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

Craniofacial surgery for facial advancement or correction of severe craniofacial malformations such as orbital hypertelorism, Crouzon's disease and Apert's syndrome may bring about great risk. Especially postoperative infection in the craniofacial surgery is a life threatening complication. Ascending infection via nasofrontal communication in frontofacial monobloc advancement, intracranial Le Fort III osteotomy, correction of hypertelorism(intracranial approach) and acute trauma of cribriform plate can lead to life threatening meningitis and meningoencephalitis. A 4 layer sealing technique for the closure of nasofrontal communication using Gelfoam , the inferiorly based, galeo-pericranial flap, rib bone graft, Tissel is a very effective method. Until the rib bone graft is completely uptaken, Gelfoam is used as a temporary blockage of bony defect and prevents displacement of rib bone graft. We used galeo-periosteal flap for the sufficient blood supply to the rib bone graft. And Tissel is used as a biologic adhesive and blockage of the surrounding gaps.
Adhesives ; Craniofacial Dysostosis ; Ethmoid Bone ; Fibrin Tissue Adhesive ; Gelatin Sponge, Absorbable ; Hypertelorism ; Meningitis ; Meningoencephalitis ; Nasal Cavity* ; Orbit ; Osteotomy ; Ribs ; Transplants

Crouzon syndrome, an autosomal dominant disorder, has characteristic features of craniosynostosis, hypertelorism, exophthalmos, maxillary hypoplasia and relative mandibular prognathism. Mutations of fibroblast growth factor receptor 2 (FGFR2) gene are associated with craniosynostotic conditions, such as Crouzon syndrome, Jackson-Weiss syndrome, Pfeiffer syndrome, Apert syndrome and Beare-Stevenson cutis gyrata. We found one child with common morphological features of Crouzon syndrome. Interestingly, she was found to have Cys342Ser mutation in FGFR2 exon lllc which has been previously observed in Jackson-Weiss syndrome. This finding supports the variable expression of FGFR2 in human and allelic heterogeneity in these apparently clinically distinct craniosynostotic conditions.
Acrocephalosyndactylia ; Child ; Craniofacial Dysostosis* ; Craniosynostoses ; Exons* ; Exophthalmos ; Humans ; Hypertelorism ; Population Characteristics ; Prognathism ; Receptor, Fibroblast Growth Factor, Type 2

Craniofacial dysostosis is considered to be one of rarely observed syndromes characterized by premature closing of all cranial sutures. The first patient was a 4-year-old male infant who had been complaining of empyema. Clinical findings showed exophthalmos, hypertelorism and facial asymmetry. Conventional radiographs demonstrated abscence of cranial sutures and underdeveloped maxilla. CT scan demonstrated the digital impressions of the inner surface of the cranial vault, enlarged and depressed sella turcica. The second patient was a 2-year-old female infant who had been complaining of facial deformity. Clinical findings showed hypertelorism and underdeveloped maxilla. Radiographs showed premature synostosis of all cranial sutures, depressed and enlarged sella turcica, and hypoplastic maxilla. 3 years after operation, her look improved. However, resurgery may be considered to decreasing intracranial pressure and for correction of facial deformity. Two interesting cases showing 'cloverleaf'skulls were presented.
Child, Preschool ; Congenital Abnormalities ; Cranial Sutures ; Craniofacial Dysostosis* ; Empyema ; Exophthalmos ; Facial Asymmetry ; Female ; Humans ; Hypertelorism ; Infant ; Intracranial Pressure ; Male ; Maxilla ; Sella Turcica ; Synostosis ; Tomography, X-Ray Computed

Craniofacial Dysostosis ; Humans ; Receptors, Fibroblast Growth Factor

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

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

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

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

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