Mühlbauer W, Anderl H, Heeckt P, Schmidt A, Zenker J, Höpner F, Schaarschmidt B
World J Surg. 1989 Jul-Aug;13(4):366-72. doi: 10.1007/BF01660749.
Craniofacial dysostosis is encountered in different congenital malformations such as Kleeblattschädel 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.
颅面骨发育不全可见于不同的先天性畸形，如 Kleeblattschädel 畸形、克鲁宗病以及阿佩尔综合征、乔森综合征、法伊弗综合征和卡彭特综合征。颅面缝过早闭合会导致颅骨出现特征性畸形，并伴有眼眶和上颌骨发育不全。手术治疗应尽早进行，以防止进一步出现功能和美观缺陷以及心理社会问题。通过颅内入路，对变形的颅骨进行松动、重塑和前移来进行矫正。32 名儿童在出生后第一年内接受了手术，最长随访时间为 8 年。28 例取得了最理想的效果。并发症发生率低于一系列在生命后期接受手术的儿童。我们主张利用现代技术、专业的儿科麻醉和术后重症监护，在婴儿期安全地进行复杂的一期颅面综合征矫正手术。
