Postpartum Nursing Care ELO D: Congenital Malformations and Perinatal Injury

Neural Tube Defects

• Most often caused by failure of the neural tube to close at either the cranial or caudal end of the spinal cord
• Result in hydrocephalus or spina bifida

Hydrocephalus

• Characterized by an increase of cerebrospinal fluid (CSF) within the ventricles of the brain
• Causes pressure changes in the brain and an increase in head size

Pathophysiology of Hydrocephalus

• Imbalance between the production and absorption of CSF or improper formation of the ventricles
• Can be congenital or acquired (tumor, sequela of infections, perinatal hemorrhage)
• Classified as noncommunicating or communicating:
  • Noncommunicating hydrocephalus results from the obstruction of CSF flow from the ventricles of the brain to the subarachnoid space.
  • Communicating hydrocephalus results when CSF is not obstructed in the ventricles but is inadequately reabsorbed in the subarachnoid space.

Manifestations of Hydrocephalus

• Signs and symptoms depend on the time of onset and the severity of the imbalance
• Classic sign is an increase in head size
• At birth, the head enlarges rapidly, the fontanelles bulge, and the cranial sutures separate
• Scalp is shiny, and the veins are dilated
• The pupils of the eyes may appear to be looking down and the sclera may be seen above the pupils
• Arnold-Chiari malformation is characterized by a foreshortened occiput suggesting pathology of the fourth ventricle, with the brain stem protruding through the cervical canal
• Dandy Walker syndrome is characterized by a prominent occiput; the condition usually involves an atresia of the foramen leading CSF out of the brain to the subarachnoid space
• The infant is often helpless and lethargic with poor muscle tone in the extremities
• The cry is shrill and high-pitched
• Irritability, vomiting, anorexia, and convulsions may occur
• In older children, headaches are a predominant symptom along with cognitive slowing, personality changes, spasticity, and other neurological signs

Diagnosis of Hydrocephalus

• Transillumination (inspection of a cavity or organ by passing a light through its walls)
• Daily head circumference measurements
• Echoencephalography, computed tomography (CT) scanning, and magnetic resonance imaging (MRI)
• Ventricular tap or puncture

Treatment of Hydrocephalus

• Diuretics (Acetazolamide and furosemide) to reduce CSF production
• Surgery to bypass or shunt the point of obstruction

Nursing Care for Hydrocephalus

• Preoperative nursing care:
  • Most often, surgical correction can be accomplished in utero via fetal surgery or shortly after birth to prevent brain damage caused by increased intracranial pressure
  • Routine preoperative care involves assessment of vital signs, skin preparation, and emotional support of the parents
  • General nursing care includes:
    • Frequent position changes to prevent hypostatic pneumonia and pressure sores. Support the head when changing positions.
    • Use a lamb's wool or sponge rubber pad under the head to prevent skin breakdown.
    • Calm, unhurried feedings in a quiet room. Place infant on their side after feeding to reduce vomiting.
    • Observe fontanelles for size and bulging. Measure and record head circumference.
    • Report signs of increased intracranial pressure (increased BP, decreased P, and signs of infection)
• Postoperative nursing care:
  • Observe for signs of increased intracranial pressure, infection, and manage pain
  • Bacterial infection is a life-threatening complication. Observe for infection at the operative site, along the shunt line, and in the diaper.
  • Signs of increased intracranial pressure include a high-pitched cry, unequal pupil size, bulging fontanelles, irritability or lethargy, poor feeding, and abnormal vital signs.
  • Positioning of the infant depends on fontanelle status.
  • Assess skin integrity.
  • Record head circumference and abdominal measurements.
  • Record I&O and observe for signs of fluid overload.
  • Provide patient education and support.

Prognosis of Hydrocephalus

• Improved with modern medication and surgical techniques to an 80% survival rate when treated early
• Approximately 1/3 of cases result in normal physical and neurological functions
• Other survivors may have varying degrees of developmental disabilities

Spina Bifida

• A group of central nervous disorders characterized by malformation of the spinal cord, also known as myelodysplasia

Pathophysiology/Manifestations of Spina Bifida

• Imperfect closure of the spinal vertebrae
• Two forms:
  • Occulta (hidden): Small opening with no associated protrusion of structures.
  • Cystica (sac or cyst): Development of a cystic mass in the midline of the opening in the spine.
    • Meningocele contains portions of the membranes and CSF.
    • Meningomyelocele contains membranes, CSF, and spinal cord and is more serious.
    • May be associated with paralysis of the legs and poor control of bowel and bladder functions.
    • Hydrocephalus is a common complication.

