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[Anesthetic experience of two patients with holoprosencephaly].

We experienced anesthetic management of two 6-month-old female patients with holoprosencephaly (HP). HP is characterized by hypoplasia of prosencephalon, facial anomalies (hypotelorism, flat nose, and/or small prolabium), abnormality of autonomic nervous system functions (hypernatremia and/or poikilothermia), and clonic convulsion. First case was a lobar type and the second case was an alobar type according to DeMyer's classification. In both cases repair of bilateral cleft lip was performed. Anesthesia was slowly induced with N2O-O2-halothane and maintained with fentanyl in N2O-O2. Body temperature (BT) was adjusted with a warming blanket while monitoring rectal temperature. In the first case clonic convulsion and bradycardias occurred during the postoperative period, which were treated with IV diazepam and isoproterenol. In the second case BT rose to 39 degrees C during postoperative period; 2 episodes of convulsions were observed; and airway obstruction occurred. They were treated accordingly. The most important points which should be kept in mind in the management of HP patients are the prevention of convulsion, adjustment of BT, maintenance of normal pulse rate and keeping a patient airway.