Clinical Experience for the Surgery in the Craniofacial Dysostosis: Reports of Nine Operative Cases.

Kang, Joon Ki; Song, Jin Un

Original Article

Journal of Korean Neurosurgical Society 1987;16(1): 103-116.

Department of Neurosurgery, Catholic University Medical College, Seoul, Korea.

ABSTRACT

The craniofacial dysostoses are due to synostosis of the basal skull sutures in the anterior cranial fossa. Patients with these abnormalities potentially have normal brains that are distorted by the misshapen skull ; shallow anterior fossa and reduction of the orbital volume, exophthalmos, and hypoplasia of the maxilla. The authors describe the operative repair and results of 9 cases of craniosynostosis. The craniofacial dysmorphic states are presumably caused by premature closure of basal skull sutures in combination with the coronary suture. In an effort to arrest and correct both the cranial and facial deformities as well as obviate the need for further radical surgery, the authors have treated 9 children with Crouzon's disease with linear suture craniectomy along the base of the skull, forehead advancement and unilateral canthal advancement techniques. A linear craniectomy along the synostotic sutures does not affect the synostotic process at the base of the skull. The advance of supraorbital margin and creating an artificial suture at the base of skull allowed for porper correction those craniofacial deformities. Following those procedure, deformity has regressed and ultimate cosmatic improvement has been obtained in 4 cases of grade III and 3 cases of the grade II and 2 cases of grade I among the 9 patients. Early neurosurgical intervention using the procedure of the forehead advancement and lateral supraorbital ridge advancement combined with linear craniectomy along the skull base suture, yields excellent results in the majority of these children.

Key Words: Craniosynostosis; Craniofacial dysostoses; Forehead advancement; Lateral canthal advancement