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

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