Newborn Care and Congenital Conditions

Newborn Care and Congenital Abnormalities

• A newborn whose mother has gestational diabetes may need CPR; refresh CPR knowledge.
• Lecture focuses on congenital abnormalities found at birth.

Hydrocephalus

• Ventricles in the brain overflow with cerebrospinal fluid due to obstruction of spinal fluid reabsorption.
• Weeks three and four of gestation are when the central nervous system forms.
• Initially, the baby's structure resembles an axe with the brain at the top and the spinal column extending down, encased in the dura.
• Cerebrospinal fluid bathes the nerves and brain tissue.
• Diagnosis:
  • Transillumination: shining a flashlight over the baby's skull in a darkened room to look for a halo effect.
  • CT scan or MRI to confirm and determine if the hydrocephalus is non-communicating (obstructed).
• Daily head measurements are necessary to monitor fluid increase in the skull.
• Treatment:
  • Permanent shunt placement to manage fluid reabsorption abnormalities.
  • Shunt extends from a ventricle in the brain to the peritoneum cavity.
  • Parents must recognize signs of increased intracranial pressure and know how to use a pump behind the ear to relieve excess fluid.
• Symptoms of increased intracranial pressure:
  • High-pitched cry
  • Excessive fussiness and irritability
  • Bulging anterior fontanelle
  • Seizures
• Post-surgery care:
  • Nurses must prevent infection at the insertion site.
  • Babies should be positioned upright to reduce strain on the neck and prevent tissue damage.
  • Frequent turning (every two hours) and use of gel pads or sheepskin to prevent skin breakdown.
  • Regular assessment using a Gravescale score to monitor skin condition.

Neural Tube Defects

• Occur around four weeks of gestation when the central nervous system is forming; failure of the neural tube to close results in spina bifida.
• Types of Spina Bifida:
  • Occulta
    • Often missed; may appear as a dimple at the base of the spine.
    • Can be hidden with a puffy area, dimple, or lipoma.
    • If asymptomatic, no intervention is needed.
  • Cystica
    • Includes meningocele and myelomeningocele, which are more severe.
    • Assessment during musculoskeletal examination involves running fingers down the spine to check for abnormalities.
  • Meningocele
    • A pouch or lipoma extends beyond the back, filled with cerebrospinal fluid.
    • Requires repair but causes less damage as there is no nerve damage.
  • Myelomeningocele
    • Most serious type; involves meninges and spinal nerves protruding into the lipoma.
    • Assess for function below the umbilicus; may result in paralysis and loss of bladder/bowel control.
• Immediate care for myelomeningocele:
  • C-section delivery is typical.
  • The baby is placed face down in the bassinet to prevent additional damage.
  • If open to the air, cover with sterile saline gauze to prevent infection.
  • Assess lower limb and bowel function over the first 24 hours.
  • Monitor urine and stool output to determine function.
  • Regular cleansing and pressure-relieving surfaces to prevent skin breakdown.

Cleft Lip and Palate

• Occurs during the first 15 weeks of fetal development.
• One of the most common congenital anomalies; affects sinuses and the hard palate.
• Cleft lip involves a fissure that can extend into the nasal passage.
• Palatoplasty (surgical repair) is ideally done before six months to ensure weight gain.
• Severe cases may require multiple surgeries.
• Cleft palate may occur with or without cleft lip and involves a hole in the hard palate.
• Requires a multidisciplinary approach:
  • Pediatrician
  • ENT surgeon
  • Speech therapist (including neonatal therapists)
• Post-operative care and feeding:
  • Minimize crying to reduce suture pressure.
  • Avoid non-nutritive sucking.
  • Clean the mouth with wet washcloths after feeding.
  • Use a special nipple to bypass the surgical site or defect.
• Breastfeeding is possible with specialized nipples.
• Maternal drug use during the first 15 weeks of pregnancy can cause cleft palate.
• Post-operative care includes:
  • Upright feeding position.
  • Slow feeding.
  • Use of longer nipples to bypass the surgical area.

Clubfoot

• Mild cases can result from the baby's position in the uterus.
• Treatment includes manipulative exercises to stretch muscles and tendons.
• X-rays determine severity; severe cases need surgery and casting.
• Passive stretching exercises are taught to parents.
• Goal is to ensure the baby can walk without problems.
• Cast care:
  • Ensure one finger can fit between cast and skin.
  • Monitor blanching of toes to ensure adequate circulation (re-blanch within three seconds).
  • Manage itching.
  • Potential need for cast removal and recasting due to baby's growth.
  • Parents may find cast removal scary due to vibration.

