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

In 1912, Crouzon reported one of craniofacial dysostosis, which was inherited as an autosomal dominant trait and characterized by craniosynostosis, midface hypoplasia, and exorbitism. In 1950, the first midface advancement was performed by means of a total facial osteotomy by Sir Harold Gillies. In 1967, Tessier accomplished complete craniofacial dysjunction by performing Le Fort III-Tessier I osteotomy on the basis of Le Fort III fracture and laid the foundation for modern craniofacial surgery. Variable combinations of the surgeries and osteotomies are performed considering functional disabilities as well as patient's age, general condition, and aesthetic problems. The nineteen-year-old female patient complained of frog-like face and showed severe exorbitism and midface hypoplasia, while the occlusion, being relatively normal. We applied modified Le Fort III osteotomy and fronto- orbital advancement. Over the follow-up period of six months, her previous normal occlusion was preserved and the exorbitism and midface deformity largely improved aesthetically.
Congenital Abnormalities ; Craniofacial Dysostosis* ; Craniosynostoses ; Female ; Follow-Up Studies ; Humans ; Orbit ; Osteotomy*

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

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

OBJECTIVETo discuss the segmental monobloc osteotomy and bi-directional distraction for the treatment of Crouzon syndrome in an infant.

METHODSA 9-month-old female infant underwent monobloc osteotomy through combined intra- and extra-cranial way. The facial skull was divided into frontal, orbital and maxillary segments. The external distractor was used to move the frontal segment, orbital segment and midface forward. The internal distractor was used to move the maxilla downward. The consolidation lasted for 3 months after distraction.

RESULTSThe osteotomy and distraction were successfully completed. The distraction distance reached 18 mm, showing by distractor. The real distraction distance of facial bone was 12 mm, documented by 3-D image. The skull deformity and severe depression of midface improved a lot. The exophthalmos and underbite were corrected. The obstructive sleep apnea also improved markedly. However, the downward movement of maxilla was limited.

CONCLUSIONSMonobloc osteotomy with external distractor, as well as maxillary distraction, could be used for Crouzon syndrome in infant. It is safe and effective method which can be performed in an early age for Crouzon syndrome with obstructive sleep apnea.

The Freeman-Sheldon syndrome (FSS) is a rare congenital syndrome, characterized with myopathy and dysplasia. The musculoskeletal and soft-tissue manifestations often require orthopedic and plastic surgery. We reported a case of 8-year-old girl with FSS operated on for scoliosis.
Anesthetics ; therapeutic use ; Child ; Craniofacial Dysostosis ; diagnosis ; surgery ; Female ; Humans ; Scoliosis ; surgery