alexa Management of Genu Varum/Valgum in Shwachman-Diamond Syndrome: A Report of Two Cases | OMICS International | Abstract
ISSN: 2168-9784

Journal of Medical Diagnostic Methods
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Case Report

Management of Genu Varum/Valgum in Shwachman-Diamond Syndrome: A Report of Two Cases

Giovanni Luigi Di Gennaro1, Elisa Pala2* and Onofrio Donzelli1
1Paediatric Orthopaedic Surgeon, Pediatric Orthopedic Department, Istituto Ortopedico Rizzoli, Bologna, Italy
2Registrar University of Bologna, Pediatric Orthopedic Department, Istituto Ortopedico Rizzoli, Bologna, Italy
Corresponding Author : Elisa Pala
Istituto Ortopedico Rizzoli
Via GC Pupilli, 1, 40136 Bologna, Italy
Tel: +393394044900
Fax: +39051331710
E-mail: [email protected]
Received June 25, 2013; Accepted August 05, 2013; Published August 08, 2013
Citation: Di Gennaro GL, Pala E, Donzelli O (2013) Management of Genu Varum/ Valgum in Shwachman-Diamond Syndrome: A Report of Two Cases. J Med Diagn Meth 2:126. doi:10.4172/2168-9784.1000126
Copyright: © 2013 Di Gennaro GL, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Shwachman’s syndrome is a rare autosomal recessive disorder characterized by exocrine pancreatic insufficiency, bone marrow failure and skeletal abnormalities. The skeletal defects in the knees are related to the asymmetrical development of the growth plates that can result in varum/valgum deformities. We describe two cases of knee deformity in SDS patients surgically treated. In case n.1, because of a severe deformity, an osteotomy of the distal femur was performed by removing a lateral wedge and stabilizing with a staple, plus lateral tibial hemiepiphysiodesis. In case n. 2 first a medial hemiepiphysiodesis of the proximal tibia with Blount staples was performed. After 18 months the staples were removed from the tibia and a medial femoral hemiepiphysiodesis was performed. In both patients satisfactory angular alignment of the knees was obtained. In patients with genu varu/valgum in SDS the general condition of the patient (values of neutropenia and thrombocytopenia) determines the timing of surgical treatment because the risk of infection is always lurking. Traditional methods, such as osteotomy or hemiepiphysiodesis, have proved to be effective in the treatment of these deformities, but must be finely adjusted, by repeating or combining these procedures to adapt to the conditions of the pathologic physes.

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