1. Dr. V. Sankara Subramanian Consultant Anaesthesiologist Dr. Jeyasekharan Hospital Nagercoil. AnaestheticManagement of Interesting Case

2. Case • Baby X a case of APERT SYNDROME with Incomplete cleft palate was posted for palatorplasty.

3. Brief Summary • Baby was full term normal delivery baby • No H/O of birth asphyxia • H/o delayed milestones (+) • H/o epilepsy (+) and was on medications • Last episode of fits 4 months back • Congenital hydrocephalus (+), VP shunt procedure done at the age of 5 years • Age 7 years, Weight – 12.5 Kgs

5. Pre op Assessment • No verbalisation • Large Head + • Syndactyly + • Macroglossia + • Micrognathia + • Maxillary hyper plasia + • CVS-RS – Clinically normal • P/A – umbilical hernia +

6. Anaesthetic Management • IV line secured with difficulty • Pre oxygenation done • Inhalation induction : 6% Sevoflurane+ N2O + O2 • Trial laryngoscopy done, epiglottis visualised • Induced with PROPOFOL 45 mg, FENTANYL 25 mic • Orally intubated with 5 size uncuffed RAE tube • Maintenance N2O + O2 + Atracurium + Sevo • Intraop – uneventful baby • Girl extubated on table, smooth postop

12. Discussion • Anticipating airway difficulty in paediatric population • Syndrome associated with cleft lip / palate • Apert Syndrome • 1906 French Neurologist first described • Mutation in a gene located on Ch:10Q • Incidence – 1:65000 • Features • Hyper hidrosis • Craniosynostosis • Chiarri malformation, Frontal Bossing, Development delay • Low set ears • Mid face – development delay • Associated ASD, VSD • Syndactly

14. Thank You