Hyperphalangia
Classification according to ICD-10 | |
---|---|
Q74.0 | Hyperphalangia of the fingers |
Q74.8 | Other specified congenital malformations of the integument |
ICD-10 online (WHO version 2019) |
The hyperphalangia (from ancient Greek ὑπέρ hyper 'over, supernumerary' and ancient Greek φάλαγξ phálanx 'trunk, finger / phalanx') is a normal anatomical feature in various aquatic vertebrates (quadrupeds), especially some dinosaurs , but also turtles .
In humans , hyperphalangia is a very rare congenital malformation with excess phalanges , the number in a finger is greater than 3.
There is usually an additional bony limb on both sides and symmetrically in the fingers and / or toes . The thumb is not affected and there are typically no other skeletal changes. In addition to the two-sided forms, there are also one-sided forms.
Must be distinguished is the Pseudohyperphalangismus in which no additional bone-conditioning is available, but an ossification center separates into two parts. Occurrence in dihydantoin embryopathy .
Synonyms are: hyperphalangism; Polyphalangism; Hyperphalangia of the digital 2-5
distribution
The cause and frequency in humans are not known, so far only a few 100 affected people have been reported.
In the context of syndromes
Hyperphalangia can occur with some syndromes :
- Catel-Manzke syndrome , synonym: Pierre Robin syndrome - hyperphalangia - clinodactyly
- Chitayat Syndrome (CHYTS)
- Devriendt Syndrome
- Hyperphalangia of the finger toe abnormality-severe pectus excavatum syndrome
- Temtamy Preaxial Brachydactyly Syndrome (TPBS)
literature
- Melvin I. Shoul, Max Ritvo: Roentgenologic and Clinical Aspects of Hyperphalangism (Polyphalangism) and Brachydactylism. In: New England Journal of Medicine. 248, 1953, p. 274, doi: 10.1056 / NEJM195302122480702 .
Individual evidence
- ↑ Paleowire
- ↑ Larousse
- ↑ a b hyperphalangia. In: Orphanet (Rare Disease Database).
- ↑ Hyperphalangia, bilateral. In: Orphanet (Rare Disease Database).
- ↑ Hyperphalangia, unilateral. In: Orphanet (Rare Disease Database).
- ↑ Dihydantoin embryopathy. In: Orphanet (Rare Disease Database).
- ↑ Beverly P. Wood, Lionel W. Young: Pseudohyperphalangism in Fetal Dilantin Syndrome. In: Radiology. 131, 1979, p. 371, doi: 10.1148 / 131.2.371 .
- ↑ I. Gunal, T. Durak, V. Oztuna, S. Seber: Various manifestations of hyperphalangism. In: Journal of hand surgery. Volume 21, Number 3, June 1996, pp. 405-407, PMID 8771491 .
- ↑ CHITAYAT SYNDROME. In: Online Mendelian Inheritance in Man . (English)
- ^ Devriendt syndrome. In: Online Mendelian Inheritance in Man . (English)
- ↑ K. Devriendt, K. Keymolen, L. Roelen, G. Van Goethem, J. Meireleire, JP Fryns: Severe short stature, hyperphalangy of the index fingers, mental retardation and facial dysmorphism. In: Clinical dysmorphology. Volume 9, Number 2, April 2000, pp. 111-114, PMID 10826622 .
- ↑ Hyperphalangia of the finger-toe anomaly-severe pectus excavatum syndrome. In: Orphanet (Rare Disease Database).
- ^ Temtamy preaxial brachydactyly syndrome. In: Orphanet (Rare Disease Database).