Maxillonasal dysostosis, described by Binder in 1962, is probably more common than one would think from the small number of cases that have been published (fewer than 40). We have seen 37 cases of this syndrome over a period of 8 years. Diagnosis of the syndrome is easy because of its characteristic anomalies of the upper lip and nose: smallness of the nose, a groove under the columella, a "half-moon" appearance of the nasal apertures, absence of the nasal spine, and projection of the chin. In addition, we have observed several associated abnormalities: convergent strabismus (2 cases), mongolism (1 case), labiomaxillopalatine cleft (1 case), and, of particular note, numerous abnormalities of the cervical spine, frequently associated with mandibular prognathism. In view of the frequency of the latter abnormalities. Binder syndrome might well be called a "nasomaxillovertebral syndrome."

Abnormalities, Multiple↗

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Craniospinal and cervicospinal malformations associated with maxillonasal dysostosis (Binder syndrome).

Craniospinal and cervicospinal malformations were found in 15 (53.5%) of 28 cases of maxillonasal dysostosis (Binder syndrome) in which a complete radiographic analysis could be done. These anomalies have not been reported previously. Analysis of the anomalies suggested a common induction factor, or, at least, a simultaneity in the development of the maxillonasal and vertebral dysplasias that characterize Binder syndrome. The following procedures are recommended: (1) examination of the cranial and cervical spine in all subjects with symptoms of Binder syndrome, and (2) regular examination of patients with spinal malformations, so that any neurologic damage may be detected early.

Abnormalities, Multiple↗

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Therapeutic aspects of maxillonasal dysostosis (Binder syndrome).

Treatment of maxillonasal dysostosis (Binder syndrome) varies according to the age of the patient. In the newborn, respiratory and alimentary problems resulting from the anomaly may necessitate the insertion of endonasal and gastric tubes. In children, occlusal problems and orofacial dysfunctions caused by maxillonasal dysostosis should be treated as soon as possible with an orthodontic mask. Once a patient reaches adulthood, surgery may be performed. If orthodontic treatment has been successful and there is normal occlusion, the surgery may consist simply of placing the muscles of the nose and upper lip in normal; anatomic position. If occlusion is abnormal, osteotomy may be performed.

Adolescent↗

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Familial osteodysplasia.

A family with characteristics that have striking similarities to the syndrome of familial osteodysplasia is reported. The inheritance pattern appears to be autosomal dominant. The typical facies has a lack of vertical facial development and a functional prognathism caused by overclosure of the mandible and opening of the gonial angle. Midfacial hypoplasia and a pointed chin are characteristic. Alveolar and sutural bone growth in the face is diminished and associated with partial dental agenesis. Variable cranial digital markings and possible synostosis are also seen. These typical facial changes are associated with other bony and systemic abnormalities. The long bones have a flask shape and occasionally have increased cortical thickness. The superior pubic ramus is often thin and scoliosis is common. The dermatoglyphic pattern demostrates a distal triradius and small whorl abnormalities. The most significant laboratory values are an increased erythrocyte sedimentation rate and C3 complement found on the erythrocytes. The plasma fibrinogen level was low in all family members.

Adult↗

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[The frontomaxillary suture. Theoretical bases and general principles of the application of postero-anterior extraoral forces to the orthopedic mask].

Each maxilla is joined to the frontal bone by a veritable fronto-maxillary sutural joint which, in the young subject, permits sliding movements. During growth, the maxilla orients itself in relation to the mechanical effects of the various structures which surround it. The location, below the frontal sinus, of this joint endows it with special properties of resistance and change in position of orientation. These anatomico-functional peculiarities explain certain results obtained by classical heavy antero-posterior of postero-anterior extra-oral forces.

Adolescent↗

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[Primary cheilorhinoplasty for congenital unilateral labiomaxillary fissure. Trial schematization of a technic].

The addition of rhinoplasty to primary cheiloplasty for cleft lip improves not only the post-operative state but also, by re-establishing the equilibrium of all the cartilaginous and musculo-periosteal structures, favourises maxillary growth. The author describes a technique and presents some results.

Bone Transplantation↗

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[Treatment of mandibular hypercondyles (argument for defense of the condylectomy)].

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Adult↗