Pediatric and Newborn Conditions Study Guide

Heart Malformations

• Heart malformations and defects can occur when the heart doesn't develop correctly during fetal development.

• These defects may not be apparent until one to two weeks after birth due to fetal circulation.

Fetal Circulation

• In fetal circulation, there are bypasses in the heart and lungs because the baby receives oxygen and waste removal from the mother.

• After birth, these shunts should close within one to two weeks, allowing blood to flow through the heart and lungs.

• If a baby has a normal heart, these changes occur without problems.

• Babies with heart defects may initially survive due to fetal circulation, but their condition can worsen as the shunts close.

Critical Congenital Heart Disease (CCHD) Screening

• Approximately 18 out of 10,000 babies are born with critical congenital heart disease.

• A screening test has been developed to detect heart defects in newborns.

• Before this test, many babies with heart defects were not diagnosed and some died after being sent home.

• The screening test involves checking oxygen saturation in the extremities.

• If circulation is normal, oxygen saturation levels should be the same in all extremities.

• A discrepancy in oxygen saturation levels may indicate a heart issue.

• The test is verified three times, and if the baby doesn't pass, further testing is done.

Risk Factors for Heart Issues

• Having only two vessels in the umbilical cord (instead of three) can be a risk factor for cardiac anomalies.

• Genetic factors and maternal conditions can also contribute to heart issues.

• Babies with Down syndrome often have heart anomalies.

• Genetic syndromes like Marfan and Turner syndrome can also have cardiac involvement.

• Fetal alcohol syndrome, rubella infections, and maternal diabetes can increase the risk of heart malformations.

• Exposure to X-rays and certain medications during pregnancy can also cause cardiac anomalies. e.g. Valchloric acid, ralentine, tetracycline, DES, lithium, Bold salt, Plumidin, Accutane and Valium

Signs and Symptoms of Cardiac Issues

• Cyanosis (bluish skin) during crying can indicate a heart problem.

• Respiratory distress includes nasal flaring, grunting, and sternal retractions.

• Tachypnea (rapid breathing) and unequal chest expansion may indicate a diaphragmatic hernia.

• Congestive heart failure can cause tachycardia, a galloping rhythm, diminished pulses, diaphoresis (sweating), edema, and an enlarged liver.

Specific Heart Defects

Transposition of the Great Arteries

• The aorta arises from the right ventricle instead of the left ventricle, and the pulmonary arteries arise from the left ventricle instead of the right.

• This causes unoxygenated blood to be pumped to the body and oxygenated blood to be pumped back to the lungs.

• Surgical correction is needed to redirect blood flow.

Coarctation of the Aorta

• The aorta is narrowed, restricting blood flow to the body.

• This can lead to backup of blood and damage to the heart and lungs.

• Surgical repair is necessary to widen the aorta.

Atrial Septal Defect (ASD)

• A hole between the two atria (upper chambers) of the heart.

• Leads to mixing of oxygenated and unoxygenated blood.

• The screening test can pick up on this because of different oxygen saturations in the extremities.

Ventricular Septal Defect (VSD)

• A hole between the two ventricles (lower chambers) of the heart.

• Oxygenated blood goes into that left ventricle and gets sucked back out of your body.

• Leads to mixing of oxygenated and unoxygenated blood.

• VSD is the most common heart defect.

Patent Ductus Arteriosus (PDA)

• The ductus arteriosus, a vessel that bypasses the lungs in fetal circulation, remains open after birth.

• Allows unoxygenated blood to bypass the lungs and enter the body's circulation.

Tetralogy of Fallot

• A combination of four heart defects: pulmonary stenosis, ventricular septal defect, overriding aorta, and enlarged right ventricle.

Associated Anomalies

• Kids who have oral, gastric, intestinal problems will also have heart issues.

• Problems with tubes, therefore the esophagus and the trachea.

Cleft Lip and Palate

• Defects in the formation of the lip and/or palate.

• Can interfere with feeding, speech, and enunciation.

• Requires surgical repair, often in a series of procedures.

