Dr. V. Sankara SubramanianConsultant Anaesthesiologist

Dr. Jeyasekharan HospitalNagercoil.

Case

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

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

Pre op Assessment

No verbalisation Large Head + Syndactyly + Macroglossia + Micrognathia + Maxillary hyper plasia + CVS-RS – Clinically normal P/A – umbilical hernia +

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

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