Prevention of Spina Bifida

• Cause is unknown
• Use of drugs or poor nutrition may contribute to the development
• Folic acid supplementation is recommended for women of childbearing age.

Treatment of Spina Bifida

• Surgical closure of the spinal vertebrae
• Multidisciplinary approach required due to potential associated complications (hydrocephalus, orthopedic problems, urinary and bowel difficulties)
• Habilitation (learning rather than relearning) is the preferred term for treatment following surgery

Nursing Care for Spina Bifida

• Main objectives are:
  • Prevention of infection
  • Prevention of injury to the sac
  • Correct positioning
  • Good skin care
  • Adequate nutrition
  • Accurate observations and charting
  • Education of the parents
  • Continued medical supervision
  • Habilitation
• Immediate care of the sac: Place a moist sterile dressing of saline or an antibiotic solution to prevent drying.
• Routine observations: Size of the sac, tears or leakage, extremity deformities and movement, head circumference, fontanelles, rectal sphincter control.
• Positioning: Prone with a pad between the legs and a small roll under the ankles to prevent pressure on the sac and postural deformities.
• Provide cuddling and sensory stimulation.
• Postoperative nursing care: Neurological assessment, infection prevention, urological monitoring, and meticulous skin care.
• Inform parents of the potential for latex allergies and food sensitivities related to those allergies.
• Provide support and guidance to parents about the disorder.
• Obtain information and resources from the Spina Bifida Association of America.

Cleft Lip

• Characterized by a fissure or an opening of the upper lip
• Resulting from the failure of the maxillary and median nasal processes to unite during embryonic development, usually between the 7th or 8th weeks of gestation
• Seems to be caused by an autosomal dominant hereditary predisposition
• More frequent in boys and may occur on one or both sides of the lip
• Common congenital anomaly, occurring in about 1 in 600 births
• Occurs more often in Asian Americans and Native Americans

Treatment of Cleft Lip

• Initial treatment is surgical repair (cheiloplasty)
• Repair is completed before age 6 months when weight gain is stabilized and the infant is free from infections
• Surgery improves the infant's sucking ability and appearance

Nursing Care for Cleft Lip

• Preoperative:
  • Complete physical exam and routine bloodwork
  • Photographs may be taken.
  • Report any oral, respiratory, or systemic infections.
  • Arm restraints may be ordered.
  • Feeding via syringe with a rubber tip or a long nipple with a large hole.
• Postoperative:
  • Prevent sucking and crying to reduce tension on the suture line.
  • Careful positioning to prevent injury to the operative site (never on the abdomen).
  • Elbow restraints and Logan bow to immobilize the upper lip.
  • Provide emotional support.
  • Provide appropriate pain relief and sedation.
  • Feedings by dropper until the wound is completely healed.

Cleft Palate

• Failure of the hard palate to fuse at the midline during the 7th to 12th weeks of gestation.
• Separation forms a passageway between the nasopharynx and the nose
• Complicates feeding
• Leads to infections of the respiratory tract and middle ear
• Cleft palate may not be readily apparent at birth
• Feeding is a problem because the cleft prevents negative pressure from being formed in the mouth, which is necessary for successful sucking
• Speech may be affected later in life

Treatment of Cleft Palate

• Goal of therapy is union of the cleft, improved feeding, improved speech, improved dental development, and nurturing a positive self-image
• Surgery is preferred between 12-18 months of age
• Requires multidisciplinary teamwork: surgeon, pediatrician, pediatric dentist, orthodontist, nurse, psychologist, speech therapist, and social worker
• Psychosocial adjustment of the family is crucial to the infant's self-image.

Postoperative Nursing Care for Cleft Palate

• Nutrition: Fluids offered by cup and soft diet. Hot foods and liquids are avoided. No sucking on straws.

