Anesthesia management in a patient with Zellweger syndrome

Abstract

Objective: Zellweger syndrome (ZS) is an autosomal recessive disorder in the spectrum of peroxisome biogenesis disorders including punctate and is defined as cerebrohepatorenal syndrome due to multiple congenital anomalies including brain, liver and kidneys. We wanted to draw attention to this issue by sharing our anesthetic experiences in a child with Zellweger syndrome.

Case: Appendectomy was planned by pediatric surgery in a 4 year old and 16 kg male patient who was diagnosed as Zellweger syndrome. Physical examination revealed large forehead, large tongue, small chinhypotony, mental motor retardation, and hepatosplenomegaly. Following the introduction of routine monitors, anesthesia were induced intravenously and maintained with sevoflurane in an 50% oxygen- 50% air mixture. The patient was intubated and ventilated with a safe pressure control mode. The patient was hemodynamically stable during surgery. After spontaneous breathing of the patient, extubation was applied smoothly.

Conclusion: Zellweger syndrome includes alarming features for the anesthesiologist. Before any procedure, the pulmonary condition should be carefully evaluated. Hepatic dysfunction may lead to a change in the metabolism of drugs based on hypoalbuminemia, coagulopathy and liver pathways. Since liver dysfunction may lead to coagulopathy, caution should be exercised when applying regional anesthesia techniques.