Neonatal Diseases MODULE E Objectives • Identify the key pathophysiologic changes that occur with each disease. • Describe the therapeutic intervention needed to treat each of the diseases. Perinatal Diseases and Other Problems with Prematurity •Retinopathy of prematurity (ROP) •Patent Ductus Arteriosus •Hypoglycemia •Cold Stress •Intraventricular & Intracerebral hemorrhaging •Bronchopulmonary dysplasia •Wilson Mikity Syndrome •Apnea of prematurity •Necrotizing enterocolitis •RDS Retinopathy of Prematurity (ROP) • Formerly known as Retrolental Fibroplasia (RLF). • Initially described in 1940/1950s following increased incidence of blindness with babies in incubators. • Incidence today: • 25 to 35% of preemies up to 35 weeks Physiology of the Developing Eye • Capillaries of retina begin branching at 16 weeks. • End of pseudoglandular period. • Capillaries begin at optic nerve and grow anteriorly toward the ora serrata which is the anterior end of the retina. • Growth is not complete until 40 weeks. • Premature infants don’t have complete growth. • As the capillary network expands, arteries and veins form in its path. • ROP is the failure of this network to develop. Oxygen and ROP • In the presence of high PaO2, the retinal vessels constrict. • Prolonged exposure to high PaO2 will lead to necrosis of the vessels (vasoobliteration). • The body attempts to correct for this by over perfusing the “good” arteries, which leads to hemorrhage in the vitreous. • This hemorrhage leads to scar tissue development and blindness. Stages and Zones of ROP • 5 stages, with 5 having the retina completely detached. • Three Zones of the eye (zone 1 is the worst) RDS - Respiratory Distress Syndrome • aka: IRDS or Hyaline Membrane Disease • Associated with lung immaturity and a deficiency in surfactant production. • Immaturity of other organ systems. • Decreased Compliance & increased WOB. • Severe hypoxemia may result in multiple organ failure. • May be associated with PPHN (PFC) or PDA. RDS - Respiratory Distress Syndrome • Symptoms worsen for first 48-72 hours. • Stabilization • Slow recovery • With progression of the disease, scar tissue replaces the normal alveolar tissue. • Hyaline Membrane Clinical Signs • • • • • • • • History of prematurity f above 60/min Grunting Retractions Flaring of nostrils Cyanosis Severe hypoxemia on blood gases Hypothermia & flaccid muscle tone X-ray Findings • Diffuse “White-out” (Radiopaque) • Atelectasis • Air bronchograms • Reticulogranular Pattern • “Fishing net” • Ground Glass Appearance Treatment • Attempt to accelerate lung maturity by pharmacological means. • Steroids • Tocolysis: Delay labor with bAdrenergic Agents • (Terbutaline) • Thermoregulation Treatment • Artificial Surfactant • CPAP or mechanical ventilation • High Frequency Ventilation • ECMO Recovery Phase • Complications • ROP • Bronchopulmonary dysplasia • Chronic lung disease (COPD for Neonates) • Intraventricular hemorrhage • Brain dysfunction • Necrotizing Enterocolitis • Intrapulmonary Hemorrhage • Full Recovery Bronchopulmonary Dysplasia • Other Name • Neonatal Chronic Lung Disease (NCLD) • Progressive chronic lung disease that presents with persistent respiratory problems at 28 days or later, radiographic changes and oxygen dependency Bronchopulmonary Dysplasia • Criteria • Preterm infants • Prolonged oxygen concentrations (O2 toxicity) • Positive pressure ventilation (barotrauma) • Patent ductus arteriosus (PDA) • Time exposure to oxygen and positive pressure • Malnutrition Bronchopulmonary Dysplasia • Not all babies with RDS develop BPD. • Pattern begins to unfold within the first 3-4 days of life that places a neonate at high risk of developing BPD. Bronchopulmonary Dysplasia • Lung Pathology • Mucosal hyperplasia of small airways. • Destruction of type I cells. • Inflammation and destruction of alveoli and capillary bed. • Lungs are cystic in some areas and atelectatic in others. Chest X-Ray • Radiology • • • • “Honeycomb” appearance Diaphragms are flattened Cystic appear (hyperlucent) Atelectasis (radiopaque) HMD to BPD – 3 Hour HMD to BPD – Day 13 HMD to BPD – Day 19 HMD to BPD – 3 Months Clinical Presentation •Tachypnea •Retractions •Mucous plugging •Hyperinflation of chest – barrel chest •Cyanotic spells •Poor ABG •Wheezing •Inadequate growth •Increased WOB •Increased oxygen consumption •Pulmonary hypertension and Cor Pulmonale Goals of Bronchopulmonary Dysplasia • Prevention of BPD. • Provide enough calories to support growth. • Wean slowly off oxygen. • Limit peak inspiratory pressures on ventilator. • CPAP or HFV • Keep FiO2 levels as low as possible. • May need to keep PaO2 levels lower. Complications of Bronchopulmonary Dysplasia • Gastroesophageal