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Peds > Chapter 21:The Newborn at Risk > Flashcards

Chapter 21:The Newborn at Risk Flashcards

Congenital Disorders

1
Q

What causes congenital malformations?

A

Genetic or environmental factors

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2
Q

What is Spina Bifida?

A

Defect in the neural arch of the lumbosacral region

Failure of the posterior laminae of the vertebrae to close; leaves an opening through which spinal meninges and spinal cord may protrude

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3
Q

Spina Bifida: What are the three types of degree of spinal cord anomalies?

A
  1. Occulta
  2. Meiningocele
  3. Myelomeningocele
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4
Q

What is spina bifida occulta?

A

A bony defect that occurs without soft tissue involvement

• no symptoms
• Dimple in the skin or a tuft of hair over the site

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5
Q

What is the spina bifida meningocele?

A

Spinal meninges protrudes through the bony defect and forms a cystic sac.

• no nerve roots involved
• No paralysis or sensory loss

Sac may rupture or perforate and cause infection leading to meningitis

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6
Q

What is spina bifida myelomeningocele?

A

Protrusion of the spinal cord and the meninges with nerve roots embedded in the wall of the cyst.

• Most severe
• Varies in severity
• Paralysis of lower trunk and legs

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7
Q

How is myelomeningocele diagnosed?

A

• Elevated alpha-fetoprotein (AFP)
• Clinical Observation
• MRI
• Monitor for associated defects
-Hydrocephalus
-Gu disorders
-Orthopedic anomalies

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8
Q

Myelomeningocele: Data collection (assessment)

A

• Routine Newborn Exam
• Observe movement and response to stimuli in lower extremities
• Measure head circumference**
-examine fontanels
• Prevent injury to sac

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9
Q

Myelomeningocele Nursing Focus:
Preventing Infection

A

• Prophylactic antibiotics
• Aseptic technique
• Prior to surgery: cover sac with sterile dressing moistened with warm sterile solution**
-moisten dressing every 2 hrs; do not let it dry**
• Protect Sac from contamination of fecal material with a protective barrier

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10
Q

Myelomeningocele Nursing Focus:
Preventing Injury

A

• Perform ROM exercises of lower extremities to prevent contracture
• Maintain prone position
• Massage knees and other bony prominences with lotion regularly, pad, and protect them from irritation

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11
Q

How would you promote family coping with Myelomeningocele?

A

• Family coping impaired due to perceived loss of ‘perfect’ newborn
• Be sensitive to caregiver needs and emotions
• Allow caregiver to express feelings and emotion
• Promote bonding

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12
Q

Myelomeningocele Family Teaching

A

• Provide information about defect
• Provide information about follow up care
• Refer to Spina Bifida Association

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13
Q

What is Hydrocephalus?

A

excess of cerebral spinal fluid (CSF) within the ventricular and subarachnoid spaces of the cranial cavity

Balance is disturbed

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14
Q

What happens when hydrocephalus occurs before the fontanelles close?

A

Suture lines separate to allow for head expansion
-rapid increase in head circumference

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15
Q

Clinical Manifestations of Hydrocephalus

A

• Excessively large head at birth
• Rapid head growth with widening cranial sutures
• Tense and fill anterior fontanelle
-towards forehead
• scalp becomes shiny and veins dilate
• Setting sun sign**
-eyes appear to be pushed downward and the sclera is visible above the iris

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16
Q

If hydrocephalus is not controlled ICP develops. What are symptom of ICP?
**

A

• irritability
• Restlessness
• Personality change
• High pitched cry
• Ataxia-poor muscle control
• Projectile vomiting
• Failure to thrive
• Seizures
• Severe headache
• Changes in LOC
• Papilledema-optic disc swelling

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17
Q

How is hydrocephalus diagnosed

A
  1. MRI
  2. Surgical-Ventriculoperitoneal shunt (VP shunt)
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18
Q

What is a VP shunt

A

procedure that drains excess CSF from the lateral ventricle through a subQ catheter.

As the child grows the catheter needs to be revised and lengthened.

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19
Q

Data Collection: Hydrocephalus

A
  1. Measure head circumference
    2.Monitor fontanelles for bulging
    3.Monitor for ICP
    -check pupils (PERRLA)
    -monitor for signs of seizures
    -monitor for lethargy/shrill cry
    4.Monitor VP shunt site for infection
    5.Frequent repositioning
    6.Support head and neck when moving
    7.Use egg crate, lambs wool, or special mattress to prevent skin breakdown
  2. Promote social interaction
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20
Q

What is a ventricular septal defect (VSD)?

