Macrodactyly
| Classification according to ICD-10 | |
|---|---|
| Q74.0 | Other congenital malformations of the upper extremity (s) and shoulder girdle |
| Q74.2 | Other congenital malformations of the lower extremity (s) and the pelvic girdle |
| ICD-10 online (WHO version 2019) | |
Under local gigantism ( . Greek μακρός macros "large", "long", δακτυλος daktylos "fingers"; "toe") refers to a relative to the other disproportionate growth, a circumscribed gigantism (gigantism) one or more fingers or toes. To differentiate it from other diseases such as B. vascular malformations , lymphangioma , tumors , exostoses , in which individual components of the finger or toe anatomy are affected, all tissues are proportionally enlarged in macrodactyly.
As with most congenital limb malformations, the cause is assumed to be a disorder during the 4th to 6th week of pregnancy.
clinic
The enlargement can already be present at birth and develop proportionally to the growth or it can only appear after the first few years of life and then show more rapid growth.
The middle rays are preferably affected. Syndactyly is more common . On the foot, the focus is on the problem of shoe supply, including the displacing effect on neighboring toes.
Differential diagnosis
The change can occur in isolation as congenital macrodactyly or in the context of:
- Hemihypertrophy
- Klippel-Trénaunay-Weber syndrome
- Lipofibromatosis
- Neurofibromatosis
- progressive macrodystrophia lipomatosa
- Proteus Syndrome
Diagnosis
An x-ray is required to delineate the differential diagnoses listed . Except for their oversize, the bony structures have not changed.
treatment
Treatment depends on the extent of the impairment and ranges from epiphysodesis to partial amputation . It takes place in connection with a pediatric orthopedic department.
Individual evidence
- ^ Carl Joachim Wirth, Abdul Kader Martini: Orthopedics and orthopedic surgery . Georg Thieme Verlag, 2003, ISBN 978-3-13-126211-0 , p. 216–.
- ↑ ICD 10 - Q74 ( Memento of the original from September 8, 2013 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice.
- ↑ F. Hefti: Pediatric Orthopedics in Practice . Springer 1998, ISBN 3-540-61480-X , page 492
literature
- RR van Rijn, JG Blickman: Differential Diagnoses in Pediatric Radiology. Thieme 2012, ISBN 978-3-13-169571-0 .
- Lexicon Orthopedics - Macrodactyly
