This document discusses various congenital chest wall anomalies including pectus excavatum, pectus carinatum, Poland syndrome, sternal defects, ectopia cordis, Jeune's syndrome, and rib cage overgrowth. It describes the causes, clinical presentations, investigations, and treatment options for each condition. The treatment sections focus on the modified Ravitch procedure, Nuss procedure, osteotomies, muscle flaps, and bracing depending on the specific anomaly.
Dr. Jakeer Hussain discusses pneumothorax, beginning with an introduction and definition. He then covers the classification of pneumothorax as either spontaneous, traumatic, or iatrogenic. Spontaneous pneumothorax is further classified as primary or secondary. The document discusses signs, symptoms, investigations including x-ray and CT scan findings, differential diagnosis, quantification methods, and various treatment options including observation, oxygen supplementation, needle aspiration, tube thoracostomy, medical or VATS pleurodesis, and open thoracotomy.
This document provides information on various congenital heart diseases. It discusses the signs, symptoms and treatments for conditions such as atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), tetralogy of Fallot (TOF), pulmonary stenosis, coarctation of the aorta and Eisenmenger's syndrome. Some key points include that ASD, VSD and PDA cause left-to-right shunts which can lead to heart failure if untreated. TOF is the most common cyanotic heart defect and causes right-to-left shunting. Symptoms of cyanotic conditions include clubbing and central cyanosis. Surgical correction
1. Chylothorax is defined as a collection of chyle in the pleural cavity resulting from a leak in the lymphatic vessels, usually from the thoracic duct which transports lymph from the body.
2. The thoracic duct anatomy is described, beginning near the kidneys and ascending through the diaphragm before crossing left and terminating near the subclavian vein. Damage to different parts of the duct can cause right or left-sided chylothorax.
3. Causes of chylothorax include traumatic injury, malignancy compressing vessels, and spontaneous/idiopathic cases. Diagnosis involves fluid analysis showing chylomicrons.
Primary ciliary dyskinesia (pcd) is an autosomal recessive genetic condition in which the microscopic cells in the respiratory system called cilia do not function normally.
The document provides an overview of the respiratory system, including its main functions and components. It describes the lungs' role in gas exchange, their anatomy, and the upper and lower airway divisions. It also outlines the stages of respiration from ventilation to gas exchange in tissues. Key parts like the pleura, alveoli, conducting and respiratory zones are defined. Respiratory protective reflexes such as coughing and swallowing are summarized as well.
The document discusses the development of the lungs and diaphragm during gestation. It notes that the lungs develop from the respiratory diverticulum which partitions into trachea, bronchi and lungs. The diaphragm develops from the partition of the foregut. Defects in this partitioning can result in conditions like esophageal atresia or tracheo-esophageal fistula. The document also lists some common lung defects seen in newborns like respiratory distress syndrome.
The Light criteria from 1972 remains a useful tool for differentiating transudative and exudative pleural effusions. While it accurately classifies most exudates, it falsely classifies around 25% of transudates as exudates, likely due to more powerful diuretic use now. If the Light criteria suggest an exudate but transudate is suspected, measuring the serum-pleural fluid protein gradient, NT-proBNP levels, or albumin gradient can help identify misclassified transudates. The Light criteria provide a good starting point but may require supplementation for accurate classification in some cases.
This document discusses erosion and resorption of the terminal phalangeal tufts (tips of the fingers and toes) in various conditions. It includes 10 figures showing examples in Raynaud's disease, diabetic gangrene, psoriatic arthritis, leprosy, hyperparathyroidism, Lesch-Nyhan syndrome, epidermolysis bullosa, progeria, familial acro-osteolysis, and pachydermoperiostosis. Each figure illustrates the characteristic bone changes and soft tissue involvement in the distal phalanges.
Dr. Jakeer Hussain discusses pneumothorax, beginning with an introduction and definition. He then covers the classification of pneumothorax as either spontaneous, traumatic, or iatrogenic. Spontaneous pneumothorax is further classified as primary or secondary. The document discusses signs, symptoms, investigations including x-ray and CT scan findings, differential diagnosis, quantification methods, and various treatment options including observation, oxygen supplementation, needle aspiration, tube thoracostomy, medical or VATS pleurodesis, and open thoracotomy.
This document provides information on various congenital heart diseases. It discusses the signs, symptoms and treatments for conditions such as atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), tetralogy of Fallot (TOF), pulmonary stenosis, coarctation of the aorta and Eisenmenger's syndrome. Some key points include that ASD, VSD and PDA cause left-to-right shunts which can lead to heart failure if untreated. TOF is the most common cyanotic heart defect and causes right-to-left shunting. Symptoms of cyanotic conditions include clubbing and central cyanosis. Surgical correction
1. Chylothorax is defined as a collection of chyle in the pleural cavity resulting from a leak in the lymphatic vessels, usually from the thoracic duct which transports lymph from the body.
2. The thoracic duct anatomy is described, beginning near the kidneys and ascending through the diaphragm before crossing left and terminating near the subclavian vein. Damage to different parts of the duct can cause right or left-sided chylothorax.
3. Causes of chylothorax include traumatic injury, malignancy compressing vessels, and spontaneous/idiopathic cases. Diagnosis involves fluid analysis showing chylomicrons.
Primary ciliary dyskinesia (pcd) is an autosomal recessive genetic condition in which the microscopic cells in the respiratory system called cilia do not function normally.
The document provides an overview of the respiratory system, including its main functions and components. It describes the lungs' role in gas exchange, their anatomy, and the upper and lower airway divisions. It also outlines the stages of respiration from ventilation to gas exchange in tissues. Key parts like the pleura, alveoli, conducting and respiratory zones are defined. Respiratory protective reflexes such as coughing and swallowing are summarized as well.
The document discusses the development of the lungs and diaphragm during gestation. It notes that the lungs develop from the respiratory diverticulum which partitions into trachea, bronchi and lungs. The diaphragm develops from the partition of the foregut. Defects in this partitioning can result in conditions like esophageal atresia or tracheo-esophageal fistula. The document also lists some common lung defects seen in newborns like respiratory distress syndrome.
The Light criteria from 1972 remains a useful tool for differentiating transudative and exudative pleural effusions. While it accurately classifies most exudates, it falsely classifies around 25% of transudates as exudates, likely due to more powerful diuretic use now. If the Light criteria suggest an exudate but transudate is suspected, measuring the serum-pleural fluid protein gradient, NT-proBNP levels, or albumin gradient can help identify misclassified transudates. The Light criteria provide a good starting point but may require supplementation for accurate classification in some cases.
This document discusses erosion and resorption of the terminal phalangeal tufts (tips of the fingers and toes) in various conditions. It includes 10 figures showing examples in Raynaud's disease, diabetic gangrene, psoriatic arthritis, leprosy, hyperparathyroidism, Lesch-Nyhan syndrome, epidermolysis bullosa, progeria, familial acro-osteolysis, and pachydermoperiostosis. Each figure illustrates the characteristic bone changes and soft tissue involvement in the distal phalanges.
