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KMID : 1155520200150010120
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Anesthesia and Pain Medicine
2020 Volume.15 No. 1 p.120 ~ p.123
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Anesthetic considerations for an adult with Wolf- Hirschhorn syndrome - A case report -
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Kim Hye-Jin
You Jun-A
Park Se-Yeon
Kim Eun-Jung
Park Soon-Ji
Kim Hee-Young
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Abstract
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Background: Wolf-Hirschhorn syndrome (WHS) includes features such as growth restriction, mental retardation, congenital heart disease, convulsions as well as microcephaly and micrognathia. Thus, the anesthesiologists may have difficulties in airway management, neuromuscular relaxation, and in maintaining hemodynamic stability.
Case: A 24-year-old man with WHS underwent surgery for closed reduction and internal fixation of the right neck of femur. His face showed features typical of patients with WHS such as a prominent glabella, hypertelorism, micrognathia, low-set malformed ears, and a down-turned mouth. Since difficult airway management was expected, a video-assisted laryngoscope was used for successful intubation. The surgery terminated without any problems under total intravenous anesthesia.
Conclusions: A patient with WHS may have a variety of problems. Therefore, through careful evaluation of the airway, cardiovascular system, and cerebral nervous system and by making an appropriate anesthetic plan, anesthesiologists can perform a safe general anesthesia in patients with WHS.
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KEYWORD
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Adult, Airway management, Chromosome abnormality, General anesthesia, Wolf-Hirschhorn syndrome
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