Total hip arthroplasty for a patient with angel-shaped phalango-epiphyseal dysplasia (ASPED). A case report.

نویسندگان

  • Hideki Warashina
  • Sinji Sakano
  • Sinji Kitamura
  • Ken-ichi Yamauchi
  • Hiroshi Kito
  • Yukiharu Hasegawa
چکیده

Angel-shaped phalango-epiphyseal dysplasia is characterized by the angel shape of the middle phalanx and severe coxarthrosis in adult life. This osteochondrodysplasia shows a further variety of heterogeneous multiple epiphyseal dysplasias. It also shows a late and dysplastic development of the femoral head that leads to osteoarthrotic changes with severe hip pain and gait disturbance. In this report, we show a 35-year-old female with Angel-shaped phalango-epiphyseal dysplasia that was treated by bilateral total hip arthroplasty. She has suffered from her coxalgia since she was 27 and since the age of 30 has been able to walk with the aid of crutches. The radiographs of her bilateral hip showed severe osteochondrotic changes with a progressive disappearance of the joint space. Total hip arthroplasties were performed to treat the osteoarthrosis on the bilateral hip joint due to Angel-shaped phalango-epiphyseal dysplasia. One year after surgery, she is pain-free, and able to walk with a cane. Based on this case, we propose that total hip arthroplasty should be considered one of the treatments for the coxopathy in patients with Angel-shaped phalangoepiphyseal dysplasia.

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Angel shaped phalangeal dysplasia, hip dysplasia, and positional teeth abnormalities are part of the brachydactyly C spectrum associated with CDMP-1 mutations.

I n 1967, Bachman described a ‘‘hereditary peripheral dysostosis’’ which affected a mother and her two children. What was later considered as multiple epiphyseal dysplasia combined with phalangeal cone shaped epiphyses was renamed angel shaped phalangoepiphyseal dysplasia (ASPED) and considered as a genetic bone marker. ASPED represents a further variety of multiple epiphyseal dysplasia and is ...

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عنوان ژورنال:
  • Nagoya journal of medical science

دوره 65 3-4  شماره 

صفحات  -

تاریخ انتشار 2002