A medical document discusses hypoxia following a road traffic accident. It describes four types of pulmonary contusion caused by direct chest compression, shearing against vertebral bodies, fractured ribs directly injuring the lung, or tearing of lung parenchyma due to prior adhesions. The document also mentions management of pulmonary contusion.
Cor pulmonale is a condition where the right ventricle of the heart enlarges and fails due to long-standing increased workload from diseases that affect the lungs like COPD. It is defined as hypertrophy and dilation of the right ventricle resulting from increased pulmonary vascular resistance. The main causes are chronic hypoxemia and pulmonary thromboembolism which lead to remodeling of the pulmonary arteries and increased pressure in the lungs. Over time, this puts strain on the right ventricle and can cause it to fail. Treatment focuses on reducing pulmonary pressures through oxygen therapy and vasodilators while managing symptoms of right heart failure.
1. Lung development begins in the fourth week of gestation and progresses through five stages - embryonic, pseudoglandular, canalicular, saccular, and alveolar.
2. Key molecular regulators of lung development include fibroblast growth factors, sonic hedgehog, retinoic acid, transforming growth factor beta, Wnts, platelet-derived growth factor and vascular endothelial growth factor.
3. Transcription factors such as NKX2-1, GLI genes, FOX family, GATA6 and SOX family also play important roles in lung development by regulating cell proliferation, branching morphogenesis and epithelial cell differentiation.
A flail chest occurs when a segment of the rib cage breaks and becomes detached from the rest of the chest wall, usually caused by two or more rib fractures in two or more places. Most commonly caused by vehicle accidents. During breathing, the detached flail segment moves in opposite direction to the rib cage, causing pain and paradoxical breathing. Management involves analgesia, intubation if needed, chest tube insertion, and physiotherapy. Outcomes range from full recovery to mortality rates of 10-25%.
This document provides an overview of vocal cord dysfunction (VCD), including its definition, presentation, potential etiologies, differential diagnosis, and treatment approaches. VCD involves adduction of the vocal cords during inhalation, exhalation, or both, resulting in respiratory symptoms. It is often misdiagnosed as asthma but requires a team-based diagnosis and treatment plan involving medical, speech therapy, and potentially psychiatric interventions.
Embryology cardiovascular system (heart development)MBBS IMS MSU
This document discusses the development of the cardiovascular system and heart in an embryo. It describes how cardiac progenitor cells migrate to form the cardiogenic field in the lateral plate mesoderm. Blood islands form and unite to create the primitive heart tube. As the embryo folds, the heart tube loops to the right and the atria shift to the left, forming the cardiac loop. The document outlines heart tube segmentation and discusses abnormalities that can occur during cardiac looping development.
Clubbing, also known as Hippocratic fingers, is a thickening of tissues at the base of fingernails and toenails such that the normal angle between the nail and digit is filled in. It is caused by increased levels of vascular endothelial growth factor (VEGF) which induces vascular changes and tissue proliferation in the nails. Common causes include lung diseases like bronchitis and cancer, as well as non-lung conditions such as liver cirrhosis. Clubbing can range from nail bed fluctuation to severe bony changes and is classified in grades based on physical appearance.
This document describes the anatomy of the lungs and pleura. It discusses the borders and lobes of the lungs, noting key landmarks like the apex and angles of Louis. It also details the fissures that divide the lungs into lobes, such as the oblique and transverse fissures. The document outlines the segmental bronchi and notes their anterior and posterior divisions. Furthermore, it describes the layers of the pleura, including the parietal and visceral pleura. Several important surface anatomy landmarks are indicated, such as Traube's area, Kronig's isthmus, and the bare area of the heart. In closing, the upper border of the liver is delineated starting at the 6th rib.
1) The lungs develop from the foregut endoderm and associated mesoderm. The endoderm forms the epithelial lining of the trachea, bronchi, and alveoli while the mesoderm forms the cartilage, muscle, and connective tissue.
2) During the 4th week, lung buds form as outgrowths of the foregut endoderm. Tracheo-esophageal ridges then fuse to separate the trachea from the esophagus. Incomplete fusion can lead to tracheo-esophageal fistulas or esophageal atresia.
3) Over successive developmental stages, the lungs continue to branch and the pleural membranes form, separating the pleural
The document discusses abnormalities that may appear on a chest x-ray and how to interpret them. It describes findings related to the lung parenchyma including consolidations, interstitial diseases, nodules, cavities, and calcifications. It also covers pleural abnormalities such as effusions, thickening, and plaques. Key signs are explained like air bronchograms, silhouetting, and Kerley lines which provide clues to diagnose conditions like pneumonia.
Flail chest is a serious chest wall injury involving fractures of three or more consecutive ribs in two places, creating a detached rib fragment. It occurs due to severe blunt trauma such as car accidents or falls. The broken rib segment moves in the opposite direction of the chest wall during breathing, making respiration difficult. Treatment involves stabilizing the chest, controlling pain, and assisting ventilation. Pneumonia and respiratory failure are complications, and mortality ranges from 10-25%.
Eisenmenger syndrome is a condition where a congenital heart defect causes pulmonary hypertension and a reversal of blood flow, leading to hypoxemia. It occurs when a longstanding left-to-right shunt causes irreversible pulmonary vascular remodeling and high pulmonary vascular resistance. Common defects that can cause Eisenmenger syndrome include ventricular septal defects, atrioventricular septal defects, and patent ductus arteriosus. Patients present with cyanosis, clubbing, and hypoxemia. Treatment focuses on managing symptoms and complications through lifestyle modifications and medications, as surgery to repair the underlying defect poses high risks. Prognosis depends on the severity of symptoms, but many patients can survive well into adulthood with proper care
Лекция. Абсцесс и гангрена легких. Abscess, lung gangrene англ..pptUpasana399630
This document discusses abscesses and gangrene of the lungs. It defines lung abscess as a pus-filled cavity in the lung bounded by granulation tissue, and gangrene of the lung as purulent-necrotic disintegration of lung tissue not separated by a capsule. Common causes are Staphylococcus and other bacteria. Abscesses form from acute infection or blood supply issues. Clinical presentation includes fever, cough, and deterioration. Treatment involves antibiotics, drainage, and sometimes surgery. Outcomes range from complete recovery to high mortality in gangrene.
This patient presented with a history of hemoptysis and a cough for one month. Investigations revealed a left upper lobe cavity with a homogenous opacity consistent with aspergilloma. The patient was diagnosed with an old treated pulmonary TB and a left upper lobe aspergilloma. Treatment included antifungal medication and bronchial arterial embolization. Aspergilloma is a rare condition where the fungus Aspergillus grows saprophytically in a pre-existing lung cavity, most commonly from prior TB.
