Airway Management in CHARGE Syndrome

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  • Kurose Makoto
    Department of Otorhinolaryngology, Sapporo Medical University School of Medicine
  • Mitsuzawa Hiroaki
    Department of Otorhinolaryngology, Sapporo Medical University School of Medicine Department of Otorhinolaryngology, Hokkaido Medical Center for Child Health and Rehabilitation
  • Nagaya Tomonori
    Department of Otorhinolaryngology, Sapporo Medical University School of Medicine
  • Takahashi Nozomi
    Department of Otorhinolaryngology, Sapporo Medical University School of Medicine Department of Otorhinolaryngology, Hokkaido Medical Center for Child Health and Rehabilitation
  • Sato Rina
    Department of Otorhinolaryngology, Sapporo Medical University School of Medicine Department of Otorhinolaryngology, Hokkaido Medical Center for Child Health and Rehabilitation
  • Himi Tetsuo
    Department of Otorhinolaryngology, Sapporo Medical University School of Medicine

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Other Title
  • CHARGE症候群における気道管理
  • 症例 CHARGE症候群における気道管理
  • ショウレイ CHARGE ショウコウグン ニ オケル キドウ カンリ

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Abstract

<p>CHARGE syndrome is a multiple malformation syndrome caused by CHD7 gene mutation. Difficulty in respiration is observed in infancy, and tracheotomy is often performed. Sixteen cases of CHARGE syndrome who had been admitted to our center in the past 20 years were retrospectively reviewed. Eleven cases (68.8%) required long-term respiratory management. The most frequent causative disease was tracheomalachia (7 cases). Tracheotomy was performed in 7 cases, and noninvasive intermittent ventilation (NIV) was performed in 3 cases. Decannulation was accomplished in 3 cases. The age at decannulation was 11 years 6 months, 12 years 1 month and 16 years 2 months respectively. In CHARGE syndrome, airway stenosis and respiratory infections are the factors that determine life expectancy, but QOL of patients and their families will be improved if decannulation is accomplished. It is beneficial for the otologist to remain involved in the management of the airway.</p>

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