Neurodevelopmental Neurodevelopmental Assessment and Care of Assessment and Care of Premature InfantsPremature Infants

Ma. Teresa C. Ambat, MDMa. Teresa C. Ambat, MDNeonatology-TTUHHSCNeonatology-TTUHHSC10/28/200810/28/2008

IntroductionIntroduction

PCPs should be vigilant in following outcomes of PCPs should be vigilant in following outcomes of prematurely born childprematurely born child

Should integrate and adapt assessments of Should integrate and adapt assessments of development, neurologic status and bahavior for each development, neurologic status and bahavior for each child at each encounterchild at each encounter

Neurodevelopmental and Behavioral Neurodevelopmental and Behavioral OutcomesOutcomes

Long-term outcome arises from complex interplay of Long-term outcome arises from complex interplay of biologic, genetic, social and environmental factorsbiologic, genetic, social and environmental factors

Additional or shifting developmental dysfunction over Additional or shifting developmental dysfunction over time, as more subtle disabilities become increasingly time, as more subtle disabilities become increasingly apparent and testable “new morbidities”apparent and testable “new morbidities”

Risk Factors for Developmetal and Behavioral Risk Factors for Developmetal and Behavioral Problems in the Preterm InfantProblems in the Preterm Infant

PrenatalPrenatalLBWLBW

GA <28 wksGA <28 wks

IUGRIUGR

Male genderMale gender

PostnatalPostnatalNeonatal seizuresNeonatal seizures

Abnormal HUS (white matter injury, Abnormal HUS (white matter injury,

PVL, Grade 3-4 IVH)PVL, Grade 3-4 IVH)

CLD Prolonged mechanical ventCLD Prolonged mechanical vent

Infections (NEC, sepsis, meningitis)Infections (NEC, sepsis, meningitis)

Feeding problems >34 wks PMAFeeding problems >34 wks PMA

ECMOECMO

Low economic statusLow economic status

Maternal depressionMaternal depression

Prevalence of Significant Disabilities in VLBWPrevalence of Significant Disabilities in VLBW

Mental retardationMental retardation 10-20%10-20%

CPCP 5-21%5-21%

BlindnessBlindness 2-11%2-11%

DeafnessDeafness 1-3%1-3%

Motor delayMotor delay 24%24%

Language problemsLanguage problems 23-42%23-42%

ADHDADHD 7-10%7-10%

Need for special educationNeed for special education 9-28%9-28%

Psychological/behavioral problemsPsychological/behavioral problems 25%25%

Correction for PrematurityCorrection for Prematurity

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

Pearls on Outcomes of Preterm InfantsPearls on Outcomes of Preterm Infants

More frequent and significant disabilities are associated More frequent and significant disabilities are associated with decreasing GA and BWwith decreasing GA and BW

Cognitive deficits > motor deficitsCognitive deficits > motor deficits

Disabilities or delays may be subtle or appear latentlyDisabilities or delays may be subtle or appear latently

Deficits in cognitive, verbal, perceptual, motor and Deficits in cognitive, verbal, perceptual, motor and visuo-motor measures may not manifest until school agevisuo-motor measures may not manifest until school age

Pearls on Outcomes of Preterm InfantsPearls on Outcomes of Preterm Infants

Up to 50% of infants born <25 wks may be found Up to 50% of infants born <25 wks may be found without disability at follow up over the 1without disability at follow up over the 1stst 3 yrs 3 yrs

Nearly all infants with normal findings on Nearly all infants with normal findings on neurodevelopmental examination at the infant’s neurodevelopmental examination at the infant’s expected due date continue to develop normallyexpected due date continue to develop normally

If no developmental delays during infancy, risk of MR or If no developmental delays during infancy, risk of MR or CP is lowCP is low

Ophthalmologic IssuesOphthalmologic Issues

Ophthalmologic problems of premature infantsOphthalmologic problems of premature infants ROP, strabismus, myopia – common ROP, strabismus, myopia – common Higher incidence of visual impairment – 45-60%Higher incidence of visual impairment – 45-60% Poor visual function may directly affect the development of Poor visual function may directly affect the development of

motor and cognitive skillsmotor and cognitive skills

Require specialized ophthalmologic testing and routine Require specialized ophthalmologic testing and routine follow-up by pediatric ophthalmologistfollow-up by pediatric ophthalmologist

ROP ScreeningROP Screening

AAP recommendation for ROP screeningAAP recommendation for ROP screening IndicationsIndications