The diaphragm develops from four sources in the embryo and matures to become a dome-shaped musculofibrous sheet that separates the thoracic and abdominal cavities. It has a central tendon to which muscular fibers attach and is the primary muscle of respiration. During inspiration, it contracts to flatten and lower, increasing the vertical space in the thorax. It has openings that allow structures like the esophagus, inferior vena cava and aorta to pass between the thorax and abdomen. The phrenic nerves provide motor innervation and allow the diaphragm to contract during breathing.
The document discusses patent ductus arteriosus (PDA), which is the failure of the ductus arteriosus to close after birth. It defines PDA, discusses its incidence, risk factors, embryology, fetal circulation, closure at birth, classification, natural history, clinical manifestations including signs and symptoms, investigations including echocardiogram and cardiac catheterization, and management including medical treatment with drugs and nonsurgical closure using devices. PDA is usually diagnosed using echocardiogram and can often be closed nonsurgically using devices like the Amplatzer duct occluder.
The document summarizes regulation of heart pumping and control of the heart rate. It discusses the Frank-Starling law where the volume of blood ejected depends on the volume present in the ventricle at the end of diastole. It also describes control of the heart by the autonomic nervous system, with the sympathetic nervous system increasing heart rate and contractility and the parasympathetic nervous system decreasing heart rate. The normal electrocardiogram waveform is also summarized.
- The document discusses pneumothorax, describing its classification, pathogenesis, clinical presentation, diagnosis, and treatment options.
- Key points include that tension pneumothorax can cause rapid deterioration and requires immediate treatment, while spontaneous pneumothorax is classified as primary or secondary depending on underlying lung health.
- Treatment involves observation for small primary pneumothoraces or procedures like aspiration, chest tube placement, or surgery depending on the size and symptoms.
This lecture covers diffusing capacity testing, specifically the single-breath carbon monoxide diffusing capacity (DLCO) test. DLCO measures the transfer of carbon monoxide across the alveolar-capillary membrane and is used to evaluate gas exchange ability. The single-breath method involves rapid inhalation of a test gas mixture containing carbon monoxide to total lung capacity, a 10 second breath hold, and analysis of exhaled gases. DLCO may be reduced in conditions involving decreased alveolar surface area or pulmonary capillary blood volume such as emphysema. Physiologic factors like hemoglobin, carboxyhemoglobin, and pulmonary blood volume also impact DLCO values.
Previous year question on pharyngeal arches embryology based on neet pg, usml...Medico Apps
Revision with a Master Quiz of 6 questions based on NEET PG Sample Questions on Pharyngeal Arches (Embryology) from Previous Year NEET PG Online Exams.
This document provides information on chest wall deformities, classifying them into four main types: sternum deformities, costal cartilage deformities, rib deformities, and combined costal cartilage and rib deformities. It describes specific conditions for each type, including cleft sternum, pectus excavatum, pectus carinatum, cervical ribs, and Poland Syndrome. Physiotherapy management is focused on postural correction, strengthening muscles, and improving mobility and lung function.
1. Pectus excavatum and pectus carinatum are the most common anterior chest wall deformities, with pectus excavatum occurring more frequently.
2. The Ravitch procedure and Nuss procedure are two common surgical techniques used to repair pectus excavatum, with Ravitch being an open resection and Nuss being minimally invasive.
3. Chest wall tumors can be either benign or malignant, with malignant tumors like fibrous histiocytoma and chondrosarcoma requiring wide resection for treatment.
A medical document discusses hypoxia following a road traffic accident. It describes four types of pulmonary contusion caused by direct chest compression, shearing against vertebral bodies, fractured ribs directly injuring the lung, or tearing of lung parenchyma due to prior adhesions. The document also mentions management of pulmonary contusion.
Cor pulmonale is a condition where the right ventricle of the heart enlarges and fails due to long-standing increased workload from diseases that affect the lungs like COPD. It is defined as hypertrophy and dilation of the right ventricle resulting from increased pulmonary vascular resistance. The main causes are chronic hypoxemia and pulmonary thromboembolism which lead to remodeling of the pulmonary arteries and increased pressure in the lungs. Over time, this puts strain on the right ventricle and can cause it to fail. Treatment focuses on reducing pulmonary pressures through oxygen therapy and vasodilators while managing symptoms of right heart failure.
1. Lung development begins in the fourth week of gestation and progresses through five stages - embryonic, pseudoglandular, canalicular, saccular, and alveolar.
2. Key molecular regulators of lung development include fibroblast growth factors, sonic hedgehog, retinoic acid, transforming growth factor beta, Wnts, platelet-derived growth factor and vascular endothelial growth factor.
3. Transcription factors such as NKX2-1, GLI genes, FOX family, GATA6 and SOX family also play important roles in lung development by regulating cell proliferation, branching morphogenesis and epithelial cell differentiation.
A flail chest occurs when a segment of the rib cage breaks and becomes detached from the rest of the chest wall, usually caused by two or more rib fractures in two or more places. Most commonly caused by vehicle accidents. During breathing, the detached flail segment moves in opposite direction to the rib cage, causing pain and paradoxical breathing. Management involves analgesia, intubation if needed, chest tube insertion, and physiotherapy. Outcomes range from full recovery to mortality rates of 10-25%.
This document provides an overview of vocal cord dysfunction (VCD), including its definition, presentation, potential etiologies, differential diagnosis, and treatment approaches. VCD involves adduction of the vocal cords during inhalation, exhalation, or both, resulting in respiratory symptoms. It is often misdiagnosed as asthma but requires a team-based diagnosis and treatment plan involving medical, speech therapy, and potentially psychiatric interventions.
Embryology cardiovascular system (heart development)MBBS IMS MSU
This document discusses the development of the cardiovascular system and heart in an embryo. It describes how cardiac progenitor cells migrate to form the cardiogenic field in the lateral plate mesoderm. Blood islands form and unite to create the primitive heart tube. As the embryo folds, the heart tube loops to the right and the atria shift to the left, forming the cardiac loop. The document outlines heart tube segmentation and discusses abnormalities that can occur during cardiac looping development.
Clubbing, also known as Hippocratic fingers, is a thickening of tissues at the base of fingernails and toenails such that the normal angle between the nail and digit is filled in. It is caused by increased levels of vascular endothelial growth factor (VEGF) which induces vascular changes and tissue proliferation in the nails. Common causes include lung diseases like bronchitis and cancer, as well as non-lung conditions such as liver cirrhosis. Clubbing can range from nail bed fluctuation to severe bony changes and is classified in grades based on physical appearance.
This document describes the anatomy of the lungs and pleura. It discusses the borders and lobes of the lungs, noting key landmarks like the apex and angles of Louis. It also details the fissures that divide the lungs into lobes, such as the oblique and transverse fissures. The document outlines the segmental bronchi and notes their anterior and posterior divisions. Furthermore, it describes the layers of the pleura, including the parietal and visceral pleura. Several important surface anatomy landmarks are indicated, such as Traube's area, Kronig's isthmus, and the bare area of the heart. In closing, the upper border of the liver is delineated starting at the 6th rib.