Postoperative Nursing Care for Cleft Lip/Palate Repair

• Preventing injury to the operative site:
  • Consult with physician about feeding methods like sucking to minimize stress and crying
  • Use comfort measures to reduce crying
• Positioning:
  • Use elbow restraints to prevent the child from placing fingers or other objects in the mouth
  • Use Logan bow position to maintain proper positioning
  • Avoid abdominal positioning
• Prevention of infection:
  • Gently cleanse sutures
  • Use antiseptic mouthwash
• Emotional care:
  • Provide cuddling and other means to express love and comfort
• Pain relief:
  • Provide pain relief or sedation as needed for the infant

Clubfoot

• Pathophysiology:
  • A congenital deformity characterized by a foot that is twisted inward or outward
  • Incidence of 1 in 1000 births
  • Mild cases can be fixed with manipulative exercises
  • True clubfoot cannot be fixed with simple exercises
  • Most common type is talipes equinovarus where the feet are turned inward and the child walks on the toes and outer border of the feet
  • Generally affects both feet
• Treatment:
  • Treatment should begin as early as possible to avoid abnormal bone and muscle development
  • Conservative treatment involves splinting or casting the foot in the correct position, performed during infancy
  • Passive stretching exercises are recommended
  • Surgery may be necessary if conservative methods are ineffective by 3 months of age
• Nursing Care:
  • Cast Care:
    • Plaster casts dry from the inside out over 24-48 hours, they should be left uncovered and protected from pressures
    • The leg and foot may be elevated on pillows to reduce swelling
    • Moving the leg with a cast requires using the palms of the hands, not fingers, to avoid damaging the skin
    • Synthetic casts are lighter, dry faster and are water-resistant
    • Toes are left exposed for observation of capillary refill and signs of poor circulation
    • If circulation is poor, the cast can be split to relieve pressure or removed and reapplied
    • The cast may need to be removed and reapplied as the infant grows
    • If surgery is performed, monitor for bleeding and vital signs
  • Educate parents about orthopedic devices, cast care, exercise, hygiene, and treatment goals
  • Provide support for financial burdens associated with treatment

Developmental Hip Dysplasia

• Pathophysiology:
  • The head of the femur is partly or completely displaced due to a shallow hip socket
  • Heredity and environmental factors play a role
  • Hip malformation, joint laxity, breech position, and maternal hormones contribute
  • Seven times more common in girls than boys
  • Early detection and treatment are crucial before ossification occurs
  • High risk in cultures where newborns are wrapped snuggly with hips adducted and extended
• Manifestations:
  • Often discovered during periodic health examinations in the first or second month of life
  • Limited abduction of the leg on the affected side
  • Asymmetry of skin folds and shortening of the femur are early signs of dislocation
  • Ortolani’s sign: a "clicking" sound as the femoral head is felt to slip back into the acetabulum
  • Barlow’s test: feeling the dislocation as the femur leaves the acetabulum when adducting and extending the hips while stabilizing the pelvis
  • Radiographic studies confirm the diagnosis
• Treatment:
  • Treatment begins immediately upon detection of the dislocation
  • Hips are maintained in constant flexion and abduction for 4-8 weeks to keep the femur within the socket
  • Pavlik harness is used for long-term immobilization for severe dislocation or when dislocation is detected later
  • Spica cast can maintain position, the length of time varies and the cast usually needs to be changed every 6 weeks
  • Surgery may be required in infants older than 18 months who don't respond to treatment
• Nursing Care:
  • Carefully observe infants during baths for signs of hip dysplasia, such as variations in buttock size, leg length, kicking with one leg only, and asymmetric skin folds
  • Note gait and posture in older children
  • Guide parents on the care and application of the Pavlik Harness
  • Neurovascular assessments need to be completed on infants in a body spica cast
  • Provide firm, plastic-covered pillows for support
  • Overhead bar and trapeze can benefit older children
  • Slightly elevate the head of the bed to help urine and feces drain away from the body
  • Frequent changes of position are essential
  • Allow older children to be involved in turning processes for a sense of control
  • Position older children on their abdomen during mealtime to ease swallowing
  • Provide toys that can be used while prone
  • Leave articles and toys within reach to promote independence
  • Frequent adjustments in home and clinical care are necessary due to rapid growth and development
  • Include the child in everyday family and play activities to encourage normal development

