Abstract
The anaesthetic management of two children with junctional epidermolysis bullosa, formerly called the letalis form, is described. Anaesthesia for children with this disease has not been described previously. Special precautions were taken to avoid mucosal injury and some customary monitoring devices were omitted. The previous anaesthetic literature, which discusses related but distinct forms of bullous skin diseases, is reviewed. The special concerns which relate to airway management in this disease are discussed.
Résumé
On décrit la conduite anethésique chez deux enfants souffrant ďépidermolyse huileuse jonctionnelle, antérieurement appelée forme mortelle. Ľanesthésie chez les enfants souffrant de cette maladie n’a pas été décrite antérieurement. On a pris des précautions particulières afin ďéviter des lésions aux muqueuses et certains appareils de monitorale habituels ont été omis. On fait une revision des écrits anesthésiques antérieurs discutant de formes reliées, mais différentes, de dermatose huileuse. On discute des inquiétudes particulières concernant ľentretien de la perméabilité des voies respiratoires dans cette maladie.
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Pearson RW, Potter B, Strauss F. Epidermolysis bullosa hereditaria lethalis. Arch Dermatol 1974; 109: 349–55.
Hruby MA, Esterly NB. Anemia in epidermolysis bullosa lethalis. Am J Dis Child 1973; 125: 696–9.
Gedde-Daht T. Sixteen types of epidermolysis bullosa. Acta Dermatovener 1981; 95: 74–87.
Tomlinson AA. Recessive dystrophic epidermolysis bullosa — the anaesthetic management of a case for major surgery. Anaesthesia 1983; 38: 485–91.
Turner TW. Two cases of junctional epidermolysis bullosa (Herlitz-Pearson). Br J Dermatol 1980; 102:97–107.
Schachner L, Lazarus GS, Dembitzer H. Epidermolysis bullosa hereditaria letalis. Br J Dermatol 1977; 96: 51–8.
James I, Wark H. Airway management during anesthesia in patients with epidermolysis bullosa dystrophica. Anesthesiology 1982; 56: 323–6.
Thompson JW, Ahmed AR, Dudley JP. Epidermolysis bullosa dystrophica of the larynx and trachea — acute airway obstruction. Ann Otol 1980; 89: 428–9.
Smith GB, Shribman AJ. Anaesthesia and severe skin disease, Anaesthesia 1984; 39: 443–55.
Wilson F. Epidermolysis bullosa — a rare disease of anaesthetic interest. Br J Anaesth 1959; 31: 26–31.
Reddy ARR, Wong DHW. Epidermolysis bullosa — a review of anaesthetic problems and case reports. Can Anaesth Soc J 1972; 19: 536–48.
Yonfa FA, Thomas JP, Labbe R, Roebuck BL, Hoar KJ, Gutierrez JF, Mackall LL. Epidermolysis bullosa — a protocol for general anesthesia and successful dental rehabilitation. Anesthesiology Review 1982; 9: 20–5.
Kubota Y, Norton ML, Goldenberg S, Robertazzi RW. Anesthetic management of patients with epidermolysis bullosa undergoing surgery. Anesth Analg 1961; 40: 244–50.
Marshall BE. A comment on epidermolysis bullosa and its anaesthetic management for dental operations. Br J Anaesth 1963; 35: 724–7.
Hamann RA, Cohen PJ. Anesthetic management of a patient with epidermolysis bullosa dystrophica. Anesthesiology 1971; 34: 389–91.
Milne B, Rosales JK. Anaesthesia for correction for oesophageal stricture in a patient with recessive epidermolysis bullosa dystrophica — a case report. Can Anaesth Soc J 1980; 27: 169–71.
Micheels J. Anaesthetic management of a child with epidermolysis bullosa. Acta Anaesth Belge 1978; 2: 141–50.
Berryhill RE, Benumof JL, Saidman LJ, Smith PC, Plumer MH. Anesthetic management of emergency cesarean section in a patient with epidermolysis bullosa dystrophica polydysplastica. Anesth Analg 1978; 57: 281–3.
Morgan WC. Dental anesthctic management of epidermolysis bullosa — a new approach. Oral surg 1975; 40: 732–5.
Ramdass T, Thangavelu TA. Epidermolysis bullosa and its E.N.T. manifestations — two case reports. J Laryngol Otol 1978; 92: 441–6.
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Holzman, R.S., Worthen, H.M. & Johnson, K.L. Anaesthesia for children with junctional epidermolysis bullosa (letalis). Can J Anaesth 34, 395–399 (1987). https://doi.org/10.1007/BF03010142
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DOI: https://doi.org/10.1007/BF03010142