GA <30 wks or BW <1500gGA <30 wks or BW <1500g Selected infants with BW 1500 – 2000g, GA >30 wks with severe Selected infants with BW 1500 – 2000g, GA >30 wks with severe

cardiorespiratory instabilitycardiorespiratory instability

Examinations usually begin at 4-6wks postnatal age or at 31-Examinations usually begin at 4-6wks postnatal age or at 31-32 wks postmenstrual age32 wks postmenstrual age

Continue every 2 wksContinue every 2 wks Once ROP is noted at any stage, examinations become more Once ROP is noted at any stage, examinations become more

frequentfrequent Can be discontinued once the retinal vessels have reached Can be discontinued once the retinal vessels have reached

the perimeter of Zone 3 – retina is “mature”the perimeter of Zone 3 – retina is “mature”

Outpatient Monitoring for Infants at Outpatient Monitoring for Infants at Risk for ROPRisk for ROP

Confirm that retinal maturation is complete Confirm that retinal maturation is complete If mature, arrange for ophthalmologic ff up at 6-9 months to monitor If mature, arrange for ophthalmologic ff up at 6-9 months to monitor

for amblyopia, strabismus and or refractive errorsfor amblyopia, strabismus and or refractive errors If immature, ophthalmologic ff-up per previous guidelinesIf immature, ophthalmologic ff-up per previous guidelines

All PT <32 wks, should undergo ophthalmologic screening All PT <32 wks, should undergo ophthalmologic screening at 6-9 months chronologoic age, at 6-9 months chronologoic age, Whether or not they were screened for ROP, developed ROP or Whether or not they were screened for ROP, developed ROP or

received tx for ROPreceived tx for ROP

All PT should have formal visual acuity screening, at least All PT should have formal visual acuity screening, at least once during preschool yearsonce during preschool years

If visual difficulties are seen, refer to appropriate resourcesIf visual difficulties are seen, refer to appropriate resources

Hearing Loss in Premature InfantsHearing Loss in Premature Infants

Overall incidence of severe congenital hearing loss: Overall incidence of severe congenital hearing loss:

1-3/1000 live births1-3/1000 live births

Hearing loss in premature infants: 2-4/100 infants born Hearing loss in premature infants: 2-4/100 infants born <32 wks<32 wks

Risk Factors for Hearing LossRisk Factors for Hearing Loss(Joint Committee on Infant Hearing)(Joint Committee on Infant Hearing)

1.1. Parental or caregiver concern re: hearing, speech, Parental or caregiver concern re: hearing, speech, language or developmental delaylanguage or developmental delay

2.2. Family history of permanent childhood hearing lossFamily history of permanent childhood hearing loss3.3. Stigmata associated with a syndrome known to cause Stigmata associated with a syndrome known to cause

hearing loss or eustachian tube dysfunctionhearing loss or eustachian tube dysfunction4.4. Postnatal infection associated with SNHL – bacterial Postnatal infection associated with SNHL – bacterial

meningitismeningitis5.5. Congenital infections – CMV, HSV, rubella, syphilis, HIV, Congenital infections – CMV, HSV, rubella, syphilis, HIV,

toxoplasmosistoxoplasmosis6.6. Syndromes associated with progressive hearing loss – Syndromes associated with progressive hearing loss –

NF, osteopetrosis, Usher syndromeNF, osteopetrosis, Usher syndrome

Risk Factors for Hearing LossRisk Factors for Hearing Loss(Joint Committee on Infant Hearing)(Joint Committee on Infant Hearing)

7.7. Neonatal indicators – hyperbilirubinemia requiring Neonatal indicators – hyperbilirubinemia requiring exchange (TB >20, needs BAER at 2months), PPHN exchange (TB >20, needs BAER at 2months), PPHN associated with mechanical ventilation or ECMOassociated with mechanical ventilation or ECMO

8.8. Nuerodegenerative disorders – Hunter syndrome, Nuerodegenerative disorders – Hunter syndrome, Friedrich ataxia, Charcot-Marie ToothFriedrich ataxia, Charcot-Marie Tooth

9.9. Head traumaHead trauma

10.10. Recurrent or persistent OM with effusion for at least 3 Recurrent or persistent OM with effusion for at least 3 monthsmonths

11.11. Prolonged use of potentially ototoxic drugsProlonged use of potentially ototoxic drugs

Screening TestsScreening Tests

Universal hearing screening recommended for all Universal hearing screening recommended for all newbornsnewborns

Methodologies for physiologic screening for hearing Methodologies for physiologic screening for hearing in newbornsin newborns

