Torsion of the Appendix Testis as a Presenting Feature of Multisystemic Inflammatory Syndrome in Children: A Case Report.

To the Editors:

An 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.

TABLE 1.

Lab Results of the Patient

Lab Results:	Initial Lab Results	Mid Admission Lab Results	Reference Range
Total leukocyte count	14.6 × 109/L (neutrophil 92.9%, lymphocytes 4%)	16.2 × 109/L (neutrophil 73%, lymphocytes 27%)	4–10 × 109/L
Hemoglobin (g/dL)	9.9	6.6	13–17 (men), 12–15 (women)
Platelet	165 × 109/L	297 × 109/L	15–400 × 109/L
Erythrocyte sedimentation rate (mm/h)	38	18
C-reactive protein (mg/L)	101	15	<5
Blood culture	Negative	—	Qualitative
Urine culture	Negative	—	Qualitative
Urine analysis	Specific gravity 1.025	—	Qualitative
	Glucose 3+
	WBC 2–4
Stool examination	Normal	—	Qualitative
Na (mmol/L)	130	132	135–145
K (mmol/L)	2.9	3.6	3.5–5
Ca (mg/dL)	8	7.6	8.6–10.3
P (mg/dL)	2.2	3.6	4–7
Blood urea nitrogen (mg/dL)	11	12	10–20
Creatinine (mg/dL)	0.66	0.67	0.5–1 (children age 3–18 years)
Aspartate aminotransferase (IU/L)	20	14	5–40
Alanine aminotransferase (IU/L)	9	8	7–40
Alkaline phosphatase (IU/L)	193	222	44–147
Total bilirubin(mg/dL)	0.2	0.4	1–1.2
Direct bilirubin (mg/dL)	0.1	0.1	<0.3
Prothrombin time (s)	19.4	16.1	11–13.5
International normalized ratio	1.44	1.19	0.9–1.2
Total protein(g/dL)	3.9	4.3	6–8
Albumin (g/dL)	2.5	2.5	3.5–5
Lactate dehydrogenase (IU/L)	422	588	50–150
Fibrinogen (mg/dL)	314		180–400
Troponin I titer (ng/L)	135.7	4.8	<19
D-Dimer (ng/mL)	2300	350	<500

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.[2-4] 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.