Family of Crouzon Syndrome Represents the Evolution of the Frontofacial Monobloc Advancement Technique: From Immediate Movement to Monobloc Distraction to Monobloc Bipartition Distraction.
Crouzon syndrome (CS) is an autosomal dominant disorder characterized by premature fusion of cranial sutures, midface and supraorbital ridge retrusion, exorbitism, and in some clinical scenarios strabismus, parrot-beaked nose, short upper lip and hypertelorbitism. Treatment of CS is overlapped with the beginning of craniofacial surgery and is grounded on morphologic and functional objectives. The authors reported on the outcomes and complications of family members (mother and 2 siblings) with CS, who were operated on by different techniques of frontofacial advancement and have attained skeletal maturity. Operations were performed in different moments throughout the last 3 decades of craniofacial surgery history. A 10-year-old Crouzon progenitor underwent a monobloc osteotomy with acute advancement, using rigid fixation and bone grafting in the osteotomy sites. An 8-year-old Crouzon daughter underwent gradual lengthening of a monobloc segment, using an external, institutionally made distracter device. In addition, a 10-year-old Crouzon son underwent gradual lengthening of a monobloc segment associated to facial bipartition, using an internal distracter device. After 30 years, the mother presented a mild relapse on the orbit level, but her children had satisfactory stable outcomes. The family members with CS have undergone different modifications of the monobloc approach based on different chronological momentum, from acute monobloc advancement, to monobloc distraction, to monobloc facial bipartition distraction.
*Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital †Division of Pediatric Neurosurgery, Department of Neurology, School of Medical Sciences, State University of Campinas (UNICAMP), Campinas, São Paulo, Brazil.
Cassio Eduardo
CE
Raposo-Amaral
Rafael
R
Denadai
Enrico
E
Ghizoni
Celso Luiz
CL
Buzzo
Cesar Augusto
CA
Raposo-Amaral
D064592:Autografts / Q000637:transplantation; D016025:Bone Transplantation / Q000379:methods; D002648:Child; D003394:Craniofacial Dysostosis / Q000235:genetics* / Q000601:surgery; D003399:Craniotomy / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D005624:Frontal Bone / Q000601:surgery; D006801:Humans; D016268:Internal Fixators; D008297:Male; D008437:Maxilla / Q000601:surgery; D009915:Orbit / Q000601:surgery; D019857:Osteogenesis, Distraction / Q000295:instrumentation / Q000379:methods*; D019340:Osteotomy, Le Fort / Q000379:methods; D019651:Plastic Surgery Procedures / Q000379:methods*; D012008:Recurrence; D013703:Temporal Muscle / Q000637:transplantation; D016896:Treatment Outcome