1) The lungs develop from the foregut endoderm and associated mesoderm. The endoderm forms the epithelial lining of the trachea, bronchi, and alveoli while the mesoderm forms the cartilage, muscle, and connective tissue.
2) During the 4th week, lung buds form as outgrowths of the foregut endoderm. Tracheo-esophageal ridges then fuse to separate the trachea from the esophagus. Incomplete fusion can lead to tracheo-esophageal fistulas or esophageal atresia.
3) Over successive developmental stages, the lungs continue to branch and the pleural membranes form, separating the pleural
The document discusses abnormalities that may appear on a chest x-ray and how to interpret them. It describes findings related to the lung parenchyma including consolidations, interstitial diseases, nodules, cavities, and calcifications. It also covers pleural abnormalities such as effusions, thickening, and plaques. Key signs are explained like air bronchograms, silhouetting, and Kerley lines which provide clues to diagnose conditions like pneumonia.
Flail chest is a serious chest wall injury involving fractures of three or more consecutive ribs in two places, creating a detached rib fragment. It occurs due to severe blunt trauma such as car accidents or falls. The broken rib segment moves in the opposite direction of the chest wall during breathing, making respiration difficult. Treatment involves stabilizing the chest, controlling pain, and assisting ventilation. Pneumonia and respiratory failure are complications, and mortality ranges from 10-25%.
Eisenmenger syndrome is a condition where a congenital heart defect causes pulmonary hypertension and a reversal of blood flow, leading to hypoxemia. It occurs when a longstanding left-to-right shunt causes irreversible pulmonary vascular remodeling and high pulmonary vascular resistance. Common defects that can cause Eisenmenger syndrome include ventricular septal defects, atrioventricular septal defects, and patent ductus arteriosus. Patients present with cyanosis, clubbing, and hypoxemia. Treatment focuses on managing symptoms and complications through lifestyle modifications and medications, as surgery to repair the underlying defect poses high risks. Prognosis depends on the severity of symptoms, but many patients can survive well into adulthood with proper care
Лекция. Абсцесс и гангрена легких. Abscess, lung gangrene англ..pptUpasana399630
This document discusses abscesses and gangrene of the lungs. It defines lung abscess as a pus-filled cavity in the lung bounded by granulation tissue, and gangrene of the lung as purulent-necrotic disintegration of lung tissue not separated by a capsule. Common causes are Staphylococcus and other bacteria. Abscesses form from acute infection or blood supply issues. Clinical presentation includes fever, cough, and deterioration. Treatment involves antibiotics, drainage, and sometimes surgery. Outcomes range from complete recovery to high mortality in gangrene.
This patient presented with a history of hemoptysis and a cough for one month. Investigations revealed a left upper lobe cavity with a homogenous opacity consistent with aspergilloma. The patient was diagnosed with an old treated pulmonary TB and a left upper lobe aspergilloma. Treatment included antifungal medication and bronchial arterial embolization. Aspergilloma is a rare condition where the fungus Aspergillus grows saprophytically in a pre-existing lung cavity, most commonly from prior TB.
The diaphragm develops from four sources in the embryo and matures to become a dome-shaped musculofibrous sheet that separates the thoracic and abdominal cavities. It has a central tendon to which muscular fibers attach and is the primary muscle of respiration. During inspiration, it contracts to flatten and lower, increasing the vertical space in the thorax. It has openings that allow structures like the esophagus, inferior vena cava and aorta to pass between the thorax and abdomen. The phrenic nerves provide motor innervation and allow the diaphragm to contract during breathing.
The document discusses patent ductus arteriosus (PDA), which is the failure of the ductus arteriosus to close after birth. It defines PDA, discusses its incidence, risk factors, embryology, fetal circulation, closure at birth, classification, natural history, clinical manifestations including signs and symptoms, investigations including echocardiogram and cardiac catheterization, and management including medical treatment with drugs and nonsurgical closure using devices. PDA is usually diagnosed using echocardiogram and can often be closed nonsurgically using devices like the Amplatzer duct occluder.
The document summarizes regulation of heart pumping and control of the heart rate. It discusses the Frank-Starling law where the volume of blood ejected depends on the volume present in the ventricle at the end of diastole. It also describes control of the heart by the autonomic nervous system, with the sympathetic nervous system increasing heart rate and contractility and the parasympathetic nervous system decreasing heart rate. The normal electrocardiogram waveform is also summarized.
- The document discusses pneumothorax, describing its classification, pathogenesis, clinical presentation, diagnosis, and treatment options.
- Key points include that tension pneumothorax can cause rapid deterioration and requires immediate treatment, while spontaneous pneumothorax is classified as primary or secondary depending on underlying lung health.
- Treatment involves observation for small primary pneumothoraces or procedures like aspiration, chest tube placement, or surgery depending on the size and symptoms.
This lecture covers diffusing capacity testing, specifically the single-breath carbon monoxide diffusing capacity (DLCO) test. DLCO measures the transfer of carbon monoxide across the alveolar-capillary membrane and is used to evaluate gas exchange ability. The single-breath method involves rapid inhalation of a test gas mixture containing carbon monoxide to total lung capacity, a 10 second breath hold, and analysis of exhaled gases. DLCO may be reduced in conditions involving decreased alveolar surface area or pulmonary capillary blood volume such as emphysema. Physiologic factors like hemoglobin, carboxyhemoglobin, and pulmonary blood volume also impact DLCO values.
Previous year question on pharyngeal arches embryology based on neet pg, usml...Medico Apps
Revision with a Master Quiz of 6 questions based on NEET PG Sample Questions on Pharyngeal Arches (Embryology) from Previous Year NEET PG Online Exams.
This document provides information on chest wall deformities, classifying them into four main types: sternum deformities, costal cartilage deformities, rib deformities, and combined costal cartilage and rib deformities. It describes specific conditions for each type, including cleft sternum, pectus excavatum, pectus carinatum, cervical ribs, and Poland Syndrome. Physiotherapy management is focused on postural correction, strengthening muscles, and improving mobility and lung function.
1. Pectus excavatum and pectus carinatum are the most common anterior chest wall deformities, with pectus excavatum occurring more frequently.
2. The Ravitch procedure and Nuss procedure are two common surgical techniques used to repair pectus excavatum, with Ravitch being an open resection and Nuss being minimally invasive.
3. Chest wall tumors can be either benign or malignant, with malignant tumors like fibrous histiocytoma and chondrosarcoma requiring wide resection for treatment.
