Ch 14 Revised

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Chapter 14Chapter 14

The Newborn with a CongenitalMalformation



Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier Inc. 2

Birth DefectsBirth Defects

z Abnormalities that are apparent at birth

z The abnormality may be of

Structure

Function

Metabolism

z May result in a physical or mental disability,

may shorten life, or may be fatal




Classifications of Birth DefectsClassifications of Birth Defects

z Malformations present at birth

May also be known as congenital malformations

z Metabolic defects (body chemistry)

z Disorders of the blood

z Chromosomal abnormalities

z Perinatal injuries




March of DimesMarch of Dimes

z ³Few birth defects can be attributed to a

single cause. The majority are thought to

result from an interplay between

environment and heredity, depending oninherited susceptibility, stage of pregnancy,

and degree of environmental hazard.´




Nervous SystemNervous System

z Neural tube defects

Most often caused from failure of neural tube to

close at either the cranial or the caudal end of

the spinal cord




HydrocephalusHydrocephalus

z Characterized by an increase in CSF within

the ventricles of the brain

Causes pressure changes in the brain

Increase in head size

Results from an imbalance between production

and absorption of CSF or improper formation of

ventricles




Hydrocephalus (continued)Hydrocephalus (continued)

z Classifications

Noncommunicating

Obstruction of CSF flow from the ventricles of the brain

to the subarachnoid space

Communicating

CSF is not obstructed in the ventricles but is

inadequately reabsorbed in the subarachnoid space




Manifestations of HydrocephalusManifestations of Hydrocephalus

z Depends upon time of

onset and severity of

imbalance

z Classic sign

Increase in size of head

Cranial sutures separate

to accommodate

enlarging mass

Scalp is shiny

Veins are dilated

Marked hydrocephalus with

³setting sun´ sign of the eyes.

Note the characteristic large head,

distended scalp veins, and full

fontanelle.




Diagnosis and Treatment

of Hydrocephalus

Diagnosis and Treatment

of Hydrocephalus

z Treatment

Medications to reduce

production of CSF

Surgery to place ashunt




Symptoms of Increasing

Intracranial Pressure

Symptoms of Increasing

Intracranial Pressure

z Increased blood pressure

z Decrease in pulse rate

z

Decrease in respirationsz High-pitched cry

z Unequal pupil size or response to light

z Bulging fontanelles

z Irritability or lethargy

z Poor feeding




Ventriculoperitoneal ShuntVentriculoperitoneal Shunt




Spina Bifida

(aka Myelodysplasia)

Spina Bifida

(aka Myelodysplasia)

z Group of CNS disorders characterized by

malformation of the spinal cord

z A congenital embryonic neural tube defect

with an imperfect closure of the spinal

vertebrae

z Two types

Occulta (hidden) Cystica (sac or cyst)




Spina BifidaSpina Bifida




Spina Bifida OccultaSpina Bifida Occulta

z Minor variation of the

disorder

z Opening is small

z No associated

protrusion of structures

z Often undetected

z May have a tuft of hair,dimple, lipoma, or

discoloration at the site




Spina Bifida Occulta

(continued)

Spina Bifida Occulta

(continued)

z Treatment generally not necessary unless

neuromuscular symptoms appear, such as

Progressive disturbances of gait

Disturbances of bowel and bladder sphincter

function




Spina Bifida CysticaSpina Bifida Cystica

z Development of a cystic mass in the midline of theopening in the spine

z Two types

z Meningocele Contains portions of the membranes and CSF

Size varies

z Meningomyelocele

More serious protrusion of membranes and spinal cordthrough the opening

May have associated paralysis of lower extremities

May have poor or no control of bladder or bowel

Hydrocephalus is a common complication




Prevention of Spina BifidaPrevention of Spina Bifida

z Mother takes folic acid 0.4mg per day prior

to becoming pregnant and/or continues to

take the folic acid supplement until the 12th

week of pregnancy




Treatment of Spina BifidaTreatment of Spina Bifida

z Surgical closure

z Prognosis is dependent upon extent of

spinal cord involvement




HabilitationHabilitation

z Patient is disabled from birth and must learn

new things

z Aim is to minimize the child¶s disability

z Constructively use all unaffected parts of the

body

z Every effort is made to help the child

develop a healthy personality so that he or she may experience a happy and productive

