Congenital Anomalies NUR 349
Popular in HumanistChldFam
verified elite notetaker
Popular in Nursing and Health Sciences
This 18 page Class Notes was uploaded by Nicole Raynor on Thursday August 25, 2016. The Class Notes belongs to NUR 349 at Molloy College taught by Paraszczuk in Fall 2016. Since its upload, it has received 6 views. For similar materials see HumanistChldFam in Nursing and Health Sciences at Molloy College.
Reviews for Congenital Anomalies
Report this Material
What is Karma?
Karma is the currency of StudySoup.
You can buy or earn more Karma at anytime and redeem it for class notes, study guides, flashcards, and more!
Date Created: 08/25/16
Chapter 54 Congenital Anomalies Down Syndrome Overview • Incidence increases to age 5 (because the recognition that there is something abnormal doesn’t always happen right away) • Higher incidence in aborted fetuses • Leading cause of death in infancy • Often results in developmental disability/ cognitive impairment/chronic illness • Most common structural defects o Heart defects, abdominal wall defects, imperforate anus, neural tube defects, cleft lip or palate, clubfoot, hip dysplasia Birth of an Infant with Congenital Anomalies • Factors the affect Response o Timing and manner of being told o Prior knowledge of the defect o Type of defect • Issues for Parents o Grief and mourning o bonding and attachment o accurate information and informed consent o Facilitating communication o Preparing for discharge Family of a Baby with a Defect • Parents experience acute feelings of grief, guilt and failure o feel shock, helplessness, isolation, fear and depression • Provide technical care, coordinate services • Impact on relationships, burden of care, financial burden, fears, pressures • Mothers/fathers respond differently • Effect on Siblings and other family members Parents of Infants with Congenital Anomalies • Four psychologic tasks for coping o Anticipatory grief o Address issues of guilt o Resumption of process of relating to infant o Understanding special needs and growth patterns Parents’ Needs • Understand routine wellbaby care/screening and growth and development • Understand how to perform any special procedures needed to care for infant • Have medical followup arranged • Referral for any special equipment required at home • Respite care for medically fragile children Nurse’s Role • Assess parent’s level of understanding of infant’s problem • Prepare parents for viewing of infant • Facilitate initial grief, assist with coping • Encourage parental participation in infant’s care • Facilitate parental adjustment to infant’s special needs • Identifying resources to meet lifelong needs Cognitive Impairment • Cognitive impairment (CI) is a general term that encompasses any type of mental difficulty or deficiency • Used synonymously with intellectual disability • Diagnosis o Made after a period of suspicion by family or health professionals o In some instances diagnosis is made at birth Cognitive Impairment: General Concepts • Mild to severe • IQ is not as important as functional assessment o Motor movement o Language o Adaptive behavior • Achieve developmental milestones slowly Children with an Intellectual Disability • These children may have limitations in both intellectual and adaptive functioning. o Social interaction o Use of language for selfexpression o Selfcare abilities • Severe limitations will require lifelong care. Nursing Management • These children have normal needs. o Positive attention, Self discovery, Growth and development • A safe environment needs to be maintained o Physical safety, Safety with strangers • Families need to actually participate in care planning. • These children may be at increased risk for other pediatric illnesses • Promote adaptive functioning • Ongoing evaluation of outcomes o Short and longterm based on needs, development • Promote adaptive functioning • Allow home routine during hospitalization o Selfcare routines o Medication schedules o Medication administration techniques o Interventions (suctioning, feeding, etc) Down Syndrome Etiology • AKA nonfamilial trisomy 21 o Extra chromosome 21 95% o Translocation of chromosomes 34% o Mosaicism, both abnormal and normal cells12% • Etiology unknown—likely multiple causality – usually occurs more with women who have children at an older age • Most common genetic cause of (Cognitive Impairment) • Occurs more often in Caucasian than AfricanAmerican families • Diagnosed by chromosome analysis – Trisomy 21 Karyotype • Incidence: 1 in 800 to 1,000 infants (~5,500/yr) o Maternal age • Age 35—risk is 1 in 400 births • Age 40—risk is 1 in 110 births • At risk for cardiac defects (monitored for during the first year of life) TQ, leukemia, hearing loss, thyroid disease, gastrointestinal problems Clinical manifestations o Hypotonia o Small head o Epicanthal eye folds (upward slant) o Protruding tongue o Flat nose/bridge o Wide, short neck o Simian crease on palm (one crease on palm) o Intellectual disability (CI) Therapeutic Management: o A number of therapies have been advocated Surgery to correct congenital anomalies Evaluation of hearing and sight Periodic testing of thyroid function o Support the family at time of diagnosis o Assist the family in preventing physical problems o Assist in prenatal diagnosis and genetic counseling A newborn assessment shows separated sagittal suture, oblique palpebral fissures, depressed nasal bridge, protruding tongue, and transverse palmar creases – TQ Breastfeeding may not be possible if the infant’s muscle tone or sucking reflex is immature. Mothers should be encouraged to pump breast milk and use special bottles for assistance with feeding. Some children with Down syndrome can breastfeed adequately. TQ The child with Down syndrome needs routine schedules and consistency. Having familiar people present, especially parents, helps to decrease the child’s anxiety. TQ Children with Down syndrome are at risk for atlantoaxial instability. Before participating in sports that put stress on the head and neck, a radiologic examination should be done. TQ Chapter 30: Care of the Child with Congenital Anomalies Heart & CNS Circulatory changes at Birth Inutero because the fetus is getting the oxygen second hand – oxygen tensions are low (pO2 40’s) Transitional and Neonatal Circulation Major changes in the circulatory system occur at birth after the first breath. o Gas exchange is transferred from the placenta to the lungs. o Fetal shunts close. o Resistance to flow in the pulmonary system decreases as systematic resistance increases. o Pulmonary vascular resistance decreases. o Marked increase in pulmonary blood flow follows. Assessment – Cardiac Initial: rapid cardiopulmonary assessment –general appearance then airway, breathing, circulation, (what is their level of consciousness) History: cardiac disease in family (links to congenital heart defects), exposure to teratogen, presence of chromosomal or other birth defect, poor weight gain, prior murmurs, respiratory difficulties or recurring URIs, cyanosis, recent Strep infection, Activity intolerance (considered this if they don’t feed well), poor feeding, fatigue Physical assessment: o Inspection: color, Chest deformities, respiratory excursion, unusual pulsations, clubbing of fingers (not going to see in an infant, takes a while to develop), nutritional status (if not feeding well). o Palpation/percussionchest/abdomen, peripheral pulses o Auscultation – HR and rhythm, point of maximum impulse (PMI) apical, character of sounds, murmurs and additional sounds, and breath sounds Diagnostic Procedures (p. 1200) Chest xray EKG 12 lead or continuous Echocardiography Exercise stress test (can’t do until children get older) Cardiac MRI Cardiac catheterization Cardiac Catheterization (p. 1212) Diagnostic o Catheter placed in either vein or artery to measure pressures or output, inject contrast (angiography) to observe flow or obtain biopsy. Interventional o Balloon catheter or other device is used to improve flow o Use to make an opening where there isn’t one o Electrophysiology studies –catheter is used to record impulses and ablate aberrant pathways Nursing Care for child undergoing cardiac catherization Preprocedure Care: o Height (length of the catheter)/weight (for anesthesia – how much sedation are we going to use) o allergy to iodine o presence of infection (yeast infection go in near the groin) o respiratory infection, and diaper rash can cancel procedure o mark peripheral pulses o obtain baseline VS and pulse ox o preprocedure education/NPO instructions (for the family if they are not old enough to understand) o roomsetup, analgesia and sedation Postprocedure care: o Monitor cardiovascular (heart rhythm, perfusion, bleeding /post sedation status: o Continuous HR/Respiratory monitoring, BP, pulse ox o keep cannulated leg straight (can be difficult because they like to keep their legs close to them) o NPO until reactive, IV until tolerating PO o strict I and O o Observe for complications –change in pulses, temp and color of extremity, changes in heart rate and rhythm, BP or respiratory status. Bleeding, diuresis due to IVs (dyes) can cause hypoglycemia in infants o Child can return to school on the third day after procedure Congenital Heart Disease Anatomic deviation present at birth and that leads to abnormal cardiac function. Classified according to structural abnormalities, functional alterations or both Etiology unknown Consequences are CHF (because of abnormal flow) and hypoxemia Left to Right Shunting Increased Pulmonary Blood Flow Ventricular Septal defect (VSD) Patent Ductus Arteiosus (PDA) *Blood is recirculated to the lungs increasing the workload of the heart