Phenylketonuria (PKU)

• Pathophysiology:
  • Faulty metabolism of phenylalanine, an amino acid found in protein foods
  • Phenylalanine hydrolase enzyme converts phenylalanine into tyrosine, but it's missing in classic PKU
  • Transmitted by an autosomal recessive gene
  • Phenylalanine builds up in the bloodstream when the infant is fed breast milk or formula
  • Phenylpyruvic acid appears in the urine in early life
  • PKU occurs more commonly in blonde and blue-eyed children
• Manifestations:
  • Severe retardation is evident in infancy
  • Infant appears normal at birth but shows delayed development by 4-6 months
  • Failure to thrive
  • Skin conditions like eczema
  • Peculiar musty odor
  • Personality disorders
  • Seizures
• Diagnosis:
  • Guthrie blood test is the most reliable test, performed on blood obtained by a heel stick
  • The test should be performed 48-72 hours after birth, after protein ingestion
  • Quantitative elevations of phenylalanine in the blood confirm the diagnosis
• Treatment:
  • Close dietary management is essential
    • Formulas: Lofenalac, Phenex-1, Phenyl-Free, and Phenex-2
    • Breastfeeding: Partial breastfeeding with Lofenalac supplementation
    • Solid foods: Introduction of solid foods low in phenylalanine at the same time as children without PKU
  • A dietitian can provide guidance and support in maintaining the diet regimen
  • Children with PKU must avoid aspartame
  • Flavoring substitutes can increase compliance
  • Frequent evaluation of blood phenylalanine levels is crucial
    • The goal is to maintain phenylalanine levels between 2-10 mg/dL
    • Levels below 2 mg/dL can result in growth retardation
    • Levels above 10 mg/dL can cause brain damage
  • Genetic counseling is important for family planning
    • Women with PKU need to follow a low-phenylalanine diet before conception
  • Sapropterin dihydrochloride (Kuvan) is the first drug available to treat this disorder

Maple Syrup Urine Disease

• Pathophysiology:
  • Caused by a defect in the metabolism of branched-chain amino acids (leucine, isoleucine, and valine)
  • Results in severe serum elevations of these amino acids
  • Leads to acidosis, cerebral degeneration, and death within 2 weeks if untreated
• Manifestations:
  • The infant appears healthy at birth but develops feeding difficulties
  • Loss of Moro reflex, irregular respirations, and convulsions
  • Urine, sweat, and earwax have a sweet or maple syrup odor (ketoacidosis)
  • Urine contains high levels of leucine, isoleucine, and valine
• Diagnosis:
  • Confirmed by blood and urine tests
• Treatment:
  • Early detection in the newborn period is critical
  • Treatment involves removing these amino acids and their metabolites from the body through hydration and peritoneal dialysis
  • Lifelong diet low in leucine, isoleucine, and valine
  • Special formulas are available
  • Exacerbations can be life-threatening and are related to abnormal leucine levels and infections

Galactosemia

• Pathophysiology:
  • The body is unable to use the carbohydrates galactose and lactose.
• Manifestations:
  • Galactosemia affects the liver, kidneys, brain, and eyes.
  • Symptoms typically appear within a few days to weeks after birth
  • Vomiting, diarrhea, failure to gain weight
  • Jaundice, lethargy, and seizures
  • Cataracts and liver damage
• Diagnosis:
  • A blood test can detect the presence of galactose in the blood
  • A urine test can detect the presence of galactose in the urine
• Treatment:
  • Remove galactose from the diet
  • Avoid lactose-containing foods, such as milk, cheese, and yogurt
  • Soy-based formula is a suitable alternative for infants
  • Long-term treatment is necessary for prevention of complications

Galactosemia

• A disorder where an enzyme is defective or missing.
• Causes a disturbance in a normal chemical reaction.
• Results in an increase of galactose in the blood and urine.
• Can result in:
  • Cirrhosis of the liver
  • Cataracts
  • Intellectual impairment
• If left untreated.
• Symptoms start abruptly and worsen gradually.
• Early warning signs are lethargy, vomiting, hypotonia, diarrhea, and failure to thrive.
• Jaundice may be present.
• Can be confirmed with blood and urine tests, including galactosemia, galactosuria, and evidence of reduced enzyme activity in red blood cells.
• Screening tests are available.
• Treatment is to avoid milk and lactose containing products in the diet.
  • This includes the nursing mother discontinuing breastfeeding.
  • Lactose-free formulas and soy protein based formulas are often substituted.

