Review article: Perioperative care of patients with epidermolysis bullosa: proceedings of the 5th international symposium on epidermolysis bullosa, Santiago Chile, December 4–6, 2008

Authors

  • KENNETH GOLDSCHNEIDER MD, FAAP,

    1. Division of Pain Management, Department of Anesthesiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
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  • ANNE W. LUCKY MD,

    1. Cincinnati Children s Epidermolysis Bullosa Center,The University of Cincinnati College of Medicine
    2. Division of Pediatric Dermatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
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  • JEMIMA E. MELLERIO MD, FRCP,

    1. Guy’s and St. Thomas’ NHS Foundation Trust, St. John’s Institute of Dermatology, London, UK
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  • FRANCIS PALISSON MD,

    1. Department of Dermatology, Facultad de Medicina, Clinica Alemana Universidad del Desarrollo, Santiago, Chile
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  • MARIA Del CARMEN VIÑUELA MIRANDA MD,

    1. Department of Anesthesiology, Clinica Alemana Universidad del Desarrollo, Santiago, Chile
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  • RICHARD G. AZIZKHAN MD, PhD, FAAP, FACS

    1. Division of General and Thoracic Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati. OH, USA

      Section Editor: Dr Jerrold Lerman
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Kenneth R. Goldschneider, MD, FAAP, Division of Pain Management, Department of Anesthesiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA (email: Kenneth.goldschneider@cchmc.org).

Summary

Epidermolysis bullosa (EB) has become recognized as a multisystem disorder that poses a number of pre-, intra-, and postoperative challenges. While anesthesiologists have long appreciated the potential difficult intubation in patients with EB, other systems can be affected by this disorder. Hematologic, cardiac, skeletal, gastrointestinal, nutritional, and metabolic deficiencies are foci of preoperative medical care, in addition to the airway concerns. Therefore, multidisciplinary planning for operative care is imperative. A multinational, interdisciplinary panel of experts assembled in Santiagio, Chile to review the best practices for perioperative care of patients with EB. This paper presents guidelines that represent a synthesis of evidence-based approaches and the expert consensus of this panel and are intended to aid physicians new to caring for patients with EB when operative management is indicated. With proper medical optimization and attention to detail in the operating room, patients with EB can have an uneventful perioperative course.

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