reflux and feeding intolerance leads to aspiration. • Decreased Ca and phosphorus (bone fractures. • Loss sight or hearing (ROP). • Chronic infections. • Pneumothorax. • Cerebral palsy. • Limit Fluid intake – develop pulmonary edema. Bronchopulmonary Dysplasia • Death is usually due to: • Cor Pulmonale • Infection • Sudden Death Discharge of patients with BPD • Home Care • Oxygen & CPT • Mechanical ventilators • Medications • Diuretics or cardiac meds • Special Attention to nutritional needs • Frequent re-admissions back into the hospital. Necrotizing Enterocolitis (NEC) • Injury to the intestinal mucosa due to hypoperfusion, hypoxia or hyperosmolar feedings. • The mucosa cannot secrete the protective layer of mucus and it becomes vulnerable to bacterial invasion. • Intestinal ischemia may result in necrosis and gangrene of the intestine. • Complication of RDS. • Highest incidence in lowest birth weight infants. Necrotizing Enterocolitis (NEC) • Intestinal dilation (distended loops of intestine with gas). • Gastric ileus (obstruction) • Abdominal distention. • Rectal bleeding • Bloody stool • Feeding is difficult. Treatment • • • • • Stop feedings. Nasogastric Suctioning Hyperalimentation IV. Antibiotics. 20% require surgery. Intraventricular Hemorrhage (IVH) • Premature infants and low birth weight infants are the greatest risk. • Diagnosed by ultrasound or CT scan. • Seen with increased incidence in children of alcoholic mothers. • 4 grades of IVH. • Grade 1 - Bleeding occurs just in a small area of the ventricles. • Grade 2 - Bleeding also occurs inside the ventricles. • Grade 3 - Ventricles are enlarged by the blood. • Grade 4 - Bleeding into the brain tissues around the ventricles. Etiology And History of IVH Grades of IVH IVH Treatment • Prevent Occurrence • Supportive Wilson-Mikity Syndrome • Seen in premature and LBW infants. • Less than 1500 grams at birth. • “Emphysema” of little babies. • Lung immaturity with rupture of the alveolar septa. • Similar to BPD except babies have not been ventilated. • Treatment is supportive. • Oxygen and mechanical ventilation. • Some question as to whether it is a separate syndrome or not. Meconium Aspiration • Disease of term or post term neonates. • Asphyxia occurs before, during or after the onset of labor. • Relaxation of the anal sphincter with release of the meconium (first stool). • Treatment is immediate suctioning & antibiotics. • Intubate with endotracheal tube and with a meconium aspirator. Meconium Aspiration • Usually associated with PFC and infection. • Pneumothorax may result from the hyperinflation. • An emergency tension pneumothorax is treated with a needle aspiration followed by chest tube insertion. Ball-Valve Effect Transient Tachypnea of the Newborn (TTN) • RDS type II. • Occurs in term or near term infants born by cesarean section. • Caused by the retention of lung fluid following birth. • Baby is born with respiratory distress and rapid f (80 – 100/min or higher). • Evaporation of lung fluid. Transient Tachypnea of the Newborn • X-ray findings are similar for RDS, TTN, and pneumonia. • Pleural effusions may be present. • May be started on broad spectrum antibiotics. • Lung maturity is found. • Usually good APGAR scores. • Frequent turning is helpful to eliminate lung fluid. Transient Tachypnea of the Newborn • ABG show oxygenation problem. • Ventilation is usually normal. • If ventilation is started, the baby will wean quickly. • Process of elimination. Tracheoesophageal Fistula or Atresia • • • • Fistula is an abnormal communication between two passages or cavities. Atresia is the absence or closure of a normal body orifice or tubular passage. TEF is a congenital abnormality resulting in respiratory distress. Most common type is an upper esophageal atresia and a lower tracheal-esophageal fistula. Diagnosis • The nurse/physician will try to pass a catheter into the stomach. • Bronchoscopy or ultrasound is used to diagnose. • May be seen on chest-x-ray. Clinical Manifestations • Constant pooling of oral, nasal and pharyngeal secretions/drooling. • Continuous or sporadic respiratory distress. • Choking on feedings. • Repeated vomiting with or after feedings. • Persistent upper lobe pneumonia or atelectasis due to aspiration. • Gastric distention. Treatment of TEF • Surgical correction is needed. • Supportive care until surgery. • Aspiration is a major concern. • A gastric feeding tube is usually placed in the esophageal pouch to remove secretions. • Keep in 30 degree upright position. • Infant is fed with a gastrostomy tube until surgery. Choanal Atresia • A congenital malformation of bone or a membrane causing partial or complete obstruction of one or both of the choana. • The