Chest wall defects and their reconstructionVivek Gs
This document discusses chest wall anatomy, functions, and various defects that can occur. It covers the history of chest wall reconstruction and describes defects that can result from trauma, tumors, infections, radiation, and congenital causes. For each type of defect, the document outlines treatment approaches such as debridement, skeletal reconstruction, flap coverage, and correction of congenital defects. Key reconstruction methods mentioned include muscle flaps, omentum, methylmethacrylate sandwiched between mesh, and transposition of regional flaps.
This document discusses diaphragmatic procedures. It begins with an overview of diaphragmatic anatomy and physiology. The main procedures described for treating congenital diseases include repair of Bochdalek hernias, the most common form of congenital diaphragmatic hernia (CDH). Repair of Morgagni hernias is also covered. For acquired conditions, diaphragmatic plication is described to treat unilateral diaphragmatic paralysis by suturing the paralyzed hemidiaphragm. Overall causes and diagnosis of diaphragmatic paralysis are reviewed.
Chest wall-Congenital anomlies and tumors.pptxPradeep Pande
1. The document provides tips for using a PowerPoint presentation on congenital anomalies and tumors of the chest wall.
2. It instructs the user to show blank slides with titles and ask students what they know about the topic before providing information on the next slide.
3. This active learning approach of revisiting topics through three iterations is recommended to reinforce learning.
4. The rest of the document contains the PowerPoint presentation on congenital chest wall deformities, tumors, classifications, clinical features, investigations, and management.
1. The document discusses several chest wall deformities including pectus excavatum, pectus carinatum, Poland syndrome, Jeune syndrome, sternal defects, kyphosis, and scoliosis.
2. Pectus excavatum is a sunken chest where the breastbone caves in. Pectus carinatum involves overgrowth of cartilage causing a protruding or "pigeon chest" deformity.
3. Other conditions discussed include Poland syndrome which is underdevelopment of chest muscles on one side, Jeune syndrome causing a narrow chest cavity, and various sternal defects including ectopia cordis.
The document discusses epiphyseal injuries around the hip, specifically slipped capital femoral epiphysis (SCFE). SCFE involves slippage of the femoral head from the neck due to weakness of the growth plate. It most commonly occurs in obese adolescents during periods of rapid growth. Presentation includes pain and limping. Imaging shows displacement of the femoral head. Treatment aims to reduce displacement and stabilize the growth plate to allow healing. Complications include avascular necrosis, premature growth plate closure, and arthritis.
Sprengel's deformity and congenital muscular torticollis are congenital musculoskeletal conditions. Sprengel's deformity involves an elevated scapula due to interrupted migration during development. It causes limited shoulder movement and deformity. Congenital muscular torticollis is caused by sternocleidomastoid muscle shortening, resulting in head tilt and facial asymmetry. Both are typically noticed at birth and can be treated conservatively or with surgery depending on severity. Physiotherapy focuses on improving range of motion and muscle strength.
The document discusses the anatomy and clinical presentation of appendicitis. It describes the location and blood supply of the appendix, noting that it can be in different positions which can affect the pain experienced. It also summarizes that the pain of appendicitis initially occurs centrally in the abdomen but becomes localized as inflammation spreads, and that variations in anatomy can cause atypical presentations of the condition.
Arnold Chiari malformation is a group of congenital anomalies affecting the posterior fossa. There are four types of Chiari malformation classified based on the degree and contents of herniation through the foramen magnum. Type I involves herniation of the cerebellar tonsils, while Type II involves herniation of the cerebellar tonsils and contents of the posterior fossa into the foramen magnum. Posterior fossa decompression is the main surgical treatment, involving enlarging the posterior fossa volume and establishing CSF flow, to relieve compression of neural structures. Complications can include CSF leaks, recurrent syringomyelia, and persistent neurological deficits.
Presentation1.pptx, radiological imaging of spinal dysraphism.Abdellah Nazeer
This document discusses radiological imaging in spinal dysraphism. It describes how various imaging modalities such as radiography, ultrasound, CT, and MRI can be used to identify and characterize different types of spinal dysraphism including open dysraphism, closed dysraphism, lipomyelomeningocele, diastematomyelia, dermal sinus, and tethered cord. Examples of imaging findings for each condition are provided with representative images to illustrate the pathology.
This document discusses various types of birth injuries in infants including injuries to the head, spine, shoulders, nerves and internal organs. It provides information on the causes, clinical presentations, diagnostic methods and treatment approaches for different birth injuries such as subgaleal hematoma, clavicle fractures, brachial plexus injuries, spinal cord injuries and liver lacerations. Risk factors for birth injuries including prolonged labor, large infant size and instrument-assisted delivery are also mentioned.
In many fetal skeletal dysplasias ,the skin and s/c tissue continues to grow at a rate proportionately greater than the long bones resulting in relatively thickened skin folds (on occasion mistaken for hydrops fetalis ) .
Polyhydraminos –common .cause –variable combination of the following –oesophageal compression by the small chest ,GI abnormalities ,micrognathia ,or hypotonia .
This document provides information on Legg Calve Perthes disease, including:
- A brief history of its discovery and description by Legg, Calve, and Perthes.
- Its definition as osteonecrosis of the femoral epiphysis in children caused by non-genetic factors.
- Presentation, diagnosis using imaging like x-rays and MRI, and classifications of severity.
- Management involves containment of the femoral head through bracing, casting, or surgery depending on the stage and prognosis. The goal is to prevent secondary arthritis by achieving a spherical femoral head.
1. Pectus excavatum (PE), or funnel chest, is the most common chest wall deformity, affecting approximately 1 in 300 births. It involves a sunken or indented sternum.
2. The Nuss procedure, introduced in 1998, is a minimally invasive surgical technique to repair PE using a curved metal bar placed underneath the sternum through small incisions. It aims to correct the deformity without removing cartilage or making large incisions.
3. Studies have found good cosmetic outcomes and high patient satisfaction following both open repair techniques and the Nuss procedure. However, the Nuss procedure has a higher risk of bar displacement requiring reoperation compared to open techniques. Long-term data on
Prenatal diagnosis of congenital anomalies 3DrAhmed Badr
This document discusses various fetal anomalies that can be detected on prenatal ultrasound scans, including definitions, prevalence, diagnostic criteria, and recommendations. It covers anomalies such as thickened nuchal fold, echogenic bowel, cardiac defects, gastroschisis, exomphalos, single umbilical artery, and hydrops fetalis. Guidelines are provided on the standard assessment of fetal anatomy and soft markers for aneuploidy during the 16-20 week anatomy scan.
- Congenital diaphragmatic hernia (CDH) and diaphragmatic eventration (DE) are congenital diaphragmatic malformations that result from abnormal development of the diaphragm. DE involves an abnormally thin diaphragm while CDH is a defect or abnormal attachment.
- For newborns with CDH, the current standard of care is delayed surgical repair after stabilization along with therapies like nitric oxide and ECMO. Surgery is rarely needed for asymptomatic DE in children.
