Anaesthesiological considerations in Costello syndrome

Anesthesia considerations for patients with de Barsy syndrome

“…While an iatrogenic etiology is a possible explanation, the repeated occurrence of hyperthermia in the same patient suggests an unknown etiology or possible underlying metabolic disorder similar to that of osteogenesis imperfecta and Costello syndrome patients. Interestingly, in two case reports of intraoperative hyperthermia in patients with Costello syndrome, isoflurane was used in one and sevoflurane in the other [14,18]. In our review, sevoflurane was the only anesthetic agent or adjuvant found in common with all 4 cases of hyperthermia.…”

Anaesthetic implications of Costello syndrome

“…Of primary importance to perioperative care are issues related to airway management. Several factors may impact on airway management including a short neck, macroglossia, hypertrophied tonsillar and supraglottic tissues, laryngeal papillomata (8) and choanal atresia (9). Two previous reports, both letters to the editor, specifically addressing anaesthetic issues report difficulty with airway management and tracheal intubation (10,11). Dearlove and Harper noted that although mouth opening was adequate, the laryngeal structures could not be identified and three attempts were necessary with cricoid pressure to achieve successful intubation (10). The arytenoids were noted to be thickened and reddened.…”

“…Benni et al. also noted difficulties with airway management including abundant tracheobronchial secretions and hypertrophy of the adenoids and the base of the tongue (11). Initial laryngoscopy revealed only the epiglottis (Cormack grade III); however, with the application of cricoid pressure, the vocal cords were visualized and tracheal intubation was accomplished.…”

“…Included in the issues related to airway management is the consideration that many of these patients will have GOR and the potential for acid aspiration (10,11). In addition to GOR, delayed gastric emptying has also been demonstrated (7).…”

Anesthetic considerations in the child with Costello syndrome: risks of cardiac arrest upon induction of anesthesia

“…Secondly, the patient was considered to have a full stomach and rapid sequence induction with succinylcholine to facilitate a rapid tracheal intubation was relatively indicated. However, the cardiac safety of succinylcholine has not been established in patients with Costello syndrome, as non‐depolarizing muscle relaxant were used in the three other cases reported on Costello syndrome and its anesthetic implications (3–5). The decision to refrain from co‐administration of atropine in conjunction with succinylcholine could be implicated in the arrest if succinylcholine promoted unopposed cholinergic stimulation on the cardiac conduction system leading to increased vagal tone and asystole.…”