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FUNCTIONAL AND ORGANIC DISEASES OF THE OESOPHAGUS AND STOMACH IN CHILDREN PART I LABARAN KAMAL UMAR (MED) INTRODUCTION The esophagus is the hollow tube that leads from the throat (pharynx) to the stomach. The walls of the esophagus propel food to the stomach not by gravity but by rhythmic waves of muscular contractions called peristalsis. FUNCTIONS OF THE OESOPHAGUS As a person swallows, food moves from the mouth to the throat, also called the pharynx (1). The upper esophageal sphincter opens (2) so that food can enter the esophagus, where waves of muscular contractions, called peristalsis, propel the food downward (3). The food then passes through the lower esophageal sphincter (4) and moves into the stomach (5). Just below the junction of the throat and the esophagus is a band of muscle called the upper esophageal sphincter. Slightly above the junction of the esophagus and the stomach is another band of muscle called the lower esophageal sphincter. When the esophagus is not in use, these sphincters contract so that food and stomach acid do not flow back up the esophagus from the stomach to the mouth. During swallowing, the sphincters relax so food can pass to the stomach. GASTROESOPHAGEAL REFLUX DISEASE- GERD Gastroesophageal reflux is the backward movement of food and acid from the stomach into the esophagus and sometimes into the mouth . Reflux may be caused by the infant’s position during feeding; overfeeding; exposure to caffeine, nicotine, and cigarette smoke; a food intolerance or allergy; or an abnormality of the digestive tract. Infants may vomit, spit up excessively, have feeding or breathing problems, and also appear irritable. Tests that help doctors diagnose the disorder include a barium study, an esophageal pH probe, a gastric emptying scan, and endoscopy. Treatment options include thickened or hypoallergenic formula for feedings, special positioning, frequent burping, histamine-2 blockers, proton pump inhibitors, and, in certain cases, metoclopramide and surgery. Nearly all infants have episodes of gastroesophageal reflux, which are characterized by wet burps, burping up, or spitting up. Wet burps typically occur shortly after eating and are considered normal. Gastroesophageal reflux becomes known as gastroesophageal reflux disease (GERD) when it Interferes with feeding and growth Damages the esophagus (esophagitis) Leads to breathing difficulties (such as coughing, wheezing, or stopping breathing) Continues beyond infancy into childhood CAUSES Healthy infants have reflux for many reasons. The circular band of muscle at the junction of the esophagus and stomach (the lower esophageal sphincter) normally keeps stomach contents from entering the esophagus. In infants, this muscle may be underdeveloped, or it may relax at inappropriate times, allowing stomach contents to move backward (reflux) into the esophagus. Being held flat during feeding or lying down after feeding promotes reflux because gravity is no longer able to help keep material in the stomach from flowing back up the esophagus. Overfeeding and drinking carbonated beverages predispose to reflux by increasing pressure in the stomach. Cigarette smoke (as secondhand smoke) and caffeine (in beverages or breast milk) relax the lower esophageal sphincter, allowing reflux to occur more readily. Caffeine and nicotine (in breast milk) also stimulate acid production so any reflux that does occur is more acidic. A food allergy or intolerance also can contribute to reflux, but this is a less common cause. Anatomic abnormalities, such as narrowing of the esophagus, partial blocking of the stomach (pyloric stenosis), or abnormal positioning of the intestines (malrotation), can initially mimic reflux. However, these abnormalities are more serious and can progress to vomiting and other symptoms of obstruction, such as abdominal pain, listlessness, and dehydration. SYMPTOMS The most obvious symptoms of gastroesophageal reflux in infants are vomiting and excessive spitting up. Reflux typically worsens in the first several months of life, peaks around 6 to 7 months of age, and then gradually lessens. Nearly all infants with reflux outgrow it by about 18 months of age. In some infants, reflux causes complications and becomes known as GERD. Such complications include irritability due to stomach discomfort, feeding problems that can result in poor growth, and “spells” of twisting and posturing that may be confused with seizures. Less commonly, small amounts of acid from the stomach may enter the windpipe (aspiration). Acid in the windpipe and breathing passages may result in coughing, wheezing, stopping breathing (apnea), or pneumonia. Many children with asthma also have reflux. Ear pain, hoarseness, hiccups, and sinusitis also can occur as a result of GERD. If the esophagus is significantly irritated (esophagitis), there may be some bleeding, resulting in iron deficiency anemia. In others, esophagitis can cause scar tissue, which can narrow the esophagus (stricture). Heartburn, a common symptom among adolescents and adults with GERD, is more commonly expressed as chest pain or abdominal pain among young children. DIAGNOSIS Tests are often not needed to diagnose gastroesophageal reflux in infants who simply have mild symptoms such as frequent spit-ups. However, if symptoms are more complicated, various tests can be performed. A barium test is the most common test. The child swallows barium, a liquid that outlines the digestive tract when x-rays are taken. Although this test can help the doctor diagnose gastroesophageal reflux, it more importantly helps the doctor identify some of the possible causes of the reflux. An esophageal pH probe is a thin flexible tube with a sensor at the tip that measures the degree of acidity (pH). Doctors pass the tube through the child’s nose, down the throat, and into the end of the esophagus. The tube is usually left in place for 24 hours. Normally, children do not have acid in their esophagus, so if the sensor detects acid, it is a sign of reflux. Doctors sometimes use this test to see whether children with symptoms such as coughing or