A Case of Dentin Dysplasia with Full Mouth Rehabilitation: A 3-year Longitudinal Study

Abstract

Dentin dysplasia, a rare hereditary disorder of dentin formation, is characterized by normal enamel but atypical dentin formation along with abnormal pulpal morphology. It is inherited as an autosomal dominant trait. It has been divided into two clinical entities: type I (radicular) and type II (coronal). Early diagnosis and initiation of effective regular dental treatments may help the patients with this condition to delay or prevent the loss of the entire dentition and help them in cope up with edentulous state in early ages. The condition undoubtedly has a negative impact on the physical and psychological well-being of the affected individual. Numerous factors have to be considered during the prosthetic rehabilitation of patients with dentin dysplasia. Treatment protocol varies according to clinical case. Although literature reports suggest general guidelines for treatment planning, the present case report describes a full mouth rehabilitation of an 8-year-old female patient with dentin dysplasia.How to cite this article: Khandelwal S, Gupta D, Likhyani L. A Case of Dentin Dysplasia with Full Mouth Rehabilitation: A 3-year Longitudinal Study. Int J Clin Pediatr Dent 2014;7(2): 119-124.

Guided endodontic treatment of multiple teeth with dentin dysplasia: a case report

“…Most published cases had rather sobering outcomes after different therapy approaches. This includes a switch to apical surgery after an unsuccessful endodontic treatment attempt [ 39 ] or leaving teeth with periapical lesions untreated [ 18 , 35 ] or tooth extraction, particularly in teeth with short roots [ 10 , 19 , 20 , 34 , 36 , 46 ]. The prognosis of endodontically treated teeth with DD might be described as uncertain.…”

“…DD, a rare disturbance of dentin formation (incidence: 1:100,000), is an autosomal dominant hereditary disease caused by a coding malfunction of the dentin sialophosphoprotein gene. The disorder is characterized by apparently normal enamel but atypical dentin formation and abnormal pulp morphology [ 10 ]. Two types of DD are distinguished based on clinical, radiological and histological findings: type I (“radicular”) and type II (“coronal”), hereinafter referred to as DD-1 and DD-2 [ 11 ].…”

“…There is no available evidence on the occurrence of apical periodontitis in teeth with DD, but several case reports suggest that these teeth may be at risk of developing apical pathologies [ 10 , 14 – 18 ].…”

VPS4B mutation impairs the osteogenic differentiation of dental follicle cells derived from a patient with dentin dysplasia type I

“…It was first reported as “rootless teeth” in 1922 by Ballschmiede, who found a family with six children manifesting with short, blunted roots and premature tooth loss. 36 The estimated incidence of DD-I is 1/100,000, 3 , 7 , 37 and thus, knowledge of this disorder mainly originates from reports of isolated cases. Individuals with DD-I frequently present with tooth hypermobility combined with diminutive roots, aberrant dentin formation, obliterated pulp chambers, and numerous periapical radiolucencies in non-carious teeth.…”

“… 1 – 4 This is also often combined with frequent periapical radiolucencies in apparently sound teeth, both in the deciduous and permanent dentitions, with an estimated incidence of 1/100 000. 5 – 7 DD-I increases the risk of early tooth loss and causes functional and esthetic disturbances. The initial information on DD-I mainly originated from reports of isolated cases owing to a low incidence.…”