life




Nursing Care of

Spina Bifida Patient

Nursing Care of

Spina Bifida Patient

z Prevent infection of, or injury to, the sac

z Correct positioning to prevent pressure on the sac

z Accurate observations and charting

z Education of the parents

z Moist, sterile dressing of saline or an antibiotic solution

may be ordered to prevent drying of the sac

z Protection from injury and maintenance of a sterile

environment for the open lesion are essential




Cleft LipCleft Lip

z Characterized by a fissure

or opening in the upper lip

z Failure of maxillary and

median nasal processes tounite during embryonic

development

z Many cases are hereditary,

others are environmentalz Appears to occur more

often in boys than girls




TreatmentTreatment

z Initial repair of cleft lip is known as

cheiloplasty cheiloplasty

z Repair occurs by 3 months of age

z Infant may have to have elbow restraints to

prevent the infant from scratching the lip

z A special syringe or bottle may be needed

to assist in feeding the child until surgeryhas occurred




Cleft PalateCleft Palate

z The failure of the hard palates to fuse at the

midline during the 7th to 12th weeks of

gestation

z Forms a passageway between the

nasopharynx and the nose




Cleft Palate: TreatmentCleft Palate: Treatment

z Goals of therapy

Union of the cleft

Improved feeding

Improved speech

Improved dental development

The nurturing of a positive self-image




Cleft Palate: Other FactorsCleft Palate: Other Factors

z Psychosocial adjustment of the family

z Follow-up care

z Home care

z Surgery between 1 year and 18 months of

age




ClubfootClubfoot

z Common deformity

z Congenital anomaly

Foot twists inward or outward

z Talipes equinovarus most common type




ClubfootClubfoot




Clubfoot: Treatment

and Nursing Care

Clubfoot: Treatment

and Nursing Care

z Started as soon as possible or bones and

muscles will continue to develop in an

abnormal manner

z Conservative treatment

Splinting or casting to hold foot in correct

position

Passive stretching exercises

z If not effective after about 3 months, surgical

intervention may be indicated




Hip DysplasiaHip Dysplasia

z Term, hip dysplasia, applies to various

degrees of deformities, subluxation or

dislocation (can be partial or complete)

z Head of femur is partly or completelydisplaced

z 7 times more common in girls

z More apparent as infant/toddler beginswalking




Developmental Hip DysplasiaDevelopmental Hip Dysplasia




Diagnostics for Hip DysplasiaDiagnostics for Hip Dysplasia

z Barlow¶s test : upon adduction and extension

of the hips (with health care provider

providing stabilization to the pelvis), may

³feel´ the dislocation actually occur z Ortolani¶s sign (or click): where health care

provider can actually feel and hear the

femoral head slip back into the acetabulum

under gentle pressure




Hip Dysplasia: TreatmentHip Dysplasia: Treatment

z Hips are maintained in constant flexion and

abduction for 4 to 8 weeks

Keeps head of femur within the hip socket

z Constant pressure enlarges and deepens

acetabulum

z Can use a P avlik harness to provide the

necessary positioningz Or surgical intervention may be necessary




Pavlik Harness, Body Cast,

and Traction

Pavlik Harness, Body Cast,

and Traction

The Pavlik harness is used in infant¶sage 1 to 6 months to maintain hips in aposition of flexion and abduction.

A, Infant in a spica body cast. This cast maintains the legs in a froglikeposition and is used to treat developmental hip dysplasia. Note that theinfant is able to move her toes freely. A diaper tucked inside prevents the

cast from becoming soiled with urine or feces.B, Traction is sometimes necessary before surgery or casting. Home careenables the child to be in familiar surroundings that will nurture growth anddevelopment.

A

B




Nursing Care of Infant/

Child in a Spica Cast

Nursing Care of Infant/

Child in a Spica Cast

z Neurovascular assessment of affected

extremities

z Place firm, plastic covered pillows beneath

the curves of the cast for support

z In the older child, a ³fracture´ bedpan should

be readily available for toileting

z Head of bed slightly elevated to help drainany body fluids away from cast

z Frequent changes of position are needed to

prevent skin breakdown




Nursing Care of Infant/Child in

a Spica Cast (continued)

