Transalar sphenoidal encephalocele and respiratory distress in a neonate: A case report

We present a full-term newborn infant who suffered from immediate postpartu m severe respiratory distress. The infant had an inspiratory strider as a r esult of a swelling of the soft palate, extending from the roof of the naso pharynx. Transoral endotracheal intubation resulted in normal saturation le vels. Histologic examination after an open biopsy showed mature neuroglial tissue. Radiology demonstrated the presence of a right parapharyngeal proce ss obstructing the nasopharynx and oropharynx and extending to the right mi ddle and posterior fossa, via the foramen ovale. After transoral debulking, the infant was extubated successfully. After an uneventful period of 5 mon ths, the patient was readmitted at our hospital for treatment of meningitis . Subsequently, the inspiratory strider recurred, and staged surgery was pe rformed. First, a transcranial approach was used to remove a large intradur al part of the process and close the defect at Meckel's cave. Two weeks lat er the retro- and parapharyngeal part of the process were removed transoral ly. Given the site of the defect of the skull base and the intradural locat ion of the process, the diagnosis is a transalar sphenoidal encephalocele. This is a rare type of basal encephalocele, and has never been reported in an infant nor known to present with respiratory distress. The pathogenesis, clinical presentation, pathology, and therapeutic implications of basal en cephaloceles are discussed.