Developmental Hip Dysplasia

• The head of the femur pops out of the hip socket.
• In-hospital care includes double diapering.
• Orthopedic doctor determines the best treatment method.
• Assessment techniques:
  • Barlow test: looking for unequal thigh creases.
  • Ortolani test: feeling for hip dislocation with leg movement.
  • X-rays confirm diagnosis.
• Treatment options:
  • Pavlik Harness: maintains hip flexion and abduction for 4-8 weeks.
  • Spica cast: used if the harness is ineffective.
• Cast care considerations:
  • Using different cas materials; fiberglass and so forth.
  • Maintaining hygiene around diaper cutout.
  • Managing limited movement within the cast.
  • Adjust expectations for developmental milestones.

Newborn Screening Tests

• Tests are performed after the baby has been on food (breast milk or formula) for at least 24 hours; typically done on the day of discharge.
• Each state determines the specific tests; all states test for at least eight specific disorders, including PKU.
• Parents can petition the state to add additional tests if there is a known family history of a genetic disorder.
• Hospital and pediatrician contact information is sent with the test samples.
• Pediatricians are responsible for contacting parents with positive results.
• Common symptoms include:
  • Lethargy
  • Poor feeding
  • Hypotonia
  • Unusual odor
  • Vomiting
• Caution against changing formula without consulting a pediatrician.

Phenylketonuria (PKU)

• Genetic disorder resulting in the accumulation of phenylalanine.
• Guthrie blood test is done 48-72 hours after birth to screen for it.
• Requires a diet low in phenylalanine.
• Monthly blood draws to monitor levels between 2 and 10 milligrams per deciliter.
• Breastfeeding is possible under dietary supervision.
• Untreated PKU can cause:
  • Mental retardation
  • Convulsions
  • Behavioral problems

Maple Syrup Urine Disease

• Defect in breaking down leucine, isoleucine, and valine.
• Causes urine and earwax to smell like maple syrup.
• Increases serum levels of leucine, isoleucine, and valine leading to acidosis, cerebral degeneration, and death if untreated within two weeks.
• Symptoms:
  • Feeding difficulties
  • Loss of Moro reflex
  • Irregular respirations
• Requires specialized formula as breast milk contains lactose, which exacerbates the condition.

Galactosemia

• Baby cannot break down galactose (sugar in milk).
• Can cause cirrhosis of the liver and neurological deficits.
• Symptoms:
  • Lethargy
  • Vomiting
  • Hypotonia
  • Diarrhea
  • Failure to thrive
  • Jaundice
• Requires stopping breastfeeding and using a special formula.

Chromosomal Abnormalities: Down Syndrome

• Most common genetic disorder causing intellectual disability.
• Characterized by specific physical traits, although severity varies.
• Common features:
  • Eyes appear closer due to a flat nasal ridge
  • Small head
  • Round face
  • Flat nose
  • Single palmar crease
  • Short, thick hands
  • Shorter pinky finger
• Associated with certain health problems, such as congenital heart defects.
• Prenatal screening includes nuchal translucency ultrasound, although results can be equivocal.

Rh and ABO Incompatibility

Rh Incompatibility

• Occurs when an Rh-negative mother carries an Rh-positive baby and blood mixes.
• The mother produces antibodies that attack the baby's red blood cells.
• Treatment may include blood transfusions.

ABO Incompatibility

• Occurs when the mother and baby have incompatible blood types (e.g., mother is type O, baby is type A or B).
• Leads to rapid breakdown of red blood cells, causing jaundice.
• Bilirubin levels rise too quickly for the liver to process.
• If untreated, can lead to:
  • Irritability
  • High-pitched cry
  • Seizures
  • Kernicterus (brain damage due to bilirubin crossing into the brain)
• Assessment includes checking the sclera for jaundice.

Jaundice Management

• Physiologic jaundice is common around day four of life.
• Assess jaundice using Kramer's rule (forehead, sternum, nipple line).
• Diagnostic procedure is measuring bilirubin blood level.
• Treatment involves:
  • Phototherapy (ultraviolet lights)
  • Frequent feedings to promote bilirubin excretion
• Monitor temperature frequently.
• Ensure adequate hydration and nutrition.
• Assess gestational age and watch for signs of hypoglycemia.