Neurodevelopmental Assessment and Care of Premature Infants Ma. Teresa C. Ambat, MD Neonatology-TTUHHSC 10/28/2008

1. Neurodevelopmental Assessment and Care of Premature Infants Ma. Teresa C. Ambat, MD Neonatology-TTUHHSC 10/28/2008

2. Introduction • PCPs should be vigilant in following outcomes of prematurely born child • Should integrate and adapt assessments of development, neurologic status and bahavior for each child at each encounter

3. Neurodevelopmental and Behavioral Outcomes • Long-term outcome arises from complex interplay of biologic, genetic, social and environmental factors • Additional or shifting developmental dysfunction over time, as more subtle disabilities become increasingly apparent and testable “new morbidities”

4. Prenatal LBW GA <28 wks IUGR Male gender Postnatal Neonatal seizures Abnormal HUS (white matter injury, PVL, Grade 3-4 IVH) CLD Prolonged mechanical vent Infections (NEC, sepsis, meningitis) Feeding problems >34 wks PMA ECMO Low economic status Maternal depression Risk Factors for Developmetal and Behavioral Problems in the Preterm Infant

5. Prevalence of Significant Disabilities in VLBW

6. Correction for Prematurity • Correction for prematurity up to 2-3 years of age when considering neurologic, developemental or behavioral issues, otherwise indicated by a standardized evaluation

7. Pearls on Outcomes of Preterm Infants • More frequent and significant disabilities are associated with decreasing GA and BW • Cognitive deficits > motor deficits • Disabilities or delays may be subtle or appear latently • Deficits in cognitive, verbal, perceptual, motor and visuo-motor measures may not manifest until school age

8. Pearls on Outcomes of Preterm Infants • Up to 50% of infants born <25 wks may be found without disability at follow up over the 1st 3 yrs • Nearly all infants with normal findings on neurodevelopmental examination at the infant’s expected due date continue to develop normally • If no developmental delays during infancy, risk of MR or CP is low

9. Ophthalmologic Issues • Ophthalmologic problems of premature infants • ROP, strabismus, myopia – common • Higher incidence of visual impairment – 45-60% • Poor visual function may directly affect the development of motor and cognitive skills • Require specialized ophthalmologic testing and routine follow-up by pediatric ophthalmologist

10. ROP Screening • AAP recommendation for ROP screening • Indications • GA <30 wks or BW <1500g • Selected infants with BW 1500 – 2000g, GA >30 wks with severe cardiorespiratory instability • Examinations usually begin at 4-6wks postnatal age or at 31-32 wks postmenstrual age • Continue every 2 wks • Once ROP is noted at any stage, examinations become more frequent • Can be discontinued once the retinal vessels have reached the perimeter of Zone 3 – retina is “mature”

11. Outpatient Monitoring for Infants at Risk for ROP • Confirm that retinal maturation is complete • If mature, arrange for ophthalmologic ff up at 6-9 months to monitor for amblyopia, strabismus and or refractive errors • If immature, ophthalmologic ff-up per previous guidelines • All PT <32 wks, should undergo ophthalmologic screening at 6-9 months chronologoic age, • Whether or not they were screened for ROP, developed ROP or received tx for ROP • All PT should have formal visual acuity screening, at least once during preschool years • If visual difficulties are seen, refer to appropriate resources

12. Hearing Loss in Premature Infants • Overall incidence of severe congenital hearing loss: 1-3/1000 live births • Hearing loss in premature infants: 2-4/100 infants born <32 wks

13. Risk Factors for Hearing Loss(Joint Committee on Infant Hearing) • Parental or caregiver concern re: hearing, speech, language or developmental delay • Family history of permanent childhood hearing loss • Stigmata associated with a syndrome known to cause hearing loss or eustachian tube dysfunction • Postnatal infection associated with SNHL – bacterial meningitis • Congenital infections – CMV, HSV, rubella, syphilis, HIV, toxoplasmosis • Syndromes associated with progressive hearing loss – NF, osteopetrosis, Usher syndrome

14. Risk Factors for Hearing Loss(Joint Committee on Infant Hearing) • Neonatal indicators – hyperbilirubinemia requiring exchange (TB >20, needs BAER at 2months), PPHN associated with mechanical ventilation or ECMO • Nuerodegenerative disorders – Hunter syndrome, Friedrich ataxia, Charcot-Marie Tooth • Head trauma • Recurrent or persistent OM with effusion for at least 3 months • Prolonged use of potentially ototoxic drugs

