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Educational Session: Newborn Emergencies Maureen D. McCollough, MD, MPH, FACEP 3/24/2010 11:00 AM - 12:00 PM Maureen McCollough MD, FACEP, FAAEM Associate Professor of Pediatrics and Emergency Medicine Keck USC School of Medicine Medical Director, Department of Emergency Medicine Los Angeles County+USC Medical Center NIGHTMARE NEONATE Term infant, usually good APGAR score, discharged home from the hospital, who returns back to the ED in extremis : S - SEPSIS S - SEIZURES I - INBORN ERRORS OF METABOLISM C - CARDIAC - Ductal dependent lesions and Left-to-Right shunts C - CONGENITAL ADRENAL HYPERPLASIA C - CNS DISASTERS - AVM’s, Child abuse, Vitamin K deficiency F - FORMULA MIXUPS I- INTESTINAL DISASTERS - volvulus, hernias, necrotizing enterocolitis T - TOXINS FEVER or SEPSIS Age at which fever means automatic admission has dropped; Admit infant less than 4 weeks old with a fever (temp > 38.0 rectally) Late onset (>1 week old) more likely to have meningitis so tap all neonates Sepsis can present subtly - abdominal distention, tachypnea, “not doing well” May not be febrile; may be hypothermic Viral infections are most common - enteroviruses; Bacterial includes E. coli, Strep Group B, Listeria Sepsis workup includes CBC, blood culture, urinalysis and culture (may have neg urinalysis but positive culture), and lumbar puncture CBC may show neutropenia or low platelets as signs of sepsis Urine - catheterize girls Suprapubic or catheterize boys Chest Xray necessary if infant has signs of lower respiratory tract disease (tachypnea, cough, retractions, grunting, nasal flaring or hypoxia) Lumbar puncture - dangerous for ill neonate to be curled up; place on a pulse ox; reportedly less dangerous sitting up; don’t cover the infant during the LP; 22 gauge with a stylet to avoid epitheliomas Up to one month - WBC - 7-22 / hp; Protein - up to 150 Antibiotics are Ampicillin and Gentamicin or Cefotaxime and Ampicillin If suspicious for herpes (vesicles, CSF shows WBC’s or high protein but negative gram stain, seizures, maternal history of herpes, or any “ill” appearing neonate), then add Acyclovir 10 mg/kg per dose q 8hrs IV SEIZURES Present more subtly than in older infants or adults: tonic seizure or migratory slow clonic movements or subtleties like lip smacking, eye deviation, bicycling or apnea Generalized tonic/clonic rare due to neurons not interacting well yet Differential diagnosis includes: CNS - child abuse, Vitamin K deficiency, hypoxic encephalopathy Infection - meningitis (bacterial or herpes) Metabolic - hypoglycemia, hypocalcemia, hypo or hypernatremia, Inborn Errors, pyridoxine deficiency Drug withdrawal from maternal opiate abuse Toxins like bilirubin Family seizures - Benign familial and 5th day fits Jitteriness is all limbs, short rapid movements, can be induced, stops with restraint of limb, gaze is normal, rarely has autonomic signs History - ask about birth trauma, infection, drug abuse, family history of neonatal seizures, formulas and home remedies Physical exam - bruising, cranial bruit for AVM, cafe-au-lait spots, jaundice, herpes vesicles Neuro exam for cranial nerves, motor exam, neonatal reflexes Treat like regular seizures with benzodiazepines, phenobarbitol, Dilantin - don’t wait for the seizure to stop on its own Be ready with airway equipment !! Check glucose and calcium right away even if seizures are stopped Glucose D10 5-10cc/kg IV (0.5-1.0 g/kg) Calcium gluconate 10% 100-300mg/kg IV (1-3ml/kg at 1ml/min) - weak cry, sounds like a bleating lamb, and extreme jitteriness are signs of hypocalcemia Consider Pyridoxine 50-100 mg IV if unresponsive to traditional therapy Workup will be dictated by the history and physical exam; admit for further workup and observation INBORN ERRORS Hereditary biochemical disorder that results in the accumulation of a metabolite that is toxic at high levels Typical metabolites are ammonia, lactic acid and ketones and usual target organ is the central nervous system Categories include urea cycle defect, organic acidemias, aminoacidopathies or galactosemia Family may have a history of previous child dying in infancy Presents usually in the neonatal period with altered mental status, lethargy, vomiting, dehydration, hypoglycemia, seizures Physical exam could reveal hepatomegaly or odor Labs show elevated levels of metabolite; may not be acidotic Ammonia - best if drawn arterially, green top tube, place on ice Lactic acid level - if acidotic Urine may show spindle shaped orotic acid crystals if urea cycle defect exists Attempt to get red and green top tubes drawn before hydration and glucose; BUT RESUSCITATION ALWAYS COMES FIRST !! Treatment includes rehydration and glucose for hypoglycemia and to decrease production and enhance elimination of the toxic metabolite HCO3 may increase ammonia passage into the CNS Continue with D 10 1/4NS at 1.5-2 times maintenance rate CARDIAC Two categories - Ductal dependent lesions and Left-to-right shunts Ductal dependent congenital heart disease: Ductus arteriosus connects pulmonary artery to aorta; shunts fetal blood away from lungs; closes first few days after birth Ductus needed