breathing difficulties have reflux. In a gastric emptying scan (milk scan), the child drinks a beverage that contains a small amount of mildly radioactive material. This material is harmless to the child. A special camera or scanner that is highly sensitive to radiation can detect where the material is in the child’s body. The camera can see how rapidly the material leaves the stomach and whether there is reflux, aspiration, or both. In upper endoscopy the child is sedated, and a small flexible tube with a camera on the end (endoscope) is passed through the mouth into the esophagus and stomach. Doctors may perform upper endoscopy if they need to see whether the reflux has caused an ulcer or irritation or if they need to obtain a sample for a biopsy. Endoscopy can also help make sure the symptoms of reflux are not due to something else such as an allergy, infection, or celiac disease. Bronchoscopy is a similar test in which doctors use an endoscope to examine the voice box (larynx) and airways. Bronchoscopy can help doctors decide whether reflux is a likely cause of lung or breathing problems. Treatment Treatment of reflux depends on the child’s age and symptoms. For infants who just have wet burps, doctors may recommend no treatment or may suggest measures such as thickening formula for feedings, special positioning, and frequent burping. Formula can be thickened by adding 1 to 3 teaspoons of rice cereal per ounce of formula. The nipple may have to be cross-cut to allow the formula to flow. Infants with reflux should be fed in an upright or semi-upright position and then maintained in an upright position for 30 minutes after eating. Infants with a food intolerance or allergy may benefit from a hypoallergenic formula. The head of the bed can be raised 6 inches (about 15¼ centimeters) to help reduce nighttime reflux. Infants should be secured in a sling fitted over the mattress or wedge to keep them from rolling or sliding down to a horizontal position on the lower end of the crib. Older children also should avoid eating 2 to 3 hours before bedtime, drinking carbonated beverages or those that contain caffeine, taking certain drugs (such as those with anticholinergic effects), eating certain foods (such as chocolate), and overeating. All children should be kept away from tobacco smoke. DRUGS If changes in feeding and positioning do not control symptoms, doctors may prescribe drugs. Several types of drugs are available for reflux: Those that neutralize acid Those that suppress acid production Those that improve the movement of the digestive tract Antacids are drugs that neutralize gastric acid. These drugs work quickly to relieve symptoms such as heartburn. For children with more severe disease, acid-suppressing drugs are required. By reducing stomach acid, these drugs lessen symptoms and allow the esophagus to heal. There are two types of acid-suppressing drugs, histamine-2 (H2) blockers and proton pump inhibitors (PPIs). H2 blockers do not suppress acid production quite as much as PPIs. Promotility drugs stimulate the movement of contents through the esophagus, stomach, and intestines. These drugs (such as metoclopramide) may help increase the strength of the lower esophageal sphincter and increase the speed at which the stomach empties. Improved gastric emptying should decrease gastric pressure, making reflux less likely to occur. Doctors used to prescribe these drugs frequently for reflux but now think they are helpful only for certain children. Surgery Rarely, reflux does not respond to nonsurgical treatment and is so severe that doctors recommend surgery. The most common surgical procedure is a fundoplication. In fundoplication, the surgeon wraps the top of the stomach around the lower end of the esophagus to make that junction tighter and decrease reflux DIAPHRAGMATIC HERNIA Hernia is a protrusion of a piece of the intestine through an abnormal opening. Some infants are born with a diaphragmatic hernia . A diaphragmatic hernia is a hole or weakening in the diaphragm (the muscle that separates the chest from the abdomen and that helps in breathing). This opening allows some of the small intestine to push through the opening, creating a bulge. Sometimes the intestine becomes trapped (incarcerated) in the opening. Sometimes incarceration cuts off the blood supply to the trapped intestine (strangulation), which can lead to a tear (perforation) and peritonitis (inflammation and usually infection of the abdominal cavity and its lining), creating a surgical emergency. A large diaphragmatic hernia can decrease lung volume and create breathing problems. Doctors do a surgical procedure to correct this type of hernia. A diaphragmatic hernia that bulges through the opening that the esophagus normally passes through (the hiatus) is called a hiatus hernia What are he different types of congenital diaphragmatic hernia? There are two kinds of CDH: A Bochdalek hernia is a hole in the back of the diaphragm. Ninety percent of CDHs are this type. A Morgagni hernia involves a hole in the front of the diaphragm. SYMPTOMS •Respiratory distress •Scaphoid abdomen •Bowel sounds heard over the hemithorax( gurgle- like noises) •Absence of breath sounds •Difficulty breathing •Fast breathing •Fast heart rate •Cyanosis (blue color of the skin) •Abnormal chest development, with one side being larger than the other DIAGNOSIS/TREATMENT X-ray: Chest protruding into the chest Ultrasound, prenatally Treatment is by surgery You are called to a delivery room after the birth of a full- term infant who has developed respiratory distress. The mother has been in excellent health and the pregnancy was uncomplicated. An ultra-sound at 16 weeks was unremarkable. Physical examination reveals a near total absence of breath sounds bilaterally. No fluid is obtained by suctioning. You immediately intubate the infant and obtain a chest radiograph, which reveals a cystic mass in the right chest that looks similar to bowel loops. Which of the following conditions is the most likely diagnose? A. Tracheosophageal fistula B. Respiratory distress syndrome C.Meconium aspiration D. Tetralogy of Fallot E. Diaphragmatic hernia END OF PART I