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Often referred to as peripheral dysostosis. Marked hyperextensibility of the fingers and precocious osteoarthritis of the hips. Characteristically, the middle phalanges represent an “angel-shaped phalanx,” which results from modification of the epiphysis, diaphysis, and metaphysis, resembling the little angels used to decorate a Christmas tree.

Extremely rare; exact incidence unknown. Autosomal dominant.

Clinical features and distinctive findings on radiologic examination of the phalanges. Angel-shaped phalanx results from a disturbance in the development of the epiphysis, diaphysis, and metaphysis resembling the little angels used to decorate a Christmas tree. The wings are formed by a diaphyseal cuff, the skirt by a cone-shaped epiphysis, and the head by the distal pseudoepiphysis. Hyperextensibility of the fingers and precocious arthritis of the hips (coxarthrosis). Hypodontia (congenital absence of up to five teeth) is often present.

Positioning may be difficult, depending on the procedure and degree of coxarthrosis. Careful direct laryngoscopy is required in the presence of dental anomalies.

Arkless-Graham Syndrome: Short hands and feet, short stature, and brachycephaly. Distinct facies characterized by prominent mandible (protruding jaw); small, broad, upturned nose with flat nasal bridge; and small mouth. Other features include skin, genital, dental, and musculoskeletal abnormalities. Mental retardation is present in 90% of children and can be severe.

Pseudohypoparathyroidism: Very rare disorder characterized by renal and/or bony anomalies caused by insensitivity to parathyroid hormone.

Warashina H, Sakano S, Kitamura S, et al: Total hip arthroplasty for a patient with angel-shaped phalango-epiphyseal dysplasia (ASPED). A case report. Nagoya J Med Sci 65:103, 2002.  [PubMed: 12580536]
Graham JM Jr, Krakow D, Tolo VT, et al: Radiographic findings and Gs-alpha bioactivity studies and mutation screening in acrodysostosis indicate a different etiology from pseudohypoparathyroidism. Pediatr Radiol 31:2, 2001.  [PubMed: 11200992]
Giedion A, Prader A, Fliegel C, et al: Angel-shaped phalango-epiphyseal dysplasia (ASPED): Identification of a new genetic bone marker. Am J Med Genet 47:765, 1993.  [PubMed: 8267010]

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