Is it safe to use fronto-facial monobloc advancement and cutting guides on adult patients with a Crouzon syndrome? Introducing two cases on 41- and 56-years-old patients.

Abstract

INTRODUCTION\nPatients with Crouzon syndrome are mainly treated in childhood by fronto-facial monobloc advancement to avoid ophthalmic, neurologic and maxillary complications. There is no reported case of a surgery on a Crouzon syndrome on adult patients in the literature. However, when faced to two cases of adult patients showing severe quality of life deterioration, our team decided to make an attempt using monobloc advancement technique.\n\n\nCASES REPORT\nTwo women aged 41 and 56 presented an untreated Crouzon syndrome and suffered of exorbitism, intracranial hypertension with chronic headaches and hypoplastic maxillary. We decided to perform fronto-facial monobloc advancement with internal distraction despite their advanced age using planned surgery and cutting guides. Distraction began seven to ten days after surgery and was of 15 mm. Distractors were taken off at 6 months. Surgical treatment corrected chronic headaches, ocular symptoms due to exorbistism and hypoplastic maxillary. Patients were very satisfied with the functional and aesthetic results. We noticed that this heavy surgery was more difficult to bear by these cases than by pediatric patients.\n\n\nCONCLUSION\nAdults with craniofacial malformations have a lower self-esteem, lower quality-of-life, and less satisfaction with their facial look as compared to individuals without facial malformations. There is also an increased risk of psychosocial problems. Despite post-operative difficulties and minor complications, our two patients were very satisfied with the functional and aesthetic results. This leads to the conclusion that surgically addressing a Crouzon syndrome on adult patients via monobloc advancement is appropriate and secure when performed by a trained team.