Nursing Care of Infant/Child in

a Spica Cast (continued)

z Toys that are small enough to ³hide´ in the

cast should not be given to the child

z Important to meet everyday needs

z A special wagon with pillows inside it for

support is one of the safest ways to

transport a child in a spica cast




Metabolic DefectsMetabolic Defects

z Inborn errors of metabolism involve a

genetic defect that may not be apparent

until after birth

z Symptoms to report would include

Lethargy, poor feeding, hypotonia, unique odor

to body or urine, tachypnea and vomiting




Phenylketonuria (PKU)Phenylketonuria (PKU)

z Faulty metabolism of phenylalanine, an

amino acid essential to life and found in all

protein foods

z Infant unable to digest this essential acidand phenylalanine accumulates in blood

and is found in the urine within the 1st week

of life

z Results in severe mental retardation




PKU (Continued)PKU (Continued)

z Infant appears normal atbirth

z By the time urine test is

positive, brain damage hasalready occurred

z Begins to show delayeddevelopment at about 4 to6 months of age

z May have failure to thrive,eczema, or other skinconditions

z Child has a musty odor

z Personality disorder

z Occurs mainly in

blonde, blue eyed

children






PKU: TreatmentPKU: Treatment

z Close dietary management

z Frequent evaluation of blood phenylalanine

level

z Child will require a synthetic food that

provides enough protein for growth and

tissue repair

z Special formulas are available for purchase,such as Lofenalac and Phenex-1




PKU: Nursing CarePKU: Nursing Care

z Teach parents importance of reading food

labels

z Following up as required with health care

provider for blood tests

z Referral to a dietician is helpful in providing

parental guidance and support

z Genetic counseling may also be indicated




Nursing TipNursing Tip

z Children with PKU must avoid the

sweetener aspartame (NutraSweet)

because it is a converted to phenylalanine in

the body




Maple Syrup Urine DiseaseMaple Syrup Urine Disease

z Defect in the metabolism of branched-chain

amino acids

z Causes marked serum elevations of leucine,

isoleucine, and valine

z Results in acidosis, cerebral degeneration,

and death within 2 weeks if not treated




Maple Syrup Urine Disease

(continued)


(continued)

z Appears healthy at birth, but problems soon

develop

z Feeding difficulties

z Loss of the Moro reflex

z Hypotonia

z Irregular respirations

z Convulsions





(continued)


(continued)

z Manifestations:

Urine, sweat, and cerumen (earwax) have a

characteristic sweet or maple syrup odor

Caused from ketoacidosis Diagnosis confirmed by blood and urine tests

M l S U i DiM l S U i Di




Maple Syrup Urine Disease:

Treatment and Nursing Care

Maple Syrup Urine Disease:

Treatment and Nursing Care

z Initial treatment

Removing the amino acids and their metabolites

from the body tissues (how?)

Lifelong diet low in amino acids leucine,isoleucine, and valine

Exacerbations are usually related to infection

and can be life threatening




GalactosemiaGalactosemia

z The body is unable to

use the carbohydrates

galactose and lactose

z An enzyme is defectiveor missing

z Results in an increased

serum galactose level

(galactosemia) and in

the urine (galactosuria)

z Galactose is present

in milk in the form of

sugar, therefore,

early diagnosis isessential




Galactosemia (continued)Galactosemia (continued)

z Manifestations

Begins abruptly,

worsens gradually

Early signs Lethargy

Vomiting

Hypotonia

Diarrhea

Failure to thrive

z Symptoms begin as

the newborn begins

to be fed

z Jaundice may bepresent

z Screening tests are

available

G l t i T t tG l t i T t t




Galactosemia: Treatment

and Nursing Care

Galactosemia: Treatment

and Nursing Care

z Milk and lactose-containing products are

eliminated from the diet

z Breastfeeding must be stopped

z Lactose-free formulas or soy-protein based

formulas are often used instead

z Parental support and education is essential




Down SyndromeDown Syndrome

z Most common of chromosomal

abnormalities

z Risk increases with

Mothers 35 years and older

Fathers 55 years and older

z Infant has mild to severe mental retardation

z Some physical abnormalities are also seen




Down Syndrome (continued)Down Syndrome (continued)

z 3 Phenotypes

z Trisomy 21 Most common

There are 3 number 21 chromosomes instead of 2 Results from nondisjunction

z Mosaicism Occurs when both normal and abnormal cells are present

Tend to be less severely affected in appearance and

intelligence

z Translocation of a chromosome A piece of chromosome in pair 21 breaks away and

attaches itself to another chromosome





z Screening for this is offered during prenatal

care starting around week 15 of gestation

Allows parents the opportunity to decide on

whether to continue or terminate the pregnancy

z Alpha-fetoprotein ( AFP) and hCG levels are

used for diagnosis

z Known as the ³Triple screen´

z Amniocentesis/CVS are most accurate

D S dD S d




Down Syndrome:

Manifestations

Down Syndrome:

Manifestations

Down syndrome. A, The typical facial appearance of an infant with Down syndrome shows the upward slant of thecanthal folds of the eyes, protruding tongue, and short, thick neck. B, The straight simian crease in the palm of thehand is a typical finding in children with Down syndrome. C, The short fifth finger is a typical finding in children withDown syndrome. The tip of the fifth finger does not extend to the distal joint of the adjoining finger.

A

B

C





z Limp, flaccid posturecaused by hypotonicityof muscles

z More difficult toposition and hold

z Contributes to heatloss

z Prone to respiratoryillnesses andconstipation due to thehypotonicity

z Incidence of acuteleukemia is higher

z Alzheimer¶s disease

more common tothose who reachmiddle adult life



Elsevier items and derived items © 2007 by Saunders, an imprint of Elsevier Inc.

Hemolytic Disease of theNewborn

Hemolytic Disease of theNewborn

Erythroblastosis Fetalis

Er throblastosis FetalisEr throblastosis Fetalis





(continued)


(continued)

z Diagnosis is confirmed by amniocentesis

and monitoring of bilirubin levels in the

amniotic fluid

z Diagnostic studies will help the physician todetermine if early interventions, such as

induction of labor or intrauterine fetal

transfusions are needed

Erythroblastosis FetalisErythroblastosis Fetalis





(continued)


(continued)

z Prevention

z Use of Rh(D) immune globulin (RhoGAM)

At 28 weeks for all Rh neg mothers

After an amnio/CVS

After birth if baby is Rh positive

After any abortion (spontaneous or induced)

If there is any trauma in pregnancy

If there is any bleeding during pregnancy






(continued)


(continued)

z Manifestations

z A direct Coombs¶ test isdone on umbilical cord blood

z Symptoms of this diseasevary

z Anemia and jaundice arepresent Anemia is caused by

hemolysis of large numbersof erythrocytes

P athological jaundice occurs

within 24 hours of delivery Liver cannot handle the

amount of hemolysis, bilirubinlevels rise rapidly

z The liver and spleenenlarge and extensiveedema may occur

z Oxygen-carrying capacityof the blood is

diminished, includingblood volume

z Infant at major risk of shock or heart failure






(continued)


(continued)

z Kernicterus is a

condition in which

bilirubin has reached

toxic levelsz Infant will have

jaundice along with

Irritability

Lethargy

Poor feeding

High-pitched shrill cry

Muscle weakness

Progresses to

opisthotonos

Seizuresz Bilirubin in the brain

tissue that has

accumulated can

cause serious braindamage and

permanent

disability

E th bl t i F t liE th bl t i F t li




Erythroblastosis Fetalis:

Treatment


Treatment

z Prompt identification

z Laboratory tests

z Drug therapy

z Phototherapy

z Exchange

transfusions, if

indicated

Erythroblastosis Fetalis:Erythroblastosis Fetalis:





Nursing Care


Nursing Care

z For phototherapy,ensure infant¶s eyes areprotected from the lights

z Although phototherapy

may prevent an increasein the level of bilirubin, ithas no effect on theunderlying cause of the jaundice

z Cover gonadsz Incubator care

z Exchangetransfusion requiresa central line (usuallythe umbilical vein)

z Nursing caresinclude Observing newborn¶s

color

Applying wet, sterile

compresses to theumbilicus, if ordered,until transfusions arecomplete




Nursing TipNursing Tip

z Assessing jaundice involves the following

The skin and the whites of the eyes assumes a

yellow-orange cast

Blanching the skin over the bony prominenceenhances the evaluation of jaundice

z Jaundice that occurs on the 1st day of life is

always pathological and requires prompt

intervention




Home PhototherapyHome Phototherapy

z Used for newborns with mild to moderate

physiological (normal) jaundice

z Less costly

z May decrease the need for hospitalization




ABO Incompatibility ABO Incompatibility

z Hemolytic disease with symptoms similar to

those of erythroblastosis can occur with

ABO incompatibility

z A mother who has an ³O´ blood type andwho gives birth to an infant with an A or B