Patent Ductus Arteriosus (PDA) Failure of the ductus arteriosus to close with LR shunt o Fetal shunt between the aorta and the pulmonary artery fails to close Symptoms: Few symptoms or CHF o Machinery murmur *place stethoscope over the 2 intercostal space where the great vessels are TQ o Risk for Bacterial Endocarditis use NSAIDS (have an anti prostaglandin effect this is used–because it can help it close) Examples of NSAIDS used: indomethacin or Motrin (ibuprofen) Treatment: Indomethecin, surgical ligation or coils placed during cardiac cath Low mortality Not open heart surgery Ventricular Septal Defect Abnormal opening between the two ventricles with LR shunt o Increased pulmonary blood flow o Some blood gets sent back to the right side Symptoms: CHF is common. Murmur present (apex over the ventricles), at risk for bacterial endocarditis. Depends on location and size Treatment: Surgical closure with purse string suture or dacron patch. Use of closure device in trials o Open heart surgery Obstructive Defects Coarctation of the Aorta Aortic Stenosis Pulmonary stenosis *Anatomic narrowing causes obstruction to blood flow Coarctation of the Aorta Narrowing of aorta near the insertion of the ductus ateriosus o Because the narrowing of the vessel – blood flow to the lower part of the body is decreased – poor cardiac output to the body Hallmark Signs: Higher BP in upper extremities bounding pulse in arms, weak/absent femoral pulses, low BP and coolness in legs Treatment: Surgically resect or enlarge with graft before age 2 to prevent hypertension or trial balloon angioplasty o Depends on the extent of the narrowing* Cyanotic Lesions with Decreased Pulmonary Blood Flow Tetralogy of Fallot Tricuspid Atresia *There is obstruction of pulmonary blood flow and a defect that enables desaturated blood to shunt from RL (blood get to the body that never reached the lungs) will never be 100% oxygen saturated at rest – at normal they will be in the high 80’s there is a mix of oxygenated and deoxygenated blood in their system Tetralogy of Fallot 4 defects: o Ventricular Septal Defect (aorta is so high up that the ventricles collapse blood from both right and left ventricle) o Pulmonic stenosis (not allowing blood flow to go through very easily) o Overriding aorta o RV hypertrophy Symptoms: o Cyanosis varies (hypoxemia) o Murmur (b/c of VSD) o Tet or blue spells occur with stress calm the baby down first o Clubbing of fingers o Squatting (when they get in a spell – helps them feel better) o poor growth *always concerned about them having cyanotic spells where they turn really blue Treatment: Surgical closure of the VSD, resect stenosis Cyanotic Lesions with Increased Pulmonary Blood Flow Transposition of the Great Vessels Truncus Arteriosus Hypoplastic Left Heart Syndrome Cyanotic Lesions with Decreased Pulmonary Blood Flow Complex anomalies Fully saturated blood mixes with desaturated blood Survival past the post natal period depends on amount of mixing Variable presentation with some desaturation and signs of CHF Staged surgeries common Higher mortality rates Transposition of the Great Vessels PA leaves the left ventricle and the aorta leaves the right 2 separate circulations unless septal defects o Aorta is supposed to be connected to the left side o Pulmonary artery to the right side o Results in mixed blood flow Manifestations: Cyanosis and evidence of CHF o They get through fetal life, after a few hours they become cyanotic (purple) because the follow is naturally going to diminish Treatment: Prostaglandin E to keep DA open then balloon septostomy then atrial switch Congestive Heart Failure Inability of heart to pump sufficient amount of blood to meet demands Etiology: structural defects, myocardial failure (cardiomyopathy or dysrhythmias) excessive demand on heart muscle (sepsis or anemia) Can be right or leftsided failure but usually occur together***** Can lead to heart muscle damage Assessment for Clinical Manifestations of CHF Impaired myocardial function tachycardia, galloping rhythm, fatigue (poor feeding), failure to thrive, ↓BP, poor perfusion, cardiomegaly o Keep in mind normal heart rate for child* Pulmonary congestion – tachypnea, dyspnea, exercise intolerance, cough, wheezing Systemic Venous Congestion – weight gain, liver and spleen enlargement, peripheral edema, neck vein distention (not easy to see in infants) **If infant with CHF has an increased respiratory rate, sweating and not feeding well alert the physician – these are early signs of congestive heart failure Common Nursing Diagnoses Decreased cardiac output Imbalanced nutrition: less than body requirements Activity intolerance Deficient knowledge Anxiety Interrupted family processes Risk for infection Risk for ineffective health maintenance Goals of Treatment Improve cardiac function – digoxin and ACE inhibitors Reduce fluid and