Abstract:

Hurler Syndrome is a rare autosomal recessive disorder associated with progressive mental retardation and glycosamine deposition in several organs. We present a kyphoscoliotic patient with Hurler syndrome scheduled for ventriculoperitoneal shunt placement and discuss the anesthetic management. The patient presented with many features complicationg all phases of anesthesia such as kyphoscoliosis, restrictive and obstructive pulmonary disease. Several problems such as hypercarbia and secretions necessitated sugammadex for reversal of neuromuscular blockade. The patients presenting with congenital metabolic syndromes are difficult to manage, either by general anesthesia or regional anesthesia. With increased lifespans, they more frequently undergo surgeries requiring general anesthesia. We are in opinion that sugammadex provides prompt recovery of muscular function without increasing secretions, and safer anesthesia.

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