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

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

OBJECTIVETo report the treatment of a case of severe Crouzon's syndrome using monobloc distraction osteogenesis and cranial vault remodeling.

METHODSThrough intra-and extra-cranial approach, monobloc osteotomy was performed and external distractor was placed. Distraction began on the 7th postoperative day at a rate of 1 mm a day, two times a day. The distractor removed after consolidation for 4 months.

RESULTSThe distraction distance attained 20 mm. The exophthalmos and cross bite were corrected completely. The severe obstructive apnea improved markedly.

CONCLUSIONSMonbloc distraction osteogenesis and cranial vault remodeling are effective and safe procedure for Crouzon's syndrome.

Child ; Craniofacial Dysostosis ; surgery ; Female ; Humans ; Osteogenesis, Distraction ; methods ; Osteotomy ; Skull ; surgery

OBJECTIVECorrection of craniofacial dysostosis with midface distraction osteotogenesis.

METHODSLe Fort III osteotomy has been employed through coronal route on patients with midface craniofacial dysostosis such as Crouzon and Apert syndrome. Then a REDII distraction device was set up, and the device bars directed. The distraction begins 6.4 days after the surgery, with a rate of 1 mm per day. When midface approaching the right position, i.e. an slightly over correction of occlusion is reached, the distraction stops and the device is held for the next 2-4 months.

RESULTSThere are 8 cases completed all the therapy with an average age of 11.9 years old. The midface had been moved averagely 9.7 mm forwards and 1.6 mm downwards. The features had been improved obviously and the occlusions reach nearly normal. The exophthalmos reduced from 20.3 mm to 11.9 mm. In cephalometry, SNA was averagely enlarged 9 degrees, and ANB enlarged 8.8 degrees. The snore during sleeping was also improved in 87.5% cases. No serious complication had occurred except minor one such as 1 case of seroma and 1 case of infection around pin on scalp. According to 5 months averagely follow-up, there is no recurrence in our list.

CONCLUSIONSMidface distraction osteotogenesis is propitious to teenage or severe cases of craniofacial dysostosis.

Adolescent ; Child ; Craniofacial Dysostosis ; surgery ; Face ; surgery ; Female ; Humans ; Male ; Osteogenesis, Distraction ; methods ; Traction ; methods

Body Height ; Child ; Craniofacial Dysostosis ; diagnosis ; Female ; Growth Disorders ; etiology ; Humans

Adult ; Child, Preschool ; Craniofacial Dysostosis ; surgery ; Craniotomy ; methods ; Female ; Humans ; Male

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