blood group constitutes the most commonly

seen ABO incompatibility

z Treatment and nursing care are the same

as with erythroblastosis




Intracranial HemorrhageIntracranial Hemorrhage

z Most common type of

birth injury

z May result from trauma

or anoxia

z Occurs more often in

preterms

z

May also occur duringprecipitate delivery or

prolonged labor

z Depending on severity,some signs/symptomsmay be Poor muscle tone

Lethargy Poor sucking reflex

Respiratory distress

Cyanosis

Twitching

Forceful vomiting High-pitched cry

Convulsions





Intracranial Hemorrhage

(continued)


(continued)

z Diagnosis

History of traumatic

delivery

CT or MRI scan Evidence of an

increase in CSF

pressure

z Treatment

Oxygen

Gentle handling

Elevated head Medications may be

prescribed

Care with feeding

because suckingreflex may be

affected






(continued)


(continued)

z If convulsion occurs, notify physician

immediately; be ready to answer the

following questions:

Were the arms, legs, or face involved? Was the right or left side of the body involved?

Was the convulsion mild or severe?

How long did it last?

What was condition of infant before and after theseizure (i.e., vital signs, skin color)?

Transient Tachypnea of theTransient Tachypnea of the




Transient Tachypnea of the

Newborn (TTN)

Transient Tachypnea of the

Newborn (TTN)

z Characterized by

Tachypnea

May also include

Chest retractions Grunting

Mild cyanosis

z Often referred to as

R espiratory Distress Syndrome,

type II

z Typically resolves

suddenly after 3

days

May be caused fromslow absorption of

fluid in lungs after

birth

z Supportive nursingand medical care

Meconium AspirationMeconium Aspiration




Meconium Aspiration

Syndrome

Meconium Aspiration

Syndrome

z In utero

Fetus expels meconium into amniotic fluid

Cord compression or other condition interrupts

fetal circulation If asphyxia or acidosis occurs, fetus may have

gasping movements that cause meconium

stained amniotic fluid to be drawn into the lungs

z At delivery Can occur if newborn inhales before nose and

mouth have been suctioned

Meconium AspirationMeconium Aspiration




Meconium Aspiration

Syndrome (continued)

Meconium Aspiration

Syndrome (continued)

z Symptoms


Nasal flaring

Retractions Cyanosis

Grunting

Rales and rhonchi

Tachypnea maypersist for several

weeks

z Treatment

Warmth

Oxygen

Supportive care NICU

Neonatal AbstinenceNeonatal Abstinence




Neonatal Abstinence

Syndrome (N AS)

Neonatal Abstinence

Syndrome (N AS)

z Fetal exposure to drugs in utero

z Many illicit drugs cross the placental barrier;

therefore, an infant born to a woman who is

an addict will suffer drug withdrawal after birth

z Infant may also have long-term

developmental and neurological deficits

Neonatal AbstinenceNeonatal Abstinence




Neonatal Abstinence

Syndrome (N AS) (continued)

Neonatal Abstinence

Syndrome (N AS) (continued)

z Symptoms

Hyperirritability

(primary sign)

Wakefulness Diarrhea

Poor feeding

Sneezing

Yawning

z Treatment

Swaddling

Quiet environment

Phenobarbital




Infant of Diabetic Mother Infant of Diabetic Mother

z Large amounts of glucose are transferred to

fetus

z Causes fetus to become hyperglycemic

z Fetal pancreas produces large amount of fetal insulin

z Leads to hyperinsulinism, along with excess

production of protein and fatty acids, often

results in an LGA newborn weighing 9

pounds (4082 g) or more (macrosomia)

Infant of Diabetic MotherInfant of Diabetic Mother




Infant of Diabetic Mother

(continued)

Infant of Diabetic Mother

(continued)

z After delivery, infant may

have low blood glucose

levels

z Has cushingoidappearance

z Size of infant makes it

look healthy, but it may

not bez May have developmental

deficits and suffer

complications of RDS

z Suffers from

Hypoglycemia

Hypocalcemia

Hyperbilirubinemiaz Requires close

monitoring for the

first 2 days of life

z Monitor for Irritability

Tremors




Question for ReviewQuestion for Review

z How can prenatal care prevent neural tube

defects?

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Ch 14 Revised