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

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