Paediatric Respiratory Reviews 16 (2015) 1–2
Contents lists available at ScienceDirect
Paediatric Respiratory Reviews
Editorial
Chest Wall Mini-symposium In pulmonary medicine the focus is appropriately on the lungs and their ability to effectively bring oxygen into the blood stream and then remove carbon dioxide in an elegant blend of bulk flow, diffusion, binding and chemical reaction. Because there is so much that can go wrong in this process, there is much to attract attention, and rightfully so. However, in doing so it is important to acknowledge the importance of the engine that drives the entire process of breathing, the respiratory pump, which is composed of the chest wall, abdomen, and respiratory muscles. This minisymposium will thoroughly explore each component of the respiratory pump. First we will explore the assessment of respiratory function through thoracoabdominal motion. While the standard approach to respiratory function is direct measurements of flow or volume from exhaled air, there are a variety of valid ways of assessing respiratory function at the thoracoabdominal surface. Many of these measurements are quantified measurements of the qualitative assessments that we often make in performing a physical assessment of a patient. Dr. Paul Seddon will discuss these in detail and how many of the more traditional tests of thoracoabdominal motion are already used in respiratory assessment [1]. The paper will also highlight how some of the newer measurements can be used to gain more precise functional data. The thorax and abdomen work in concert and deficiencies in one unit can have clear impact on the other. Dr. Howard Panitch will review the impact of abdominal wall defects on the diaphragm and respiration [2]. Providing a thorough exploration of the immediate physiologic abnormalities that these defects can produce, the options for therapy and then the long term prognosis and impact on physiologic function are discussed. The anterior chest wall produces the most common thoracic abnormalities in pectus excavatum and pectus carinatum. Pectus defects are easily recognised, but often difficult to thoroughly understand due to the varied conditions in which they occur, ranging from connective tissue diseases to neuromuscular disease with respiratory muscle weakness. Dr. Anastassios Koumbourlis takes the reader through the pectus defects comprehensively in discussing the multiple genetic links and proposed etiologies and their clinical presentation [3]. The different decision points for surgical correction and the options available for repair currently are discussed and contrasted with how past surgical approaches, highlighting how and why the current techniques are used. Moving to the spine and posterior chest wall Drs. Greg Redding and Oscar Mayer explore chest wall and spine disease caused but primary abnormalities in skeletal and muscular support. Dr. Redding’s paper describes in some detail early onset scoliosis
http://dx.doi.org/10.1016/j.prrv.2014.10.011 1526-0542/ß 2014 Elsevier Ltd. All rights reserved.
(EOS), which is typically associated with structural skeletal deformity of the vertebrae or ribs [4]. Unlike in more straight forward juvenile or adolescent onset scoliosis, in EOS the scoliosis often occurs due to asymmetric chest wall and spine growth that can produce a complex three dimensional abnormality involving scoliosis, kyphosis, spinal rotation, and rarely lordosis. This produces variable physiologic abnormalities in impacting respiratory muscle function and a progressive restrictive respiratory disease that is often more complex and severe than that seen in less severe scoliosis. Dr. Redding emphasizes the unique approach needed when treating patients with EOS compared to less complex later onset scoliosis. The paper contrasts growth sparing procedures and variable insertion/support points to correct the defect and harness growth potential as opposed to suppression of spinal and chest wall growth in correcting the deformity. The thorax grows by coincident growth of the ribs and spine and defects in either, or both, can cause significant chest wall hypoplasia. Dr. Mayer reviews general concepts related to chest wall hypoplasia and its clinical significance in discussing examples of hypoplasia due to abnormal spine growth (Jarcho-Levin syndrome) and abnormal chest wall growth (Jeune syndrome) [5]. These conditions require aggressive surgical intervention that involves unique approaches, much different that needed for EOS. In the final chapter, Dr. Mayer discusses the impact of neuromuscular disease on scoliosis and chest wall distortion using the disparate examples of spinal muscular atrophy (SMA) and Duchenne muscular dystrophy (DMD) [6]. In SMA the neuropathy produces progressive muscle atrophy that typically affects the intercostal muscles more than other respiratory muscles and often produces severe chest wall distortion in addition to severe scoliosis with the chest wall distortion often preceding the scoliosis. However, in DMD the myopathy affects the diaphragm prominently and then the skeletal muscles quite diffusely. This typically produces a scoliosis and in some cases a secondary chest wall distortion that is usually not nearly as severe as that in SMA. Because of this, and that the onset of thoracospinal disease is typically earlier in SMA than in DMD, the treatment approach is often dramatically different often with use of growth sparing interventions in SMA, but more conventional spinal fusion in DMD. Thoracospinal functional impairment is relatively easy to irecognise, compared to airway and parenchymal function, but it is often hard to quantify, and harder to treat. I hope that you find this mini-symposium a useful review of the important role of the chest wall and spine in the operation of the respiratory system.
