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PEDIATRIC ANESTHESIOLOGY

Children with Infantile Neuronal Ceroid Lipofuscinosis Have an Increased Risk of Hypothermia and Bradycardia During Anesthesia

Ning Miao, MD^*, Sondra W. Levin, MD, Eva H. Baker, MD, PhD, Rafael C. Caruso, MD^||, Zhongjian Zhang, MD, PhD, Andrea Gropman, MD^¶#, Deloris Koziol, PhD^**, Robert Wesley, PhD^**, Anil B. Mukherjee, MD, PhD, and Zenaide M. N. Quezado, MD^*

From the *Department of Anesthesia and Surgical Services, NIH Clinical Center; Section on Developmental Genetics, Program on Developmental Endocrinology and Genetics, Eunice Kennedy Shriver, National Institute of Child Health and Human Development, Bethesda, Maryland; Department of Pediatrics, Walter Reed Army Medical Center, Washington, DC; Diagnostic Radiology Department, NIH Clinical Center; ||Visual Function Section, Ophthalmic Genetics and Visual Function Branch, National Eye Institute, Bethesda, Maryland; ¶Department of Neurology, Children’s National Medical Center, Washington, DC; #Medical Genetics Branch, National Human Genome Research Institute; and **Biostatistics and Clinical Epidemiology Service, Office of the Director, NIH Clinical Center, National Institutes of Health, Bethesda, Maryland.

Address correspondence and reprint requests to Zenaide Quezado, MD, Department of Anesthesia and Surgical Services, National Institutes of Health Clinical Center, National Institutes of Health, 10 Center Dr., MSC-1512, Building 10, Room 2C624, Bethesda, MD 20892-1512. Address e-mail to zquezado{at}nih.gov.

Abstract

BACKGROUND: Neuronal ceroid lipofuscinoses (NCLs) are a group of autosomal recessive neurodegenerative diseases characterized by lysosomal accumulation of autofluorescent material in neurons and other cell types. The infantile NCL (INCL) subtype is rare (1 in >100,000 births), the most devastating of childhood subtypes, and is caused by mutations in the gene CLN1, which encodes palmitoyl-protein thioesterase-1.

METHODS: To investigate the incidence of hypothermia and bradycardia during general anesthesia in patients with INCL, we conducted a case-control study to examine the perianesthetic course of patients with INCL and of controls receiving anesthesia for diagnostic studies.

RESULTS: Eight children with INCL (mean age 25 mo [range, 10-32] at first anesthetic) and 25 controls (mean age 44 mo [range, 18-92]) underwent 62 anesthetics for nonsurgical procedures. Patients with INCL had neurologic deficits including developmental delay, myoclonus, and visual impairment. Patients with INCL had lower baseline temperature (36.4 ± 0.1 vs 36.8 ± 0.1, INCL versus controls, P < 0.007), and during anesthesia, despite active warming techniques, had significantly more hypothermia (18 vs 0 episodes, P < 0.001) and sinus bradycardia (10 vs 1, P < 0.001) compared with controls. INCL diagnosis was significantly associated with temperature decreases during anesthesia (P < 0.001), whereas age, sex, and duration of anesthesia were not (P = NS).

CONCLUSIONS: We report that patients with INCL have lower baseline body temperature and during general anesthesia, despite rewarming interventions, are at increased risk for hypothermia and bradycardia. This suggests a previously unknown INCL phenotype, impaired thermoregulation. Therefore, when anesthetizing these children, careful monitoring and routine use of warming interventions are warranted.