論文

査読有り 国際誌
2017年12月

Perioperative management of a patient with Coffin-Lowry syndrome complicated by severe obesity: A case report and literature review.

Medicine
  • Mikako Hirakawa
  • ,
  • Tasuku Nishihara
  • ,
  • Kazuo Nakanishi
  • ,
  • Sakiko Kitamura
  • ,
  • Sonoko Fujii
  • ,
  • Keizo Ikemune
  • ,
  • Kentaro Dote
  • ,
  • Yasushi Takasaki
  • ,
  • Toshihiro Yorozuya

96
49
開始ページ
e9026
終了ページ
記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.1097/MD.0000000000009026

RATIONALE: Coffin-Lowry syndrome (CLS) is a rare inherited disease with specific clinical features, such as mental retardation, facial dysmorphism, and cardiac abnormality. In particular, the characteristic facial features of CLS, including retrognathia and large tongue, are associated with difficult ventilation and/or intubation, which is a serious problem of anesthesia management. However, case reports on anesthesia management of CLS are very limited as there are only two published English reports till date. In this case report, we discuss anesthetic and postoperative considerations in patients with CLS, focusing on difficult airway management, and summarize past reports including some Japanese articles. PATIENT CONCERNS: A 25-year-old man with CLS was planning to undergo laminectomy because of progressive quadriplegia caused by calcification of the yellow ligament. We suspected difficulty in airway management because of several factors in his facial features, short thyromental and sternomental distances in computed tomography, severe obesity, and sleep apnea syndrome. DIAGNOSES: Difficult airway was suspected. However, because of mental retardation, awake intubation was considered difficult. INTERVENTIONS: We selected bronchofiberscope-guided nasotracheal intubation, maintaining spontaneous breathing under moderate sedation with a propofol target-controlled infusion. OUTCOMES: Airway management was safely performed during anesthesia induction. LESSONS: In many patients with CLS, difficult intubation was reported, and sedation or slow induction maintaining spontaneous breathing was mainly selected for anesthesia induction. Spontaneous breathing should be maintained during anesthesia induction in case of CLS patients.

リンク情報
DOI
https://doi.org/10.1097/MD.0000000000009026
PubMed
https://www.ncbi.nlm.nih.gov/pubmed/29245289
PubMed Central
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5728904
ID情報
  • DOI : 10.1097/MD.0000000000009026
  • ISSN : 0025-7974
  • PubMed ID : 29245289
  • PubMed Central 記事ID : PMC5728904

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