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[Anesthetic experience of two patients with holoprosencephaly]
Insights
Anesthetic management for infants with holoprosencephaly (HP) requires careful monitoring of body temperature and seizure prevention. Successful surgical repair of bilateral cleft lip was achieved in two young patients with HP.
Area of Science:
- Anesthesiology
- Pediatric Surgery
- Medical Genetics
Background:
- Holoprosencephaly (HP) is a congenital disorder resulting from incomplete forebrain division.
- HP presents with characteristic facial anomalies, autonomic dysfunction, and potential neurological complications like seizures.
- This study focuses on anesthetic considerations for surgical repair in infants diagnosed with HP.
Observation:
- Two 6-month-old female patients with different types of holoprosencephaly (lobar and alobar) underwent bilateral cleft lip repair.
- Anesthetic induction utilized N2O-O2-halothane, with maintenance via fentanyl in N2O-O2.
- Intraoperative and postoperative management included vigilant monitoring and adjustment of body temperature, heart rate, and airway patency.
Findings:
- The first patient experienced postoperative seizures and bradycardia, managed with diazepam and isoproterenol.
- The second patient developed hyperthermia, seizures, and airway obstruction, requiring appropriate interventions.
- Both cases highlight the complex physiological challenges associated with anesthetic management in HP patients.
Implications:
- Effective anesthetic protocols for holoprosencephaly patients must prioritize seizure prevention and control.
- Maintaining normothermia and hemodynamic stability are critical during and after surgical procedures.
- Careful airway management is essential due to the potential for obstruction in HP patients.
Abstract:
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.