By Ghazi M. Rayan, Joseph Upton III
Physicians usually have simply partial wisdom of universal congenital hand and top extremity anomalies and their linked syndromes. Surgeons more often than not locate those syndromes to be abstruse and congenital hand stipulations can symbolize an enigma even to pediatric geneticists. This publication is designed to function a realistic, up to date reference that would allow practitioners and scholars in a number of disciplines to simply realize the commonest congenital higher extremity anomalies and syndromes. In overall, 37 congenital top extremity anomalies and 127 syndromes are mentioned. Salient and customary offering positive factors are defined intimately and illustrated by means of fine quality electronic colour photos each time attainable. additionally, correct heritage info is integrated on such facets as occurrence, etiology, pathogenesis and findings in other places within the body.
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Extra info for Congenital Hand Anomalies and Associated Syndromes
Rayan, J. 1007/978-3-642-54610-5_4, © Springer-Verlag Berlin Heidelberg 2014 29 30 4 Vascular Malformations Associated Syndromes Klippel-Trenaunay syndrome Blue rubber bleb nevus syndrome CLOVES syndrome Bockenheimer syndrome Glomuvenous Parkes-Weber syndrome PHOST PTEN Sturge-Weber syndrome Proteus syndrome Maffucci syndrome GAPO syndrome Cowden syndrome Klippel-Trenaunay Syndrome Klippel-Trenaunay Syndrome AKA KTS (abbreviation) Klippel-Trenaunay-Weber syndrome Angioosteohypertrophy syndrome Osteohypertrophic-varicose-nevus syndrome Hallmarks A triad of vascular malformations, soft tissue and skeletal hypertrophy and capillary malformations (CM).
B The CM and soft tissue hypertrophy of the central portion of the hand have displaced the less affected peripheral digits. The atrophic skeleton is 4 Vascular Malformations associated with disuse. c Despite surgery the right arm is often stiff and marginally functional. Truncal CLVMs and scoliosis are common with upper limb involvement Klippel-Trenaunay Syndrome Fig. 2 KTS a Despite early surgery this CLVM has continued to grow. Lymphatic vesicles on the chest wall continue to ulcerate and macerate.
Compression at the root level within the paraspinal regions must be considered. As with the long bones a pseudoarthrosis may be seen at the forearm level. The proximal half of the radius is most common. These typically present in early childhood with a slight radial deviation of the wrist caused by a slight shortening of the radius with or without a subtle dysplasia and/or subluxation of the radial head. These lesions are typically not painful. With growth the clinical and radiologic changes become more apparent.