1314 deformity with mesomelic dwarfism of both upper and lower limbs. It is. the commonest variety of mesomelic dwarfism(3). Madelung deformity may also be seen associated with diverse disorders such as mucopolysaccharidosis, Turner's syndrome, achondroplasia, dyschondroplasia (Ollier's disease), multiple exostosis and multiple epiphyseal dysplasia. Madelung's original description of the lesion...

INTRODUCTION This systematic review aims to answer three research questions concerning the management of hereditary multiple osteochondromas of forearm in children: What is the best available evidence for the currently employed surgical procedures? What patient characteristics are associated with better prognosis? What disease characteristics are associated with better prognosis? METHODS We s...

BACKGROUND Moisturizing, heating and filtering gases inspired via the mechanical ventilation (MV) circuits help to reduce the adverse effects of MV. However, there is still no consensus regarding whether these measures improve patient prognosis, shorten MV duration, decrease airway secretion and lower the incidence of ventilator associated pneumonia (VAP) and other complications. OBJECTIVES T...

We report a mouse model of multiple osteochondromas (MO), an autosomal dominant disease in humans, also known as multiple hereditary exostoses (MHE or HME) and characterized by the formation of cartilage-capped osseous growths projecting from the metaphyses of endochondral bones. The pathogenesis of these osteochondromas has remained unclear. Mice heterozygous for Ext1 or Ext2, modeling the hum...

PURPOSE Deformities of the forearm and shortening of the ulna occur in 30 % of patients with hereditary multiple exostoses (HME), leading to radial head dislocation and loss of movement. Several surgical techniques have been described for treatment, and the aim of our study was to present clinical and radiographic results at skeletal maturity in 15 children that underwent the surgical procedure...

Introduction: Cerebral cortical development is a highly complex process influenced by environmental, genetic and functional abnormalities. Hemimegalencephaly (HME) is a rare brain malformation that involves overgrowth of one hemisphere. Clinically macrocephaly, mental retardation, contralateral hemiparesis, hemianopsia and intractable epilepsy may be present. Diagnosis is mainly done with image...

BACKGROUND Chest wall osteochondroma is a rare tumor in children. Even though the potential for malignant transformation or serious intrathoracic complications is low, it has led some centers to advocate surgical management of these bony tumors. We present our experience of the surgical management of costal osteochondromata. METHODS Between January 1, 2006 and November 1, 2012 we saw 854 pati...