No abstract available.
Adult* ; Craniofacial Dysostosis* ; Humans

Craniofacial Dysostosis ; Humans ; Receptors, Fibroblast Growth Factor

Craniofacial dysostosis, a well defined. rare syndrome first described by Crouzon in 1912, characteristically shows frontal bosses, prognathism, exophthalmos, exotropia, optic nerveatrophy and maxillary hypoplasia. Three cases of familial occurrence are presented, and according to their history, 11 of 13 members in 5 generations of their family are suspected to have been afflicted. A brief review of related literature is described.
Craniofacial Dysostosis* ; Exophthalmos ; Exotropia ; Family Characteristics ; Humans ; Prognathism

The authors present a case of Crouzon's disease with advanced craniostenosis and exophthalmos. Extensive subperiosteal resection of the vault and base of the skull resulted obvious decompression of the brain and the orbit. It should be emphasized that the removal of osseous partitions penetrating deep into the sulci is the chief point of surgical procedures to prevent incarceration of cerebral gyrus at the inner surface of the skull.
Brain ; Craniofacial Dysostosis* ; Craniosynostoses ; Decompression ; Exophthalmos ; Orbit ; Skull

A monstrous faced, 60 years old famale patient was first seen in our clinic on May 1, 1976, complaining of diffuse abdominal pain. Throughout physical examination and x-ray study revealed a craniofacial dysostosis, which is rare in this age group, and other lab. data seemed normal.
Abdominal Pain ; Craniofacial Dysostosis* ; Humans ; Middle Aged ; Physical Examination

Crouzon's disease, first described by Crouzon in 1912, is characterized by craniosynostosis, exorbitism and midface hypoplasia. As well, it has been known to be transmitted as an autosomal dominant trait. Clinically, Crouzon's disease not only has aesthetic problems, but also many functional disabilities, such as increased intracranial pressure, hydrocephalus, visual disturbance. difficulty in nasal breathing and malocclusion. The surgical correction of Crouzon's disease includes: 1) frontal bone advancement and release of the craniosynostosis; 2) correction of the midface retrusion; and 3) other ancillary procedures for better aesthetic results. The authors performed Le Fort III advancement for the correction of midface retrusion in 5 cases of Crouzon's disease. These advancements were performed at about the age of 15 in consideration of facial bone growth disturbance, psychosocial effects, and the recurrence of class III malocclusion after operation. The average advancement of the midface was 9. 2 mm. We experienced favorable aesthetic and functional results without severe complication in all cases or relapse of the advanced segment.
Craniofacial Dysostosis* ; Craniosynostoses ; Facial Bones ; Frontal Bone ; Hydrocephalus ; Intracranial Pressure ; Malocclusion ; Recurrence ; Respiration

Distraction osteogenesis has become an alternative technique to treat craniomaxillofacial anomalies. It was initially used to treat mandibular dysplasia and now it is applied to other regions of the craniofacial skeleton. We now present our clinical experience of midface distraction with the use of rigid external distraction for the treatment of an 8-years-old girl with midface hypoplasia in Crouzon's disease, who had undergone fronto-orbital advancement at the age of 6. We performed midface advancement by Le Fort III osteotomy with rigid external distraction system(RED II, KLS Martin, Jacksonville, FL). The active distraction was initiated on the 3rd postoperative day and was continued until the 20th postoperative day for 18 days. The rate of distraction can be adjusted during this time according to clinical judgment and cephalometric assessment. On completion of distraction, the RED II was left in place without activation for 25 days for rigid retention. The RED II was then removed and an orthodontic facemask was applied with elastic traction for 6wks. The total amount of distraction was 18.5mm, 28.5mm, 10.5mm, 14.5mm at right inferior orbital rim, left inferior orbital rim, right intraoral, left intraoral area respectively. The photography, cephalometry, and 3D CT(3 dimensional computed tomography) show that facial convexity was improved. We could correct midface deficiency successfully by LeFort III osteotomy and rigid external distraction.
Cephalometry ; Craniofacial Dysostosis* ; Female ; Humans ; Judgment ; Orbit ; Osteogenesis, Distraction ; Osteotomy ; Photography ; Skeleton ; Traction

A case of Crouzon's disease, in spite of the relatively small number of cases reported until recently. The patient was 3years old male and hospitalized Woo-Sok University hospital 5 May, 1970. the appearance of the patient was striking type of facial deformity. There is a marked frontal boss forming a prominent bregmatic tumour, hooked nose, a small receding maxilla and a pronounced prognathism in wthich the teeth of the lower jaw protrude several centimetres beyond those of the upper. Proptosis and subnormal mentality was present, Cerebral decompression had been performed to the patient for his exophthalmos, it was aggreviated after the operation and thus enucleation was done finally.
Congenital Abnormalities ; Craniofacial Dysostosis* ; Decompression ; Exophthalmos ; Humans ; Jaw ; Male ; Maxilla ; Nose ; Prognathism ; Strikes, Employee ; Tooth

Normally the cranial bones are closed finally in early adulthood; thus, premature closure of one or more sutures of the skull results in a head deformity producing damaging effects to the brain and eyes. The authors presented a case of Crouzon's disease which was characterized by shallow orbit, exophthalmos, maxillary hypoplasia and a prominent jaw. And the literatures relating to this disease were briefly reviewed.
Brain ; Congenital Abnormalities ; Craniofacial Dysostosis* ; Exophthalmos ; Head ; Jaw ; Orbit ; Skull ; Sutures

Crouzon's disease is a peculiar type of craniosynostosis which is premature closure of the suture, and deformities of craniofacial bone. Such a many deformities are caused by continuing brain growth unrelated to damaged bone growth. The authors presented a case of Crouzon's disease in a 4-year old male patient who has characteristic clinical findings with particular sensory neural type hearing loss and generalized seizure.
Bone Development ; Brain ; Child, Preschool ; Congenital Abnormalities ; Craniofacial Dysostosis* ; Craniosynostoses ; Hearing Loss ; Humans ; Male ; Seizures ; Sutures