabadi, Shabnam
title: Torsion of the Appendix Testis as a Presenting Feature of Multisystemic Inflammatory Syndrome in Children: A Case Report
date: 2021-07-27
journal: Pediatr Infect Dis J
DOI: 10.1097/inf.0000000000003283
sha: 1189e4b2bf32b83f13bc55344dd7404a75145834
doc_id: 697415
cord_uid: z8zbr1ri

nan

A Case Report

A n otherwise healthy 8-year-old boy presented to the pediatric emergency ward because of a 2-day history of abdominal pain, followed by acute onset of rapidly progressive swelling and pain in the right testis. He had no history of trauma, dysuria or fever. He underwent emergency surgery because of suspicion of testicular torsion. The intraoperative finding was torsion of the right appendix testis.

After surgery, the patient developed fever with subsequent generalized rash and conjunctivitis and attended the pediatric ward of a tertiary hospital. There was a history of an upper respiratory tract infection in his father 1 month before his admission.

On physical examination, he appeared dehydrated. Vital signs included the following: temperature 39.5 °C, pulse rate of 120 beats/min, blood pressure 100/50 mm Hg and respiratory rate of 30/min. He had bilateral conjunctival erythema, maculopapular rash all over the trunk, abdominal distension with a generalized tenderness, normal breathing sound and a pansystolic murmur grade 2/6 in the left sternal border. The upper and lower extremities had nonpitting edema. The site of the recent testicular surgery was clear without erythema or discharge. He had a normal cremasteric reflex. No lymphadenopathy was detected, and other parts of the physical examination were normal. Oxygen saturation was detected 99% at room air. The primary and midadmission laboratory results are summarized in Table 1 .

The electrocardiogram was normal. The echocardiography revealed a moderate right-sided pleural effusion with good left and right ventricular systolic function and normal coronary arteries.

Considering the recent pandemic and noticeable elevation of inflammatory markers, nasopharyngeal swab polymerase chain reaction test and immunoglobulin assay for severe acute respiratory syndrome coronavirus (SARS-CoV) 2 were done. The nasopharyngeal swab was negative for coronavirus-2 or influenzas (type A and B) RNA. The SARS-CoV-IgM was negative (0.23 Au/mL), but IgG was positive (5.81 Au/mL).

Regarding the patient's clinical presentations and laboratory findings, he was diagnosed as multisystemic inflammatory syndrome in children (MIS-C), and treatment with IV immunoglobulin, aspirin and corticosteroid was initiated. After therapy, the general condition improved and was discharged after 7 days.

MIS-C is a postviral inflammatory vasculopathy in children and adolescents characterized by persistent fever, multiorgan dysfunction, laboratory markers of significant inflammation, lack of an alternative diagnosis and prior SARS-CoV-2 infection or exposure. 1 The most frequently encountered initial features include persistent fever, along with dermatologic, mucocutaneous and GI features. 1 To the best of our knowledge, there are only a few reported cases of coronavirus disease 2019 infection presenting with testicular pain. [2] [3] [4] However, there was no previous report on MIS-C presenting with scrotal pain and testis appendix torsion. Our patient presented with appendix testis torsion and immediately after the surgery developed fever, gastrointestinal, cardiovascular, hematologic and mucocutaneous findings. Torsion of the appendix testis is generally a self-limiting condition and the cause of scrotal pain in half of children 7-12 years of age. 5 We report this case to bring awareness of atypical presenting features of MIS-C. Considering the increased emergence of MIS-C cases and lack of proper understanding of early diagnostic clinical features and treatment, there is an urgent need for more investigation on clinical presentations, treatment and outcomes. W ith interest, we read the article by

Araújo et al 1 about a 17-year-old female who was diagnosed with Guillain-Barré syndrome (GBS), and subtype acute, demyelinating inflammatory polyneuropathy, 8 days after onset of a mild coronavirus disease 2019 infection. Surprisingly, cerebrospinal fluid (CSF) investigations were positive for RNA of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). 1 The patient profited from IV immunoglobulins. 1 The study is appealing but raises concerns.

The main limitation is that the test upon which SARS-CoV-2 RNA was detected in the CSF was not specified. Although a control mock CSF tested negative for the virus, there is no mentioning if the applied test was validated for CSF testing. There is also no discussion about the possibility that the test was false positive. Sensitivity and specificity of the applied real-time polymerase chain reaction were not provided. No information about the test-retest reliability was provided. There were also no repeated CSF investigations for the virus during follow-up, why it remains unknown for how long the virus could be proven in the CSF.

A second limitation is that there was no discussion about the pathophysiologic implications of the test result. Because GBS is an immunologic and not an infectious disorder, it is rather unlikely that presence of the virus in the CSF had a direct pathophysiologic consequence. Anyhow, presence of SARS-CoV-2 is not uncommon. Particularly in patients experiencing meningitis or encephalitis, SARS-CoV-2 has been repeatedly found in the CSF. [2] [3] [4] In immunemediated complications of SARS-CoV-2, however, SARS-CoV-2 is usually absent in the CSF. In a study of 220 patients with SARS-CoV-2-associated GBS, collected until the end of December 2020, CSF was investigated for the virus in 56 cases but was found in none of them. 5 Absence of the virus in the CSF was explained by the assumption that the virus never enters the CSF or that it enters the CSF but remains only for a short time before invading neurons or endothelial cells. An argument for the temporary presence of the virus in the CSF is that virus RNA has been found on autopsy studies in neurons and endothelial cells of the frontal lobe. 6 There is also no discussion via which pathway the virus had entered the CSF. Speculations in the literature include retrograde migration of the virus along cranial or peripheral nerves, hematogenic spread, or intracellular transport in leukocytes via the blood-brain barrier.

Missing are the results of the cerebral magnetic resonance imaging with contrast medium. Because GBS can manifest as Bickerstaff encephalitis, it is crucial to know if there was immune encephalitis of the brainstem or not. In this respect, it should be mentioned if there was involvement of cranial nerves, the respiratory muscles or the bulbar muscles. Because GBS may be complicated by autonomic involvement, we should know if the patient ever developed autonomic dysfunction.

Although SARS-CoV-2-associated GBS is more prevalent in adults compared with children or adolescents, there is increasing evidence that also younger ages can be affected. In the study of 220 patients with SARS-CoV-2associated GBS, 6 patients were below age 18 years. 5 A shortcoming of Table 1 1 is that no reference limits were provided.

Overall, the interesting study has limitations which challenge the results and their interpretation. There is a need to address these limitations to strengthen the conclusions.

Multisystem inflammatory syndrome in children (MIS-C) and the coronavirus pandemic: current knowledge and implications for public health

Abdominal and testicular pain: an atypical presentation of COVID-19

Testicular pain as an unusual presentation of COVID-19: a brief review of SARS-CoV-2 and the testis

Orchiepididymitis in a boy with COVID-19

Appendix testes torsion

First report of SARS-CoV-2 detection in cerebrospinal fluid in a child with Guillain-Barré syndrome

Infectious and immunemediated central nervous system disease in 48 COVID-19 patients

A first case of meningitis/encephalitis associated with SARS-coronavirus-2

COVID-19-associated meningoencephalitis complicated with intracranial hemorrhage: a case report

Guillain-Barre syndrome in 220 patients with COVID-19

Central nervous system involvement by severe acute respiratory syndrome coronavirus -2 (SARS-CoV-2)

To the Editors:W e appreciate the interest in our article 1 and the opportunity to respond to the comments. In the published report, we present a pediatric case of COVID-19-associated GBS