2
Editorial / Paediatric Respiratory Reviews 16 (2015) 1–2
References [1] Seddon P. Options for Assessing and Measuring Chest Wall Motion. Paediatr Respir Rev 2015;16(1):10–7. [2] Panitch HB. Pulmonary complications of abdominal wall defects. Paediatr Respir Rev 2015;16(1):3–9. [3] Koumbourlis AC. Pectus deformities and their impact on pulmonary physiology. Paediatr Respir Rev 2015;16(1):18–24. [4] Redding G. Primary Thoraco-spinal Disorders of Childhood. Paediatr Respir Rev 2015;16(1):25–9. [5] Mayer OH. Chest wall hypoplasia- Principles and Treatment. PaediatrcRespir Rev 2015;16(1):30–4.
[6] Mayer OH. Scoliosis and the impact in neuromuscular disease. NMDz paper on scoliosis Paediatr Respir Rev 2015;16(1):35–42.
Oscar Henry Mayer*, Associate Professor of Clinical Pediatrics, Perelman School of Medicine at The University of Pennsylvania, Division of Pulmonary Medicine, The Children’s Hospital of Philadelphia, 3501 Civic Center Boulevard, Philadelphia, PA 19104 *Tel.: +215 590 3749; fax: +215 590 3500 E-mail address: [email protected]
Contents lists available at ScienceDirect
Paediatric Respiratory Reviews
Editorial
Chest Wall Mini-symposium In pulmonary medicine the focus is appropriately on the lungs and their ability to effectively bring oxygen into the blood stream and then remove carbon dioxide in an elegant blend of bulk flow, diffusion, binding and chemical reaction. Because there is so much that can go wrong in this process, there is much to attract attention, and rightfully so. However, in doing so it is important to acknowledge the importance of the engine that drives the entire process of breathing, the respiratory pump, which is composed of the chest wall, abdomen, and respiratory muscles. This minisymposium will thoroughly explore each component of the respiratory pump. First we will explore the assessment of respiratory function through thoracoabdominal motion. While the standard approach to respiratory function is direct measurements of flow or volume from exhaled air, there are a variety of valid ways of assessing respiratory function at the thoracoabdominal surface. Many of these measurements are quantified measurements of the qualitative assessments that we often make in performing a physical assessment of a patient. Dr. Paul Seddon will discuss these in detail and how many of the more traditional tests of thoracoabdominal motion are already used in respiratory assessment [1]. The paper will also highlight how some of the newer measurements can be used to gain more precise functional data. The thorax and abdomen work in concert and deficiencies in one unit can have clear impact on the other. Dr. Howard Panitch will review the impact of abdominal wall defects on the diaphragm and respiration [2]. Providing a thorough exploration of the immediate physiologic abnormalities that these defects can produce, the options for therapy and then the long term prognosis and impact on physiologic function are discussed. The anterior chest wall produces the most common thoracic abnormalities in pectus excavatum and pectus carinatum. Pectus defects are easily recognised, but often difficult to thoroughly understand due to the varied conditions in which they occur, ranging from connective tissue diseases to neuromuscular disease with respiratory muscle weakness. Dr. Anastassios Koumbourlis takes the reader through the pectus defects comprehensively in discussing the multiple genetic links and proposed etiologies and their clinical presentation [3]. The different decision points for surgical correction and the options available for repair currently are discussed and contrasted with how past surgical approaches, highlighting how and why the current techniques are used. Moving to the spine and posterior chest wall Drs. Greg Redding and Oscar Mayer explore chest wall and spine disease caused but primary abnormalities in skeletal and muscular support. Dr. Redding’s paper describes in some detail early onset scoliosis
http://dx.doi.org/10.1016/j.prrv.2014.10.011 1526-0542/ß 2014 Elsevier Ltd. All rights reserved.
