Neurodevelopemental Assessment and Care of Premature


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Neurodevelopemental Assessment and Care of Premature

  1. 1. <ul><li>Neurodevelopmental Assessment and Care of Premature Infants </li></ul><ul><li>Ma. Teresa C. Ambat, MD </li></ul><ul><li>Neonatology-TTUHHSC </li></ul><ul><li>10/28/2008 </li></ul>
  2. 2. Introduction <ul><li>PCPs should be vigilant in following outcomes of prematurely born child </li></ul><ul><li>Should integrate and adapt assessments of development, neurologic status and bahavior for each child at each encounter </li></ul>
  3. 3. Neurodevelopmental and Behavioral Outcomes <ul><li>Long-term outcome arises from complex interplay of biologic, genetic, social and environmental factors </li></ul><ul><li>Additional or shifting developmental dysfunction over time, as more subtle disabilities become increasingly apparent and testable “new morbidities” </li></ul>
  4. 4. Risk Factors for Developmetal and Behavioral Problems in the Preterm Infant <ul><li>Prenatal </li></ul><ul><li>LBW </li></ul><ul><li>GA <28 wks </li></ul><ul><li>IUGR </li></ul><ul><li>Male gender </li></ul><ul><li>Postnatal </li></ul><ul><li>Neonatal seizures </li></ul><ul><li>Abnormal HUS (white matter injury, </li></ul><ul><li>PVL, Grade 3-4 IVH) </li></ul><ul><li>CLD Prolonged mechanical vent </li></ul><ul><li>Infections (NEC, sepsis, meningitis) </li></ul><ul><li>Feeding problems >34 wks PMA </li></ul><ul><li>ECMO </li></ul><ul><li>Low economic status </li></ul><ul><li>Maternal depression </li></ul>
  5. 5. Prevalence of Significant Disabilities in VLBW Mental retardation 10-20% CP 5-21% Blindness 2-11% Deafness 1-3% Motor delay 24% Language problems 23-42% ADHD 7-10% Need for special education 9-28% Psychological/behavioral problems 25%
  6. 6. Correction for Prematurity <ul><li>Correction for prematurity up to 2-3 years of age when considering neurologic, developemental or behavioral issues, otherwise indicated by a standardized evaluation </li></ul>
  7. 7. Pearls on Outcomes of Preterm Infants <ul><li>More frequent and significant disabilities are associated with decreasing GA and BW </li></ul><ul><li>Cognitive deficits > motor deficits </li></ul><ul><li>Disabilities or delays may be subtle or appear latently </li></ul><ul><li>Deficits in cognitive, verbal, perceptual, motor and visuo-motor measures may not manifest until school age </li></ul>
  8. 8. Pearls on Outcomes of Preterm Infants <ul><li>Up to 50% of infants born <25 wks may be found without disability at follow up over the 1 st 3 yrs </li></ul><ul><li>Nearly all infants with normal findings on neurodevelopmental examination at the infant’s expected due date continue to develop normally </li></ul><ul><li>If no developmental delays during infancy, risk of MR or CP is low </li></ul>
  9. 9. Ophthalmologic Issues <ul><li>Ophthalmologic problems of premature infants </li></ul><ul><ul><li>ROP, strabismus, myopia – common </li></ul></ul><ul><ul><li>Higher incidence of visual impairment – 45-60% </li></ul></ul><ul><ul><li>Poor visual function may directly affect the development of motor and cognitive skills </li></ul></ul><ul><li>Require specialized ophthalmologic testing and routine follow-up by pediatric ophthalmologist </li></ul>
  10. 10. ROP Screening <ul><li>AAP recommendation for ROP screening </li></ul><ul><ul><li>Indications </li></ul></ul><ul><ul><ul><li>GA <30 wks or BW <1500g </li></ul></ul></ul><ul><ul><ul><li>Selected infants with BW 1500 – 2000g, GA >30 wks with severe cardiorespiratory instability </li></ul></ul></ul><ul><ul><li>Examinations usually begin at 4-6wks postnatal age or at 31-32 wks postmenstrual age </li></ul></ul><ul><ul><li>Continue every 2 wks </li></ul></ul><ul><ul><li>Once ROP is noted at any stage, examinations become more frequent </li></ul></ul><ul><ul><li>Can be discontinued once the retinal vessels have reached the perimeter of Zone 3 – retina is “mature” </li></ul></ul>
