“Pain in the Neck”: Acute-Onset Neck Pain in an Adolescent Girl

A 10-year-old girl presents to the pediatric emergency department (ED) with acute onset of sharp neck pain and torticollis that began on awakening the morning of presentation. She has had shoulder pain for the past 3 days and in addition developed pain in her neck the morning of presentation. This morning she developed numbness in her legs and inability to walk. She has no associated headache, dizziness, fever, or urinary or bowel incontinence.She had a recent ED visit for flank pain due to constipation treated with polyethylene glycol 9 days earlier. Her medical history includes asthma and eczema. She is fully immunized. She has had a 12-lb (5.4-kg) weight loss in the previous 7 weeks.Her initial vital signs are as follows: temperature, 98.8°F (37.1°C); pulse, 104 beats/min; blood pressure, 138/91 mm Hg; respiratory rate, 20 breaths/min; and oxygen saturation, 100% on room air. She is generally well-appearing but in acute distress secondary to neck pain. She is lying in bed holding her neck laterally flexed to the right, refusing to move it, and she cries out in pain with any attempt to move her head to midline. She has abdominal tenderness with normal bowel sounds and no hepatosplenomegaly or masses. Her neurologic examination is significant for 3/5 strength in the upper extremities and inability to move the lower extremities spontaneously except toe wiggling. Sensation is intact throughout. She has a flexor plantar response bilaterally, but no deep tendon reflexes could be elicited in her upper or lower extremities. She is refusing to sit up or move in bed due to neck pain.A complete blood cell count, comprehensive metabolic panel, C-reactive protein level, and creatine phosphokinase level were all normal.Neck pain, a common chief complaint in the pediatric ED, is most often musculoskeletal, traumatic, or infectious in origin. The typical etiologies include muscular spasm; cervical spine injury; peritonsillar, retropharyngeal, or other deep neck abscess; and meningitis or epidural abscess. Less commonly, neck pain can be secondary to arthritis, congenital malformations, vertebral anomalies, and posterior fossa or cervical spinal tumors. (1) It is important to take a thorough history with attention to the timing and specific characteristics of pain, including quality, radiation, aggravating or relieving factors, and any associated symptoms to help build a differential diagnosis and determine the need for radiologic and laboratory investigation. (2)Neurology was consulted and recommended gabapentin and ketorolac treatment for pain. She was admitted for further evaluation and management. The following day she was noted to have normal strength and reflexes in the upper extremities but 2/5 strength, hypotonia, and absent reflexes in the lower extremities, including absent Babinski sign. She also developed urinary retention requiring Foley catheter placement. Magnetic resonance imaging (MRI) of the brain and complete spine was performed and demonstrated a solidly enhancing lesion in the thoracic cord at T5-T6 through T6-T7 with a peripherally enhancing single cystic component extending cranially to approximately C5 (Fig 1). There were areas of hypointensity in the upper thoracic portion consistent with hemorrhage, as well as T2 hyperintensity extending cranially to the craniocervical junction (Fig 2) and caudally to the conus (Fig 3), consistent with edema. Neurosurgery was consulted and recommended dexamethasone treatment to reduce edema. The patient was brought to the operating room for laminoplasty and tumor resection, after which she experienced lower extremity paralysis and urinary incontinence. Surgical pathology demonstrated a diffuse midline glioma, H3 K27M mutation (World Health Organization grade IV). Oncology discussed treatment options with the family and noted that