Brown Bag Lunch Monday, April 3, 2017 Gross Motor Delays Brown Bag Lunch Monday, April 3, 2017

Spring-Board ARTICLE Clinical Report: Motor Delays: Early Identification and Evaluation. Pediatrics 2013 Rooted in Focus groups of pediatricians asking for more guidance on these issues Audience: pediatric primary care providers States that the most commonly used screening instruments have not been validated on children with motor delays… Proposes algorithm for identification & early evaluation in the medical home based on surveillance, screening instruments, observation and additional w/u Not evidence based per se

Existing Guidelines – AAP 2006 Council on Children with Disabilities, Section on Developmental Behavioral Pediatrics. Bright Futures Steering Committee; Medical Home Initiatives for Children with Special Needs Project Advisory Committee. Identifying infants and young children with developmental disorders in the medical home; an algorithm for developmental surveillance and screening. Pediatrics. 2006; 118(1): 405-420. For all development, not specific to gross motor Currently being revised.

2006 AAP Algorithm For Identifying Developmental Delays Surveillance at all well visits Scheduled/Reflex Dev Screening Follow-up &/or Refer THE BASICS OF WHAT WE DO Limited details Focus on identification & Refer

2013 Proposed Algorithm Adjustments Build on recommendations for surveillance & screening If identify gross motor delays, initiate W/U by PMD High tone  MRI and refer at the same time Low or normal tone  CK and thyroid tests (to identify muscular dystrophies and/or acquired hypothyroidism) Dysmorphic, heart failure, organomegaly  Genetics w/u with microarray & referral

2013 Clinical Report ALGORITHM

But Let’s take a step back, What do we know? Parents are reliable reporters of gross motor development, but surveillance & screening are important to identify what is normal/abnormal Early identification can help outcomes Physical therapy Treatment for specific conditions Prognosis Family planning

DDX GROSS MOTOR DELAY DDX: Central CNS, Peripheral CNS, NM disease, metabolic, genetic Types: Transient vs. persistent vs. progressive vs. regressive Isolated vs. global development delays Prevalence: % with mild delays? 0.3% population with Cerebral palsy less with NM disorders (muscular dystrophies, SMA, myasthenia gravis) and genetic disorders (storage disease, mitochondrial diseases, others) Acquired hypothyroid Developmental coordination disorders = 6% of kindergarteners

Proposed Algorithm - Surveillance Address development at all check-ups Listen & attend to parental concerns Document & maintain development history Make accurate observations of the child Identify risk and protective factors Maintain accurate record of the process & findings

Proposed Algorithm – DEFINING DELAY Specific screening test at 9, 18 & 30 months (consider again at 48 months) And at other ages if concerns identified ASQ (Bell curve of normal) See ASQ Questions

Proposed Algorithm – DefiniNG Delay Observed skills, GROSS MOTOR DELAY if unable to do these things: 9 months: roll bilaterally, sit well, symmetric movements, no handedness, grasps & transfers What about the 9 month old that doesn’t pull to stand? What about the 15 month old that is not yet walking? 18 months: sits, stands, walks independently, grasps & moves small objects 30 months: loss of previous skills, subtle gross/fine and language skills 48 months: loss of skills

SURVEILLENCE & SCREENING – AVERAGE AGES Marked delay beyond these ages warrants attention, but does not necessarily signify a neuro-motor disease…

EXPANDED HISTORY – FOCUSED ON MOtOR

EXPANDED HISTORY - MOTOR BIRTH History (if < 36 weeks, correct until age 24 months) PMH – Chronic medical conditions, failure to thrive, heart failure, features FH – Genetic syndromes SH – opportunities for gross motor development

EXAM PearLS Watch posture, play and spontaneous movements during history Growth parameters – failure to thrive? Head circumference? HEENT – drooling, ptosis, feeding, facial features RESP – tachypnea, retractions, airway control CV – signs of heart failure ABD - Organomegaly NEURO-MUSCULAR: CN - red reflex, facial expressions, EOM, PERRLA, tongue movements/fasciculations, shoulder shrug Strength – observe function, body posture & movement, muscle bulk and atrophy, joint flexibility, grasp quality, Gower sign in older children Tone – slip through, ventral suspension, truncal tone in young infant; scarf sign & popliteal sign, persistent primitive reflexes, reflexes Movement – dyspraxia, coordination, gait, tremor Vision/hearing screening

Deciphering Tone Increased tone flags: Low tone flags: Reaches milestones early, asymmetric or out of order (roll to tummy 1st, handedness earlier than 18 months, stands before sits) Upper motor neuron signs (increased reflexes, babinski positive, rigid)  concerned for CP, get brain MRI Low tone flags: LMN signs (low reflexes, floppy baby, scarf sign and popliteal sign) low muscle bulk and strength  concerned for metabolic or NM disorder, get thyroid studies and CK levels, consider genetics microarray testing

RED FLAGS FOR PROMPT REFERRAL

Other next steps Mild abnormalities with no red flags Observation with scheduled follow-up Resources: Early Intervention +/-subspecialists Prompt referral for red flags Children with significant delays should be identified as having special needs Trigger care coordination and chronic care management paradigms.

CritiqueS 1st: Took issue with statement that PEDS & ASQ were not validated for gross motor identification 2nd: 1) validity of screening tools is sufficient; 2) surveillance should include additional steps of interpreting, discussing and closing the loop on referrals; 3) No study has demonstrated the NNS for blood testing in children with gross motor delays; 4) Need more support with referral care coordination.

What does this mean for Us? Who do you refer to early intervention or have closer follow-up? Who do you refer to specialists? Who do you test (MRI, thyroid, CK, microarray)? Which specialists do you like? Neurology Genetics PM&R?