Torticollis and Hypoxia in a Febrile 3-Year-Old Girl

A previously healthy immunized 3-year-old girl presents to the pediatric emergency department with neck pain. She has 11 days of fever, rhinorrhea, and cough with a maximum temperature of 104°F (40°C) on the day of presentation. She now has difficulty breathing while supine and difficulty moving her neck. Associated complaints include decreased oral intake and urine output and 1 episode of non-bloody diarrhea. Her mother denies any rashes, oral lesions, mucosal changes to the eyes, drooling, swelling of her extremities, choking or gagging episodes, or emesis. There is no recent travel or trauma. Vitals signs are as follows: temperature 102°F (38.9°C), heart rate 176 beats/min, respirations 40 breaths/min, blood pressure 119/66 mm Hg, and oxygen saturation 85% on room air. She is toxic appearing, with respiratory distress and left-sided torticollis with limited active and passive range of motion. Shotty anterior cervical lymph nodes are present bilaterally. She is able to open her mouth. She is tachypneic with diminished breath sounds throughout the left side and tachycardic without rubs, murmurs, or gallops. The abdominal exam is normal. Irritability is present, but the neurologic exam is otherwise normal. She has a negative Kernig sign. Capillary refill is less than 2 seconds, and there are no rashes, bruises, diaphoresis, or cyanosis. Her saturation improves (99%) on 1-L oxygen supplementation. The following tests are obtained as part of the institutional multisystem inflammatory syndrome in children protocol and for the prolonged fever: complete blood count, complete metabolic panel, inflammatory markers, troponin, pro-brain natriuretic peptide, blood culture, respiratory viral panel, and SARS-CoV-2 IgG antibody levels. A chest radiograph (Figure 1) and computed tomographic (CT) scan with intravenous contrast of the neck are obtained. She is positive for respiratory syncytial virus and negative for a current SARS CoV-2 infection on polymerase chain reaction testing. Her laboratory evaluation is notable for elevated neutrophils and bands despite a normal white count, microcytic anemia, mild thrombocytosis, and elevated inflammatory markers. Her blood culture is negative. In addition, she has a mildly elevated anion gap with a lactic acidosis, which corrects to normal range after a 20-ml/kg bolus of normal saline. Her laboratory evaluation is summarized in Table 1.The differential diagnosis for acquired torticollis includes trauma, infection, central nervous system (CNS) and bone pathology, drug reaction, ocular pathology, and paroxysmal conditions. With a fever, specific diagnoses to consider include a retropharyngeal abscess, acute cervical lymphadenitis, viral myositis, foreign body aspiration, Grisel syndrome (nontraumatic subluxation of the atlantoaxial joint), pneumonia with a parapneumonic effusion, cervical osteomyelitis, and cervical discitis.1,2 Kawasaki disease presenting as a retropharyngeal abscess was also considered, in addition to COVID-19 and multisystem inflammatory syndrome in children. Other considerations for neck stiffness included meningitis and peritonsillar abscess.Her chest x-ray (Figure 1) demonstrated diffuse opacification of the left lung. She had a normal para- and retropharyngeal space without atlantoaxial subluxation on the CT scan. A chest ultrasound on admission showed a medium-sized left pleural effusion and trace right pleural effusion. She was diagnosed with a superimposed bacterial pneumonia complicated by a parapneumonic effusion after a respiratory syncytial virus infection. Her torticollis was hypothesized to be secondary to referred pain.1Torticollis is a lateral head tilt associated with the chin rotated toward the contralateral side. It is a clinical sign and not a diagnosis. Congenital torticollis is the most common form.1 Infection and trauma are the most common causes of acquired torticollis.3,4 Children are vulnerable to cervical spine injuries with high-velocity traumatic injuries. Atlantoaxial subluxation is of particular concern during pediatric traumatic injuries when torticollis is a presenting sign.4 Grisel syndrome, however, is a nontraumatic atlantoaxial subluxation. It is associated with head and neck infections as well as