Secondary Conditions Associated with Prematurity
Bronchopulmonary Dysplasia
Bronchopulmonary dysplasia, or BPD, refers to the chronic respiratory
condition which frequently develops in significantly preterm infants who
have required ventilatory support. This condition typically presents with
chronic respiratory distress, changes in the respiratory tract itself
- such as increased airway resistance, pulmonary edema, pulmonary hypertension
- persistent supplemental oxygen dependence, and radiographic aberrancies.
BPD may develop following respiratory interventions in the premature neonatal
period which involve mechanical ventilation and oxygen administration.
In addition to the development of chronic lung disease, infants with
BPD may also be affected by problems with growth/nutrition, developmental
progression, and cardiovascular functioning. Gastroesophageal reflux is
common in preterm infants and presents a risk of chronic aspiration that
may aggravate the already existing lung injury of BPD. Bronchopulmonary
dysplasia additionally places the infant at risk for problems with neurodevelopment,
such as learning disabilities, cerebral palsy, seizure disorders, sensory
impairments, and problems with speech production.
Premature infants commonly require respiratory support because their
lungs aren't fully developed. This support typically consists of supplemental
oxygen therapy, continuous positive airway pressure (CPAP), and/or mechanical
ventilation. For infants whose lungs are not yet making surfactant, synthetic
surfactant must be administered exogenously, as mechanical ventilation
in the absence of adequate surfactant may induce atelectrauma (lung injury
from low pressure) from the continuing ventilation of lung tissue with
poor surface tension, or volutrauma, from the overextension and rupture
of normal lung tissue. Appropriate surfactant therapy typically improves
respiratory outcome and decreases incidence of BPD. Clinicians should
be aware of the various problems that may present in the infant who has
been diagnosed with BPD.
Infants with BPD often go home on supplemental oxygen. The average length
of time the oxygen is needed is about three months; however, some infants
may require oxygen therapy for up to one year. Infants who sustain BPD
have the most problems during the first year of life. Colds and respiratory
infections are not uncommon, and children tend to get sicker than unaffected
peers. When they do develop a cold or respiratory infection, they require
oxygen therapy more frequently. BPD may retard growth in significantly
affected infants due to elevated energy requirements from increased respiratory
effort. A few babies will continue to have respiratory problems, such
as asthma, into adulthood.
The previous material was taken in small part from the Baby Awareness
and Support through Interactive Computer Systems (BASICS); a project supported
by the U.S. Department of Education and the University of Kentucky.
D'Angio, C.T., & Maniscalco, W.M. (2004). Bronchopulmonary
dysplasia in preterm infants: Pathophysiology and management strategies.
Pediatric Drugs, 6(5), 303-330.
Eisenberg, J.D. (2000). Chronic respiratory disorders.
In R.E. Nickel & L.W. Desch (Eds.), The physician's guide to caring
for children with disabilities and chronic conditions (pp. 622-624).
Baltimore, Maryland: Paul Brookes Publishing.
Retinopathy of Prematurity (ROP)
Retinopathy of Prematurity, usually referred to as ROP, is a condition
which often occurs in infants who are born prematurely. Visual impairments
frequently result from significant ROP, with blindness occurring in severe
cases. Historically ROP was seen as being the direct result of extensive
oxygen therapy in the postnatal period of a preterm infant. It is now
known that other factors may also be involved.
Around the fourth month of gestation the retinal blood vessels begin
growing from the area of the optic nerve in the back of the eye toward
the front of the eye, with vascularization typically complete at birth.
The preterm infant's retinal/visual development is interrupted by birth,
and the events that follow (oxygen therapy, ventilation, surfactant therapy,
etc.) somehow induce changes in the normal pattern of blood vessel growth.
Retinopathy of prematurity involves the proliferation of retinal blood
vessels in an abnormal pattern. When the vessel growth stops, this may
result in retinal scar formation. Contraction of this retinal scar may
actually cause the retina to detach - inducing blindness. ROP represents
the most common cause of retinal damage in infants. Infants with ROP are
additionally more likely to experience myopia, strabismus, glaucoma, and
blindness.
What cause the abnormal growth of blood vessels in ROP is still not completely
understood. Extensive postnatal oxygen therapy remains strongly correlated.
Premature infants should be evaluated regularly by an ophthalmologist
while in the neonatal intensive care unit, and at subsequent visits to
the clinician during the first few months of life. Early detection of
ROP may permit timely intervention, such as laser cauterization, which
may stop disease progression and avoid/reduce retinal detachment.
The previous material was taken in small part from
the Baby Awareness and Support through Interactive Computer Systems (BASICS);
a project supported by the U.S. Department of Education and the University
of Kentucky.
Desch, L.W. (2000). Visual and hearing impairments. In
R.E. Nickel & L.W. Desch (Eds.), The physician's guide to caring
for children with disabilities and chronic conditions (pp. 269).
Baltimore, Maryland: Paul H. Brookes Publishing.
Menacker, S.J. & Batshaw, M.L. (1997). Vision: Our
window to the world. In M.L. Batshaw (Ed.), Children with disabilities
(pp. 220-221). Baltimore, Maryland: Paul H. Brookes Publishing.