- In adults, an elevated diaphragm is attributed to a congenital cause only after ruling out other etiologies, and surgery may be indicated for symptomatic DE or when it
This document discusses various types of ventral hernias. It defines ventral hernias as hernias that occur in the abdominal wall. It then provides details on the anatomy of the abdominal wall and the pathophysiology of hernia formation. Several specific types of ventral hernias are described including epigastric, umbilical, incisional, lumbar, and parastomal hernias. For each type, the document outlines characteristics, clinical presentation, complications, and treatment approaches. Surgical repair is generally recommended, and mesh placement is often used to reinforce repairs.
A 3-year-old girl presented with a limp in her right leg and was diagnosed with coxa vara. Coxa vara is a decrease in the angle between the femoral neck and shaft to less than 120 degrees. It can be congenital, developmental, or secondary to other bone diseases. Surgical management involves subtrochanteric osteotomy to correct the angle, which is fixed internally with plates or screws. Postoperative care includes casting for 8-12 weeks until radiographic healing is seen. Complications can include recurrence, physeal injury, or leg length discrepancy.
Storyboard on Acne-Innovative Learning-M. pharm. (2nd sem.) CosmeticsMuskanShingari
Acne is a common skin condition that occurs when hair follicles become clogged with oil and dead skin cells. It typically manifests as pimples, blackheads, or whiteheads, often on the face, chest, shoulders, or back. Acne can range from mild to severe and may cause emotional distress and scarring in some cases.
**Causes:**
1. **Excess Oil Production:** Hormonal changes during adolescence or certain times in adulthood can increase sebum (oil) production, leading to clogged pores.
2. **Clogged Pores:** When dead skin cells and oil block hair follicles, bacteria (usually Propionibacterium acnes) can thrive, causing inflammation and acne lesions.
3. **Hormonal Factors:** Fluctuations in hormone levels, such as during puberty, menstrual cycles, pregnancy, or certain medical conditions, can contribute to acne.
4. **Genetics:** A family history of acne can increase the likelihood of developing the condition.
**Types of Acne:**
- **Whiteheads:** Closed plugged pores.
- **Blackheads:** Open plugged pores with a dark surface.
- **Papules:** Small red, tender bumps.
- **Pustules:** Pimples with pus at their tips.
- **Nodules:** Large, solid, painful lumps beneath the surface.
- **Cysts:** Painful, pus-filled lumps beneath the surface that can cause scarring.
**Treatment:**
Treatment depends on the severity and type of acne but may include:
- **Topical Treatments:** Such as benzoyl peroxide, salicylic acid, or retinoids to reduce bacteria and unclog pores.
- **Oral Medications:** Antibiotics or oral contraceptives for hormonal acne.
- **Procedures:** Such as chemical peels, extraction of comedones, or light therapy for more severe cases.
**Prevention and Management:**
- **Cleanse:** Regularly wash skin with a gentle cleanser.
- **Moisturize:** Use non-comedogenic moisturizers to keep skin hydrated without clogging pores.
- **Avoid Irritants:** Such as harsh cosmetics or excessive scrubbing.
- **Sun Protection:** Use sunscreen to prevent exacerbation of acne scars and inflammation.
Acne treatment can take time, and consistency in skincare routines and treatments is crucial. Consulting a dermatologist can help tailor a treatment plan that suits individual needs and reduces the risk of scarring or long-term skin damage.
Storyboard on Skin- Innovative Learning (M-pharm) 2nd sem. (Cosmetics)MuskanShingari
Skin is the largest organ of the human body, serving crucial functions that include protection, sensation, regulation, and synthesis. Structurally, it consists of three main layers: the epidermis, dermis, and hypodermis (subcutaneous layer).
1. **Epidermis**: The outermost layer primarily composed of epithelial cells called keratinocytes. It provides a protective barrier against environmental factors, pathogens, and UV radiation.
2. **Dermis**: Located beneath the epidermis, the dermis contains connective tissue, blood vessels, hair follicles, and sweat glands. It plays a vital role in supporting and nourishing the epidermis, regulating body temperature, and housing sensory receptors for touch, pressure, temperature, and pain.
3. **Hypodermis**: Also known as the subcutaneous layer, it consists of fat and connective tissue that anchors the skin to underlying structures like muscles and bones. It provides insulation, cushioning, and energy storage.
Skin performs essential functions such as regulating body temperature through sweat production and blood flow control, synthesizing vitamin D when exposed to sunlight, and serving as a sensory interface with the external environment.
Maintaining skin health is crucial for overall well-being, involving proper hygiene, hydration, protection from sun exposure, and avoiding harmful substances. Skin conditions and diseases range from minor irritations to chronic disorders, emphasizing the importance of regular care and medical attention when needed.
A congenital heart defect is a problem with the structure of the heart that a child is born with.
Some congenital heart defects in children are simple and don't need treatment. Others are more complex. The child may need several surgeries done over a period of several years.
The Children are very vulnerable to get affected with respiratory disease.
In our country, the respiratory Disease conditions are consider as major cause for mortality and Morbidity in Child.
Applications of NMR in Protein Structure Prediction.pptxAnagha R Anil
This presentation explores the pivotal role of Nuclear Magnetic Resonance (NMR) spectroscopy in predicting protein structures. It delves into the methodologies, advancements, and applications of NMR in determining the three-dimensional configurations of proteins, which is crucial for understanding their function and interactions.
Dr. Tan's Balance Method.pdf (From Academy of Oriental Medicine at Austin)GeorgeKieling1
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Organization
Academy of Oriental Medicine at Austin
Academy of Oriental Medicine at Austin
Academy of Oriental Medicine at Austin
About AOMA: The Academy of Oriental Medicine at Austin offers a masters-level graduate program in acupuncture and Oriental medicine, preparing its students for careers as skilled, professional practitioners. AOMA is known for its internationally recognized faculty, award-winning student clinical internship program, and herbal medicine program. Since its founding in 1993, AOMA has grown rapidly in size and reputation, drawing students from around the nation and faculty from around the world. AOMA also conducts more than 20,000 patient visits annually in its student and professional clinics. AOMA collaborates with Western healthcare institutions including the Seton Family of Hospitals, and gives back to the community through partnerships with nonprofit organizations and by providing free and reduced price treatments to people who cannot afford them. The Academy of Oriental Medicine at Austin is located at 2700 West Anderson Lane. AOMA also serves patients and retail customers at its south Austin location, 4701 West Gate Blvd. For more information see www.aoma.edu or call 512-492-303434.
The biomechanics of running involves the study of the mechanical principles underlying running movements. It includes the analysis of the running gait cycle, which consists of the stance phase (foot contact to push-off) and the swing phase (foot lift-off to next contact). Key aspects include kinematics (joint angles and movements, stride length and frequency) and kinetics (forces involved in running, including ground reaction and muscle forces). Understanding these factors helps in improving running performance, optimizing technique, and preventing injuries.