  11. 11. Outpatient Monitoring for Infants at Risk for ROP <ul><li>Confirm that retinal maturation is complete </li></ul><ul><ul><li>If mature, arrange for ophthalmologic ff up at 6-9 months to monitor for amblyopia, strabismus and or refractive errors </li></ul></ul><ul><ul><li>If immature, ophthalmologic ff-up per previous guidelines </li></ul></ul><ul><li>All PT <32 wks, should undergo ophthalmologic screening at 6-9 months chronologoic age, </li></ul><ul><ul><li>Whether or not they were screened for ROP, developed ROP or received tx for ROP </li></ul></ul><ul><li>All PT should have formal visual acuity screening, at least once during preschool years </li></ul><ul><li>If visual difficulties are seen, refer to appropriate resources </li></ul>
  12. 12. Hearing Loss in Premature Infants <ul><li>Overall incidence of severe congenital hearing loss: </li></ul><ul><li>1-3/1000 live births </li></ul><ul><li>Hearing loss in premature infants: 2-4/100 infants born <32 wks </li></ul>
  13. 13. Risk Factors for Hearing Loss (Joint Committee on Infant Hearing) <ul><li>Parental or caregiver concern re: hearing, speech, language or developmental delay </li></ul><ul><li>Family history of permanent childhood hearing loss </li></ul><ul><li>Stigmata associated with a syndrome known to cause hearing loss or eustachian tube dysfunction </li></ul><ul><li>Postnatal infection associated with SNHL – bacterial meningitis </li></ul><ul><li>Congenital infections – CMV, HSV, rubella, syphilis, HIV, toxoplasmosis </li></ul><ul><li>Syndromes associated with progressive hearing loss – NF, osteopetrosis, Usher syndrome </li></ul>
  14. 14. Risk Factors for Hearing Loss (Joint Committee on Infant Hearing) <ul><li>Neonatal indicators – hyperbilirubinemia requiring exchange (TB >20, needs BAER at 2months), PPHN associated with mechanical ventilation or ECMO </li></ul><ul><li>Nuerodegenerative disorders – Hunter syndrome, Friedrich ataxia, Charcot-Marie Tooth </li></ul><ul><li>Head trauma </li></ul><ul><li>Recurrent or persistent OM with effusion for at least 3 months </li></ul><ul><li>Prolonged use of potentially ototoxic drugs </li></ul>
  15. 15. Screening Tests <ul><li>Universal hearing screening recommended for all newborns </li></ul><ul><li>Methodologies for physiologic screening for hearing in newborns </li></ul><ul><ul><li>Auditory brainstem response (ABR) </li></ul></ul><ul><ul><li>Evoked otoacoustic emission (EOAE) </li></ul></ul>
  16. 16. Follow-up Testing and Medical Evaluation <ul><li>Infants who refer in both ears  diagnostic ABR within 2 wks </li></ul><ul><li>Unilateral abnormal results  ff-up testing within 3 months </li></ul><ul><li>Ff-up testing: full diagnostic frequency ABR to measure threshold, evaluation of middle ear function, observation of behavioral response to sound, parental report of emerging communication and auditory behaviors </li></ul>
  17. 17. Follow-up Testing and Medical Evaluation <ul><li>Any infants at risk for progressive or delayed hearing loss  close audiologic monitoring at least q6 months for the first 3 years) </li></ul><ul><li>Hearing assessment at 1 year in all infants born at < 32 wks (even if hearing screen is passed) </li></ul><ul><li>PCP should monitor all infants for normal hearing and language development and refer any infant with delays for hearing assessment </li></ul>
  18. 19. Referrals <ul><li>Once an infant is diagnosed with a true hearing loss, the following referrals should be made: </li></ul><ul><li>Complete evaluation by an otolaryngology or otology specialist who has experience with infants </li></ul><ul><li>Genetic evaluation and counseling (hearing loss with no definite etiology) </li></ul><ul><li>Pediatric ophthalmology (evaluate for additional sensory loss) </li></ul><ul><li>Developmental pediatric, neurology, cardiology, and or nephrology as indicated by other clinical findings and known associated problems with syndromes </li></ul>
  19. 20. Habilitation/Management <ul><li>Early intervention services to enhance acquisition of developmentally appropriate language skills </li></ul><ul><li>Amplification systems – hearing aids </li></ul><ul><li>Cochlear implant: profound, bilateral SNHL, no benefit from hearing aids, no medical conditions that will interfere with procedure, realistic expectations from family </li></ul><ul><li>“ Stimulation” or “mapping” sessions </li></ul>
