• Journal of the korean academy of Pediatric Dentistry
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• v.34 no.4
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• pp.672-678
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• 2007

Anatomic abnormalities of teeth development include the presence of a single maxillary central incisor at midline instead of two central incisors. The incidence of cases with a single maxillary central incisor is approximately 1 in every 50,000 live births. single median maxillary central incisor(SMMCI) may be a integral manifestation of any one of a number of syndromes. It has been reported an association with several midline disorders which have varied in severity. A SMMCI has also been reported as an isolated finding. But in some cases, it has been considered as an incomplete expression of autosomal dominant holoprosencephaly. So any patient who appears for treatment with a SMMCI should be referred for a detailed medical examination to exclude other associated systemic abnormalities and for mutation analysis to facilitate correct diagnosis and the provision of appropriate genetic counseling. Early orthodontic treatment is needed to minimalize emotional trauma of child. This case report was about three patients with a SMMCI as isolated finding. The purpose of these case reports was to present cases of single maxillary central incisor not associated with other disorders.

• Archives of Plastic Surgery
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• v.34 no.4
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• pp.528-530
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• 2007

Purpose: Holoprosencephaly(HPE) is a rare developmental defect due to incomplete cleavages of the prosencephalon during the third week of fetal development. Chromosomal anomalies, genetic syndrome, teratogen, or genetic disorder of non-syndromic HPE are usually accepted as etiology. The consequences of prechordal mesoderm defect are varying degrees of deficit of midline facial development, especially the median nasal process(premaxilla), and incomplete morphogenesis of the forebrain. We experienced a case of lobar HPE with complete cleft lip and palate. Methods: A female newborn infant was born at $38^{+6}$ weeks' gestational age via NSVD. The infant's birth weight was 3.6 kg, height 52 cm, and head circumference 32.5 cm, showing microcephaly, flat nose, median complete cleft lip & palate, and hypotelorism, along with defects of midfacial development including losses of premaxilla, philtrum, nasal septum, and columella. Results: There were no specific findings noted from the head and neck X-ray and tests for endocrine and metabolic disorders, but clinical characteristics of midface and dysgenesis corpus callosum on brain MRI were seen, so that this case was diagnosed with HPE. Conclusion: HPE is divided into three categories of alobar, semilobar, and lobar prosencephaly according to the degree of cerebral hemisphere separation. Assesment of patient's brain abnormality and malformation is essential in determining the extent and benefit of surgical intervention. This case was included in the lobar type HPE which shows relatively good prognosis compared with other types and reconstruction of median complete cleft lip & palate and midfacial defects will be performed.