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 retro- and 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.
Skip Nav Destination
Article navigation
1 January 1999
Electronic Article|
January 01 1999
Transalar Sphenoidal Encephalocele and Respiratory Distress in a Neonate: A Case Report
Address correspondence to E.A.M. Mylanus, Department of Otorhinolaryngology, University Hospital Nijmegen, Philips van Leydenlaan 15, Postbus 9101, 6500 HB Nijmegen, The Netherlands.
Pediatrics (1999) 103 (1): e12.
Article history
Received:
March 31 1998
Accepted:
July 20 1998
Citation
Emmanuel A.M. Mylanus, Henry A.M. Marres, José Vlietman, Louis A.A. Kollée, Hans Peter M. Freihofer, Henk O.M. Thijssen, Joost de Vries, Pieter Wesseling; Transalar Sphenoidal Encephalocele and Respiratory Distress in a Neonate: A Case Report. Pediatrics January 1999; 103 (1): e12. 10.1542/peds.103.1.e12
Download citation file:
0 Comments
Comments Icon
Comments (0)
Sign in
Don't already have an account? Register
Pay-Per-View Access
$25.00