  20. 21. White Matter Injury <ul><li>Periventricular leukomalacia (PVL) – white matter injury in preterm infants </li></ul><ul><li>Results from insults to the developing brain 23-32 wks </li></ul><ul><li>Incidence: 5-15% of those born GA<32 wks </li></ul><ul><li>US: echodensity in periventricular white matter adjacent to lateral ventricles  cystic changes </li></ul><ul><li>Outcomes: MR, CP, developmental delay, visual impairments </li></ul>
  21. 22. Intraventricular Hemorrhage <ul><li>Occurs in ~35-50% of infants born <35 wks </li></ul><ul><li>Grade III IVH associated with 30% risk of CP/MR and 50% risk of developmental disability </li></ul><ul><li>Intraparenchymal hemorrhage associated with 70% risk of CP/MR and 90% risk for developmental disability </li></ul>
  22. 24. Care and Assessment of Shunted Neonates <ul><li>Closely observe for long-term complications </li></ul><ul><li>Over-drainage related problems </li></ul><ul><ul><li> collapse of thin cortex, subdural effusion/hematoma, craniosynostosis </li></ul></ul><ul><ul><li>Evidenced by sunken fontanel, overlapping sutures </li></ul></ul><ul><ul><li>MX: positional precautions, upgrade or readjustment of valve settings </li></ul></ul><ul><li>Wound breakdown </li></ul><ul><li>Shunted infants with myelomeningocele </li></ul><ul><ul><li>Arnold-Chiari II malformation </li></ul></ul><ul><ul><li>Risk for brainstem dysfunction secondary to compression (retropulsion of head, stridor, drooling, increased tone in extremities) </li></ul></ul>
  23. 25. Neurologic Surveillance <ul><li>Perform periodic neurologic examinations </li></ul><ul><li>Tone (passive resistance) </li></ul><ul><li>Strength (active resistance) </li></ul><ul><li>DTR </li></ul><ul><li>Coordination, station and gait </li></ul><ul><li>Use assessments over time to establish prognosis </li></ul><ul><li>Single encounters provide a mere snapshot of ongoing developmental trajectories </li></ul>
  24. 26. Neurologic Surveillance <ul><li>Abnormal tone can be suggestive of CP or </li></ul><ul><li>Maybe transient  resolve in the 1 st year w/o sequelae </li></ul><ul><li>May evolve from one form to another (hypotonia  hypertonia) </li></ul><ul><li>Multidisciplinary evaluation </li></ul><ul><li>Extensive evaluations as necessary: MRI, cytogenetic studies, metabolic work-up </li></ul>
  25. 27. Findings of Abnormal Tone <ul><li>Hypotonia </li></ul><ul><li>Exaggerated head lag </li></ul><ul><li>Excessive ‘slip through’ when held at the shoulders </li></ul><ul><li>Poor head control </li></ul><ul><li>Poor truncal tone  exaggerated curve in ventral suspension </li></ul><ul><li>Persistent hypotonia + decreased DTRs </li></ul><ul><li>Hypertonia </li></ul><ul><li>Spastic form </li></ul><ul><li>Dyskinetic form with rigid extension </li></ul><ul><li>Early rolling over </li></ul><ul><li>Hypertonia with leg extension </li></ul><ul><li>Absent weight bearing </li></ul><ul><li>Persistent/early feeding problems </li></ul>
  26. 28. Neurologic Surveillance <ul><li>Assess for persistence or delay of reflexes </li></ul><ul><li>Primitive reflexes </li></ul><ul><ul><li>Moro, tonic labyrynthe, asymmetric tonic neck </li></ul></ul><ul><ul><li>Appear and readily elicited in the 1 st 3 months  disappear by 6-8 months </li></ul></ul><ul><li>Postural reflexes </li></ul><ul><ul><li>Complex, self-protective reflexes involving righting, protection and equilibrium movements </li></ul></ul><ul><ul><li>Slow to evolve in children with CNS injury </li></ul></ul>
  27. 29. Developmental Surveillance <ul><li>Assess neurodevelopmental and behavioral status </li></ul><ul><ul><li>Developmental tools – screening tools, not diagnostic tools </li></ul></ul><ul><ul><li>Parent questionnaires, history and discussion during clinical encounter </li></ul></ul><ul><li>Consult appropriate specialists if results are concerning </li></ul>
  28. 31. AAP Algorithm for Developmental Surveillance and Screening Pediatrics Vol 118, July 2006 <ul><li>Developmental surveillance incorporated at every well-child visit </li></ul><ul><li>If any concerns  standardized developmental screening tests </li></ul><ul><li>Regular screening tests: 9, 18 and 30 (24) month visits </li></ul><ul><li>Children diagnosed with developmental disorders: children with special health care needs requiring chronic condition management </li></ul>
  29. 32. Developmental Surveillance