1.1. Auditory brainstem response (ABR)Auditory brainstem response (ABR)

2.2. Evoked otoacoustic emission (EOAE)Evoked otoacoustic emission (EOAE)

Follow-up Testing and Medical Follow-up Testing and Medical EvaluationEvaluation

1.1. Infants who refer in both ears Infants who refer in both ears diagnostic ABR within 2 diagnostic ABR within 2 wkswks

2.2. Unilateral abnormal results Unilateral abnormal results ff-up testing within 3 ff-up testing within 3 monthsmonths

Ff-up testing: full diagnostic frequency ABR to measure Ff-up testing: full diagnostic frequency ABR to measure threshold, evaluation of middle ear function, observation threshold, evaluation of middle ear function, observation of behavioral response to sound, parental report of of behavioral response to sound, parental report of emerging communication and auditory behaviorsemerging communication and auditory behaviors

Follow-up Testing and Medical Follow-up Testing and Medical EvaluationEvaluation

3.3. Any infants at risk for progressive or delayed hearing loss Any infants at risk for progressive or delayed hearing loss close audiologic monitoring at least q6 months for the close audiologic monitoring at least q6 months for the first 3 years)first 3 years)

4.4. Hearing assessment at 1 year in all infants born at Hearing assessment at 1 year in all infants born at << 32 32 wks (even if hearing screen is passed)wks (even if hearing screen is passed)

5.5. PCP should monitor all infants for normal hearing and PCP should monitor all infants for normal hearing and language development and refer any infant with delays language development and refer any infant with delays for hearing assessmentfor hearing assessment

ReferralsReferrals

Once an infant is diagnosed with a true hearing loss, Once an infant is diagnosed with a true hearing loss, the following referrals should be made:the following referrals should be made:

1.1. Complete evaluation by an otolaryngology or otology specialist Complete evaluation by an otolaryngology or otology specialist who has experience with infantswho has experience with infants

2.2. Genetic evaluation and counseling (hearing loss with no definite Genetic evaluation and counseling (hearing loss with no definite etiology)etiology)

3.3. Pediatric ophthalmology (evaluate for additional sensory loss)Pediatric ophthalmology (evaluate for additional sensory loss)

4.4. Developmental pediatric, neurology, cardiology, and or Developmental pediatric, neurology, cardiology, and or nephrology as indicated by other clinical findings and known nephrology as indicated by other clinical findings and known associated problems with syndromesassociated problems with syndromes

Habilitation/ManagementHabilitation/Management

Early intervention services to enhance acquisition of Early intervention services to enhance acquisition of developmentally appropriate language skillsdevelopmentally appropriate language skills

Amplification systems – hearing aidsAmplification systems – hearing aids Cochlear implant: profound, bilateral SNHL, no benefit Cochlear implant: profound, bilateral SNHL, no benefit

from hearing aids, no medical conditions that will from hearing aids, no medical conditions that will interfere with procedure, realistic expectations from interfere with procedure, realistic expectations from familyfamily

““Stimulation” or “mapping” sessionsStimulation” or “mapping” sessions

White Matter InjuryWhite Matter Injury

Periventricular leukomalacia (PVL) – white matter Periventricular leukomalacia (PVL) – white matter injury in preterm infantsinjury in preterm infants

Results from insults to the developing brain 23-32 wksResults from insults to the developing brain 23-32 wks Incidence: 5-15% of those born GA<32 wksIncidence: 5-15% of those born GA<32 wks US: echodensity in periventricular white matter US: echodensity in periventricular white matter

adjacent to lateral ventricles adjacent to lateral ventricles cystic changes cystic changes

Outcomes: MR, CP, developmental delay, visual Outcomes: MR, CP, developmental delay, visual impairmentsimpairments

Intraventricular HemorrhageIntraventricular Hemorrhage

Occurs in ~35-50% of infants born <35 wksOccurs in ~35-50% of infants born <35 wks

Grade III IVH associated with 30% risk of CP/MR and Grade III IVH associated with 30% risk of CP/MR and 50% risk of developmental disability50% risk of developmental disability

Intraparenchymal hemorrhage associated with 70% Intraparenchymal hemorrhage associated with 70% risk of CP/MR and 90% risk for developmental risk of CP/MR and 90% risk for developmental disabilitydisability

Care and Assessment of Shunted NeonatesCare and Assessment of Shunted Neonates

Closely observe for long-term complicationsClosely observe for long-term complications

