Intra-and postoperative management in a patient with holoprosencephaly

Abstract

Intra-and postoperative management of patients with holoprosencephaly is very difficult due to great variations in blood pressure and/or body temperature conbined with respiratory problems.
Case report: The patient was a female with holoprosencephaly type IV complicated by cleft lip and Palate VSD, diabetes insipidus, hypernatremia and respiratory problems. Plastic surgry of lip was performed at the age of 11 month.
Anesthesia was introduced after fentanyl citrate and diazepam i.v. with no premedication, and the operation was done under oxide/fentanyl citrate/enflurane anesthesia. During the operation, central, peripheral and rectal temperatures were monitored.
During and after the operation, variations in body temperature were great, but other vital signs and serum electrolytes were stable.