Case 2: Upper Airway Obstruction in a Previously Healthy 3-Year-Old Boy

A previously healthy, fully immunized 3-year-old boy presents to the emergency department (ED) with 2 days of fever, fatigue, poor oral intake, vomiting, and diarrhea. Examination shows a nontoxic patient with dry lips and moist mucus membranes, mobile, nontender, small and scattered cervical lymphadenopathy, no tonsillar erythema, supple neck, and mild diffuse tenderness to abdominal palpation. In the ED, the patient is febrile to 102.0 °F and tachycardic to 155 beats per minute; otherwise, the patient is hemodynamically stable with blood pressure of 101/53, respiratory rate of 24 breaths per minute, and O2 saturation of 98%. Laboratories are significant for leukopenia with neutrophilia (white blood cell 4.95 k/mm3, 82% neutrophils), mild thrombocytopenia (platelets 154 k/mm3), elevated inflammatory markers (C-reactive protein (CRP) 18 mg/dL, procalcitonin 25 ng/mL), and a bicarbonate level of 17 mEq/L. A respiratory antigen panel is positive for adenovirus and parainfluenza 4. A blood culture is collected. He is admitted to the hospital and treated empirically with intravenous (IV) ceftriaxone and fluids for possible sepsis in the setting of fever, tachycardia, and elevated inflammatory markers. Vomiting and diarrhea improve, and his CRP initially downtrends to 6.5 mg/dL.Over the next few days, the patient continues to refuse oral intake and has sustained fevers to 102.0 °F. Laboratories show severe neutropenia with an absolute neutrophil count of 0.24 k/mm3, thrombocytopenia with platelets of 87 k/mm3, and rising CRP to 14 mg/dL. His blood culture shows no growth. A chest radiograph reveals right lower lung opacities and a small pleural effusion, which is treated with ceftriaxone. A lateral view of the neck is not obtained at this time. He develops a barking cough with intermittent stridor and difficulty handling secretions. Examination shows an ill-appearing, tired, drooling child, with cervical lymphadenopathy and submandibular fullness, and positioning himself with his neck extended and his chin tilted upward. Oropharyngeal examination performed with a pediatric intensivist present at the bedside reveals normal tonsils and uvula without erythema or exudates. Intravenous steroids and racemic epinephrine nebulizer treatments are initiated. The stridor improves with racemic epinephrine; however, no other symptoms significantly improve. CT of the neck is obtained (Figure 1).The clinical presentation of this patient is concerning for acute upper airway obstruction. Given their smaller tracheal width and immature cartilage, children are at a higher risk of respiratory failure from upper airway obstruction compared with adults.1 Local edema from infection narrows the airway, making airway obstruction a life-threatening condition. Upper airway obstruction has a wide variety of infectious and noninfectious etiologies (Table 1). This patient’s fever, abrupt symptom onset, and elevated inflammatory markers suggest an infectious etiology. The differential diagnoses include laryngotracheobronchitis (croup), bacterial tracheitis (BT), peritonsillar abscess (PTA), retropharyngeal abscess (RPA), and diphtheria.Barking cough, hoarseness, and inspiratory stridor are suggestive of croup. Croup causes edema of the larynx, trachea, and large bronchi. Most cases of croup are viral, primarily caused by parainfluenza viruses. While imaging is not required for the diagnosis, narrowing of the subglottic and glottic area, known as the “steeple sign,” is usually seen on x-ray, as seen in this patient, who was obtained after the patient developed stridor (Figure 2).2 However, while cough is highly sensitive and specific for croup, drooling is not typically associated with croup.3 Treatment includes corticosteroids, and if symptomatic at rest, racemic epinephrine is used. Although the patient has parainfluenza and stridor that improves with racemic epinephrine, he has other signs and symptoms that cannot be explained by croup.BT is a rare but dangerous infection of the subglottic trachea, usually occurring after an upper respiratory viral illness. Inflammation from the viral immune response predisposes the trachea to bacterial infection, most commonly with S. aureus, S. pneumoniae, or H. influenzae.4 BT is more common in immunocompromised patients or patients with tracheostomies. Patients present with shortness of breath, dysphonia, and stridor associated with fever, cough, and thick, mucopurulent secretions that may further narrow or obstruct the airway.5 Patients are often toxic appearing, but rarely drool or maintain airway patency via neck extension, as the current patient does.Peritonsillar abscess (PTA) is a deep neck infection that is typically caused by Streptococcus pyogenes and Staphylococcus aureus.6 PTA usually follows either viral or bacterial pharyngitis. Local spread of the infection or bacterial superinfection leads to pus accumulation between the tonsil and its capsule.7 Most common signs and symptoms include fever and sore throat. Physical examination shows the uvula deviating away from the side of the abscess, drooling, and a muffled voice. Trismus can occur if the infection is severe. Treatment includes incision and drainage and antibiotics.6 This diagnosis is less likely in the current