A

Abnormal opening between the right and left ventricle.
Loud harsh murmur
Surgical repair with patch

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21
Q

What is atrial septal defect (ASD)?

A

Abnormal opening between the right and left atria.
Surgical repair with a patch or sutures

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22
Q

What is patent ductus arteriosus?

A

Vascular channel between the left pulmonary artery and descending aorta.

Needed in fetal circulation to bypass nonfunctioning lungs

Typically closes during first week of life

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23
Q

If PDA fails to close the blood flow is reversed and does what?

A

floods the lungs

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24
Q

What med is administered in attempt to close PDA?

A

Indomethacin; prostaglandin inhibitor

if not successful surgical correction will be done

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25
PDA is common with what newborns?
preterm newborns and newborns with Down Syndrome
26
How is PDA diagnosed?
Widened pulse pressure with bounding pulses. Systolic murmur
27
What is Tetralogy of Fallot (TOF)
Cyanotic heart defect 4 types of heart defects: 1. Pulmonary Stenosis-narrowing of the upper portion of the right ventricle of the valve cusps 2. Ventricular Septal Defect-abnormal opening in the septum between right and left ventricle 3. Overriding Aorta-aorta straddles the ventricle septum, overriding the VSD; allows a shunt of unsaturated blood from right ventricle into aorta 4. Right Ventricular Hypertrophy-increase in size of right ventricle
28
How does one with TOF present?
1. Infant presents with feeding problems and poor weight. 2. Anoxic spells or "tet" spells that can lead to LOC -treatment= knees to chest or squatting position 3. Requires surgical correction
29
Manifestations of Cleft Lip & Palate
1. Unilateral or bilateral 2. Small opening or involve the entire palate 3. May have nasal deformities or dental disorders
30
How is Cleft lip/palate diagnosed?
1.Physical appearance confirms 2. insert gloved finger into newborns mouth to assess palate
31
How is Cleft lip/palate treated?
Surgery between 6 months - 5 years Repair needed to ensure proper speech, nutrition, and dentition. May require several surgeries. Caregiver emotional support.
32
Post Op care: Cleft lip/palate
Elbow restraints Logan bow or butterfly closure Do NOT put anything in their mouth High risk for aspiration and malnutrition Provide adequate pain control
33
Cleft lip/palate family teaching
1. Sucking is important to speech development. 2. Holding the baby upright while feeding helps avoid choking. 3. Burp the baby frequently because a large amount of air is swallowed during feeding. 4. Don't tire the baby. Limit feedings to 20-30 mins max 5. Feed strained foods slowly from the side of the spoon in small amounts. 6. Don't be alarmed if food seeps through the cleft and out the nose 7. Have babies ears checked at any time they have a cold or upper respiratory infection. 8. No "baby talk" for speech development 9. Try to understand early talking without trying to correct the infant 10. Good mouth care is important. 11. Early dental care is essential to observe teething and prevent caries
34
Having no anal orifice is known as?
Imperforate Anus
35
Clinical Manifestations for Imperforate Anus
1. No anal orifice, may be a dimple 2. No meconium stool in 24 hours 3. Abdominal distention
36
Imperforate Anus is diagnosed and treated how?
Diagnosed: X Ray studies Treatment: Surgery- extent and type depends on defect May need colostomy
37
Nursing Care: Imperforate Anus
1. Family Education for colostomy care 2. Clean site with soap and water 3. Diaper the baby in a usual way
38
Abnormal protrusion of a part of an organ through a weak spot is known as?
A hernia
39
True or False Most hernias can be surgically repaired?
True
40
What is a diaphragmatic hernia?
Displacement of abdominal organs into left chest. Pushes heart to right and compresses left lung. Requires surgery
41
What is a Omphalocele hernia?
Abdominal contents protrude into the root of the umbilical cord. Cover with moistened gauze Requires surgery
42
What is a umbilical hernia?
Incomplete closure of umbilical cord. Common in preterm and African American infants. Bowel strangulation Surgery if not self corrected
43
Inguinal hernia
Primarily in males Intestines slip into scrotal sac Bowel strangulation possible Requires surgery
44
Omphalocele hernia: At birth what should be done until surgery
cover immediately with moist gauze and plastic wrap
45
What is hypospadias?