Necrotizing Enterocolitis (NEC)
Necrotizing enterocolitis is a potentially life-threatening condition
occurring in approximately 2-5 % of very low birth weight infants. NEC
carries an estimated 20% mortality rate. It represents the most frequent
gastrointestinal emergency occurring in neonatal intensive care units.
The etiology of NEC appears to be related to multiple factors, including
preterm delivery, hypoxia, administration of enteral tube feedings, and
toxicity to intestinal microorganisms. Of these factors, only prematurity
is consistently correlated with the occurrence of NEC. Even though NEC
has been recognized as a disease entity for a century, the exact pathogenesis
remains unclear.
Infants with necrotizing enterocolitis typically present with abdominal
distention, sluggishness, vomiting, and/or abdominal tenderness, which
may progress to more severe symptoms of intestinal bleeding or actual
perforation if gone unchecked. Most experts agree that NEC develops due
to the interaction of three basic factors: premature administration of
enteral tube feedings; ischemia of the intestinal wall related to the
action of vasoconstrictive substances, such as platelet activating factor
(PAF); and inflammatory response to bacterial toxins.
Treatment of NEC within the NICU setting involves the judicious management
of enteral tube feeding protocols, nasogastric suctioning to decrease
bowel distention, intravenous fluids administration, and often antibiotic
prophylaxis. Severely affected cases frequently require surgical resection
of the bowel to remove the necrotic tissue. It has been established for
some time that breast milk feeding offers some protection against the
development of NEC. Such is the importance in high risk infants that donor
human milk may be used when the mother is unable to provide needed lactation.
Breast milk may additionally be fortified to insure optimal nutrient provision.
Bernbaum, J.C., & Batshaw, M.L. (1997). Born too soon,
born too small. In M.L. Batshaw (Ed.), Children with disabilities
(pp.126-127). Baltimore, Maryland: Paul H. Brookes Publishing.
Lee, J.S., & Polin, R.A. (2003). Treatment and prevention
of necrotizing enterocolitis. Seminars in Neonatology, Dec 8(6),
449-450.
Updegrove, K. (2004). Necrotizing enterocolitis: The evidence
for use of human milk in prevention and treatment. Journal of Human
Lactation, Aug 20(3), 335-339.
Pellegrini, M., Lagrasta, N., Campos,
S.J., Garcia, C., & Marzocca, G. (2004). Necrotizing enterocolitis
in the preterm infant: Etiopathogenic considerations. Recent Progress
in Medicine (Italian journal), Aug 95(7-8), 384-387.
Intraventricular Hemorrhage (IVH)
Intraventricular hemorrhage refers to bleeding that occurs into the normally
fluid-filled spaces of the brain, or ventricles. The term may also be
used to describe bleeding which occurs into the brain tissue itself near
the ventricles. Preterm infants are at a significant risk for IVH, with
infants born most prematurely experiencing the greatest risk. IVH rarely
occurs in term infants.
IVH in preterm infants is related to the immaturity of blood vessels
in the brain. These vessels may easily rupture, allowing leakage of blood
into the surrounding tissue. Additionally, preterm infants often have
difficulty regulating blood flow to the brain. Alterations in brain blood
volume may further increase the chance of vessel rupture. Typically IVH
will occur during the first three days of life. Infants sustaining significant
IVH may experience long term effects such as hydrocephalus, learning disabilities,
brain injury, seizures, hearing or visual deficits, motor problems, and/or
delays in development. Hydrocephalus is the most common clinical complication
of IVH.
Intraventricular hemorrhages are graded into four levels according to
type, location, and severity of bleed. The majority of IVH's are Grade
I or II, and are generally considered "mild". Typically these
infants do as well as unaffected infants of similar gestational age. Infants
with Grade III and IV have increasing risk of brain injury and subsequent
developmental problems. Very low birth weight infants (less than 1000g)
and/or infants of 28 weeks or less gestation frequently develop cerebral
palsy (as many as 17 %) or other neurodevelopmental sequalae. Long-term
effects are most common in babies with Grade IV IVH, and/or those who
have developed hydrocephalus requiring treatment.
The previous material was taken in part from the Baby
Awareness and Support through Interactive Computer Systems (BASICS); a
project supported by the U.S. Department of Education and the University
of Kentucky.
Colvin, M., McGuire, W., & Fowlie, P.W. (2004). Neurodevelopmental
outcomes after preterm birth. British Medical Journal, 329, 1390-1393.
Retrieved online December 23, 2004 from http://bmj.bmjjournals.com/.
Periventricular Leukomalacia (PVL)
Periventricular leukomalacia is a term used to describe the damage and
subsequent necrosis which occurs in white matter areas of the brain. Preterm
infants are at risk for developing PVL due to both the immaturity of brain
structures and blood pressure regulating systems at birth. PVL may occur
as a result of either lack of blood flow to the white matter area or by
pressure created by ventricles filled with blood. Neurodevelopment may
be retarded or altered due to white matter injury. In significant cases
of PVL, neurological disorders, including cerebral palsy and/or mental
retardation may result. Preterm infants with PVL constitute approximately
35-40% of children with cerebral palsy (Nickel, 2000).