sodium retention –diuretics, possible fluid and sodium restriction Decrease cardiac workload (swaddling – upright position*) Increase tissue oxygenation o **O2 only as ordered–may be detrimental in some cases Decreasing Workload on the Heart Limit feeding time (feed more frequently). Elevate the head of the bed ↓ work of breathing Maintain normal body temperature, treat infection Provide uninterrupted rest. (cluster care) Engage in selflimiting activities. Provide oxygen (depending upon the lesion). Interventions Medications o Digoxin increases cardiac output, decreases venous pressure, decreases edema Check apical pulse–parameters should be ordered o Depending on the age of the child and their normal heart rate – it will depend if you will hold the med Ordered in microgram small doses, overdose may be lethal Dig toxicity – nausea, vomiting, anorexia, bradycardia, dysrhythmias Never stop abruptly or makeup missed dose Should be given at the same time every day o ACE inhibitorsBP before and after o Captopril o Diuretics – K loss can potentiate digoxin o Use K+ sparing diuretics Feeding the Infant with Congestive Heart Failure Feed the infant or child in a relaxed environment; frequent, small feedings may be less tiring. o Limit feeding time to no more than 30 minutes Hold the infant in an upright position. This may provide less stomach compression and improve respiratory effort. If the child is unable to consume appropriate amount during 30minute feeding every 3 hours, consider nasogastric feeding. Monitor for increased tachypnea, diaphoresis, or feeding intolerance (vomiting). Concentrating formula to 30 kcal/oz may increase caloric intake without increasing infant’s work. Additional interventions o Conserve energy – organize nursing activities, keep child calm, adjust feedings, prevent cold stress o Maintain nutrition and monitor fluid balance o Parent teaching Evaluation – o Optimal state of health o Parents understand instructions Hypoxemia PaO2 that is less than normal that can lead to decrease in tissue oxygenation causing impaired functioning and acidosis Usually cause is desaturated blood that does not get to the lungs enters systemic circulation Clinical Manifestations – cyanosis, polycythemia, clubbing of fingers, squatting *can damage pulmonary vasculature Hypercyanotic Spells “Tet” spells Seen in infants with obstruction of pulmonary blood flow (Tetralogy of Fallot) Caused by sudden infundibular spasm(↓ pulmonary blood flow and ↑ RL shunting) caused by feeding, defecation, stress, crying Prompt recognition and treatment o Calm the baby first o Kneechest position, comforting, 100% O2 by mask, morphine subcutaneous or IV o Maintain hydration to ↓ blood viscosity reduce risk of CVAs Kneechest position Infective Endocarditis (IE) Infection of cardiac valves and endocardium Bacteria, fungus or viral o Streptococcus viridans ↑risk with Prosthetic heart valves, shunts complex defects (turbulent flow pathogens entrapped) Diagnosed by blood culture and Echocardiogram Prevention and early recognition is key IV antibiotics for 2 to 8 weeks Antibiotic Prophylaxis for Children at Risk for IE Teach parents of high risk children about both the need for prophylactic antibiotics Dental procedures, including cleaning, that may induce gingival or mucosal bleeding Tonsillectomy and/or adenoidectomy o Amoxicillin is Taken orally 1 hour before procedure Surgery and/or biopsy involving respiratory or intestinal mucosa Incision and drainage of infected tissue Invasive genitourinary and gastrointestinal procedures Nursing care of the Family Help with adjustment shock, anxiety, guilt Educate – about condition, good nutrition, medications and other tx, child’s education according to developmental age Help copingwhen and how to contact HCP, encouraging activity Prepare for procedures or surgery –environment and equipment Nursing Care of the Family Postop care: NICU or PICU first Dysrhythmia assessment, respiratory status – ventilation then O2 by mask/NC with mist but avoid chilling Chest tubes to suction the water sealremoved 13 days postop Strict I and O provide rest –cluster nursing activities Adequate pain management Neural Tube Defects Failed closure of neural tube during fetal life May involve entire length of the neural tube or small portion Etiology multifactorial Incidence declining o To prevent: Folic acid supplementation at child bearing age o 0.4 mg is recommended o Antenatal diagnosis and termination of pregnancy Spina Bifida Failure of osseous spine to close SB occulta Not visible externally o Usually lumbosacral, L5S1 o Skin indications dimple, angiomas o Fluid leaking from the opening o Not usually detected prenatally SB cystica Saclike protrusion o Meningocele – meninges, spinal fluid, no nerves o Myelomeningocele – all Varying and serious degrees of neurologic deficit Myelomeningocele