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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
Q

PDA is common with what newborns?

A

preterm newborns and newborns with Down Syndrome

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26
Q

How is PDA diagnosed?

A

Widened pulse pressure with bounding pulses.

Systolic murmur

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27
Q

What is Tetralogy of Fallot (TOF)

A

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

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28
Q

How does one with TOF present?

A
  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
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29
Q

Manifestations of Cleft Lip & Palate

A
  1. Unilateral or bilateral
  2. Small opening or involve the entire palate
  3. May have nasal deformities or dental disorders
30
Q

How is Cleft lip/palate diagnosed?

A

1.Physical appearance confirms
2. insert gloved finger into newborns mouth to assess palate

31
Q

How is Cleft lip/palate treated?

A

Surgery between 6 months - 5 years

Repair needed to ensure proper speech, nutrition, and dentition.

May require several surgeries.

Caregiver emotional support.

32
Q

Post Op care: Cleft lip/palate

A

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
Q

Cleft lip/palate family teaching

A
  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
Q

Having no anal orifice is known as?

A

Imperforate Anus

35
Q

Clinical Manifestations for Imperforate Anus

A
  1. No anal orifice, may be a dimple
  2. No meconium stool in 24 hours
  3. Abdominal distention
36
Q

Imperforate Anus is diagnosed and treated how?

A

Diagnosed: X Ray studies
Treatment: Surgery- extent and type depends on defect
May need colostomy

37
Q

Nursing Care: Imperforate Anus

A
  1. Family Education for colostomy care
  2. Clean site with soap and water
  3. Diaper the baby in a usual way
38
Q

Abnormal protrusion of a part of an organ through a weak spot is known as?

A

A hernia

39
Q

True or False
Most hernias can be surgically repaired?

A

True

40
Q

What is a diaphragmatic hernia?

A

Displacement of abdominal organs into left chest.

Pushes heart to right and compresses left lung.

Requires surgery

41
Q

What is a Omphalocele hernia?

A

Abdominal contents protrude into the root of the umbilical cord.

Cover with moistened gauze

Requires surgery

42
Q

What is a umbilical hernia?

A

Incomplete closure of umbilical cord.

Common in preterm and African American infants.

Bowel strangulation

Surgery if not self corrected

43
Q

Inguinal hernia

A

Primarily in males

Intestines slip into scrotal sac

Bowel strangulation possible

Requires surgery

44
Q

Omphalocele hernia: At birth what should be done until surgery

A

cover immediately with moist gauze and plastic wrap

45
Q

What is hypospadias?

A

Urethra opening on ventral (underside) side of penis instead of tip.

Cannot void in normal standing position.

Surgical repair 6 &18 months

46
Q

What is epispadias?

A

Urethra is on dorsal (top) side of the penis

Cant void in normal standing position

Surgical repair

47
Q

True or False
Circumcision should be delayed with hypospadias & epispadias

A

True
Foreskin is used in repair

48
Q

What is exstrophy of the bladder?

A

Anterior surface of the urinary bladder lies open on the lower abdomen

49
Q

With exstrophy of the bladder why is a “waddling gait” formed?

A

due to widely split symphysis pubis and rotated hip sockets

50
Q

External sex organs are incompletely or abnormally formed is known as?

A

Ambiguous Genitalia

51
Q

Ambiguous genitalia: how is sex determined

A

Not possible to determine based on observation.

Establish genetic sex ASAP

Requires surgical reconstruction

Parent will need support

52
Q

With ambiguous genitalia is it easier to construct male or female sex organs?

A

Female.
Possible to construct a functional vagina surgically and to administer hormones.
It is less possible to adequately fixa penis

53
Q

How do parents with newborns with ambiguous genitalia feel?

A

Guilt
Anxiety
Confusion

54
Q

What is congenital talipes equinovarus commonly known as

A

Clubfoot

55
Q

What is clubfoot?

A

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
Q

How is clubfoot treated?
-nonsurgical
-surgical

A

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
Q

What is the Denis Browne splint?

A

Splint with shoes
may be used to maintain correction for another 6 months or longer

58
Q

With developmental dysplasia of the hip what is important?

A

Recognize the disorder before the child walks

59
Q

Dysplasia of the hip is more common in what gender?

A

Females

60
Q

What might be detected with dysplasia of the hip?

A

*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
Q

Treatment: Developmental dysplasia of the hip

A
  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
Q

Nursing Process: Infant in an orthopedic cast

A
  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
Q

What is Phenylketonuria (PKU)?

A

Recessive hereditary defect of metabolism.
When untreated causes intellectual disability.

64
Q

Clinical manifestations of PKU **

A

Frequent vomiting

Mental disability

Aggressive or hyperactive behaviors

Convulsions

Eczema

Musty smell to urine

65
Q

Treatment for PKU

A
  1. Formula low in phenylalanine
  2. Diet low in phenylalanine
    -omit most grains, meat, fish, dairy products, eggs, nuts, and legumes
66
Q

What is congenital hypothyroidism?

A

absence of a thyroid or inability of the thyroid gland to secrete thyroid hormone

67
Q

Clinical Manifestations: Congenital Hypothyroidism

A
  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
Q

Treatment for Congenital Hypothyroidism

A
  1. Monitor T3 & T4 and TSH levels
  2. Administer replacement thyroid hormone (levothyroxine)
  3. Medication therapy is needed for life.
69
Q

What is Trisomy 21

A

Down Syndrome

Physical and cognitive challenges

Higher risk for women older than 35 yrs

Various screenings during pregnancy detect trisomy 21 in utero.

70
Q

Trisomy 21 clinical manifestations

A
  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
Q

Treatment: Trisomy 21

A

Physical characteristics determine medical and nursing management

Caregivers need strong support and education

Peds (3 decks)

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