Syndactyly
| Classification according to ICD-10 | |
|---|---|
| Q70 | Syndactyly |
| ICD-10 online (WHO version 2019) | |
In human medicine , syndactyly (from ancient Greek σύν syn 'together' and δάκτυλος dáktylos 'finger') refers to a congenital anatomical malformation of the limbs ( dysmelia ), which is characterized by an agglomeration or non-separation of fingers or toes . A distinction is made between cutaneous (membranous), osseous or total syndactylia.
If there is a bony fusion of 2 phalanges of a finger or a toe, with the missing finger or toe joint , one speaks of a symphalangia or symphalangism.
distribution
The peculiarity is innate and arises in the period of about the 5th to 7th week of development of the human embryo . Syndactyly occurs in 1 in 2,000–3,000 births.
root cause
The adhesions or non-separation can occur accidentally (see amniotic ligament syndrome ) or through alcohol consumption during pregnancy, but can also be a symptom of genetically determined peculiarities in 20–40% of cases .
Syndactyly 3/4 (i.e. of the middle and ring fingers) is a common feature in children with triploidy . Syndactyly also occurs more than average in Aarskog syndrome , Adams-Oliver syndrome , Carpenter syndrome , Eaton-McKusick syndrome , Fraser syndrome , Fuhrmann syndrome , Pierre Robin sequence and other syndromes . In Smith-Lemli-Opitz syndrome a syndactyly of 2/3 of the toes is often found.
classification
According to the Orphanet database , the following classification exists:
- SD1 , syndactyly type 1, formerly zygodactyly
- SD2 , synonym: synpolydactyly
- SD3
- SD4 , synonym: Haas type polysyndactyly
- SD5 , synonym: postaxial syndactyly with metacarpal synostosis
- SD6 , synonyms: syndactyly, type middle; Unilateral Syndactyly of Digital 2-5
- SD8
Clinical manifestations
A functional impairment occurs only to a limited extent, so that there is usually no need for an operative correction. At least on the foot, there is no major cosmetic impairment, while the risk of complications is significantly higher than on the hand.
diagnosis
In some cases, syndactylia can be detected in a child prenatally as part of prenatal ultrasound examinations (often with fine ultrasound ).
Differential diagnosis
A distinction must be made between pseudosyndactyly , which is formed by mutilation in the context of epidermolysis bullosa dystrophica type Hallopeau-Siemens . Syndactyly or pseudosyndactyly only develops in infancy or toddlerhood. Operational separation is possible.
therapy
In the case of a surgical correction, this is done in most cases before the age of three in order to avoid incorrect growth and joint deformation, especially if fingers are not of the same length.
The respective characteristics as well as the options for surgical treatment of syndactyly vary, among other things. a. depending on whether the peculiarity is caused by a fusion of bones or bone cuts (osseous syndactyly) or by a skin connection (similar to a webbing , so-called cutaneous syndactyly).
See also
Web links
Individual evidence
- ^ Wilhelm Gemoll : Greek-German school and hand dictionary . G. Freytag Verlag / Hölder-Pichler-Tempsky, Munich / Vienna 1965.
- ↑ a b F. Hefti: Pediatric orthopedics in practice. Springer 1998, ISBN 3-540-61480-X . P. 398
- ↑ W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. , 2nd ed., P. 111, 1996, ISBN 3-540-60224-0 .
- ^ Rudolf Häring , Hans Zilch : Textbook surgery . Walter de Gruyter, Berlin 1986, ISBN 3-11-009657-9 .
- ↑ The Fetal Alcohol Syndrome - University Children's Hospital Münster
- ↑ Syndactyly type 1. In: Orphanet (database for rare diseases).
- ^ Syndactyly type 2. In: Orphanet (database for rare diseases).
- ^ Syndactyly type 3. In: Orphanet (database for rare diseases).
- ^ Syndactyly type 4. In: Orphanet (database for rare diseases).
- ^ Syndactyly type 5. In: Orphanet (database for rare diseases).
- ^ Syndactyly type 6. In: Orphanet (database for rare diseases).
- ^ Syndactyly type 8. In: Orphanet (database for rare diseases).
