Successfully Tracheostomy and Monobloc Advancement with External Frontofacial Distraction Followed by Simultaneous Adenoidectomy, Conchotomy, and Septoplasty in Severe Late Crouzon Syndrome

A 10-year-old male patient presented with Severe Late Crouzon Syndrome, turri-brachycephaly, andsevere exorbitism of both eyes, visual loss, beaked nose and maxilla hypoplasia. The head circumference was54 cm 90 precentile. Patient had a high arched palate, tonsil hypertrophy type III malocclusion. Fromthe multidisciplinary meeting, in the first stage it was decided to secure airway Tracheostomythen immediately followed by Monobloc Advancement External Frontofacial Distraction. Then inthe second stage, after removal rigid external distractor, 9 months surgery, patientunderwent simultaneous procedures Adenoidectomy, Conchotomy, Septoplasty. Thus, patientgot stable follow-up, Nasendoscopy Tracheostomy devices removed. After 11 follow-up.Visual function still not getting better. However noseand hypoplasia, circumference, malocclusion weregetting much reduced, happier more active.