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.