Over fifty percent of children who have incurred some degree of PVL have
normal intelligence. However, it is important for the clinician to be
alert for signs of developmental and/or cognitive delay, as early intervention
will improve outcome.
The above material was taken in part from the Baby Awareness and Support
through Interactive Computer Systems (BASICS); a project supported by
the U.S. Department of Education and the University of Kentucky.
Nickel, R.E. (2000). Cerebral palsy. In R.E. Nickel &
L.W. Desch (Eds.), The physician's guide to caring for children with
disabilities and chronic conditions, (pp. 143). Baltimore, MD: Paul
H. Brookes Publishing.
Cerebral Palsy: Potential Neurological Complications
Preterm infants, particularly those with birth weights less than 1500g
and/or gestational ages of less than 32 weeks, are at risk for delays
or deficits in neurodevelopment. Aberrant neuromotor function observed
in the first year of life may resolve with maturation (transient dystonias)
or evolve into frank cerebral palsy. Cerebral palsy is most strongly associated
with white matter injury occurring in very preterm infants who have sustained
periventricular leukomalacia (PVL) and/or Grade IV intraventricular hemorrhage.
Preterm infants with PVL constitute approximately 35-40% of children with
cerebral palsy (Nickel, 2000). Children who do not develop cerebral palsy
still may experience motor impairments which may not become evident until
they become school age.
Neuromotor disturbances represent the most common hidden disability occurring
in children of preterm birth. Additional risk factors for neuromotor dysfunction
include: frequent episodes of apnea and bradycardia during the neonatal
period; severe bronchopulmonary dysplasia; and male gender. The former
two factors may induce hypoxia of the brain tissues. Neurodevelopmental
dysfunction is also positively associated with lower cognitive outcome.
It is important for the clinician to be alert for signs/symptoms of neurosensory
or neuromotor dysfunction, as timely intervention may improve outcome.
Bracewell, M., & Marlow, N. (2002). Patterns of motor
disability in very preterm children. Mental Retardation and Developmental
Disabilities Research Reviews, 8(4), 241-248.
Colvin, M., McGuire, W., and Fowlie, P.W. (2004). Neurodevelopmental
outcomes after preterm birth. British Medical Journal, 329, 1390-1393.
Retrieved online 12/23/04 from www.bmj.com.
Janvier, A., Khairy, M., Kokkotis, A., Cormier, C., Messmer,
D., & Barrington, K. (2004). Apnea is associated with neurodevelopmental
impairment in very low birth weight infants. Journal of Perinatology,
24(12), 763-768. Retrieved online 12/26/04 from PubMed.com
Nickel, R.E. (2000). Cerebral palsy. In R.E. Nickel &
L.W. Desch (Eds.), The physician's guide to caring for children with
disabilities and chronic conditions, (pp. 143). Baltimore, MD: Paul
H. Brookes Publishing
Perlman, J.M. (2001). Neurobehavioral deficits in premature
graduates of intensive care: Potential medical and neonatal environmental
risk factors. Pediatrics, 108(6), 1339-1346. Retrieved online12/26/04
from www.pediatrics.org.
Intellectual (Cognitive) Disability
Infants born significantly preterm often experience some degree of hypoxia
during the perinatal and neonatal periods typically related to respiratory
and cardiovascular system immaturity. Hypoxia of brain tissues may result
in permanent damage. Additionally, bronchopulmonary dysplasia, intraventricular
hemorrhage (with or without hydrocephalus), and/or periventricular leukomalacia
may occur in very premature infants and further contribute to destruction
of brain tissue. Hypoxia-related injury sustained in the area of the hippocampus
and/or basal ganglia may contribute to neurobehavioral deficits as the
infant matures. Very sick low birth weight infants frequently have low
levels of thyroxine - despite normal thyrotropin levels. Because thyroid
hormones are necessary for normal brain development, these infants may
be at risk for neurodevelopmental abnormalities, such as lower IQ scores,
cerebral palsy, and learning disabilities. Hyperbilirubinemia is also
a frequent problem in sick preterm infants and may injure neurons - even
when the bilirubin level is not severely elevated. Finally, glucocorticoids
(steroids) administered to prevent or treat lung problems in the neonatal
period may alter the structure of the developing brain, particularly in
the area of the hippocampus.
Perlman, J.M. (2001). Neurobehavioral deficits in premature
graduates of intensive care: Potential medical and neonatal environmental
risk factors. Pediatrics, 108(6), 1339-1346. Retrieved online12/26/04
from www.pediatrics.org
Short, E.J., et al. (2003). Cognitive and academic consequences
of bronchopulmonary dysplasia and very low birth weight: 8 year outcomes.
Pediatrics, 112(5), 359-356. Retrieved online 12/26/04 from http://www.pediatrics.org
Yeh, T.F., et al. (2004). Outcomes at school age after
postnatal dexamethasone therapy for lung disease of prematurity. New
England Journal of Medicine, 350(13), 1304-1313.