Abstract
The anaesthetic management of children with glycogen-storage disease type lia (Pompe’s disease) presents a variety of challenges. A modification of a femoral nerve block, the inguinal paravascular block, as described by Winnie, was used in conjunction with intravenous ketamine to provide anaesthesia for a diagnostic muscle biopsy in a 5.5-month-old infant with Pompe’s disease. A peripheral nerve stimulator was used to locate the femoral nerve in lieu of eliciting a paraesthesia.
Résumé
La conduite anesthésique des enfants atteints de maladie d’entreposage de glycogène de type lia présente une variété de défis. Une modification du block du nerf fémoral, le block paravasculaire inguinal telle que décrite par Winnie, a été utilisée simultanément avec l’induction intraveineuse de kétamine afin de fournir l’anesthésie pour une biopsie musculaire diagnostique chez un enfant âgé de 5.5 mois atteinte d’une maladie de Pompe.
Un stimulateur nerveux périphérique était utilisé afin de localiser le nerf fémoral et de provoquer la pares-thésie.
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References
Nihill MR, Wilson DS, Hugh-Jones K. Generalized glycogenosis type II (Pompe’s disease). Arch Dis Child 1970; 45: 122–9.
Ellis FR. Neuromuscular disease and anaesthesia. Br J Anaesth 1974; 46: 603–12.
Winnie AP, Ramamurthy S, Durran, Z. The inguinal paravascular technic of lumbar plexus anesthesia. The“3-in-l block”. Anesth Analg 1973; 52: 989–96.
Wright B. A new use for the block-aid monitor. Anesthesiology 1969; 30: 236–7.
Harris LC, Powell G, Brown OW III.Pompe’s disease. Pediatr Clin North Am 1978; 25: 861–7.
Caddell JL.Whittemore R. Observations of generalized glycogenosis with emphasis on electrocar-diographic changes. Pediatrics 1962; 29: 743–63.
Hernandez A Jr.Marchesi V, Goldring D, Kissane J, Hartmann AF. Cardiac glycogenosis. J Pediair 1966; 68: 400–12.
Ehlers KH, Hagstrom JWC, Lukas PS, Redo SF, Engle MA. Glycogen-storage disease of the myocardium with obstruction to left ventricular outflow. Circulation 1962; 25: 96–109.
Rees A, Minhas K, Solinger R. Echocardiographic evidence of outflow tract obstruction in Pompe’s disease (Glycogen-storage disease of the heart). Am JCardiol 1976; 37: 1103–6.
Hohn AR, Lowe CU, Sokal JE, Lambert EC. Cardiac problems in glycogenosis with specific reference to Pompe’s disease. Pediatrics 1965; 35: 313–21.
Bharti S, Serrano M, DuBrow I et al. The conduction system in Pompe’s disease. Pediatr Cardiol 1982; 2: 25–32.
Muller OF, Bellet S.Ertrugrul A. Glycogen-storage disease: report of a case with generalized glycogenosis and review of the literature. Circulation 1961; 23: 261–8.
Adams FH, Emmanouilides GC. Heart Disease in Infants, Children, and Adolescents. Baltimore: Williams and Wilkins, 1983.
Abajian JC, Mellish RWP, Browne AF, Perkins FM, Lambert DH, Mazuzan JE. Spinal anesthesia for surgery in the high-risk infant. Anesth Analg 1984; 63: 359–62.
Loubser P, Kyoung S, Cohen S. Adverse effects of spinal anesthesia in a patient with idiopathic hypertrophic subaortic stenosis. Anesthesiology 1984; 60: 228–30.
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Rosen, K.R., Broadman, L.M. Anaesthesia for diagnostic muscle biopsy in an infant with Pompe’s disease. Can Anaesth Soc J 33, 790–794 (1986). https://doi.org/10.1007/BF03027132
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DOI: https://doi.org/10.1007/BF03027132