Split hand/foot malformation: Report of 13 cases in a family with variable presentation
Introduction: Split hand/foot (SHSF) malformation is characterized by variable suppression of central digital rays. It is a rare congenital hand and foot anomaly usually showing autosomal dominant pattern of inheritance. We report this rare anomaly of hand and foot in a family with 13 cases to discu...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2014-01-01
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| Series: | Journal of Orthopedics, Traumatology and Rehabilitation |
| Subjects: | |
| Online Access: | http://www.jotr.in/article.asp?issn=0975-7341;year=2014;volume=7;issue=1;spage=53;epage=55;aulast=Singh |
| _version_ | 1818341738640572416 |
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| author | Vidya Bhushan Singh Abhilekh Mishra Amit Kumar Chaurasia Pankaj Kumar Lakhtakia |
| author_facet | Vidya Bhushan Singh Abhilekh Mishra Amit Kumar Chaurasia Pankaj Kumar Lakhtakia |
| author_sort | Vidya Bhushan Singh |
| collection | DOAJ |
| description | Introduction: Split hand/foot (SHSF) malformation is characterized by variable suppression of central digital rays. It is a rare congenital hand and foot anomaly usually showing autosomal dominant pattern of inheritance. We report this rare anomaly of hand and foot in a family with 13 cases to discuss its inheritance pattern and clinical features. Observation: One family of 27 members has been identified in which 13 members are affected. The disease has been running dominantly in the family and variably affecting both males and females. Morphologically there is partial or complete absence of central second to fourth digital rays with variable syndactyly and bony changes in adjacent rays. Conclusion: SHSF malformation is type of longitudinal arrest of development of central digital rays. Various theories regarding causation of disease have been proposed. The presentation of disease varies due to varying severity of involvement. Early appropriate surgical intervention is essential in selected cases to achieve a good functional limb. |
| first_indexed | 2024-12-13T16:03:34Z |
| format | Article |
| id | doaj.art-7dcd4b71fe56401896b8be86212d9bf5 |
| institution | Directory Open Access Journal |
| issn | 0975-7341 |
| language | English |
| last_indexed | 2024-12-13T16:03:34Z |
| publishDate | 2014-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Journal of Orthopedics, Traumatology and Rehabilitation |
| spelling | doaj.art-7dcd4b71fe56401896b8be86212d9bf52022-12-21T23:39:06ZengWolters Kluwer Medknow PublicationsJournal of Orthopedics, Traumatology and Rehabilitation0975-73412014-01-0171535510.4103/0975-7341.134017Split hand/foot malformation: Report of 13 cases in a family with variable presentationVidya Bhushan SinghAbhilekh MishraAmit Kumar ChaurasiaPankaj Kumar LakhtakiaIntroduction: Split hand/foot (SHSF) malformation is characterized by variable suppression of central digital rays. It is a rare congenital hand and foot anomaly usually showing autosomal dominant pattern of inheritance. We report this rare anomaly of hand and foot in a family with 13 cases to discuss its inheritance pattern and clinical features. Observation: One family of 27 members has been identified in which 13 members are affected. The disease has been running dominantly in the family and variably affecting both males and females. Morphologically there is partial or complete absence of central second to fourth digital rays with variable syndactyly and bony changes in adjacent rays. Conclusion: SHSF malformation is type of longitudinal arrest of development of central digital rays. Various theories regarding causation of disease have been proposed. The presentation of disease varies due to varying severity of involvement. Early appropriate surgical intervention is essential in selected cases to achieve a good functional limb.http://www.jotr.in/article.asp?issn=0975-7341;year=2014;volume=7;issue=1;spage=53;epage=55;aulast=Singhmalformationsplit footsplit hand |
| spellingShingle | Vidya Bhushan Singh Abhilekh Mishra Amit Kumar Chaurasia Pankaj Kumar Lakhtakia Split hand/foot malformation: Report of 13 cases in a family with variable presentation Journal of Orthopedics, Traumatology and Rehabilitation malformation split foot split hand |
| title | Split hand/foot malformation: Report of 13 cases in a family with variable presentation |
| title_full | Split hand/foot malformation: Report of 13 cases in a family with variable presentation |
| title_fullStr | Split hand/foot malformation: Report of 13 cases in a family with variable presentation |
| title_full_unstemmed | Split hand/foot malformation: Report of 13 cases in a family with variable presentation |
| title_short | Split hand/foot malformation: Report of 13 cases in a family with variable presentation |
| title_sort | split hand foot malformation report of 13 cases in a family with variable presentation |
| topic | malformation split foot split hand |
| url | http://www.jotr.in/article.asp?issn=0975-7341;year=2014;volume=7;issue=1;spage=53;epage=55;aulast=Singh |
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