African Journal of Paediatric Surgery About APSON | PAPSA  
Home About us Editorial Board Current issue Search Archives Ahead Of Print Subscribe Instructions Submission Contact Login 
Users Online: 708Print this page  Email this page Bookmark this page Small font size Default font size Increase font size 
 
TECHNICAL INNOVATION
Year : 2014  |  Volume : 11  |  Issue : 1  |  Page : 3-7

Reconstruction of bilateral tibial aplasia and split hand-foot syndrome in a father and daughter


1 Department of First Medical, Ludwig Boltzmann Institute of Osteology, Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, Hanusch Hospital; Department of Paediatric, Orthopaedic Hospital of Speising, Vienna, Austria
2 Department of Paediatric, Orthopaedic Hospital of Speising, Vienna, Austria
3 Department of First Medical, Ludwig Boltzmann Institute of Osteology, Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, Hanusch Hospital, Vienna, Austria

Correspondence Address:
Ali Al Kaissi
Department of First Medical, Ludwig Boltzmann Institute of Osteology, Hanusch Hospital of WGKK and AUVA Trauma Center Meidling, Hanusch Hospital, Vienna
Austria
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-6725.129201

Rights and Permissions

Background: Tibial aplasia is of heterogeneous aetiology, the majority of reports are sporadic. We describe the reconstruction procedures in two subjects - a daughter and father manifested autosomal dominant (AD) inheritance of the bilateral tibial aplasia and split hand-foot syndrome. Materials and Methods: Reconstruction of these patients required multiple surgical procedures and orthoprosthesis was mandatory. The main goal of treatment was to achieve walking. Stabilization of the ankle joint by fibular-talar-chondrodesis on both sides, followed by bilateral Brown-procedure at the knee joint level has been applied accordingly. Results: The outcome was with improved function of the deformed limbs and walking was achieved with simultaneous designation of orthotic fitting. Conclusion: This is the first study encompassing the diagnosis and management of a father and daughter with bilateral tibial aplasia associated with variable split hand/foot deformity without foot ablation. Our patients showed the typical AD pattern of inheritance of split-hand/foot and tibial aplasia.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed6599    
    Printed100    
    Emailed0    
    PDF Downloaded208    
    Comments [Add]    

Recommend this journal