Article Text
Abstract
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Background and Aims Treacher-Collins syndrome (TCS) is an autosomal dominant disorder with craniofacial deformities arising from developmental anomalies of branchial arches. We report a patient with TCS presenting for caesarean section (CS) and how regional anaesthesia circumvented the management of an almost-impossible airway.
Results An obese 31-year-old primigravida (BMI 42) required Ex-utero Intrapartum Treatment (EXIT) procedure for management of foetal mandibular hypoplasia. To facilitate EXIT, she was planned for elective CS under general anaesthesia (GA). The patient presented with preterm labour, prompting earlier action. Airway examination revealed micrognathia, microstomia, jaw malocclusion, and thick short neck. She was previously tracheostomised twice. There was documented difficult airway during recent surgery. Fibreoptic techniques had failed due to copious secretions. There was an unfavourable laryngeal view (grade 4 Cormack-Lehane) despite using a hyperangulated blade video laryngoscope. After discussion with relevant stakeholders, she underwent semi-urgent CS without EXIT the following day after consideration for the maternal airway. A combined spinal-epidural was performed with ultrasound guidance. Hyperbaric bupivacaine 11.5mg and fentanyl 15mcg was delivered intrathecally. Epidural space was located at 7.5cm from skin and a catheter was inserted. CS was uneventful.
Conclusions We described a parturient with exquisite airway challenges (obesity, TCS with craniofacial deformities, previous tracheostomies, and known difficult airway) mitigated with regional anaesthesia. With improvements in healthcare, patients with congenital diseases are expected to survive to adulthood. Being cognizant of the anaesthetic challenges associated with their condition and planning for it is essential for clinical management. Early involvement of a multidisciplinary team facilitates discussion and planning of perioperative management.