Thoracic insufficiency syndrome: Approaches to assessment and management.

Thoracic insufficiency syndrome (TIS) was described in 2003 as the inability of the thorax to support normal respiration or lung growth. TIS includes a broad and disparate group of typically degenerative thoracospinal conditions. Although TIS arises due to a heterogeneous group of disorders and thus its incidence is not well quantified, general approaches to management and treatment exist. Evolving imaging techniques and measurements of health-related quality of life augment tests of pulmonary function to quantify disease burden, longitudinally and pre- and post-intervention. Intervention is primarily via growth-sparing surgery, for which several device options exist, to preserve vertical growth prior to a definitive spinal fusion at skeletal maturity.

Chest wall hypoplasia--principles and treatment.

The chest is a dynamic structure. For normal movement it relies on a coordinated movement of the multiple bones, joints and muscles of the respiratory system. While muscle weakness can have clear impact on respiration by decreasing respiratory motion, so can conditions that cause chest wall hypoplasia and produce an immobile chest wall. These conditions, such as Jarcho-Levin and Jeune syndrome, present significantly different challenges than those faced with early onset scoliosis in which chest wall mechanics and thoracic volume may be much closer to normal. Because of this difference more aggressive approaches to clinical and surgical management are necessary.

Scoliosis and the impact in neuromuscular disease.

Scoliosis can alter respiratory mechanics by changing the orientation of the muscles and joints of the respiratory system and in severe forms can put a patient at risk of severe respiratory morbidity or respiratory failure. However, perhaps the most important factor in determining the pulmonary morbidity in scoliosis is the balance between the "load" or altered respiratory mechanics and the "pump" or the respiratory muscle strength. Therefore, scoliosis in patients with neuromuscular disease will both lead to increased "load" and a weakened "pump", an exceptionally unfortunate combination. While progressive neuromuscular disease by its nature does not respond favorably to attempts to improve respiratory muscle strength, the natural approach of early proactive management of the "load" and in the case of scoliosis a variety of different strategies have been tried with variable short term and long term results. Figuring this out requires both an understanding of the underlying pathophysiology of a particular neuromuscular condition and the available options for and timing of surgical intervention.

Early changes in pulmonary function after vertical expandable prosthetic titanium rib insertion in children with thoracic insufficiency syndrome.

BACKGROUND: The vertical expandable prosthetic titanium rib (VEPTR) has been inserted in children with thoracic insufficiency syndrome for the last decade to expand and support the chest and allow for further lung growth. There are minimal published data evaluating the postoperative change in lung function after VEPTR insertion. We hypothesize that there will be a significant increase in lung function after VEPTR insertion, and the earlier the insertion, the greater the improvement. METHODS: The Chest Wall Disorders Study Group Database, containing data before and after VEPTR insertion from 7 different centers, was queried for spirometry and lung volume measurements, and the data were analyzed to assess the short-term effect on lung function of VEPTR placement. RESULTS: There was a statistically significant decrease in forced vital capacity, forced expiratory volume in 1 second as a percent of predicted, an increase in residual volume (RV) that did not reach statistical significance, and there was no change in total lung capacity at the first postoperative visit (7.7 +/- 4.8 months). There was a significant decrease in Cobb angle. There was no correlation between absolute change in any pulmonary function and Cobb angle age at the time of surgery. CONCLUSIONS: Although there is a clinically and radiographically apparent expansion of the thorax after VEPTR insertion, there is no similar improvement in lung volume, and instead there is a decrease in forced vital capacity and increase in residual volume, the explanation for which requires further study. This lack of change in pulmonary function after VEPTR insertion suggests that the benefit of VEPTR insertion may lie more in stabilizing the thorax and improving respiratory mechanics measured in other ways.

Pediatric palliative, end-of-life, and bereavement care.

The pediatric hospitalist plays an integral role in providing palliative, end-of-life, and bereavement care for children and families. This article focuses on a multifaceted approach to this domain of care in which the physician is a key member of an interdisciplinary team. We believe that we can improve quality of life and relieve suffering only by paying attention to the medical, emotional, spiritual, and practical needs and goals of dying children and their loved ones.