title: [Chiari type II malformation with brain stem paroxystic dysfunction. What can we do?].
abstract: The Chiari type II malformation is the main cause of death in children with myelomeningocele, usually due to respiratory dysfunction, and unfortunately there is no effective cure. Episodic symptoms of brain stem dysfunction are frequent. We report a child with Chiari type II malformation and brain stem dysfunction who required tracheotomy and who was fed through a nasogastric tube from the age of 1.5 months. He first presented episodic symptoms at the age of 8 months and died at 9 months due to respiratory dysfunction despite intensive care. We also report our experience in the last 12 years with four patients with Chiari type II malformation and episodic symptoms of brain stem dysfunction. Both patients who required mechanical ventilation died. Neither surgical decompression nor intensive care prevented the fatal outcome, which was unpredictable and inevitable. Prognosis may be more positive in less severe cases, because the two patients that did not require mechanical ventilation show favorable outcome to date.
authors.affiliation: Sección de Neuropediatría. Unidad de Cuidados Intensivos Pediátricos. Hospital Infantil Universitario Miguel Servet. Zaragoza. España. jlopezp@hmservet.insalud.es
authors.forename: J
authors.initials: J
authors.lastname: López-Pisón
authors.forename: R
authors.initials: R
authors.lastname: Cabrerizo de Diago
authors.forename: A
authors.initials: A
authors.lastname: Ramírez Gómara
authors.forename: M
authors.initials: M
authors.lastname: Cuadrado Martín
authors.forename: C
authors.initials: C
authors.lastname: Boldova Aguar
authors.forename: J
authors.initials: J
authors.lastname: Melendo Gimeno
mesh_terms: D001139:Arnold-Chiari Malformation / Q000150:complications* / Q000628:therapy; D001927:Brain Diseases / Q000150:complications; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008297:Male; D012121:Respiration, Artificial