Location and magnitude of defect determine nature and extent of impairment If defect below 2nd lumbar vertebra o Flaccid paralysis of lower extremities o Sensory deficit Not necessarily uniform on both sides of defect Therapeutic Management Prevent infection Assessment of neuro and associated anomalies – check for hydrocephalus Early closure surgery in fetus or 1272 hrs of life o Prevent stretching of other nerve roots and further damage o Sac easily ruptured, Prone to leakage of CSF Musculoskeletal concerns assess functioning, promote ambulation, prevent contractures, correct deformities, prevent problems R/T immobility (obesity, skin breakdown) Bladder and Bowel functioning o Neurogenic bladder (are they voiding or dribbling)/oozing stool/rectal prolapse o Promote bladder emptying and continence by intermittent catheterizations, medications, surgery o Bowel regimens, enemas, surgery Nursing Considerations Preop Care of the Sac o Delicate handling, keep prone, sterile saline dressings, prevent contamination (stool) Assess for infection (check temperature frequently), increased ICP and complications (bladder, bowel, rectum) monitor Postop carepositioning, feeding, monitor elimination Parent teaching and family support o There is no known cause of this Hydrocephalus Accumulation of CSF in the ventricles of the brain causing dilation of the ventricles Caused by: o impaired absorption of CSF within the subarachnoid space (communicating) o Obstruction to flow of CSF through ventricular system (noncommunicating) Commonly associated with myelomeningocele (in 80%85% of SB cases) after closure May develop secondary to other condition (injury or hemorrhage in premies) Monitoring head circumference and fontanel tension Manifestations: o Irritability, poor appetite, rapid head growth with observable distended scalp veins Serial imaging—ventricle size Treatment o drain excessive CSF (ventriculostomy or shunt) o Treatment of complications o Manage problems related to psychomotor development o Most important – keep ventricle from getting overly large* VP Shunt –if there is increased ICP we can let the physician know – because shunt is not working properly VP Shunt Complications Infection: shunt malfunction, o fever and inflammation of tract, abdominal pain Malfunction: usually obstruction (lemon pudding – have to be drained) o increased ICP, worsening neurologic status/altered LOC If significant –Emergent revision or removal of shunt with external ventricular drainage (EVD) put in place Endoscopic Third Ventriculostomy In place of a shunt an opening is made in the floor of the third ventricle to allow drainage of excessive fluid High success rate in those >2 Complications: post op bleeding and infection, closure of the stoma Nursing Considerations Assessment o ICP in infants, assess fontanels and head circumference, when older behavioral signs o Infection Postopmaintain position, to maintain drainage o External drain or pumping of valve (surgeon order) Manifestations of ↑ICP Infant o Poor feeding or vomiting o Irritability /restlessness, lethargy o Highpitched cry o Bulging fontanel o Increased head circumference o Separation of cranial sutures o Distended scalp veins o Eyes deviated downward ("setting sun" sign) o Increased or decreased response to pain Child o Headache o Diplopia o Mood swings o Slurred speech o Papilledema (after 48 hours) o Altered level of consciousness o Nausea and vomiting, especially in the morning Latex Allergy Identified as serious health hazard when a child with Spina Bifida experienced anaphylaxis due to latex allergy SB patients at high risk for latex allergy due to repeated exposure to latex products from multiple surgeries and repeated urinary catheterizations Nursing consideration: avoid using any latex products Question 1 Which of the following would indicate pulmonary venous congestion is occurring in an infant with ventricular septal defect? a. Increase in the depth of respiration b. Crackles noted with auscultation c. Murmur noted with auscultation d. Hepatomegaly noted with palpation Question 2 A 2 month old girl is admitted to the unit for cardiac surgery. Preoperative orders state: Obtain weight, keep NPO and infant’s head elevated at a 45 degree angle. Which action would be the most appropriate? a. Defer weighing and use the weight obtained at the most recent clinic visit b. Place the infant on the scale in a supine position to obtain weight. c. Weigh the infant in a infant seat and subtract the weight of the seat. d. Elevate the infant’s head with pillows on the scale and the subtract the weight of the pillows
Are you sure you want to buy this material for
You're already Subscribed!
Looks like you've already subscribed to StudySoup, you won't need to purchase another subscription to get this material. To access this material simply click 'View Full Document'