Osvaldo Bernardo Muchanga-GASTROINTESTINAL INFECTIONS AND GASTRITIS-2024.pdfOsvaldo Bernardo Muchanga
GASTROINTESTINAL INFECTIONS AND GASTRITIS
Osvaldo Bernardo Muchanga
Gastrointestinal Infections
GASTROINTESTINAL INFECTIONS result from the ingestion of pathogens that cause infections at the level of this tract, generally being transmitted by food, water and hands contaminated by microorganisms such as E. coli, Salmonella, Shigella, Vibrio cholerae, Campylobacter, Staphylococcus, Rotavirus among others that are generally contained in feces, thus configuring a FECAL-ORAL type of transmission.
Among the factors that lead to the occurrence of gastrointestinal infections are the hygienic and sanitary deficiencies that characterize our markets and other places where raw or cooked food is sold, poor environmental sanitation in communities, deficiencies in water treatment (or in the process of its plumbing), risky hygienic-sanitary habits (not washing hands after major and/or minor needs), among others.
These are generally consequences (signs and symptoms) resulting from gastrointestinal infections: diarrhea, vomiting, fever and malaise, among others.
The treatment consists of replacing lost liquids and electrolytes (drinking drinking water and other recommended liquids, including consumption of juicy fruits such as papayas, apples, pears, among others that contain water in their composition).
To prevent this, it is necessary to promote health education, improve the hygienic-sanitary conditions of markets and communities in general as a way of promoting, preserving and prolonging PUBLIC HEALTH.
Gastritis and Gastric Health
Gastric Health is one of the most relevant concerns in human health, with gastrointestinal infections being among the main illnesses that affect humans.
Among gastric problems, we have GASTRITIS AND GASTRIC ULCERS as the main public health problems. Gastritis and gastric ulcers normally result from inflammation and corrosion of the walls of the stomach (gastric mucosa) and are generally associated (caused) by the bacterium Helicobacter pylor, which, according to the literature, this bacterium settles on these walls (of the stomach) and starts to release urease that ends up altering the normal pH of the stomach (acid), which leads to inflammation and corrosion of the mucous membranes and consequent gastritis or ulcers, respectively.
In addition to bacterial infections, gastritis and gastric ulcers are associated with several factors, with emphasis on prolonged fasting, chemical substances including drugs, alcohol, foods with strong seasonings including chilli, which ends up causing inflammation of the stomach walls and/or corrosion. of the same, resulting in the appearance of wounds and consequent gastritis or ulcers, respectively.
Among patients with gastritis and/or ulcers, one of the dilemmas is associated with the foods to consume in order to minimize the sensation of pain and discomfort.
- Video recording of this lecture in English language: https://youtu.be/RvdYsTzgQq8
- Video recording of this lecture in Arabic language: https://youtu.be/ECILGWtgZko
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
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2. CAUSES
Pectus Excavatum
Pectus Carinatum
Poland syndrome.
Sternal defect.
Ectopia cordis and pentalogy of
Cantrell.
Jeune’s syndrome / Asphyxiating
thoracic dystrophy.
Rib cage overgrowth
Responsible for 90 % of
defects
Inadequate growth
(Aplasia and hypoplasia)
3. PECTUS EXCAVATUM
Funnel chest, is the most common
congenital abnormality of the chest wall.
It is characterized by variable
depression of the sternum and lower
costal cartilages.
Pectus anomalies can be evident early
in life and are 3X more common in
boys.
Its association with scoliosis and Marfan
syndrome suggests a possible link with
connective tissue disorders. Studies
show a pattern of inheritance as high as
4. CLINICAL PRESENTATION AND INVESTIGATIONS
C/O: Asymptomatic in most cases but limited physical activity in severe
depression.
Examination: Asymmetrical sternal depression starting from the second
costal cartilage. Associated heart defects in 1.5 % and Marfan syndrome
in 15 % of cases.
Investigations
CT scan and Haller index (Transverse Ǿ / Antro-posterior Ǿ ). 3.25 is
indication for surgery.
Echocardiography.
Pulmonary function test.
5. TREATMENT
Indications:
Symptoms of limited activity, psychological depression.
Restrictive lung disease as measured by pulmonary function tests.
Cardiac pressure, pulmonary atelectasis and Haller BT index that is > 3.25.
Cardiac malformations (mitral valve prolapse or the existence of
arrhythmia).
Recurrence after previous corrective surgery.
Appropriate timing:
Puberty
5 – 20 years
Adult has less malleable chest wall and hence Nuss procedure is
contraindicated.
6. TYPES OF CORRECTIVE INTERVENTION
TRANSVERSE STERNAL OSTEOTOMY WITH
SUBPERICHONDRIAL COSTAL CARTILAGE RESECTION
(MODIFIED RAVITCH PROCEDURE).
MINIMALLY INVASIVE REPAIR (NUSS PROCEDURE).
MAGNETIC MINI MOVER PROCEDURE.
THE VACUUM BELL.
7. MODIFIED RAVITCH PROCEDURE
A transverse incision is made in the anterior chest below the nipple line corresponding with
the future inframammary fold.
Skin flaps are then elevated to expose the body of the sternum and affected costal
cartilages. Up to the level of sterno-manubrial joint.
After optimal exposure, the pectoralis muscles are dissected off the cartilages and
elevated bilaterally to the level of the costochondral joints.
The perichondrium corresponding with the affected costal cartilages is opened sharply.
The cartilages of the 3rd, 4th, and 5th ribs are exposed, grasped, and divided sharply from
their insertion into the sternum. Laterally, a 1-cm rim of cartilage is left in continuation to
the adjacent rib for further growth plate development.
The next step is the correction of the sternal angulation, which is achieved by performing a
transverse osteotomy and by removing a wedge of bone at the anterior surface of the
sternum. This is then re-approximated with heavy sutures
A closed drainage system is left in place, the pectoral muscle flaps are re-approximated in
the midline, and the skin is closed in layers.
8. MINIMAL INVASIVE NUSS PROCEDURE
Minimal invasive procedure utilizing
videoscope, a pectus bar removed after 2-3
years.
The main complication after Ravitch is
recurrence, the Nuss procedure is more
painful, wound infection, pneumothorax and
overcorrection are among the complications.
9. PECTUS CARINATUM
Is the second most common congenital abnormality
of the chest wall. More common in males (4:1). The
defect is usually identified in the early years of
puberty.
The body of the sternum and adjacent costal
cartilages protrude, creating a defect similar to a
pigeon's breast.
Although most defects are symmetrical, they can
also be asymmetric and combined with pectus
excavatum.
According to a classification based on the salient
part of the sternum, two variants can be identified:
chondrogladiolar and chondromanubrial.
Asymptomatic but in severe form pain on lying
10. TREATMENT
NON OPERATIVE OPERATIVE
A brace that provides
anteroposterior compression
results in progressive remodeling
of the chest.
