Anaesthetic Challenges in a Paediatric Patient with Escobar Syndrome-Difficult Airway and Postoperative Pneumothorax.

Abstract

Escobar syndrome (ES) is an autosomal recessive disorder characterised by the presence of pterygia in cervical, antecubital and popliteal regions. Anaesthesiologist encounter notable challenges in this syndrome, especially airway management due to associated malformations like cleft lip/palate, micrognathia, syngnathia, ankyloglossia, neck contracture, cervical spine fusion, limited neck extension and craniofacial dysmorphism. In addition to difficult airway, anaesthesiologist may encounter other perioperative challenges. Here, we report a paediatric patient with ES, who required general anaesthesia for laparoscopic inguinal hernia repair and orchidopexy. Initial attempt with video laryngoscope failed due to inability to visualise epiglottis. Subsequent attempt with fibreoptic bronchoscope also failed due to rapid decrease in oxygen saturation. He was finally intubated with fibreoptic bronchoscope along with oxygen insufflation with a 3mm internal diameter polyvinylchloride endotracheal tube inserted nasally and connected to oxygen supply. Further perioperative challenges faced were intraoperative hyperthermia and postoperative pneumothorax with mediastinal shift. To the best of our knowledge, this is the first case reporting pneumothorax with mediastinal shift as a postoperative complication and use of oxygen insufflation through nasal tube during fibreoptic intubation in paediatric patient with ES.