High risk of peri‐implant disease in periodontal Ehlers–Danlos Syndrome. A case series

Abstract

ObjectivesPeriodontal Ehlers–Danlos syndrome (pEDS) has recently been delineated as a molecularly defined cause of early severe periodontitis. Here we report that implant treatment failed in three affected individuals from one family.Materials and MethodsLongitudinal data before and after implant treatment were examined for three individuals with genetically confirmed pEDS in the course of a large‐scale pedigree analysis.ResultsMost detailed information was available for individual 1 in whom first periodontal bone loss was diagnosed at age 16 years. Rapid progression resulted in multiple tooth extractions at age 23 years and interforaminal placement of four implants. After primary implant success, peri‐implant bone loss accompanied by highly inflamed tissues and receding gums led to explantation five years later. In individual 2, severe periodontitis was diagnosed at age 15 years and resulted in extraction of all mandibular teeth at age 28 years. Four interforaminal implants were placed. Peri‐implant bone loss was diagnosed four years later, when up to three implant threads were exposed. Individual 3 showed complete tooth loss at age 29 years. He was restored with ten implants and removable prosthesis. Peri‐implant bone loss was diagnosed radiologically eight years later, when seven implant threads were exposed.ConclusionThis is the first report on severe peri‐implant bone loss in pEDS. Retention of teeth as long as possible is the primary objective in pEDS as satisfying prosthetic solutions are missing. Further evaluation of dental management in individuals with pEDS is needed to develop concise treatment guidelines.

Atrophic violaceus pretibial plaques: when the clue is the oral mucosa

“…Kapferer-Seebacher et al 1 reported on 93 patients with pEDS; our family's clinical features were similar ( Table 1). Dental implants have been reported to be successful in patients with other forms of EDS, but pEDS has shown worse results, 2 as in the father of our family, whose implants lasted < 1 year.…”

Surgical-orthodontic treatment in patients with Ehlers–Danlos syndrome: a report of two familial cases

“…Rinner et al (2018) reported a series of three familial cases of periodontal-type EDS with implant failures secondary to severe peri-implantitis despite appropriate periodontal management. The bone loss was amplified by the procedure, leading to the conclusion that there is a strong mechanical influence on the implant prognosis in these three patients [22]. Therefore, the biological mechanisms of osseointegration seem to be preserved without major and specific complications in the context of the EDS; however, clinical studies involving larger cohorts of patients are needed.…”

“…The main challenge in oral cares of patients with EDS is the preservation of a functional dentition as long as possible and prevention of any infectious pathology, which can be addressed by regular specialized follow-ups and implementation of adapted. Early preventive and conservative care and motivation for oral hygiene to prevent complex or potentially iatrogenic fixed or adjunctive prosthetic rehabilitation are also essential and integrated in the treatment plan [13,16,22].…”

Classification, nosology and diagnostics of Ehlers-Danlos syndrome

“…38 Recently, leukencephalopathy and peri-implant disease were described as additional features of pEDS. 39,40 In 2016, bi-allelic AEBP1 mutations were described in an autosomal recessive EDS variant, featuring joint hypermobility with joint dislocations, hyperextensible, translucent and redundant skin, poor wound healing with abnormal scarring, easy bruising and gastrointestinal, urogenital, cardiovascular and skeletal abnormalities. Its features overlap with several EDS types, particularly cEDS, mcEDS and spEDS.…”

“…Often this is not possible and therefore there is still room for new types. This is, among other things, due to: -the clinical overlap between many of these EDS types Journal of Biomedicine and Translational Research, 5 (2) 2019, [34][35][36][37][38][39][40][41][42][43][44][45][46] -absence of a pathogenic variant in any of the known EDS associated genes in an important proportion of EDS patients. -the presence of associated features which do not fit into one of the existing types.…”