Omphalocele and Gastroschisis

• Omphalocele: Abdominal contents herniate through the umbilical cord into a sac.

• Gastroschisis: Bowel herniates through an abdominal wall defect.

• After feeding kids with this syndrome, the kid is gonna have abdominal distension because it's can't go anywhere. It's gonna have to come back where you could. It can be bile stain. The stomach's gonna be distended.

Imperforated Anus

• Absence or blockage of the anal opening.

• Requires surgical correction.

Musculoskeletal System

Hip Dysplasia

• Abnormal development of the hip joint.

• Can range from subluxation to complete dislocation.

• Often treated with bracing or triple diapering.

Clubfoot

• Deformity of the foot that turns inward and downward.

• May be positional or due to bone involvement.

• Treatment ranges from exercises to surgery.

GU

Bladder Issues

• The skin over the bladder is not closed.

Hypospadias

• The opening of the urethra is on the underside of the penis.

• Circumcision is contraindicated.

Epispadias

• The opening of the urethra is on the upper side of the penis.

• Circumcision is contraindicated.

Undescended Testes

• One or both the absence of one or both of the testes. Is you feel up the you feel in the scrotum. You feel the testes that is down into the sac itself

Metabolic Disorders

Phenylketonuria (PKU)

• Inability to metabolize phenylalanine, leading to brain damage.

• Treated with a special diet to avoid phenylalanine.

Congenital Hypothyroidism

• Thyroid gland defect.

• Treated with thyroid hormone replacement therapy.

Maple Syrup Urine Disease

• Metabolic disorder that causes urine to smell like maple syrup.

Screening

• Testing the babies for at the very beginning was the hemalalimuria. That's the most common amino acid disorder.

• Maple Syrup Urine Disease (MSUD), Congenital Hypothyroidism (CH), PKU and Galactosemia also requires screen testing as well.

Characteristics

Down Syndrome

• Short fingers, a single palmar crease (simian crease), shorter neck, and eye shaped like an Asian

Injuries

Acquired Traumatic Birth Injuries

• Fractures: Skull fractures, fractured clavicles (most common), humerus, and femurs.

Erb's Duchenne Paralysis

• Damage to nerve endings during delivery.

• Causes paralysis of the arm.

• Arm kind of will locate in when there it's hanging, Morhawk's not there.

Facial Palsy

• Paralysis of facial nerves due to what: The way they're positioned or how they have their hand or something else or if they were in kinda jammed in there with that. Is that flattened on one side.

Phrenic Nerve Damage

• Damage to the phrenic nerve, affecting diaphragm function and breathing, it's gonna affect your breathing, Cyanosis, irregular thoracic respirations with no abdominal movement on inspiration.

Cranial Hemorrhages

• Subdural, subarachnoid, epidural, intraventricular hemorrhages.

  • Blood in the spinal fluid is know you're have a rectal

• Most will be absorbed.

Respiratory Issues

• Nasal flaring, grunting, sternal retractions are indicators.

Transient Tachypnea of the Newborn (TTN)

• Respiratory distress without cyanosis.

• Self-limiting, resolves within 72 hours.

Respiratory Distress Syndrome (RDS)

• Respiratory distress with cyanosis.

• Due to lack of surfactant.

Meconium Aspiration Syndrome (MAS)

• Respiratory distress caused by meconium in the lungs.

• Associated with fetal distress during labor, diabetic or hypertensive moms, difficult delivery, post-term infants.

Persistent Pulmonary Hypertension of the Newborn (PPHN)

• Persistent fetal circulation due to non-functioning lungs.

• Can be caused by respiratory distress syndrome, diaphragmatic hernias, neonatal pneumonia, sepsis.

Polycythemia

• High hematocrit level.

• Seen more in preemies and kids who are starved inside, high hemagglis.They have high hemagglis, so they're gonna be red.

Loss and Grief

• The grieving process starts with the grieving process. We take pictures of them. We make little boxes for them.

Ethics Committee

Ethics Discussion

• When is it too early to save a baby? How much is too much? Are we trying to save babies that really shouldn't be saved? How do we decide? How do the parents decide? Who gets to decide?