Urethra opening on ventral (underside) side of penis instead of tip. Cannot void in normal standing position. Surgical repair 6 &18 months
46
What is epispadias?
Urethra is on dorsal (top) side of the penis Cant void in normal standing position Surgical repair
47
True or False Circumcision should be delayed with hypospadias & epispadias
True Foreskin is used in repair
48
What is exstrophy of the bladder?
Anterior surface of the urinary bladder lies open on the lower abdomen
49
With exstrophy of the bladder why is a "waddling gait" formed?
due to widely split symphysis pubis and rotated hip sockets
50
External sex organs are incompletely or abnormally formed is known as?
Ambiguous Genitalia
51
Ambiguous genitalia: how is sex determined
Not possible to determine based on observation. Establish genetic sex ASAP Requires surgical reconstruction Parent will need support
52
With ambiguous genitalia is it easier to construct male or female sex organs?
Female. Possible to construct a functional vagina surgically and to administer hormones. It is less possible to adequately fixa penis
53
How do parents with newborns with ambiguous genitalia feel?
Guilt Anxiety Confusion
54
What is congenital talipes equinovarus commonly known as
Clubfoot
55
What is clubfoot?
Foot is inverted, the heel is drawn up, and forefoot is adducted. May be unilateral or bilateral Most common congenital foot deformity Can be seen on ultrasound in utero
56
How is clubfoot treated? -nonsurgical -surgical
Nonsurgical: use plastic splints or cast -cast changed frequently until radiographic evidence of correction Surgical: Used if no response to nonsurgical -involves lengthening of Achilles tendon and operating on bony structures -must be 10 or older
57
What is the Denis Browne splint?
Splint with shoes may be used to maintain correction for another 6 months or longer
58
With developmental dysplasia of the hip what is important?
Recognize the disorder before the child walks
59
Dysplasia of the hip is more common in what gender?
Females
60
What might be detected with dysplasia of the hip?
***audible click when examining the newborn using the Barlow sign & Ortolani maneuver** 1. Asymmetry of gluteal folds 2. Limited abduction of the affected hip 3. Apparent shortening of femur
61
Treatment: Developmental dysplasia of the hip
1. Use 2-3 diapers to keep legs abducted in a frog position 2. Use of brace, traction, or casting 3. May need surgical correction followed by a spica cast
62
Nursing Process: Infant in an orthopedic cast
1. Reinforce teaching about cast care. 2. Determine caregivers ability to understand and cooperate with infants care 3. Emotional support for family 4. Observe for signs that cast is drying evenly after application 5. Check toes for circulation and movement 6. Check skin at the edges of the cast for signs of pressure or irritation 7. Observe for signs of shock and bleeding during postop 8. Comfort 9. Meet growth and development needs of infant 10. Relieving pain and discomfort
63
What is Phenylketonuria (PKU)?
Recessive hereditary defect of metabolism. When untreated causes intellectual disability.
64
Clinical manifestations of PKU **
Frequent vomiting Mental disability Aggressive or hyperactive behaviors Convulsions Eczema Musty smell to urine
65
Treatment for PKU
1. Formula low in phenylalanine 2. Diet low in phenylalanine -omit most grains, meat, fish, dairy products, eggs, nuts, and legumes
66
What is congenital hypothyroidism?
absence of a thyroid or inability of the thyroid gland to secrete thyroid hormone
67
Clinical Manifestations: Congenital Hypothyroidism
1. S/S appear about 6 weeks of age 2. Depressed nasal bridge 3. Large tongue 4. Puffy eyes and face 5. Hoarse cry 6. Skin is dry and cool 7. Chronic constipation ** 8. Enlarged abdomen (poor muscle tone) ** 9. Poor feeders 10. Cry very little 11. Sleep for a long period of time
68
Treatment for Congenital Hypothyroidism
1. Monitor T3 & T4 and TSH levels 2. Administer replacement thyroid hormone (levothyroxine) 3. Medication therapy is needed for life.
69
What is Trisomy 21
Down Syndrome Physical and cognitive challenges Higher risk for women older than 35 yrs Various screenings during pregnancy detect trisomy 21 in utero.
70
Trisomy 21 clinical manifestations
1. Brachycephaly- shortness of head 2. Short stature 3. Upward slanted eyes with epicanthic fold 4. Short flattened bridge of nose 5. Protruding tongue 6. Deep crease on palm 7. Low muscle tone 8. Heart anomalies 9. Thick skin on neck
71
Treatment: Trisomy 21
Physical characteristics determine medical and nursing management Caregivers need strong support and education

Peds (3 decks)

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