Transalar Sphenoidal Encephalocele and Respiratory Distress in a Neonate: A Case Report

We present a full-term newborn infant who suffered from immediate postpartum severe respiratory distress. The infant had an inspiratory stridor as a result of a swelling of the soft palate, extending from the roof of the nasopharynx. Transoral endotracheal intubation resulted in normal saturation levels. Histologic examination after an open biopsy showed mature neuroglial tissue. Radiology demonstrated the presence of a right parapharyngeal process obstructing the nasopharynx and oropharynx and extending to the right middle and posterior fossa, via the foramen ovale. After transoral debulking, the infant was extubated successfully. After an uneventful period of 5 months, the patient was readmitted at our hospital for treatment of meningitis. Subsequently, the inspiratory stridor recurred, and staged surgery was performed. First, a transcranial approach was used to remove a large intradural part of the process and close the defect at Meckel’s cave. Two weeks later the retroand parapharyngeal part of the process were removed transorally. Given the site of the defect of the skull base and the intradural location 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 encephaloceles are discussed. Pediatrics 1999;103(1). URL: http:// www.pediatrics.org/cgi/content/full/103/1/e12; basal encephalocele, respiratory distress, inspiratory stridor, meningitis. ABBREVIATIONS. MRI, magnetic resonance imaging; CT, computed tomography; CSF, cerebrospinal fluid. This article reports a pharyngeal mass consisting of mature neuroglial tissue causing life-threatening respiratory distress and meningitis in a full-term newborn infant. A combination of clinical, radiologic, and pathologic findings led to the diagnosis and transcranial and transoral surgical treatment of a transalar sphenoidal encephalocele. A multidisciplinary approach was essential in the successful management of the patient.