patient, as he had bilateral airway edema without trismus.Retropharyngeal abscess (RPA) is a deep neck infection of the oropharynx caused by S. aureus, S. pneumoniae, and H. influenza that commonly follows an upper respiratory tract infection, causing suppurative adenitis in the lymph nodes within the retropharyngeal space.7 Patients present with high fever, dysphagia, odynophagia, and drooling. Patients with RPA characteristically keep their necks in flexion because of pain with extension,6 whereas the current patient preferred to maintain an extended neck, making this diagnosis less likely. Lateral neck radiographs show widening of prevertebral soft tissue.6,8Diphtheria is a life-threatening, toxin-mediated infection caused by Corynebacterium diphtheriae. Diphtheria is rare in the United States because of routine vaccination. Diphtheria is characterized by a thick, gray, adherent pseudomembrane that covers the tonsillar and retropharyngeal area. If this pseudomembrane extends into the larynx or becomes dislodged, fatal airway obstruction can occur.9 Fever, sore throat, cervical lymphadenopathy, and dysphagia are common symptoms. Diphtheria is diagnosed via throat culture and can be complicated by cardiac and neurologic conditions such as myocarditis and palatal palsy.10 Given that the patient was vaccinated and did not have a pseudomembrane on examination, this diagnosis was unlikely.Computed tomography (CT) of the neck demonstrated tonsillitis, cervical adenopathy, and laryngeal edema with thickening extending to the trachea, causing slight narrowing of the subglottic airway (Figure 1). Flexible nasolaryngoscopy revealed mucopurulent secretions, as well as edematous epiglottis and arytenoids. The patient was diagnosed with epiglottitis based on direct visualization of the epiglottis with supporting radiographic images.Epiglottitis can cause life-threatening obstruction of the upper airway. Edema of the epiglottis or subglottic structures can cause a “ball-valve” airway obstruction,11 which is particularly concerning in children as the subglottis constitutes the narrowest part of the airway and is made of highly pliable cartilage as compared with adults.1 The natural patient response, and clinical hallmark of epiglottitis, is “tripod positioning,” where the patient leans forward, extends the neck, and tilts the chin upward to increase airflow through the trachea.12 Additional signs and symptoms in pediatric patients include fever, tachycardia, stridor, and dysphagia.12,13 Historically, epiglottitis is associated with Haemophilus influenzae type B. Since the introduction of the conjugate vaccine in 1987, cases have been rare and have been associated with nontypeable Haemophilus influenzae, Streptococcus species, and Staphylococcus aureus.14,15Epiglottitis is a clinical diagnosis defined as inflammation of the epiglottis or surrounding structures on visual inspection.13 Radiological imaging may confirm the diagnosis. A lateral cervical spine radiograph demonstrates epiglottic edema, known as the “thumb sign,” and narrowing or complete loss of the pocket between the epiglottis and the tongue, known as the “vallecula sign.”16 A CT scan shows thickening of the epiglottis or aryepiglottic folds.12Patients should be kept in a comfortable position. Any maneuver that may obstruct the airway or cause acute agitation should be avoided.14 Respiratory status can quickly deteriorate, so airway specialists should be consulted as soon as possible.17 Furthermore, because the diagnosis is made clinically, laryngoscopy can worsen clinical outcomes if the airway is not previously secured.18Treatment is based on likely causative organisms. Current recommendations endorse the use of a third-generation cephalosporin and an antistaphylococcal agent.19 Corticosteroids and epinephrine are recommended to improve airway edema.14 Supportive care, including IV hydration and inhaled humidified oxygen, has been shown to attenuate the risk of upper airway infection.18The patient is transferred to the pediatric intensive care unit for airway monitoring. IV ceftriaxone and clindamycin are continued to complete a 10-day course. The patient is also treated with methylprednisolone followed by a prednisone taper and inhaled budesonide. He improves without requiring intubation and is safely discharged home.Epiglottitis is a clinical diagnosis that presents with fever, dysphagia, stridor, and drooling, seen mainly in children under 5 years of age. The “tripod sign” of a patient leaning forward, extending the neck, and tilting the chin upward is pathognomonic.12Diagnosis is made by visual inspection; radiological imaging may confirm the diagnosis, including the “thumb sign” and “vallecula sign” on a lateral chest radiograph.20The mainstay of treatment is to keep patients comfortable and avoid maneuvers that may obstruct the airway. Treatment includes third-generation cephalosporins and an antistaphylococcal agent. Corticosteroids and nebulized epinephrine reduce airway edema. Supportive hydration may be indicated.14,18,19Patients should be monitored for airway obstruction, which can be sudden and fatal.18The Haemophilus influenzae type B vaccine is the best protection against epiglottitis. Nontypeable H influenzae, Staphylococcal, and Streptococcal organisms are the common causative agents.14