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Results of the Tessier integral procedure for correction of Treacher Collins syndrome.

A new procedure is proposed for correction of the malformations of severe cases of Treacher Collins syndrome in the areas of the maxilla, mandible, and orbit. The operation essentially consists of a rotation of the midfacial segment around a transverse axis at the frontonasal angle. The midface is rotated forward anteriorly and downward posteriorly. It is stabilized by cranial grafts impinged into the temporal bone and the maxilla. The mandible is lengthened either by V shape osteotomy or bone grafting of the ramus. The mandible is fixed in an overjet class III dental occlusion. A posterior bite wafer is maintained for 2 months. The construction of the orbital cavity is completed with cranial bone grafts. The Tessier integral procedure for Treacher Collins syndrome has been done in one stage in six patients and in two stages in five patients. Sufficient data were available for analysis of only four patients.

Adolescent↗

[Facio-auriculo-vertebral syndrome: description of a case].

The authors report the case of a fourteen months old girl who presented with emisomal, uropoietic and vertebral anomalies associated with neurologic handicap. These features can be considered characteristic of facio-auriculo-vertebral spectrum. This syndrome was not frequently described in Italian pediatric literature.

Female↗

[Hydrocephalus and the Goldenhar syndrome].

Two female babies with Goldenhar syndrome have, the first an aqueductal stenosis, the second repetitive spells of pain and strain of the anterior fontanel. Aqueductal stenosis, hydrocephalus or intracranial hypertension are not previously described in this syndrome.

Child↗

[Goldenhar syndrome and other ear abnormalities in HR-PCT].

The use of CT in hospitals in which also ENT departments are located will be useful in cases of osseous congenital disorders of the ear. In combination with the clinical and audiometric investigations CT of the temporal bone will be helpful for the indication of the operations to ameliorate the hearing.

Adult↗

Applications of computer graphics in craniofacial surgery.

Three-dimensional surface images can be reconstructed from CT scans. These are particularly useful in the study and management of patients with congenital and acquired craniofacial deformities. The principles of 3-D reformation, the production of the surface images, and recent advances in modification of these images for specific clinical needs are presented.

Adult↗

Periorbital myectomy for essential blepharospasm.

Essential blepharospasm is considered to be an incomplete form of Meige dystonic syndrome. Different medical and surgical treatments have been tried with a low success rate on a long-term basis. We are presenting a patient in whom the recently described technique of periorbital myectomy was applied.

Blepharospasm↗

[Lipoma of the corpus callosum as a constituent of Goldenhar syndrome].

A newborn infant with clinical signs of the Goldenhar-syndrome was found to have a lipoma of the corpus callosum documented both by cranial sonography and computerized tomography. The association of lipoma of the corpus callosum and Goldenhar-syndrome is discussed and in view of the recent literature, it is concluded that lipoma of the corpus callosum like other recently observed intracranial lesions is indeed an additional manifestation within the spectrum of the Goldenhar-syndrome.

Brain Neoplasms↗