Early operation in craniofacial dysostosis

Craniofacial dysostosis is encountered in different congenital malformations such as Kleeblattschadel deformity, Crouzon's disease, and Apert's, Chotzen's, Pfeiffer's, and Carpenter's syndromes. Premature closure of cranial and facial sutures leads to characteristic disfigurement of the skull with orbital and maxillary hypoplasia. Operative treatment should be performed as early in life as possible to prevent further functional and esthetic deficiencies, and psychosocial problems. Correction is done by an intracranial approach with mobilization, remodeling, and advancement of the deformed skull. Thirty-two children have been operated during the first year of life with a maximum follow-up of 8 years. Most favorable results were obtained in 28 cases. The rate of complications were lower than in a series of children operated on later in life. We advocate that complex 1-stage corrections of craniofacial syndromes may be safely carried out during infancy utilizing modern techniques, expert pediatric anesthesia, and postoperative intensive care.