Postsurgical pneumoencephaly in a patient with Klippel-Feil syndrome

A 49-year-old woman presented with occipital headaches, dizziness, photophobia and vomiting for 2 weeks' status post posterior scalp mole removal by a general surgeon. The physical examination revealed occipital lesions with foul smelling purulent discharge mixed with clear fluid drainage, webbed neck with decreased range of motion, facial asymmetry and a low posterior hairline. A CT of head showed occipital skull defect providing a communicating pathway for scalp infection, an atlas fusion defect and an extensive pneumoencephaly. Further imaging showed low-lying cerebellar tonsils and fusion of the body of second and third cervical vertebrae. Klippel-Feil syndrome was diagnosed and the patient was successfully managed with duraplasty of occipital dura, placement of a lumbar drain and antibiotics. In patients with findings suggestive of Klippel-Feil syndrome and lesions proximal to the CNS, appropriate neuroimaging and possible neurosurgical consult should be considered prior to any surgical intervention.