Down Syndrome

• One of the most common chromosomal abnormalities.
• Occurs in around 1 in 700 births in the US.
• Most common cause of genetic intellectual disability.
• There are three phenotypes of Down Syndrome:
  • Trisomy 21- 95% of cases
  • Mosaicism
  • Translocation
• Screening begins in the first trimester with an ultrasound and nuchal translucency assessment.
• Second trimester quad test that involves testing blood for levels of AFP, UE, inhibin-A, and hCG.
• Pregnant-associated plasma protein A (PAPP-A) can also indicate a risk for Down syndrome.
• Diagnosed by clinical manifestations.
• Clinical signs are present at birth, which include:
  • Close set, upward slanting eyes
  • Small head
  • Round face
  • Flat nose
  • Protruding tongue
  • Short and thick neck
  • Simian crease
  • Short and thick hands
  • Little finger curved and doesn’t extend past the distal joint
  • Wide space between the first and second toes
  • Underdeveloped muscles and loose joints
  • Physical growth may be slower than normal.
  • The child is often intellectually limited and has been found to have IQ’s in the borderline to low-average range.
  • Congenital heart deformities are associated.
  • Resistance to infection is poor, and they are prone to respiratory and ear infections.
  • There are often speech and hearing problems.
  • Lifespan has increased but they are at a higher risk for acute leukemia and Alzheimer’s disease.

Hemolytic Disease of the Newborn (Erythroblastosis Fetalis)

• A disorder that becomes apparent during fetal life or soon after birth.
• Occurs when an Rh-negative mother and an Rh-positive father produce an Rh-positive fetus.
• Mother’s body creates antibodies that destroy fetal blood cells, leading to anemia in the fetus and newborn.
• Early detection and prophylactic treatment with Rh immune globulin (RhoGAM) has made it rare today.
• Extensive maternal health history is gathered, including previous Rh sensitizations.
• Indirect Coombs’ test is used to detect the presence of Rh-positive antigens.
• Amniocentesis is done to confirm the diagnosis.
• The fetal RH status can be determined noninvasively with free DNA in maternal plasma.
• Prevention is accomplished by using RhoGAM at 28 weeks of gestation and within 72 hours of delivery.
• Also administered after spontaneous or therapeutic abortion, amniocentesis, and after bleeding during pregnancy.
• A direct Coombs’ test is done on cord blood at delivery.
• Symptoms include:
  • Anemia
  • Jaundice
  • Excessive immature RBCs
  • Enlarged liver and spleen
  • Extensive edema
• Bilirubin toxicity can lead to kernicterus.
• Treatment can be done before or after birth and includes:
  • In utero intravascular transfusion of packed RBCs
  • Exchange transfusion
  • Phototherapy
  • Intravenous immunoglobulin (IVIG)
• Nursing care for phototherapy includes:
  • Protecting the infant’s eyes from the lights.
  • Protecting gonads from heat damage.
  • Observing for maculopapular rash.
  • Observing for jaundice and bronze baby syndrome
  • Monitoring for pressure areas.
  • Maintaining adequate fluid volume.
  • Monitoring the infant’s temperature.
  • Observing for neurological deficit, such as twitching or lethargy.
  • Providing feedings and assisting with breastfeeding.
  • Providing reassurance and education to parents.

