Depicting External Crown Resorption in Syndromic Amelogenesis Imperfecta Associated With Junctional Epidermolysis Bullosa

To characterize External Crown Resorption (ECR) in patients with Syndromic Amelogenesis Imperfecta (SAI) associated with junctional epidermolysis bullosa (JEB).

A multicentre retrospective study was conducted at two centers (Germany and Chile). Analyzed variables included dentition, tooth, surface, age, severity, progression, treatment strategy, and clinical outcome.

One hundred and seventy teeth affected with ECR (60 primary, 110 permanent) from 30 patients were included. Patients presented an average of 5.7 ± 4.0 affected teeth. Within this cohort, in primary dentition, incisors represented 88% of affected teeth, 8% canines, and 3% molars. In permanent dentition, 62% were incisors, 19% molars, 10% canines, and 9% premolars. Eight teeth showed two independent initiation sites, giving 178 affected surfaces in 170 teeth; 85.4% of these surfaces were incisal/occlusal, 12.9% proximal, and 1.7% vestibular/palatal. Age of detection varied from 8 months to 8.7 years (primary dentition) and from 5.2 to 22 years in permanent dentition. Severity analysis revealed that posterior teeth presented a higher proportion of severely compromised molars (61.9%), while anterior teeth presented a higher proportion of more moderate (57.4% permanent) and mild (37.7% primary) compromise. Observed progression intervals, based on 39 teeth, ranged from 4 months to 9.3 years. Imaging studies, treatment strategies, and their outcomes are presented. Restoration was associated with a lower frequency of further structural loss than preventive strategies alone. A provisional radiographic screening protocol is proposed.

ECR can affect primary and permanent dentition, leading to tooth loss if left unrestored. The radiographic screening protocol for early diagnosis and treatment of ECR proposed in this article can aid in preserving teeth.