Successfully Tracheostomy and Monobloc Advancement with External Frontofacial Distraction Followed by Simultaneous Adenoidectomy, Conchotomy, and Septoplasty in Severe Late Crouzon Syndrome
Keywords:Adenoidectomy, conchotomy, septoplasty, crouzon syndrome.
A 10-year-old male patient presented with Severe Late Crouzon Syndrome, with turri-brachycephaly, and
severe exorbitism of both eyes, visual loss, beaked nose and maxilla hypoplasia. The head circumference was
54 cm and 90 precentile. Patient had a high arched palate, tonsil hypertrophy and type III malocclusion. From
the multidisciplinary meeting, in the first stage it was decided to first secure the airway with Tracheostomy
then immediately followed by Monobloc Advancement with External Frontofacial Distraction. Then in
the second stage, after removal of the rigid external distractor, by 9 months after first surgery, the patient
underwent a simultaneous procedures of Adenoidectomy, Conchotomy, and Septoplasty. Thus, after patient
got stable in follow-up, Nasendoscopy and Tracheostomy devices was removed. After 11 months follow-up.
Visual function still not getting better. However turri-brachycephaly, exorbitism of both eyes, beaked nose
and maxilla hypoplasia, head circumference, arched palate, tonsil hypertrophy and the malocclusion were
getting much reduced, and the patient getting happier and more active.
How to Cite
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.