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Family of Crouzon Syndrome Represents the Evolution of the Frontofacial Monobloc Advancement Technique: From Immediate Movement to Monobloc Distraction to Monobloc Bipartition Distraction

Raposo-Amaral, Cassio Eduardo MD, PhD*; Denadai, Rafael MD*; Ghizoni, Enrico MD, PhD*,†; Buzzo, Celso Luiz MD, MSc*; Raposo-Amaral, Cesar Augusto MD*

doi: 10.1097/SCS.0000000000001949
Clinical Studies
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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.

Address correspondence and reprint requests to Cassio Eduardo Raposo-Amaral, MD, PhD, Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital, Av. Adolpho Lutz, 100, Caixa Postal: 6028, Campinas, São Paulo, CEP: 13084–880, Brazil; E-mail: cassioraposo@hotmail.com

Received 14 February, 2015

Accepted 1 May, 2015

The authors report no conflicts of interest.

© 2015 by Mutaz B. Habal, MD.