Over-drainage related problemsOver-drainage related problems collapse of thin cortex, subdural effusion/hematoma, collapse of thin cortex, subdural effusion/hematoma,

craniosynostosiscraniosynostosis Evidenced by sunken fontanel, overlapping suturesEvidenced by sunken fontanel, overlapping sutures MX: positional precautions, upgrade or readjustment of valve settingsMX: positional precautions, upgrade or readjustment of valve settings

Wound breakdownWound breakdown

Shunted infants with myelomeningoceleShunted infants with myelomeningocele Arnold-Chiari II malformationArnold-Chiari II malformation Risk for brainstem dysfunction secondary to compression Risk for brainstem dysfunction secondary to compression

(retropulsion of head, stridor, drooling, increased tone in extremities)(retropulsion of head, stridor, drooling, increased tone in extremities)

Neurologic SurveillanceNeurologic Surveillance

Perform periodic neurologic examinationsPerform periodic neurologic examinations1.1. Tone (passive resistance)Tone (passive resistance)

2.2. Strength (active resistance)Strength (active resistance)

3.3. DTRDTR

4.4. Coordination, station and gaitCoordination, station and gait

Use assessments over time to establish prognosisUse assessments over time to establish prognosis Single encounters provide a mere snapshot of ongoing Single encounters provide a mere snapshot of ongoing

developmental trajectoriesdevelopmental trajectories

Neurologic SurveillanceNeurologic Surveillance

Abnormal tone can be suggestive of CP orAbnormal tone can be suggestive of CP or Maybe transient Maybe transient resolve in the 1 resolve in the 1stst year w/o sequelae year w/o sequelae May evolve from one form to another (hypotonia May evolve from one form to another (hypotonia

hypertonia)hypertonia)

Multidisciplinary evaluationMultidisciplinary evaluation Extensive evaluations as necessary: MRI, cytogenetic Extensive evaluations as necessary: MRI, cytogenetic

studies, metabolic work-upstudies, metabolic work-up

Findings of Abnormal Tone Findings of Abnormal Tone

HypotoniaHypotonia1.1. Exaggerated head lagExaggerated head lag

2.2. Excessive ‘slip through’ Excessive ‘slip through’ when held at the shoulderswhen held at the shoulders

3.3. Poor head controlPoor head control

4.4. Poor truncal tone Poor truncal tone exaggerated curve in exaggerated curve in ventral suspensionventral suspension

5.5. Persistent hypotonia + Persistent hypotonia + decreased DTRsdecreased DTRs

HypertoniaHypertonia1.1. Spastic formSpastic form

2.2. Dyskinetic form with rigid Dyskinetic form with rigid extensionextension

3.3. Early rolling overEarly rolling over

4.4. Hypertonia with leg Hypertonia with leg extensionextension

5.5. Absent weight bearingAbsent weight bearing

6.6. Persistent/early feeding Persistent/early feeding problemsproblems

Neurologic SurveillanceNeurologic Surveillance

Assess for persistence or delay of reflexesAssess for persistence or delay of reflexes

Primitive reflexesPrimitive reflexes Moro, tonic labyrynthe, asymmetric tonic neckMoro, tonic labyrynthe, asymmetric tonic neck Appear and readily elicited in the 1Appear and readily elicited in the 1stst 3 months 3 months disappear by disappear by

6-8 months6-8 months

Postural reflexesPostural reflexes Complex, self-protective reflexes involving righting, protection Complex, self-protective reflexes involving righting, protection

and equilibrium movementsand equilibrium movements Slow to evolve in children with CNS injurySlow to evolve in children with CNS injury

Developmental SurveillanceDevelopmental Surveillance

Assess neurodevelopmental and behavioral statusAssess neurodevelopmental and behavioral status Developmental tools – screening tools, not diagnostic Developmental tools – screening tools, not diagnostic

toolstools Parent questionnaires, history and discussion during Parent questionnaires, history and discussion during

clinical encounterclinical encounter

Consult appropriate specialists if results are concerningConsult appropriate specialists if results are concerning

AAP Algorithm for Developmental AAP Algorithm for Developmental Surveillance and ScreeningSurveillance and Screening Pediatrics Vol 118, July Pediatrics Vol 118, July 20062006

Developmental surveillance incorporated at every well-Developmental surveillance incorporated at every well-child visitchild visit

If any concerns If any concerns standardized developmental standardized developmental screening testsscreening tests

Regular screening tests: 9, 18 and 30 (24) month visitsRegular screening tests: 9, 18 and 30 (24) month visits

Children diagnosed with developmental disorders: Children diagnosed with developmental disorders: children with special health care needs requiring chronic children with special health care needs requiring chronic condition managementcondition management

Developmental SurveillanceDevelopmental Surveillance