[Anesthesia for an Eleven Year Old Girl with Sjögren-Larsson Syndrome].

Abstract

Sjögren-Larsson syndrome (SLS) is an autosomal recessive disease characterized by a triad of congenital ichthyosis, spastic quadriplegia and mental retardation. An 11-year-old girl (body weight 30 kg) diagnosed as SLS was admitted with Benett fracture of the right thumb. She was monitored with standard protocol. General anesthesia was induced by rapid induction method with propofol 50 mg and rocuronium 0.6 mg - kg⁻¹. She was ventilated with bag and mask, and intu- bated without difficulty. Neuromuscular function was continuously assessed by 40 mA-TOF-stimulation re- sponses with acceleromyography immediately after induction of general anesthesia by TOF-Watch® SX (Organon Ireland, division of MSD, Ireland). Anesthe- sia was maintained with total intravenous anesthesia with propofol 6-8-10 mg⁻¹ · hr⁻¹ in oxygen. No abnor- mal responses were observed by TOF-Watche SX despite the neuromuscular disease. Residual effect of the neuromuscular blocking agent was successfully reversed by sugammadex and she was extubated without any respiratory trouble. She was discharged on the postoperative day 1 without complications.