P2D125: “clivus Osteomyelitis Presenting as Headache and Torticollis in an Immunocompetent Six-year-old Female”

Introduction: Central skull base osteomyelitis (CSBO) is an uncommon but potentially life-threatening entity. It is often a complication of nasopharyngeal infection and has been reported in both immunocompromised and immunocompetent children. Pediatricians must have a high index of suspicion for CSBO and should initiate prompt treatment to prevent disease extension and decrease mortality rates. We present a case of a six-year-old immunocompetent female with a history of headache and no medical history, in whom a mass was detected around the clivus on magnetic resonance imaging.

Case Description: A 6-year-old girl without any relevant history, presented to our hospital with symptoms of 1 month onset consistent with frontal, oppressive, and non-pulsatile headache that radiated to occipital and cervical regions, with consequent movement limitation and subjective upper and lower extremity weakness. Physical examination revealed marked limitations of neck motion. A non-contrast MRI of the brain had been performed 2 weeks prior to her visit to our hospital and showed no lesions of concern. A repeat contrast MRI of the brain performed at our institution showed an expansive lesion replacing the clivus and extending anteriorly to the posterior wall of the rhinopharynx.
Laboratory examinations showed no abnormalities. The patient underwent an emergency endoscopic transsphenoidal approach which revealed erosion of the anterior clivus wall and purulent secretion. The samples obtained during the procedure showed inflammatory tissue, clivus tissue cytometry showed poor cellularity and normal B lymphocytes with no abnormal lymphoid population. Staphylococcus aureus multisensitive was detected in blood cultures and in the clival collection sample. These findings lead to the diagnosis of CSBO. The patient was treated with a six-week course of cephalexin and acute phase reactants monitoring every week until the treatment was completed.

Discussion: Central skull base osteomyelitis is a rare but potentially life-threatening complication of nasopharyngeal infections.1 It is more commonly associated with diabetic and older or immunodeficient patients, but it has also been reported in immunocompetent pediatric patients 2. In the pediatric population, it is often caused by the contiguous spread of microorganisms. Diagnosing this entity in children is challenging since symptoms are non-specific or vague, and signs of infection may be absent in immunocompetent patients. The typical radiologic finding of CSBO is non-specific soft tissue inflammation rather than bone marrow infiltration, as seen in our present case.3 The standard treatment for CSBO is long- term intravenous antibiotics and should be initiated empirically until the causative microorganism is confirmed by culture.

Conclusion: The present case shows the importance as pediatricians of being aware and considering CSBO in the potentially differential diagnosis of headache and torticollis in the ER. Neuroimaging is useful for diagnosing CSBO and aggressive culture guided antibiotics are imperative to prevent complications and decrease mortality rates.