Complex Airway Management in Pierre Robin Sequence with Failed Intubation: Case Report and Lesson Learned

Abstract

Background : Pierre Robin Sequence (PRS) is a rare condition with a global prevalence of 1 in 8,500-14,000 live births, characterized by micrognatia, glossoptosis, and upper airway obstruction. Reporting this case is essential to enriching the literature on PRS management, especially in Indonesia, where case data is still limited.

Case Illustration : A 1 year ten months old child with PRS, palatoschizis, laryngomalacia, secondary hypothyroidism, and mandibular hypoplasia. The patient will undergo mandibular osteodistraction to address airway obstruction and improve facial structure. Since birth, the patient has shown feeding difficulties and airway obstruction. At five months of age, an echocardiography examination revealed a patent ductus arteriosus (PDA). The patient was fasted for 6 hours before surgery, intravenous access was established, and premedication with midazolam 0.05mg/kgBB. Preoxygenation, induction with ketamine 1mg/kgBB, intubation with spiral ETT No. 3.5 and atracurium 0,5mg/kgBB were given, and maintenance anaesthesia using sevoflurane. Close monitoring was done to ensure the patient's stability. Postoperatively, the patient was transferred to the intensive care unit with intravenous paracetamol analgesic and close monitoring to prevent complications.

Conclusion This case highlights the importance of meticulous airway planning in children with Pierre Robin Sequence (PRS) and a history of failed intubation. Successful anaesthetic management was achieved through preoperative airway assessment, adequate preoxygenation, successful tracheal intubation before administration of neuromuscular blockade, and maintenance with sevoflurane. Given the potential for difficult ventilation and intubation in PRS, appropriate preparation for advanced airway techniques and multidisciplinary collaboration are essential.