Resumo

RODRIGUEZ MIRALRIO, Mario Eduardo; TOXTLE ROMAN, Marco Antonio  e  HUESCA QUINTERO, Carlos Javier. Ohtahara syndrome associated with H-type tracheoesophageal fistula. Bol. Med. Hosp. Infant. Mex. [online]. 2013, vol.70, n.5, pp.380-386. ISSN 1665-1146.

Background. Ohtahara syndrome is an early infantile epileptic encephalopathy characterized by frequent tonic spasms, partial seizures and occasional myoclonus. Interictal EEG characteristically shows a pattern of burst of spikes alternating with phases of suppression of brain electrical activity (''burst-suppression''). Clinical manifestations usually begin before 20 days of life. The few cases reported in the literature do not mention associated congenital malformations. Case report. We report the first case of a 6-month-old male infant with Ohtahara syndrome associated with H-type tracheoesophageal fistula. Conclusions. The association between Ohtahara syndrome and tracheoesophageal fistula may be due to a fortuitous presentation without any relationship with the neurological syndrome.

Palavras-chave : Ohtahara syndrome; seizures; burst-suppression; tracheoesophageal fistula.

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