The brace must be worn 14 hours
a day for at least 2 years.
Surgical correction is often
reserved for those who fail
conservative management or in
noncompliant patient
Modified Ravitch with
multiple ostiotomies in
combinded anomalies.
Minimal invasive
subcutaneous C shaped
bar.
11. POLAND’S SYNDROME
Poland's syndrome is a rare congenital anomaly in which
there are varying degrees of hypoplasia or aplasia of the
thoracic wall components.
It occurs in ~1 in 30,000 live births and is rarely bilateral.
The only consistent anomaly in this syndrome is the
underdevelopment or absence of the pectoralis muscles.
Associated anomalies of the rib cage are present in up to
60% of cases, including hypoplasia or aplasia of the ribs and
their cartilages.
Other deformities include amastia, athelia, bradydactyly or
syndactyly, and occasionally hypoplasia of the subclavian
vessels.
12. CLINICAL PRESENTATION AND TREATMENT
Hypoplasia of the pectoral muscles rarely causes functional limitation in the ipsilateral
extremity, perhaps due to compensation by other muscle groups.
Costochondral defects and absence of ribs can result in disfiguring anomalies. In
addition, women may have amastia, athelia, or ectopia of the nipple.
The surgical treatment of Poland's syndrome is largely cosmetic and is
individualized according to the anomalies present.
The concavity of the chest wall at the site of the defective pectoral muscle can be
filled with muscle flaps or implants.
In women with amastia, reconstruction should be performed when full development of
the normal contralateral breast is completed.
Techniques for repairing the costochondral anomalies in Poland's syndrome are
adaptations to the Ravitch technique. When multiple ribs are hypoplastic or absent,
contralateral costal grafts can be used.
Wedge osteotomies are performed as needed to correct the abnormal curvature or
rotations in the sternum.
13. STERNAL DEFECTS
Sternal defects are divided into four
categories based on tissue coverage of the
heart:
(1) Sternal cleft or bifid sternum,
(2) Thoracic ectopia cordis,
(3) Cervical ectopia cordis, and
(4) Thoracoabdominal ectopia cordis
14. TYPES OF STERNAL CLEFT
Sternal clefts/bifid sternums are
malformations that occur as the sternal
elements fail to fuse.
Sternal clefts are classified as complete or
partial, with partial defects being either
superior or inferior.
Superior defects are the most common and
are usually not associated with other
anomalies.
Sternal clefts may be
“U”-shaped (proximal to the 4th cartilage)
or “V”-shaped (reaching the xiphoid
process).
Inferior sternal clefts are usually associated
15. CLINICAL PRESENTATION AND INVESTIGATIONS
Patients with sternal clefts may present as a spectrum from
asymptomatic to recurrent respiratory infections and may
have a dramatic appearance when the child cries or coughs.
Surgery is advocated in even asymptomatic patients to
protect the heart, relief parental anxiety, and decrease
respiratory infections.
Perioperative evaluation should include an echocardiogram,
chest radiographs, and 3D CT scan to completely evaluate
the bony abnormality.
If there are concerns for vascular anomalies, then a contrast
CT scan is justified.
16. TREATMENT
Primary repair is performed at 3 months
of age before the thoracic cage
becomes rigid. Neonatal repair has also
been advocated.
Approximation can be achieved by
removing an inferior wedge of cartilage,
or converting a partial sternal defect to
a complete.
If approximation is not possible then
chondrotomies and clavicular fracture
lateral to the protuberant medial third
has been advocated.
If decompensation occurs, the
remaining options include autologous
tissue flaps, myocutaneous flaps, or
prosthetic materials.
17. ECTOPIA CORDIS AND PENTALOGY OF CANTRELL
Cantrell, Haller, and Ravitch in 1958 described a syndrome consisting of five
embryologic defects:
(1) Ectopia cordis,
(2) Deficiency of the anterior diaphragm,
(3) Supraumbilical abdominal wall defect,
(4) Defect in lower sternum, and
(5) Congenital intracardiac anomalies.
Other anomalies such as cleft palate and clubfeet were also described with
the syndrome.
18. CLINICAL PRESENTATION
More than half the cases of Cantrell's pentalogy present with the
complete syndrome.
Ectopia cordis is present in 63% of cases and is called cervical, thoracic,
or thoracoabdominal depending on the area of cardiac evisceration.
The most common intracardiac anomalies associated with the
syndrome include (VSD) , (ASD) and tetralogy of Fallot.
The diagnosis can be made in the first trimester of pregnancy by
ultrasonography.
Recently, MRI has been used to delineate the anatomic details of the
pentalogy.
19. TREATMENT
When the diagnosis is made early in pregnancy, the physician should
counsel the parents about the high mortality associated with the
syndrome. Termination of pregnancy may be an option.
There are several methods for correcting the different components of the
syndrome. The main objective is to cover the eviscerated heart with
skin, muscle flap, or synthetic material, without returning the heart to the
thoracic cavity . This is because these patients usually have reduced
thoracic capacity.
In a later surgery, the osseous defect should be corrected. With the
advent of pediatric cardiovascular surgery, the mortality of patients with
intracardiac defects has been reduced substantially.
Patients with the worst prognosis are those who present with complex
heart defects and those with cervical or thoracic ectopia cordis.
20. JEUNE’S SYNDROME / ASPHYXIATING
THORACIC DYSTROPHY
A rare autosomal recessive disorder. It was first described
by Jeune in 1955 in a pair of siblings with severely narrow
thoraces.
A genetic locus has been identified on chromosome 15q13
as well as in the IFT80 gene encoding intraflagellar proteins
in a subset of patients.
Prenatal diagnosis are polyhydramnios, abnormal small
thorax, short limbs and unidentifiable fetal respiratory
movements.
21. PRESENTATION
Clinical manifestations of this syndrome
may include dwarfism with short ribs,
short limbs, fixed elevated clavicles,
and a high incidence of C-1 stenosis.
The clinical spectrum runs from a mild
form where the chest may support
adequate respirations to a severe form
where the chest is narrowed in both
transverse and vertical axis, with
horizontal ribs and irregular
costochondral junctions.
In the severe form, the rigidity of the
chest does not allow chest wall
expansion leading to ventilator
dependence and respiratory failure.
22. TREATMENT
The main goal of expanding the thoracic
volume leading to improved lung expansion.
Multiple procedures are described that
involve a median sternotomy with placement
of graft material to expand the thoracic cavity.
Staged procedures, Davis and colleagues
have described a lateral thoracic expansion
technique where the ribs and underlying
tissue are divided in a staggered fashion and
fixed with titanium plates to permit chest
expansion in a gradual manner.
Vertical expandable titanium rib (VEPTR)
procedure has been described by Campbell
and colleagues in patients with Jeune's
syndrome as a technique to improve chest
wall movement.
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