obstruction results in asphyxia since infants are nose breathers early in life. • Respiratory Distress subsides when the baby cries. Diagnosis • A catheter or probe fails to pass through the infant’s nose. Often the nose has a large accumulation of thick secretions. • If the obstruction is a membrane, it may be punctured to provide relief of the respiratory distress. Clinical Manifestations • Clinical Signs • • • • Respiratory distress Cyanosis Retractions Pooling of nasal secretions Treatment • Treatment • Insertion of an oral airway to facilitate mouth breathing. • If distress continues, then intubate and ventilate. Diaphragmatic Hernia • CDH is a congenital condition in which the abdominal organs herniate into the chest cavity through the diaphragm. • Life threatening condition. • Lung tissue is compressed. Diaphragmatic Hernia • Most common defect is in the posterolateral region of the diaphragm in an area called the foramen of Bochdalek. • Left side herniation is more frequent (8590%). • Stomach, spleen & intestines can enter the chest. • Scaphoid (boat shaped) Abdomen is present. Diaphragmatic Hernia • The baby will be in respiratory distress at birth. • PMI may be shifted. • Breath sounds diminished. • Bowel sounds can be heard over lung fields. • Confirmed with chest x-ray. • Lungs are hypoplasitc (underdeveloped). Treatment of Diaphragmatic Hernias • Orogastric tube is inserted to remove air. • Do not manually ventilate these infants. • Overdistension of stomach will worsen problem. • Intubate to prevent air in the stomach and intestines. • High Frequency Ventilation, ECMO • High mortality rate. • Pneumothorax is common. Treatment of Diaphragmatic Hernias •Prenatal ultrasound can accurately diagnose a CDH in utero (in utero repair has been successfully accomplished)!! Persistent Pulmonary Hypertension of the Newborn (PPHN) • Formerly Persistent Fetal Circulation (PFC) • Pulmonary hypertension after birth caused by asphyxia and which prevents the transition of fetal to newborn circulation. • It may be a primary disorder or a secondary disorder: • • • • • • RDS TTN Pneumonia Cold Stress Meconium aspiration Diaphragmatic hernia Persistent Pulmonary Hypertension of the Newborn (PPHN) • Blood is shunted Right to Left across the ductus arteriosus. • The Apgar is usually 5 or less at 1 and 5 minutes. Signs and Symptoms • • • • Tachypnea Retractions Cyanosis Breath sounds are clear if no pulmonary disease is present. • Refractory to oxygen therapy (true shunt). • Difference in pre & post ductal blood gases. Diagnostic Testing • Hyperoxia Test • If PaO2 does not increase with 100% oxygen, suspect a cardiac shunt • Not specific for PFC • Compare preductal and postductal PaO2 • If shunt is present Preductal > Postductal. • 15 to 20 mm Hg and with FiO2 • Hyperoxia-Hyperventilation Test • Most definitive. • Hyperventilate until PaCO2 is 20 – 25 mm Hg • Alkalosis will reduce pulmonary hypertension and PaO2 will improve. • Echocardiography – ultrasound of the heart • Cardiac Catheterization Treatment for PPHN • Oxygen therapy to maintain PaO2 greater than 50 – 60 mm Hg. • Mechanical ventilation. • Nitric Oxide • ECMO, HFV • Keep glucose and electrolytes normal. Pneumothorax • • • • • • Cyanosis Tachypnea Grunting Nasal flaring PMI is shifted Diminished or absent breath sounds Confirmation of a Pneumothorax • Transillumination • Bed Side • Chest x-ray Treatment of Pneumothorax • Emergency treatment . • Needle Aspiration • 2nd intercostal space • Chest Tube. • Given the baby 100% oxygen until chest tube is inserted. Infections • Pneumonia – infection in the lungs. • Septicemia – infection in the bloodstream. • Meningitis – infection/inflammation of the covering of the brain and spinal cord. • Urinary Tract Infections • Conjunctivitis – infection or inflammation of the eye. • Omphalitis – infection/inflammation of the umbilical stump. Pneumonia • Transplacental • Acquired at birth • Amniotic fluid. • Premature rupture of membranes greater than 12-24 hours (PROM). • Postnatal • Invasive lines. • Respiratory equipment. • Hospital Personnel. Pneumonia • Premature infants are at greater risk. • Group B Beta Hemolytic Streptococci & Escherichia Coli are the most common organisms. • PFC is usually a consequence of pneumonia. Diagnosis of Pneumonia • Chest x-ray • Very difficult to distinguish between Pneumonia, RDS & TTN. • Culture and Sensitivity. Postnatally Acquired Pneumonia • Klebsiella • Pseudomonas • Methicillin-Resistant Staphylococcus (MRSA) • Resistant to penicillin type drugs. • Candida Albicans (fungal). Viruses that affect the Newborns • • • • • • Herpes Virus Respiratory Syncytial Virus (RSV) Rubella Adenovirus Cytomegalovirus Chlamydia