Nager syndrome (Nageracrofacialdysostosis) is an inherited disorder of facial, limb and skeletal morphogenesis; an autosomal dominant condition with marked variability.

Aneonate weighing 1.5 kg was born out of an emergency LSCS at 34 weeks gestation. The neonate had clinical features of craniofacial anomalies: malar hypoplasia, severe mandibular hypoplasia with retrognathia, downward slanted palpebral fissures, low set ears, high nasal bridge, hair line extending to cheeks, high narrow hard palate, soft palate agenesis and choanal atresia; limb anomalies: radial limb defects with bilateral flexed elbow joints, flexion deformity of multiple jointsand lefthipdislocation; Cardiac anomalies: ASD, VSD and COA. Neonate was apnoeic and cyanosed at birth; neonatal resuscitation was done as per NALS guidelines. Bag and mask ventilation was adequate with an oral airway. In an attempt to place a definitive airway, laryngoscopy was attempted; it was difficult due to facial anomalies. Laryngeal Mask Airway failed to produce a seal for adequate ventilation. Adult Fibreoptic bronchoscope was tried as paediatricfibreoptic was not available; it failed to advance beyond oral cavity, therefore, surgical tracheostomy was done to secure the airway.

Neonate can present with difficult airway due to congenital anomalies making neonatal resuscitation challenging. A sequential algorithmic approach with multidisciplinary involvement can help maintain oxygenation and avoid mortality and morbidity.

Nager syndrome, craniofacial, mandibular hypoplasia, tracheostomy