Is It Safe To Use Frontofacial Monobloc Advancement and Cutting Guides on Adult Patients with Crouzon Syndrome? Introducing 2 Cases on 41- and 56-Year-Old Patients.

World Neurosurg

Department of Maxillofacial Surgery, Clocheville Hospital, Centre Hospitalier Universitaire de Tours, Tours, France; Craniofacial Surgery, Clocheville Hospital, Centre Hospitalier Universitaire de Tours, Tours, France; Université of Medecine François Rabelais, Tours, France.

Published: September 2019

Background: Patients with Crouzon syndrome are mainly treated in childhood by frontofacial monobloc advancement to avoid ophthalmic, neurologic, and maxillary complications. There is no reported case of surgery on adult patients with Crouzon syndrome in the literature. However, when faced with 2 cases of adult patients showing severe quality of life deterioration, our team decided to make an attempt using monobloc advancement technique.

Case Description: Two women aged 41 and 56 presented with untreated Crouzon syndrome and suffered from exorbitism, intracranial hypertension with chronic headaches, and hypoplastic maxillary. We decided to perform frontofacial monobloc advancement with internal distraction despite their advanced age using planned surgery and cutting guides. Distraction began 7-10 days after surgery and was of 15 mm. Distractors were taken off at 6 months. Surgical treatment corrected chronic headaches, ocular symptoms due to exorbitism, and hypoplastic maxillary. Patients were satisfied with the functional and aesthetic results. We noticed that this heavy surgery was more difficult to bear by these adults than children.

Conclusions: Adults with craniofacial malformations have a lower self-esteem, lower quality of life, and less satisfaction with their facial look as compared with individuals without facial malformations. There is also an increased risk of psychosocial problems. Despite postoperative difficulties and minor complications, our 2 patients were satisfied with the functional and aesthetic results. This led to the conclusion that surgically addressing adult patients with Crouzon syndrome via monobloc advancement is appropriate and secure when performed by a trained team.

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http://dx.doi.org/10.1016/j.wneu.2019.05.207DOI Listing

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