#36280 Neuraxial anaesthesia in a patient with coffin-siris syndrome – a case report

• DOI: 10.1136/rapm-2023-esra.317
• Publication Date: 2023-10-10T10:32:09Z
• Authors: Filipa Farias; Alexandrina Jardi...; João Pinho; Ivanete Peixer; Jorge Paulos; Teresa Cenicante
• Keywords: Coffin

<h3></h3> <b>Please confirm that an ethics committee approval has been applied for or granted:</b> Not relevant (see information at the bottom of this page) <h3>Background and Aims</h3> Coffin-Siris syndrome (CSS) is a rare genetic disorder, with less than 250 molecularly confirmed cases worldwide. It characterized by growth restriction, developmental delay, craniofacial malformations, range heart, gastrointestinal, genitourinary nervous system abnormalities. These abnormalities may present anaesthetic challenge mainly due to difficult airway management, respiratory complications poor patient cooperation. The available literature on CSS approach consists 10 case reports, only one describing regional anaesthesia technique. <h3>Methods</h3> A 14-year-old female was scheduled bilateral proximal tibial hemiepiphysiodesis. Preoperative evaluation significant obstructive sleep apnoea, IgA deficiency several infections over last year hypertrophic cardiomyopathy. history doubtful delayed emergence from general anaesthesia, despite recovery spontaneous ventilation, in past procedures. Physical examination revealed obesity, short neck macroglossia. deep sedation accomplished intravenously propofol fentanyl, maintained sevoflurane 1,5%, ensuring ventilation through laryngeal mask airway. An L3-L4 epidural block performed ropivacaine 0,5%. ASA standard monitoring bispectral index were applied, multimodal analgesia ensured. <h3>Results</h3> Hemiepiphysiodesis successfully under proposed technique, combining neuraxial sedation. perioperative period uneventful. <h3>Conclusions</h3> patients can be challenging anaesthesiologist syndrome's malformation spectrum, cardiac structural disease, lack reassuring literature. Neuraxial successful safe selected