to remain open to supply blood to either the lungs or to the systemic circulation Ductus closes usually in the first day of life but can be delayed several days Ductal dependent neonate gets into trouble when the ductus closes Usually presents in the first week of life with sudden change in color or respiratory rate Lungs dependent Defect is usually a right-to-left intracardiac shunt like: Tetralogy of Fallot, Tricuspid atresia, Truncus Arteriosus, Pulmonary stenosis Ductus closes ----> neonate presents cyanotic because of decreased blood flow to lungs Oxygen will not correct the hypoxia Systemic circulation dependent Defect is usually a severe obstruction to flow to the systemic circulation like: Coarctation of the aorta, severe Aortic stenosis, Hypoplastic left heart Ductus closes ----> neonate presents hypotensive or with poor circulation to lower extremities because of decreased blood flow to the systemic circulation Exam includes blood pressure and palpation of pulses in all extremities; O2 saturation will be decreased in the lower extremities Treatment includes Airway, Breathing, Circulation Prostaglandin E1 is a potent vasodilator, especially to ductal tissue; start at 0.05-0.1mcg/kg/min IV infusion; side effects include hypotension, apnea Dopamine and Dobutamine sometimes needed for better cardiac output Left-to-right intracardiac shunts present with congestive heart failure Usually presents at end of first month or during first few months of life Example is a large ventricular septal defect Normally, pulmonary vascular pressures drop during first months of life ---> any pre-existing left-to-right shunt, like a VSD, will worsen as these pulmonary pressures decrease ---> more and more blood flows to the lungs, putting the child into congestive heart failure Neonates present with slower onset of symptoms: difficulty feeding, sweating or tiring during feeds, poor weight gain, tachypnea Exam will show tachypnea, ?rales, enlarged liver; neonates do not have pitting edema Treatment includes admission and diuresis CONGENITAL ADRENAL HYPERPLASIA Adrenal insufficiency resulting from deficient enzyme needed to produce cortisol Results in deficiency of cortisol and aldosterone, and accumulation of androgens Clinically results in adrenal hyperplasia, salt wasting, dehydration, hyponatremia, hyperkalemia, azotemia, acidosis, and sometimes cardiovascular collapse Females have enlarged clitoris and fused labia; males have hyperpigmented scrotums Attempt to get red and green top tubes drawn before Hydrocortisone given; BUT RESUSCITATION ALWAYS COMES FIRST !! Treatment includes hydration and Hydrocortisone 25mg IV and admission Hyperkalemia is usually well tolerated, but treat if arrhythmias are present CNS DISASTERS Child Abuse Vitamin K deficiency – Vit K not given at birth; CNS, skin, GI bleeding Cerebral AVM INTESTINAL DISASTERS: Volvulus, Incarcerated Hernia, Diaphragmatic Hernia, Necrotizing Enterocolitis VOLVULUS Congenital malrotation of the midgut portion of the intestine - during the 5 - 8th week of embryonic life, the intestine projects out of the abdominal cavity, rotates 270 degrees and returns into the abdomen; if the rotation is not right, the intestine will not be “fixed down” correctly at the mesentery ------> at risk for volvulus Volvulus is the twisting of a loop of bowel about its mesenteric base attachment True medical emergency because necrotic bowel can occur within hours of onset of the twisting Presents one of three ways: - sudden onset of bilious vomiting and abdominal pain - history of “feeding problems” with bilious vomiting that now appears like a bowel obstruction - failure to thrive with severe feeding intolerance (least common) BILIOUS (green) VOMITING IN NEONATES IS ALWAYS WORRISOME AND IS A TRUE EMERGENCY !! abdomen may or may not be distended depending upon location of the volvulus; if obstruction is high, abdomen may not be distended; abdomen may be “blue” if bowel is already ischemic / necrotic Pain is a constant pain, not intermittent; neonate may be jaundiced Hematochezia is a late, BAD sign ! Labs - nothing classic except dehydration and acidosis Abdominal plain film - classic “double bubble sign” - paucity of gas (airless abdomen) with two air bubbles - one in the stomach and one in the duodenum - plain film can also be entirely normal Upper GI - considered the gold standard - small intestine is rotated to right side of the abdomen; contrast narrows at site of obstruction “cork-screwing”; spiraling of small bowel about the superior mesenteric artery Need to diagnose this life threatening process EMERGENTLY !!! Re-hydrate the infant aggressively; place an NG tube Antibiotics: Ampicillin, Clindamycin and Gentamicin When the diagnosis is being considered, contact the Pediatric Surgeon on-call immediately; the sooner the child gets to the OR, the lower the morbidity and mortality - some peds surgeons will take an ill appearing neonate with BILIOUS vomiting to the OR directly without any additional diagnostic tests FORMULA MIXUPS and TOXINS Mixing formula incorrectly Any other liquids, powders or herbs given for a variety of reasons – colic, spitting up, vomiting, constipation, diarrhea