[Anesthetic Management of an Adrenoleukodystrophy Patient for Intrathecal Baclofen Therapy].

A 34-year-old man with adrenoleukodystrophy (ALD) was scheduled for pump system insertion of intrathecal baclofen therapy under general anesthesia. ALD, a rare genetic disorder, is associated with a total body increase in long chain fatty acids caused by defective degradation, and includes various nervous system abnormalities, muscular weakness, in addition to adrenal insufficiency. He had contracture of the both legs, and muscular weakness of the left hand, and Mallampati class III, but no respiratory disability. In the operating room, we administered hydrocortisone 100 mg for steroid coverage, and low-dose midazolam, and fentanyl. As spontaneous breathing remained, we could easily see epiglottis and arytenoid cartilage by McGRATH. Therefore we selected rapid-induction of anesthesia with thiamylal, and rocuronium 40 mg, under cricoid pressure. We avoided propofol. Anesthsia was maintained with sevoflurane and remifentanil, monitoring BIS and train of four. No more rocuronium was administered, and anesthesia was uneventful. Intrathecal baclofen therapy is given to patients who have severe contracture. When we selected general anesthesia, we should be aware of the possibility of muscular weakness, and cannot intubate cannot ventilate scenario.

[A child with Klippel-Feil syndrome in whom GlideScope was effective for tracheal intubation].

GlideScope videolaryngoscope (GlideScope, herein-after referred to as "GS", Verathon Medical, Bothell, WA, USA), with a high-resolution camera positioned on a blade, enables operators to confirm the position of the larynx and a tube through clear view, thereby conducting intubation safely in a patient whose neck is difficult to be bent back. As the blade is slim, GS is indicated for use in children whose oral cavity is narrow. We herein report safe and smooth intubation with GS in a child with Klippel-Feil syndrome in whom difficult intubation was predicted.

[Fiberoptic tracheal intubation through a laryngeal mask airway in a pediatric patient with treacher collins syndrome].

A 6-month-old girl with Treacher Collins syndrome was scheduled for tracheotomy because of severe airway obstruction. During slow induction of anesthesia with inhalation of sevoflurane, assisted mask ventilation was successfully performed using oropharyngeal airway. Either direct laryngoscope or GlideScope Video Laryngoscope could not reveal any part of the epiglottis (Cormack and Lehane grade 4). Even fiberoptic bronchoscopic examination assisted by GlideScope Video Laryngoscope gave a poor view of the pharynx on the video monitor. Laryngeal mask airway (LMA) was inserted easily and allowed adequate ventilation, through which fiberoptic intubation was successfully achieved. We cut the LMA short in order to pass the 3 mm tracheal tube until the glottis through it.