Jeune syndrome (JS) is an autosomal recessive disease also known as asphyxiating thoracic dystrophy. A narrow bell-shaped thoracic wall and short extremities are the most typical features of the syndrome. Prognosis in JS depends on the severity of the pulmonary hypoplasia caused by the chest wall deformity. Most patient deaths are due to respiratory problems at early ages. Herein, we report a c...

PURPOSE Stabilization and replacement of ribs is still a challenge, because most available systems for intramedullary and extramedullary fixation are less than perfect. We present our experience with a modified device, which compensates for several disadvantages in other methods. DESCRIPTION Originating from the Strasbourg Thoracic Osteosyntheses System (STRATOS [MedXpert GmbH, Heitersheim, G...

Blunt chest wall trauma is a major cause of morbidity and mortality, especially in the presence of a flail chest where paradoxical inward movement of the flail segment in inspiration is found. Patients with a flail chest require aggressive pain control, pulmonary toilet, and often intubation and mechanical ventilation to establish an internal pneumatic stabilization of the flail segment. This m...

Nonoperative and operative management for early onset scoliosis has changed significantly since the Harrington era 50 plus years ago. Surgeons learned quickly that a spine fusion in growing child can result short thorax development of thoracic insufficiency syndrome. Techniques were developed refined over subsequent decades to allow spinal growth, control chest wall deformity, limit pulmonary d...

Gillam, G. L., McNicol, K. N., and Williams, H. E. (1970). Archives of Disease in Childhood, 45, 789. Chest deformity, residual airways obstruction and hyperinflation, and growth in children with asthma. Part II: Significance of chronic chest deformity. Studies were made on 56 10-11-year-old asthmatic children with onset of symptoms before 3 years of age, frequent and persistent attacks, and ob...

STUDY DESIGN Case series. PURPOSE The objective of this study was to investigate the change in pulmonary function in adult patients with a spinal deformity who underwent spinal corrective surgery. OVERVIEW OF LITERATURE Degenerative lumbar and/or thoraco-lumbar deformities are is often prominent in adult spinal deformity cases, whereas a thoracic deformity involving the chest wall is incons...

conclusions negative pressure inspiration is significantly impaired by an unstable chest wall. restoring mechanical stability of the fractured ribs improves respiratory outcomes similar to baseline values. results a wide variation in the mean inspiratory volumes and peak flows were measured between specimens; however, the effect of a flail chest wall and the subsequent internal fixation of the ...

Background: Non-invasive ventilation is an established treatment for chronic respiratory failure due to chest wall deformity. There are few data available to inform the choice between volume and pressure ventilators. The aim of this study was to compare pressure and volume targeted ventilation in terms of diurnal arterial blood gas tensions, lung volumes, hypercapnic ventilatory responses, slee...