Nursing Care for a Newborn with Hemolytic Disease

• Monitor newborn's body temperature to provide a neutral thermal environment
• Check laboratory values and report any abnormal levels or spread of jaundice discoloration.
• Provide information to the newborn's parents about his condition and treatment interventions

Intracranial Hemorrhage

• Most common type of birth injury, can occur due to trauma or anoxia
• Occurs more often in preterm infants with fragile blood vessels
• Can also occur during precipitate delivery, prolonged labor, or when the newborn's head is large compared to the mother's pelvis
• Location of hemorrhage can be subdural, subarachnoid, or intraventricular

Manifestations of Intracranial Hemorrhage

• Signs can occur suddenly or gradually depending on severity
• Poor muscle tone
• Lethargy
• Poor sucking reflex
• Respiratory distress
• Cyanosis
• Twitching
• Forceful vomiting
• High-pitched, shrill cry
• Convulsions
• Opisthotonos posturing
• Tense, bulging fontanelle
• Pupil dilation or inequality

Treatment of Intracranial Hemorrhage

• Place infant in incubator for temperature control, administer oxygen, and continuous observation
• Administer vitamin K to control bleeding
• Administer phenobarbital for twitching or convulsions
• Prophylactic antibiotics and vitamins may be used

Nursing Care for Intracranial Hemorrhage

• Handle infant gently and elevate head to decrease intracranial pressure
• Feed carefully as the sucking reflex may be affected and infant vomits easily
• Monitor for signs of increased intracranial pressure: perform neurochecks, monitor vital signs, measure head circumference, palpate fontanelles
• Observe and document the character of convulsions to help determine the location of the bleeding
• Assist the healthcare provider with procedures such as lumbar puncture and aspiration of subdural hemorrhage

Transient Tachypnea of the Newborn (TTN)

• Usually occurs after cesarean section birth or rapid vaginal delivery
• Thought to be caused by slow absorption of lung fluid after birth

Manifestations of TTN

• Characterized by tachypnea, chest retractions, grunting, and mild cyanosis
• Resolves within 3 days

Treatment for TTN

• Supportive care
• Providing warmth
• Energy conservation
• Supplemental oxygen

Meconium Aspiration Syndrome (MAS)

• Occurs when fetus or newborn aspirates meconium-stained amniotic fluid into the lungs
• Can occur during prolonged labor or if the infant takes their first breath before the nose and mouth are suctioned

Manifestations of MAS

• Respiratory distress, including nasal flaring, retractions, cyanosis, grunting, rales, and rhonchi

Treatment for MAS

• Supportive care
• Providing warmth
• Energy conservation
• Supplemental oxygen
• Intubation and mechanical ventilation may be necessary

Neonatal Abstinence Syndrome (NAS)

• Occurs when a fetus is exposed to drugs such as opiates, amphetamines, tranquilizers, or multiple illicit drugs while in utero
• The infant born to an addicted mother is physiologically dependent and experiences withdrawal symptoms after birth

Manifestations of NAS

• Body tremors and hyperirritability
• Wakefulness, diarrhea, poor feeding, sneezing, and yawning

Treatment of NAS

• Provide a quiet environment
• Swaddling
• Reduce external stimuli
• Close observation for seizures

Infant of a Diabetic Mother

• Diabetes in the mother can present various problems for the newborn
• Effects depend on the duration and control of the mother's condition
• Well-controlled diabetes leads to minimal adverse effects
• Poorly controlled diabetes can lead to serious complications
• High glucose levels are transferred to the fetus, leading to hyperglycemia, hyperinsulinism, and macrosomia.
• Infant may be large for gestational age (LGA)

Manifestations of Infant of a Diabetic Mother

• Infant may experience birth injuries due to size
• Hypoglycemia after birth due to loss of maternal glucose and overproduction of insulin
• Round, puffy face due to increased subcutaneous fat
• May have developmental deficits, respiratory distress syndrome (RDS), or congenital anomalies
• Infants with poorly controlled diabetes in the mother may be small for gestational age due to poor placental perfusion, and experience hypoglycemia, hypocalcemia, and hyperbilirubinemia

Nursing Care of Infant of a Diabetic Mother

• Close monitoring of vital signs
• Early feeding and frequent assessment of blood glucose levels for the first 2 days of life
• Observe for irritability, tremors, and respiratory distress

Nursing Care for Infants with Congenital Anomalies and Perinatal Injuries

• Emphasize the importance of addressing individual needs based on specific conditions
• Provide emotional support to the infant and parents
• Encourage family involvement in care
• Educate families on the importance of early intervention and long-term follow-up