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DIAPHRAGM AND HIATUS HERNIA Anatomy of diaphragm Diaphragm Diaphragmatic communications Physiology Normal anatomy of LES Normal anatomy of LES Normal anatomy of LES Hernia-peritoneum diverticulum Clasification Congenital Accuired Congenital hernia Congenital diaphragmatic hernia Incidence 1 : 2000-5000 live birth 8 % of all major congenital anomalies mortality rate nearing 70 percent CDH accounts > 1% of total infant mortality in USA Cost per new case CDH = 250 000 $ Diaphragm Development Causes The cause of CDH is largely unknown CDH can occur as part of a multiple malformation syndrome Karyotype abnormalities have been reported in 4% of infants with CDH Congenital Diaphragmatic Hernias (CDH) Types of Congenital Diaphragmatic Hernias (CDH) – – – – Bochdalek Morgagni Diaphragmatic eventration Central tendon defects Bochdalek Hernia Postero-lateral diaphragmatic hernia Most common manifestation of CDH, accounting for more than 95% of cases Majority of Bochdalek hernias (80-85%) occur on the left side of the diaphragm – A failure of the diaphragm to completely close during development. – Herniation of the abdominal contents into the chest – Pulmonary hypoplasia Morgagni Hernia anterior defect of the diaphragm referred to as Morgagni’s, retrosternal, or parasternal hernia accounts for approximately 2% of all CDH cases characterized by herniation through the foramina of Morgagni which are located immediately adjacent to the xyphoid process of the sternum majority occur on the right side of the body and are generally asymptomatic Diaphragmatic eventration abnormal displacement (i.e. elevation) of part or all of an otherwise intact diaphragm into the chest cavity diaphragm is thinner in the region of eventration, allowing the abdominal viscera to protrude upwards thinning is thought to occur because of incomplete muscularisation of the diaphragm Minor forms of diaphragm eventration are asymptomatic Congenital Diaphragmatic Hernias (CDH) Left sided CDH is a 2 - 4 cm posterolateral defect Right lobe of liver can occupy most of hemithorax in rt side defect Hepatic veins may drain ectopically into right atrium Lung and liver may be fused Prenatal Diagnosis ultrasonography diagnosis (as early as the second trimester) Mediastinal shunt Viscera herniation (stomach, intestines, liver*, kidneys, spleen and gall bladder) Abnormal position of certain viscera inside the abdomen Stomach visualization out of its usual position Intrauterine growth retardation* Polyhydramnios* Fetal hydrops* * bad prognosis Fetal diafragmatic hernia: Ultrasound diagnosis Prenatal MR Imaging - single-shot turbo spinecho (HASTE)- of congenital diaphragmatic hernia Prenatal MR Imaging of congenital diaphragmatic hernia Pulmonary hypoplasia Anatomopathology show of CDH Prenatal Counseling multidisciplinary team patient's obstetrician perinatologist geneticist surgeon social worker Prenatal management Glucocorticoids Thyrotropin-releasing hormone Fetal surgical therapy (Antenatal surgical intervention, In utero tracheal occlusion ) Delivery Room Management affected infants should be delivered in a specialized center require positive pressure ventilation in the delivery room. to prevent distension of the gastrointestinal tract and further compression of the pulmonary parenchyma, a double-lumen nasogastric or orogastric tube of large caliber is placed to act as a vent. early intubation Postnanal Diagnosis Respiratory distress Scaphoid abdomen Auscultation of the lungs reveals poor air entry Shift of the heart to the side opposite Postnanal Diagnosis left-sided CDH Radiograph in a male neonate shows the tip (large arrow) of the nasogastric tube positioned in the left hemithorax. Note the marked apex leftward angulation of the umbilical venous catheter (small arrow). Right congenital diaphragmatic hernia Radiograph in a male neonate shows that the nasogastric tube (arrow) deviates to the left of the thoracic vertebral bodies as it passes through the inferior portion of the thorax Postnatal management Mechanical ventilation Nitric Oxide Surfactant Surgery Operative approach The defect in the diaphragm Patch repair of a large defect Evolving Therapies In utero repair Liquid ventilation Pulmonary transplantation Pharmacology – Prostacyclin derivatives – Calcium channel blockers – Phosphodiesterase inhibitors Prognosis Pulmonary recovery: When all resources, are provided, survival rates range from 40-69%. Long-term morbidity: Significant long-term morbidity, including chronic lung disease, growth failure, gastroesophageal reflux, and neurodevelopmental delay, may occur in survivors. ADULT DIAPHRAGMATIC HERNIA Classification ?Asymptomatic congenital diaphragmatic hernia Posttraumatic or postoperative Hiatus hernia Posttraumatic hernia Symptoms Uncomplicated: – Similar woth GERD – Respiratory symptoms – Cardiac arrhythmia, ischemic heart disease\ Complications: – Strangulation: acute respiratory and digestive symptoms, very difficult to assess on clinical examination Diagnostic Plain thoracic X-Ray Nasogastric tube + X-ray Barium or Gastrographin studies if nonemergency CT-scan Treatment Approach: – Laparotomy vs laparoscopy – Thoracotomy vs thoracoscopy – Urgent vs chronic disease Reintegration of viscus Resection of peritoneal sac Close the defect in diaphragm – Suturing – Mesh HIATAL HERNIA Hiatal Hernia Defined (Also called Diaphragmatic Hernias) Protrusion of the stomach upward into the mediastinal cavity through the esophageal hiatus of the diaphragm – Sliding • 90% of cases – Rolling (paraesophageal) Sliding Hiatal Hernia The esophagus passes through the diaphragm and connects to the stomach. When a sliding hiatal hernia is present, part of the stomach moves up through an opening (hiatus) in the diaphragm. The presence of a hiatal hernia increases the risk for gastroesophageal reflux Paraesophageal Hiatal Hernia The fundus and possibly portions of the stomach’s greater curvature, rolls through the esophageal hiatus and into the thorax beside the esophagus A Comparison of the normal stomach, sliding hiatal hernia and rolling hiatal hernia Diagnostic Tools Barium Swallow CXR Endoscopy with biopsy Stool for quiac Esophageal manometry Diagnostic Tools Key Features of Hernias Sliding hiatal hernia – Heartburn – Regurgitation – Chest pain – Dysphagia – Belching Paraesophageal hernia – Feeling of fullness and breathlessness after eating – Feeling of suffocation – Cheat pain that mimics angina – Symptoms worse in recumbent position Symptoms Complications – Slow bleed – Anemia – Pulmonary Aspiration Risk Factors Increased intraabdominal pressure – – – – – Obesity Pregnancy Bending Coughing Weight lifting Age Medical Treatment Goals – Aimed at relieving symptoms and prevent complications • Bleeding – Reduce regurgitation of stomach contents into esophagus • Medications – – – Includes antacids and histamine receptor antagonists (Pepcid and Reglan) Neutralizes stomach acidity Decrease acid production Surgical Intervention Used when medical therapy fails to control symptoms Surgery is extensive and produces frequent complications Hiatal hernia tends to recur after surgery – Laparoscopic Nissen Fundoplication Postoperative Care Risk for bleeding, infection and organ injury Respiratory Care NG tube Management Nutritional Care Results Complications Temporary dysphagia Gas bloat syndrome (avoid carbonated beverages) Atelectasis, pneumonia Obstructed NG tub Reccurrent GERDe RARE: – Mediastinitis – Fistula Complications