TL;DR: This report documents a unique case of rough hypoplastic amelogenesis imperfecta with apparent anterior oligodontia and multiple anomalies of the associated mesenchymally derived tissues.
TL;DR: This case presenting with a rare syndrome characterized by enamel dysplasia and multiple unerupted teeth with large solid fibrous pericoronal lesions manifesting with odontogenic fibroma-like features shows an anterior open bite malocclusion and gingival overgrowths.
Abstract: This article reports on a case presenting with a rare syndrome characterized by enamel dysplasia and multiple unerupted teeth with large solid fibrous pericoronal lesions manifesting with odontogenic fibroma-like features. Our case shows in addition to these findings an anterior open bite malocclusion and gingival overgrowths. These overgrowths exhibit the microscopic features of the multiple pericoronal odontogenic fibroma-like lesions that appear to be the hallmark of this syndrome. This unusual case brings the total number documented in the literature to 5, all of which were reported from South Africa.
TL;DR: It was concluded that prosthetic dental replacement in this patient would be difficult because of the distorted jaw relationship and large alveolar ridges, and craniofacial reconstruction might be compromised by obstruction of the nasal airways, difficulty in intubation and postoperative respiratory problems.
Abstract: Structured Abstract
Authors – Roberts TS, Stephen L, Beighton P
Aims and Objectives – Documentation of dental and orthodontic implications of osteoglophonic dysplasia (OGD).
Settings and Participants – Case report describing oral and dental manifestations of a female with OGD, aged 39 years, who was first documented three decades ago.
Results – This rare genetic disorder manifests with gross stunting of stature, associated with severe craniofacial malformation and multiple unerupted teeth. Radiographically, multiple lucent lesions were present in the tubular bones and mandible as well as several impacted teeth.
Conclusion – We concluded that prosthetic dental replacement in this patient would be difficult because of the distorted jaw relationship and large alveolar ridges. Equally, craniofacial reconstruction might be compromised by obstruction of the nasal airways, difficulty in intubation and postoperative respiratory problems.
TL;DR: Two case reports of hypoplastic amelogenesis imperfecta with oligodontia, multiple unerupted teeth, pulpal calcifica tion, taurodontism and anterior deep bite who were provided with functional and esthetic rehabilitation are presented.
Abstract: Amelogenesis Imperfecta (AI) represents a group of developmental conditions, genomic in origin, which affect the structure and clinical appearance of enamel of all or nearly all the teeth in a more or less equal manner. It is usually inherited either as an X-linked, autosomal dominant or autosomal recessive trait. The enamel may be hypoplastic, hypomineralised or both and affected teeth may be discolored, sensitive or prone to disintegration. Diagnosis is based on the family history, pedigree plotting and meticulous clinical observation. The treatment of patients with AI should start with early diagnosis and intervention to prevent latter restorative problems. Herein, we present two case reports of hypoplastic amelogenesis imperfecta with oligodontia, multiple unerupted teeth, pulpal calcifica tion, taurodontism and anterior deep bite who were provided with functional and esthetic rehabilitation.