Aplasia of the first and second branchial arches.
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We report a case of pycnodysostosis and review 54 cases of this syndrome in the Japanese literature, with special emphasis on oral and maxillofacial findings. Common findings were as follows: hypoplasia of maxilla and mandible, hypopneumatization of the maxillary sinuses, loss of mandibular angle, a grooved palate, and malpositioned teeth.
The so-called hairy or teratoid polyp is a rare lesion of bigerminal origin that comprises elements derived from both ectodermal and mesodermal cell lines. In this article we report the presence of meningothelial elements in a hairy polyp, a previously undescribed component of this entity. The lesion was characterized by a pedunculated outgrowth from the hard palate. The surface of the outgrowth was covered by squamous epithelium and a central core of fibroadipose tissue, pilosebaceous glands, cleftlike pseudovascular spaces, and groups of epithelioid cells. These reticulated and cellular foci had the immunohistochemical and ultrastructural features of meningothelial tissue.
1. By means of radiographies, of microscopic serial sections and of a cardboard geometric reconstruction (magnification 80), an investigation has been made of the more complete "anterior" face of cephalothoracopagus rabbit with anchyoty, synoty, otocephaly, prosophthalmy and cebocephaly. 2. All details are organized according to a well precise plan: a. all structures touching the principle plane of symmetry are fused; b. everything which would exist beyond that plane did not develop. 3. The heads of mallei are fused. The nearly normal auditory ossicles build a transversal chain. The bases of stapes are thickened. The centres of ossification of the heads are fused but normal. Each Corpus incudis shows a normal centre of ossification. 4. The outer epithelial rudiments of the tympanic membranes remain separated by a cleft. 5. The derivatives from the first branchial arch are defective, the anterior processes of malleus being fused. The lack of Meckel's cartilages induces cebocephaly, which provokes itself prosophthalmy and otocephaly. 6. There is a clear tendency-even in the deeply altered regions-towards a harmonious symmetry.
The correction of oral and maxillo-facial mal-formations causes lesions to sensitive nerves due to the invasive and traumatic surgery techniques required. Generally neuro-sensory and cutaneous-mucosal defects are reversible, with full recovery within 6-12 months. In a follow-up study (2 and 8 years) we found altered tactile and pain sensitivity in 43% of patients at subjective evaluation, with objective alteration in 17% of the group. Dental sensitivity evaluated by a pulp test showed an increase in for all teeth tested; values were higher for superior than for inferior teeth. Despite the increased pulp sensitivity threshold, due to the surgical damage, no signs of pulp disease were detected. Quality of life was not affected even by such a high subjective change in sensitivity. We therefore believe that patients undergoing orthognathic surgery must be fully informed; they must be made to understand the risks involved, and that damage may occur to sensitive nerves following surgery. All surgeons, including those with experience in the field, should continually seek to improve their skill and learn new techniques, in order to reduce complications to a minimum.
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The development of the tongue begins as known, in the floor of the primitive oral cavity, when the human embryo is four weeks old. More specifically, the tongue develops from the region of the first three or four branchial arches during the period that the external face develops. Malformations of the tongue, are structural defects, present at birth and happening during embryogenesis. The most common malformations are: 1. Aglossia 2. Microglossia, which is always combined with other defects and syndromes, like Moëbius syndrome 3. Macroglossia, which is commonly associated with cretinism, Down's syndrome, Hunter's syndrome, Sanfilippo syndrome and other types of mental retardation 4. Accessory tongue 5. Long tongue 6. Cleft or Bifid tongue, condition very usual in patients with the orodigitofacial syndrome 7. Glossitis Rhombica Mediana, a developmental malformation? 8. Lingual thyroid. Malformations are extensively analysed and discussed.
Hemifacial hyperplasia is a developmental disorder characterized by a marked unilateral asymmetry. The etiology is unknown, although several factors have been described. It involves not only the hard tissues of the face, but the soft tissues as well. A case report of an adult woman with hemifacial hyperplasia highlighting the clinical significance is presented.
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Since Castillo-Morales developed the Orofacial Regulation Therapy for children with Down syndrome in the mid 1970s, close observation of orofacial symptoms in the growing child has led to new findings. Primary orofacial signs are present at birth through age one; secondary alterations develop with untreated school-age children. A synopsis of the most important disorders in children with Down syndrome is given. Findings that relate to malfunction are summed up; these findings can be influenced by Orofacial Regulation Therapy.
Clinical management of articulation dysfunctions is one area in which the joint efforts of dentistry and speech pathology are particularly beneficial. Those articulation deviations discussed result from: (1) deformities in the upper lip, teeth, mandible and the hard and soft palates; (2) introduction of dentures; (3) difficulties in adjusting to esophageal speech; and (4) special problems associated with cleft palate/cleft prepalate. Team management of the individual with cleft palate and/or cleft prepalate was also reviewed. While orthodontists and prosthodontists provide the technology for correct speech production, speech pathologists furnish therapy for cultivating speech or modifying defective articulation patterns. Speech pathologists also serve as liaisons with respect to patients and other professionals. The authors believe that, ideally, clinicians from all disciplines should assume a holistic attitude in treating organic and functional human pathologies. Such interdisciplinary clinical cooperation is especially effective between dentistry and speech pathology. The concept of team management of cleft palate/cleft prepalate is an excellent example of how concomitant treatment effects total patient care. However, joint research and educational ventures also contribute to this objective and should continue to be encouraged.
Oromandibular and limb syndromes present particular types of orofacial lesions. Six personal cases and about one hundred cases reported in the literature are reviewed using Hall's classification. Etiopathogenic mechanisms are being increasingly related to fetal epigenetic and environmental factors.
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