15. Screening Tests • Universal hearing screening recommended for all newborns • Methodologies for physiologic screening for hearing in newborns • Auditory brainstem response (ABR) • Evoked otoacoustic emission (EOAE)

16. Follow-up Testing and Medical Evaluation • Infants who refer in both ears  diagnostic ABR within 2 wks • Unilateral abnormal results  ff-up testing within 3 months • 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

17. Follow-up Testing and Medical Evaluation • Any infants at risk for progressive or delayed hearing loss  close audiologic monitoring at least q6 months for the first 3 years) • Hearing assessment at 1 year in all infants born at < 32 wks (even if hearing screen is passed) • PCP should monitor all infants for normal hearing and language development and refer any infant with delays for hearing assessment

19. Referrals • Once an infant is diagnosed with a true hearing loss, the following referrals should be made: • Complete evaluation by an otolaryngology or otology specialist who has experience with infants • Genetic evaluation and counseling (hearing loss with no definite etiology) • Pediatric ophthalmology (evaluate for additional sensory loss) • Developmental pediatric, neurology, cardiology, and or nephrology as indicated by other clinical findings and known associated problems with syndromes

20. Habilitation/Management • Early intervention services to enhance acquisition of developmentally appropriate language skills • Amplification systems – hearing aids • Cochlear implant: profound, bilateral SNHL, no benefit from hearing aids, no medical conditions that will interfere with procedure, realistic expectations from family • “Stimulation” or “mapping” sessions

21. White Matter Injury • Periventricular leukomalacia (PVL) – white matter injury in preterm infants • Results from insults to the developing brain 23-32 wks • Incidence: 5-15% of those born GA<32 wks • US: echodensity in periventricular white matter adjacent to lateral ventricles  cystic changes • Outcomes: MR, CP, developmental delay, visual impairments

22. Intraventricular Hemorrhage • Occurs in ~35-50% of infants born <35 wks • Grade III IVH associated with 30% risk of CP/MR and 50% risk of developmental disability • Intraparenchymal hemorrhage associated with 70% risk of CP/MR and 90% risk for developmental disability

24. Care and Assessment of Shunted Neonates • Closely observe for long-term complications • Over-drainage related problems •  collapse of thin cortex, subdural effusion/hematoma, craniosynostosis • Evidenced by sunken fontanel, overlapping sutures • MX: positional precautions, upgrade or readjustment of valve settings • Wound breakdown • Shunted infants with myelomeningocele • Arnold-Chiari II malformation • Risk for brainstem dysfunction secondary to compression (retropulsion of head, stridor, drooling, increased tone in extremities)

25. Neurologic Surveillance • Perform periodic neurologic examinations • Tone (passive resistance) • Strength (active resistance) • DTR • Coordination, station and gait • Use assessments over time to establish prognosis • Single encounters provide a mere snapshot of ongoing developmental trajectories

26. Neurologic Surveillance • Abnormal tone can be suggestive of CP or • Maybe transient  resolve in the 1st year w/o sequelae • May evolve from one form to another (hypotonia  hypertonia) • Multidisciplinary evaluation • Extensive evaluations as necessary: MRI, cytogenetic studies, metabolic work-up

27. Hypotonia Exaggerated head lag Excessive ‘slip through’ when held at the shoulders Poor head control Poor truncal tone  exaggerated curve in ventral suspension Persistent hypotonia + decreased DTRs Hypertonia Spastic form Dyskinetic form with rigid extension Early rolling over Hypertonia with leg extension Absent weight bearing Persistent/early feeding problems Findings of Abnormal Tone

28. Neurologic Surveillance • Assess for persistence or delay of reflexes • Primitive reflexes • Moro, tonic labyrynthe, asymmetric tonic neck • Appear and readily elicited in the 1st 3 months  disappear by 6-8 months • Postural reflexes • Complex, self-protective reflexes involving righting, protection and equilibrium movements • Slow to evolve in children with CNS injury

29. Developmental Surveillance • Assess neurodevelopmental and behavioral status • Developmental tools – screening tools, not diagnostic tools • Parent questionnaires, history and discussion during clinical encounter • Consult appropriate specialists if results are concerning

31. AAP Algorithm for Developmental Surveillance and ScreeningPediatrics Vol 118, July 2006 • Developmental surveillance incorporated at every well-child visit • If any concerns  standardized developmental screening tests • Regular screening tests: 9, 18 and 30 (24) month visits • Children diagnosed with developmental disorders: children with special health care needs requiring chronic condition management

32. Developmental Surveillance