Abstract

Background:Hajdu-Cheney Syndrome (HCS) is a rare hereditary bone metabolism disorder characterized by acro-osteolysis, short stature, craniofacial changes, periodontitis and premature tooth loss. Extensive search of the current literature revealed no reports of implant placement in patients with HCS.Case Report:A 22-year old woman with osteoporosis, generalized advanced chronic periodontitis and premature tooth loss was referred to the Postgraduate Clinic of Periodontology, University of Athens-Greece. The patient was diagnosed in 2001 with HCS. The patient received non-surgical periodontal treatment and several teeth were extracted due to extensive alveolar bone loss.After careful consideration of the possible implications deriving from the patient’s condition and having taken her young age into account, initially, a dental implant was placed in the upper right first premolar region. Specific protocols such as longer healing periods were implemented, so five years after placement and successful osseointegration of this implant, four additional dental implants were placed in the posterior regions of the maxilla and the mandible. Prosthetic rehabilitation followed 6 months after implant placement. Upon completion of periodontal treatment, the patient was enrolled in a periodontal maintenance program.Results:Clinical and radiographic examination of the patient during the periodontal maintenance program after implant placement revealed no abnormalities in the implant region.Conclusion:Patients with HCS suffer from periodontitis, bone destruction and premature tooth loss. This case indicates the successful osseointegration of dental implants in patients with HCS. However, further research is required in order to determine the predictability of dental implant placement in those patients.

Highlights

  • BackgroundHajdu-Cheney Syndrome (HCS) is a rare hereditary bone metabolism disorder characterized by acro-osteolysis, short stature, craniofacial changes, periodontitis and premature tooth loss

  • Hajdu-Cheney syndrome (HCS) or acro-osteolysis is an extremely rare autosomal dominant congenital bone metabolism disorder characterized by excessive abnormalities of the cranial, spinal and facial bone structures, severe1874-2106/16 2016 Bentham Open576 The Open Dentistry Journal, 2016, Volume 10 progressive resorption of the distal phalanges and generalized osteoporosis [1, 2]

  • HCS is an osteolytic syndrome presenting multiple clinical features such as, osteoporosis, short stature, craniofacial and skull changes, periodontitis and premature tooth loss and it is often referred in the current literature as arthro-dentoosteodysplasia [5], cranio-skeletal dysplasia with acro-osteolysis [6], hereditary osteodysplasia with acro-osteolysis [7] and acro-osteolysis with osteoporosis in skull and mandible [8]

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Summary

Background

Hajdu-Cheney Syndrome (HCS) is a rare hereditary bone metabolism disorder characterized by acro-osteolysis, short stature, craniofacial changes, periodontitis and premature tooth loss. The patient received non-surgical periodontal treatment and several teeth were extracted due to extensive alveolar bone loss. After careful consideration of the possible implications deriving from the patient’s condition and having taken her young age into account, initially, a dental implant was placed in the upper right first premolar region. Specific protocols such as longer healing periods were implemented, so five years after placement and successful osseointegration of this implant, four additional dental implants were placed in the posterior regions of the maxilla and the mandible. Upon completion of periodontal treatment, the patient was enrolled in a periodontal maintenance program

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