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1/3. Occlusal guidance of two Kabuki make-up syndrome patients: case reports.

    Kabuki make-up syndrome (KMS) has been reported since 1981 by Niikawa et al. Complications of KMS were moderate mental retardation, skeletal and dermatoglyphic abnormalities. A 7 year-old boy, who had severe permanent tooth deficiency, anterior open bite, tongue thrust and mild mental retardation, was referred to our clinic. Oral characters of another patient were an anterior open bite, transposition of maxillary central and lateral incisor. Orthodontic treatment in two patients is reported and suggested future treatment plans in these patients is given.
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ranking = 1
keywords = tooth
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2/3. A syndrome of hemimaxillary enlargement, asymmetry of the face, tooth abnormalities, and skin findings (HATS).

    Hemimaxillofacial dysplasia and segmental odontomaxillary dysplasia appear to be the same syndrome, having the common features of unilateral abnormalities of bone, teeth, gums, and skin. oral manifestations are the hallmark of this condition. Those affected are generally recognized in childhood and may have partial anodontia, abnormal spacing of the teeth, delayed eruption, and gingival thickening of the affected segment. Reported cutaneous manifestations include facial asymmetry, Becker's nevus, "hairy nevus," lip hypopigmentation, discontinuity of the vermilion border, depression of the cheek, and erythema. The oral lesions do not appear to be progressive. We describe a child with features consistent with hemimaxillofacial dysplasia/segmental odontomaxillary dysplasia. Findings of a biopsy specimen from the cheek confirmed the presence of a Becker's nevus. Cutaneous findings reported in the previous 31 cases are reviewed and summarized. The acronym HATS (hemimaxillary enlargement, asymmetry of the face, tooth abnormalities, and skin findings) is introduced to reflect the spectrum of abnormalities in bone, teeth, and skin that may be seen in this developmental disorder.
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ranking = 5.2465244409989
keywords = tooth, eruption
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3/3. Transmaxillary osteogenesis distraction combined with orthopedics and orthodontics in the correction of a severe retrusion of the upper maxilla.

    A patient having adequate records and diagnosed as having Binder syndrome is presented. Nasomaxillary hypoplasia requires a definitive treatment, use of bone grafts, upper maxillary osteotomies, and advancement or a combination of both. Bone grafts can be reabsorbed, and complete maxillary advance modifies normal occlusion in a certain way, because the posterior sector is not compromised. The aim of this case treatment is to create a new osseous surface that makes the eruption of the permanent dental pieces easier and gives the correct skeletal position to the upper maxilla with distraction osteogenesis combined with an orthopedic appliance, transmaxillary segment osteotomy, and subsequent orthodontics. The absence of the osseous surface in the upper maxilla and the presence of supernumeraries in the anterior region determined the permanence of most of the temporary dental pieces, resulting in the impossibility of making the exchange to permanent dental pieces. The technique and the devices are simple and easy to manipulate. After the treatment, an adequate dental relation is restored as well as effective advance of the upper maxillary bone; thus, an excellent functional and stable esthetic result is achieved, avoiding any complication of velopharingeal incompetence.
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ranking = 0.24652444099893
keywords = eruption
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