A Interdisciplinary Concept of Oral Rehabilitation in a Nonsyndromic Autosomal Dominant Oligodontia with a Novel Mutation of PAX9. A Clinical Report
Agenesis of one or more teeth is one of the most common of human developmental anomalies . Oligodontia is a very rare condition, has a population prevalence of 0.03% to 0.07% and occurs most frequently in females at a ratio of 3:2. The most frequently missing teeth are the maxillary lateral incisors, followed by the mandibular second premolars and the mandibular central incisors . The etiology of tooth agenesis may vary from physical obstruction or disruption of the dental lamina, space limitation and functional abnormalities of the dental epithelium or failure of initiation of the underlying mesenchyme .
It may also occur as part of a systemic genetic syndrome or can also be due to an isolated condition (nonsyndromic oligodontia) like mutation in LTBP3 , or mutation in the homeobox gene MSX1 or paired domain transcription factor PAX9. The absence of permanent teeth may cause several clinical problems and the inconvenience to patients will vary, depending on the age, specific teeth and the number of teeth that are missing. This clinical report describes a rare case of non-syndromic oligodontia in a eight years old girl in mixed dentition stage who had problems with aesthetics, mastication, and phonation.
Since, the therapeutic concept of oral rehabilitation by multi-disciplinary approaches are decisive for a successful treatment outcome an early diagnosis, and comprehensive treatment planning with good coordination and timing of the individual treatment phases are presented. Case Report A 8 year-old female patient (proband) reported to the department of pediatric dentistry with a chief complaint of multiple decayed teeth in lower left quadrant.
The patients past medical history and the family history were not contributory. It was patient’s first visit to a dentist. Extra-oral examination revealed no abnormalities of the skin, hair or nails. Intraoral examination, revealed grossly decayed teeth in relation to 54, 65, 84 and hypoplastic teeth in relation to 64, 74 and 55. A few retained carious deciduous teeth were present in relation to 53, 55, 65, 81, and 85 with huge midline diastema. The proband (III:9) Orthopantomographic (OPG) examination revealed agenesis of eighteen permanent teeth including third molars. The missing teeth were 12,13,15,17,18,22, 23,25,27,28,33,35,36,38,43,45,46,48.
It may also occur as part of a systemic genetic syndrome or can also be due to an isolated condition (nonsyndromic oligodontia) like mutation in LTBP3 , or mutation in the homeobox gene MSX1 or paired domain transcription factor PAX9. The absence of permanent teeth may cause several clinical problems and the inconvenience to patients will vary, depending on the age, specific teeth and the number of teeth that are missing. This clinical report describes a rare case of non-syndromic oligodontia in a eight years old girl in mixed dentition stage who had problems with aesthetics, mastication, and phonation.
Since, the therapeutic concept of oral rehabilitation by multi-disciplinary approaches are decisive for a successful treatment outcome an early diagnosis, and comprehensive treatment planning with good coordination and timing of the individual treatment phases are presented. Case Report A 8 year-old female patient (proband) reported to the department of pediatric dentistry with a chief complaint of multiple decayed teeth in lower left quadrant.
The patients past medical history and the family history were not contributory. It was patient’s first visit to a dentist. Extra-oral examination revealed no abnormalities of the skin, hair or nails. Intraoral examination, revealed grossly decayed teeth in relation to 54, 65, 84 and hypoplastic teeth in relation to 64, 74 and 55. A few retained carious deciduous teeth were present in relation to 53, 55, 65, 81, and 85 with huge midline diastema. The proband (III:9) Orthopantomographic (OPG) examination revealed agenesis of eighteen permanent teeth including third molars. The missing teeth were 12,13,15,17,18,22, 23,25,27,28,33,35,36,38,43,45,46,48.
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