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Characteristics of Hajdu-Cheney syndrome and specifics of the full denture: case report

Publication at First Faculty of Medicine, Second Faculty of Medicine |
2010

Abstract

The case report presents a male 48 years old who suffers from Hajdu-Cheney syndrome. AD transmission into the 4th generation was proved by genealogical examination.

Seven members in the pedigree were affected, suspect primogenitor originate from Armenia. In proband typical clinical and radiographic findings were discovered, e.g. clubbed fingers, phalangeal acroosteolysis, brachycephalic skull with basilar impression and multiple Wormian bones in the coronal and lambdoid sutures.

X-ray survey discovered compression fracture of the lumbar vertebra L2, hypoplastic sinuses and edentulous upper and lower jaws. Severe osteoporosis of lumbar spine and osteopenia of femoral necks was proved by densitometry.

From this reason the treatment with antiresorptive drugs (bisphosphonates) was introduced and high bone turnover was normalized. Early loss of teeth and alveolar prořebone atrophy significantly affected the quality of his life.

The objective of the report is to show the process of providing a patient with a complete denture in a less typical manner, with emphasis on retention and stability in the case of extreme atrophy and osteoporosis of jawbones, which are associated with this syndrome. A reconstruction of lower jaw by dental bone implants is discussed from aspects of both possible osteolysis due to Hajdu-Cheney syndrome and osteonecrosis of jaws because of long term bisphosphonates therapy.