(EOS), which is typically associated with structural skeletal deformity of the vertebrae or ribs [4]. Unlike in more straight forward juvenile or adolescent onset scoliosis, in EOS the scoliosis often occurs due to asymmetric chest wall and spine growth that can produce a complex three dimensional abnormality involving scoliosis, kyphosis, spinal rotation, and rarely lordosis. This produces variable physiologic abnormalities in impacting respiratory muscle function and a progressive restrictive respiratory disease that is often more complex and severe than that seen in less severe scoliosis. Dr. Redding emphasizes the unique approach needed when treating patients with EOS compared to less complex later onset scoliosis. The paper contrasts growth sparing procedures and variable insertion/support points to correct the defect and harness growth potential as opposed to suppression of spinal and chest wall growth in correcting the deformity. The thorax grows by coincident growth of the ribs and spine and defects in either, or both, can cause significant chest wall hypoplasia. Dr. Mayer reviews general concepts related to chest wall hypoplasia and its clinical significance in discussing examples of hypoplasia due to abnormal spine growth (Jarcho-Levin syndrome) and abnormal chest wall growth (Jeune syndrome) [5]. These conditions require aggressive surgical intervention that involves unique approaches, much different that needed for EOS. In the final chapter, Dr. Mayer discusses the impact of neuromuscular disease on scoliosis and chest wall distortion using the disparate examples of spinal muscular atrophy (SMA) and Duchenne muscular dystrophy (DMD) [6]. In SMA the neuropathy produces progressive muscle atrophy that typically affects the intercostal muscles more than other respiratory muscles and often produces severe chest wall distortion in addition to severe scoliosis with the chest wall distortion often preceding the scoliosis. However, in DMD the myopathy affects the diaphragm prominently and then the skeletal muscles quite diffusely. This typically produces a scoliosis and in some cases a secondary chest wall distortion that is usually not nearly as severe as that in SMA. Because of this, and that the onset of thoracospinal disease is typically earlier in SMA than in DMD, the treatment approach is often dramatically different often with use of growth sparing interventions in SMA, but more conventional spinal fusion in DMD. Thoracospinal functional impairment is relatively easy to irecognise, compared to airway and parenchymal function, but it is often hard to quantify, and harder to treat. I hope that you find this mini-symposium a useful review of the important role of the chest wall and spine in the operation of the respiratory system.
2
Editorial / Paediatric Respiratory Reviews 16 (2015) 1–2
References [1] Seddon P. Options for Assessing and Measuring Chest Wall Motion. Paediatr Respir Rev 2015;16(1):10–7. [2] Panitch HB. Pulmonary complications of abdominal wall defects. Paediatr Respir Rev 2015;16(1):3–9. [3] Koumbourlis AC. Pectus deformities and their impact on pulmonary physiology. Paediatr Respir Rev 2015;16(1):18–24. [4] Redding G. Primary Thoraco-spinal Disorders of Childhood. Paediatr Respir Rev 2015;16(1):25–9. [5] Mayer OH. Chest wall hypoplasia- Principles and Treatment. PaediatrcRespir Rev 2015;16(1):30–4.
[6] Mayer OH. Scoliosis and the impact in neuromuscular disease. NMDz paper on scoliosis Paediatr Respir Rev 2015;16(1):35–42.
Oscar Henry Mayer*, Associate Professor of Clinical Pediatrics, Perelman School of Medicine at The University of Pennsylvania, Division of Pulmonary Medicine, The Children’s Hospital of Philadelphia, 3501 Civic Center Boulevard, Philadelphia, PA 19104 *Tel.: +215 590 3749; fax: +215 590 3500 E-mail address: [email protected]