ear, nose, and throat surgeries and was considered high on the differential during this case prior to obtaining the CT scan.5–7Upper respiratory tract infections, retropharyngeal and parapharyngeal abscess, cervical adenitis, and sternocleidomastoid myositis have been associated with torticollis.4,6 Osteomyelitis, discitis, and pyomyositis should be considered if there is any concern for muscular or bony tenderness on exam. Rarely, Kawasaki disease and juvenile idiopathic arthritis have also been noted as the underlying diagnosis.8–11 Although rare, pneumonia has been described as a cause of torticollis with referred pain from pleural irritation, with upper lobe pneumonia more commonly cited as underlying pathophysiology.12 Additional life-threatening causes of pediatric torticollis include spinal and epidural hematomas, posterior fossa and spinal cord tumors, and suppurative jugular thrombophlebitis. CNS signs such as hypertension and bradycardia concerning for raised intracranial pressure, optic disk edema (indicating increased intracranial pressure), cranial nerve deficits, change in gait, extremity muscle weakness, or changes in bowel and bladder function may help to delineate CNS pathology from other causes of torticollis.1,4 Neck tilt can also be a compensatory mechanism for diplopia, which may reflect ocular or CNS pathology.It is important to ascertain if there is airway compromise in a patient exhibiting torticollis. Retropharyngeal abscess, parapharyngeal abscess, or brain stem mass can present with signs of upper airway obstruction or loss of airway maintenance. Pediatric patients may present with respiratory distress and hypoxia, as in this case, which may suggest an upper or lower respiratory tract infection. However, diaphragmatic paralysis because of CNS lesions must also be considered in context of the exam. Certain risk factors may increase predisposition to a particular cause. For example, children with Trisomy 21 have an increased risk of atlantoaxial instability, or a spinal or epidural hematoma may be more likely in the context of a hematologic disorder.Acquired torticollis is typically a transient clinical sign when the underlying pathology is appropriately treated. Diagnosing atlantoaxial subluxation is key to mitigating spinal trauma. Imaging typically includes x-rays with open-mouth odontoid, anterior-posterior, and lateral views. Since an odontoid view may be difficult to obtain in a pediatric patient with torticollis, a CT scan with contrast can be helpful in this evaluation, while also assessing for superficial and deep neck space infections or masses. If a CT scan demonstrates atlantoaxial subluxation, a magnetic resonance image is a better tool for diagnosing nonosseous spinal cord injury.13 Neurosurgery involvement is key in determining if immediate intraoperative surgical intervention including cervical traction is needed vs conservative management with benzodiazepines for muscle relaxation and watchful waiting. Head imaging is warranted in the presence of CNS symptoms to rule out intracranial pathology. Otherwise, symptomatic management can include cervical collar placement and nonsteroidal anti-inflammatory drugs to help with inflammation and pain.14,15The patient initially received empirical broad-spectrum antibiotics with ceftriaxone and vancomycin at meningitic dosing. Ibuprofen was given for antipyresis and pain management. A thoracostomy tube was used to drain the parapneumonic effusion after admission. She was quickly weaned off supplemental oxygen. Her antibiotic regimen was narrowed to ceftriaxone and sulfamethoxazole/trimethoprim and subsequently amoxicillin/clavulanic acid and sulfamethoxazole/trimethoprim for a total of 4 weeks. The patient’s torticollis was the first sign to improve after admission.Pneumonia complicated by a parapneumonic effusion can present as acquired torticollis. Prolonged respiratory symptoms, hypoxia, and a febrile illness increase the likelihood of this diagnosis.It is important to determine if the torticollis is secondary to atlantoaxial subluxation on imaging since neurosurgical management would be indicated.Acquired torticollis secondary to infectious causes such as a parapneumonic effusion resolves with adequate pain management and with resolution of the primary illness.