Amelogenesis imperfecta (AI) is a collection of diverse inherited disorders featuring dental-enamel defects in the absence of significant nondental symptoms. AI phenotypes vary and are categorized as hypoplastic, hypocalcified, and hypomaturation types. Phenotypic specificity to enamel has focused research on genes encoding enamel-matrix proteins. We studied two families with autosomal-dominant...

Amelogenesis imperfecta (AI) is a group of hereditary disorders primarily characterized by developmental abnormalities in the quantity and/or quality of enamel. There are some reports suggesting an association between AI and generalized gingival enlargement. This paper describes the clinical findings and oral management of two siblings presenting both AI and hereditary gingival fibromatosis (HG...

This manuscript describes an interdisciplinary approach over a period of 8 years combining surgical and prosthodontic treatment of a young patient diagnosed with hypocalcified-type amelogenesis imperfecta and anterior open bite. The treatment procedures included transitional restorations, orthodontic treatment, and maxillofacial surgery with a one-piece Le Fort I osteotomy, bilateral mandibular...

Amelogenesis Imperfecta (AI) is a genetic disease affecting primary and permanent tooth enamel. The incidence varies between 1:700 to 1:4000. The clinical findings include enamel defects, tooth sensitivity, poor dental aesthetics, reduced vertical dimension, dentin dysplasia, and pulpal calcification. Effective treatment planning should incorporate numerous factors such as the patient’s age, di...

This paper describes treatment planning and dental rehabilitation of permanent maxillary incisors of an 11-year old patient affected by hypoplastic Amelogenesis imperfecta using CEREC chair side technology. The treatment was conducted in a multidisciplinary approach and followed by orthodontic treatment using self-ligating brackets to achieve optimum results. The teeth were treated endodontical...

BACKGROUND Kohlschütter-Tönz syndrome is a rare neurodegenerative disorder presenting with intractable seizures, developmental regression, and characteristic hypoplastic dental enamel indicative of amelogenesis imperfecta. Recently, mutations in ROGDI were identified in part of Kohlschütter-Tönz syndrome cases, but the siblings reported here do not have a mutation in the ROGDI gene, showing tha...