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

JA Clinical Reports

Nami Kakuta¹  Yuta Uemura¹  Yasuo M. Tsutsumi¹  Katsuya Tanaka¹  Eisuke Hamaguchi¹  Katsuyoshi Kume¹  Yoko Sakai¹  Shinji Kawahito¹ 

[1] Department of Anesthesiology, Tokushima University Hospital;

关键词: Barber-Say syndrome;    Macrostomia;    Cutis laxa;    General anesthesia;   

DOI  :  10.1186/s40981-016-0033-x

来源: DOAJ

【 摘 要 】

Abstract 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.

【 授权许可】

Unknown