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Blepharo-canthal deformities in patients following craniofacial surgery.

Functional problems and deformities of the eyes have become a major concern in the surgical treatment of ortital hypertelorism and craniofacial dysostosis, as experience with skeletal relocation for these disorders has been gained. Comprehensive preoperative and postoperative study and measurements of the bony orbits, the globes, the lids, the canthi, and the nose are necessary for the evaluation of present techniques and the design of alternative procedures. Some of the deformities of the blepharo-canthal complex are part of the congenital malformation. Other distortions, seen only postoperatively, are secondary to specific surgical maneuvers. Awareness of these primary and secondary factors has led to better soft tissue reconstruction at the time of the bony orbital translocations or the craniofacial disjunction. We describe the techniques which we have found especially useful in avoiding and correcting these postoperative blepharocanthal deformities.

Acrocephalosyndactylia↗

Dermatoglyphics in Saethre-Chotzen syndrome: a family study.

The dermatoglyphic findings in a Cuban family with the Saethre-Chotzen syndrome are reported. The family consisted of the parents who were first cousins and their three children. A new classification of zygodactylous patterns was used. Characteristic dermatoglyphic patterns which appeared in these cases were representative of the syndrome. Dermatoglyphics also helped to discover minor expressions of syndactyly and showed that all the members of the family had zygodactylous patterns on palms and soles.

Acrocephalosyndactylia↗

Rehabilitation aspects of Apert's syndrome.

Two children with the unusual condition known as Apert's syndrome are presented. Both have the typical manifestations of the syndrome: craniosynostosis, exorbitism, hypertelorism, maxillary hypoplasia, dental malocclusion, cleft palate, compound syndactyly of the hands and simple syndactyly of the feet. A review of the latest surgical procedures for correction of these defects is presented and the importance of early physical and social rehabilitation is stressed.

Acrocephalosyndactylia↗

[Acrocephaly, cranial asymmetry, brachydactyly, syndactyly, progressive senile dementia--a new type of acrocephalosyndactyly?].

Clinicogenetic assessment of a family whose six members proved to be carriers of an identical pathological trait allowed the delineation of the clinical picture of the hereditary disease described. Its major components include congenital cranial and facial abnormalities, malformed extremities characteristic of acrocephalosyndactyly, and a progressive pattern of psychic disturbances and higher cortical dysfunctions arising in old age. The first to come are increasing acoustic agnosia and disconnected delusions (stage of psychotic disorders) which are followed by progressive overall mental retardation with concomitant deactualization and disappearance of delusional feelings (stage of dementia). It has been suggested that cases of this kind make up a new (viz., the sixth) type of acrocephalosyndactyly with an autosomal dominant pattern of hereditary transmission. The mutant gene appears to be pleiotropic and characterized by complete penetrance and slightly varying expressivity.

Acrocephalosyndactylia↗

[Miniplate osteosynthesis in craniofacial surgery].

The easily malleable Champy miniplates are suitable for fixation not only in the viscero- but also in the neurocranium in craniofacial surgery of complex craniofacial deformities. The miniplate osteosynthesis stabilizes the skeletal segments in their new position and function so securely that intermaxillary fixation is no longer necessary. This modality of internal fixation is applicable even in small children and infants allowing total osteotomy and mobilisation of the cranium and the facial skeleton at this early age. The miniplates may also prevent restenosis after radical stripping of prematurely closed sutures. The plates and screws are usually removed 6 to 12 months later through small stab incisions. The craniofacial surgery groups in Munich and Innsbruck have successfully applied this form of fixation in 10 selected children with severe malformations, their age ranging from 3 months to 14 years. The technique and results are demonstrated in appropriate examples. The pros and cons of the miniplate osteosynthesis versus other methods of fixation are discussed. During a period of nearly 3 years (1980-1982) no complications have been observed.

Acrocephalosyndactylia↗