In lower socioeconomic groups and in areas of the world with deficient standards of hygiene (e.g., developing regions), Epstein–Barr virus (EBV) tends to infect children at an early age, and infectious mononucleosis (IM) is uncommon. In areas with higher standards of hygiene, infection with EBV is often delayed until adulthood, and IM is more prevalent. IM is a benign disease that commonly causes sore throat, pharyngitis, or tonsillitis. It does not often lead to dyspnea but they will likely have some associated dysphagia and odynophagia.
Differential diagnoses of such presentation could be infectious causes such as Ludwig’s angina, epiglottitis, peritonsillar abscess, diphtheria, Kikuchi’s disease, and IM. Some of the noninfectious causes can include local trauma, angioedema, foreign bodies, or neoplasms.
EBV-induced IM normally resolves on its own after a few weeks or months, although there can be multiple complications involving pulmonary, neurological, hematological, and hepatic systems. Tracheal obstruction causing respiratory failure is the most common cause of death/most lethal complication of IM. Here, we report a 22-year-old male who presented with tender cervical lymphadenopathy, fever, and near-fatal tracheal obstruction.
A 22-year-old tribal boy presented in the emergency department of tertiary care hospital in central India with complaints of high grade, intermittent fever associated with chills and rigors for 5 days. He also had a history of breathlessness for 3 days. He also had complaints of swelling over the right side of the neck which progressed for 5 days. There was no history of abdominal pain, nausea, vomiting, weight loss, headache, cough, rhinorrhea, and rash. He had no past history of illness, recent travel history, and animal bites. He denied a history of drug or tobacco consumption.
On admission, his vital signs revealed a pulse rate of 112/min, blood pressure of 110/70 mmHg, respiratory rate of 32/min, temperature of 98.6° Fahrenheit, and oxygen saturation of 90% on room air. Neurological examination was done and Glasgow Coma Scale was 15/15. On local examination, he had multiple right-sided anterior and posterior cervical lymphadenopathy of size 4 cm × 5 cm which was firm, tender, and not matted [Figure 1a]. There was a tracheal deviation to the left side. There was no torticollis. The swelling was causing airway compression and stridor. Oropharyngeal examination was normal. On respiratory system examination, the patient was having tachypnea and the use of accessory muscles for respiration was prominent. Bilateral wheeze was present on auscultation. Abdomen examination revealed no organomegaly and was nontender.
As the patient had respiratory distress, tachypnea, and near-complete airway obstruction, oral intubation was attempted by the emergency room physician but failed due to obstruction. After this emergency, a tracheostomy was done in the emergency room by the on-call anesthetist and expert ear, nose, and throat (ENT) specialist, and the patient was shifted to the isolated cubicle in the intensive care unit to prevent cross-transmission.
Laboratory investigations and its finding are depicted in Table 1.
Peripheral smear showed predominantly normocytic, normochromic red blood cells. No atypical lymphocytes were seen. Chest X-ray showed a mediastinal mass on the right side compressing the trachea and tracheal deviation to the left, bilateral lung fields were clear, and there were no signs of acute cardiopulmonary disease. Ultrasonography of the neck was done which was suggestive of right-sided cervical lymphadenopathy. The lymph nodes were round in shape, had heterogeneous echotexture, and were matted. Ultrasonography of the abdomen was normal and showed no hepatomegaly, splenomegaly, or splenic infarct. High-resolution computed tomography of the thorax was done which was suggestive of soft-tissue density lesion noted in the right paratracheal region displacing and partially compressing trachea and displacement of the adjacent vessels to the contralateral side probably lymph nodal mass. There was evidence of soft-tissue density lesion in the carinal and subcarinal region, possibly enlarged lymph nodes [Figure 1b].
A cervical lymph node biopsy was taken and sent for histopathological examination. Section of the biopsy revealed monocytoid B-cell lymphocytes with round, angulated nuclei, and some contain clear cytoplasm admixed with neutrophils on histopathology, partially effaced architecture, increased immunoblasts, and atypical lymphocytes in sinusoids, and polymorphous inflammatory cells suggesting the diagnosis of IM [Figure 2]. IM was suspected and a Paul–Bunnell test was done which was positive.
Tracheostomy procedure done swiftly by the ENT specialist was lifesaving in our patient. The patient was treated with injectable corticosteroids intravenous methylprednisolone 1 g once daily for 5 days, injection ceftriaxone 1 g intravenous twice daily, nonsteroidal anti-inflammatory drugs, intravenous hydration, and other supportive management. Existing guidelines suggest the use of corticosteroids only when complications such as pharyngeal edema, pharyngotonsillitis, and airway obstruction occur. The tracheostomy tube was removed after 1 week.
EBV-causing IM usually has a benign and self-limited course. In 90% of cases of IM, the etiology is EBV and in 10% it is cytomegalovirus. Young children and the elderly are very mildly affected, whereas young people experience a prodrome of lethargy and malaise, which is followed by fever, lymphadenopathy, and pharyngitis. The most serious complications leading to mortality are splenic rupture and meningoencephalitis, but upper respiratory tract blockage could be fatal. Waldeyer’s ring which consists of tonsils, adenoids, and other lymphoid tissue inflammation causes tonsillar hypertrophy in IM, but only rarely does this inflammation lead to airway compromise and admission to hospital. This airway obstruction may only rarely need intubation and usually resolves with corticosteroids.
Massive tonsillar hypertrophy, pharyngeal edema, or both are seen in some patients, resulting in upper airway blockage and mortality. Only five patients in a trial of 467 people with IM who were hospitalized presented with potentially fatal airway blockage and two of these did not respond to medical treatment. In the case of Ludwig’s angina with a difficult airway (cannot intubate and cannot ventilate) and thrombocytopenia, emergency percutaneous tracheostomy for secure airway has already been described. According to one published case report, two adolescent mononucleosis patients needed tonsillectomy to breathe correctly.
Although it has not been properly explored, the use of corticosteroids to alleviate IM issues has been advised based on anecdotal evidence. In mild, uncomplicated cases, corticosteroids are not required, and they should not be given to everyone with IM. Some of the theoretical concerns are that there could be immunomodulation of the virus leading to several malignancies.
Kikuchi’s disease was kept in the differential diagnosis as it has a similar presentation of cervical lymphadenopathy with fever. Suspicion of Kikuchi’s disease was ruled out by histopathology report as there was the absence of prominent plasmacytoid lymphocytes and eosinophilic necrotic debris. There were no karyorrhectic areas, absence of starry skin formation by plasmacytoid lymphocytes and immunoblasts, and absence of histiocytic phagocytosis. The presence of neutrophils which is rare to absent in Kikuchi’s disease.
Airway compromise as described in this patient is a rare consequence of IM which can be a cause of mortality in such cases. Timely interventions such as emergency tracheostomy can save the life. As described in this case, a deviated trachea needs careful identification to avoid complications related to tracheostomy can be avoided. Although not practiced in our case, a point-of-care ultrasound may help identify and mark tracheal rings. We also wish to suggest that an early diagnosis of the etiology can better help in the early initiation of treatment.
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The authors followed applicable EQUATOR Network guidelines (http://www.equator-network.org/), notably the CARE guideline, during the conduct of this report.
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The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
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