General anesthesia of a Japanese infant with Barber-Say syndrome : a case report

• Hamaguchi, Eisuke

• Tsutsumi, Yasuo M.

• Kume, Katsuyoshi

• Sakai, Yoko

• Kakuta, Nami

• Uemura, Yuta

• Kawahito, Shinji

• Tanaka, Katsuya

抄録

Background: Barber-Say syndrome (BSS) is a very rare congenital disorder characterized by macrostomia, cutis laxa, and other features. We report our experience of performing general anesthesia on a Japanese child with BSS. Case presentation: A bilateral repair of the corners of the mouth under general anesthesia was planned for an 18-month-old male with macrostomia; the child was 75 cm in height and weighed 9.9 kg. As insertion of the peripheral intravenous catheter was difficult, it was inserted before the surgery by a pediatrician. The patient wore a mask and was ventilated manually after loss of consciousness with intravenous anesthesia. A mask for adults provided a superior fit and was effective in preventing air leakage from the corners of the mouth. After rocuronium was administered, the larynx was spread with a Macintosh laryngoscope. There was no laryngeal anatomical abnormality, and tracheal intubation was readily possible. The operation was completed without incident. Stiffening of both arms occurred for several seconds one hour after the operation ended, but the patient did not develop other complications. Conclusions: Mask ventilation and the insertion of an intravenous catheter may be difficult in the general anesthesia of patients with BSS, and anesthetic management requires caution.

収録刊行物

• JA Clinical Reports

  JA Clinical Reports 2 11-, 2016-06-02

  Japanese Society of Anesthesiologists|Springer Nature

キーワード

• Barber-Say syndrome
• Macrostomia
• Cutis laxa
• General anesthesia

詳細情報

• CRID: 1050569094143774208
• NII論文ID: 120006939618
• ISSN: 23639024
• Web Site: http://repo.lib.tokushima-u.ac.jp/114560
• 本文言語コード: en
• 資料種別: journal article
• データソース種別:
  • IRDB
  • CiNii Articles