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abnormal vertebral development in utero that can include abnormal shape, fusion, or partial/missing vertebrae. Is often associated with other skeletal or organ abnormalities
congenital scoliosis
aquired curvature over time associated w/ nm disease, related to inability of muscular system to support thorax for upright positioning
neuromuscular scoliosis
physical presentation of a child w/ scoliosis
Asymmetrical posture- uneven shoulder, trunk lean, rib hump
functional presentation of a child with scoliosis
deteriorating ability to sit, asymmetrical weight shifting
2 main characteristics of the normal thorax
stable volume, ability to change volume
most common cause of thoracic insufficiency syndrome
scoliosis (limits lung growth due to rib cage and spinal deformities)
how is thoracic insufficiency syndrome diagnosed?
x-ray, MRI, pulmonary function tests, physical exam, genetic testing for associated conditions
nonsurgical management for scoliois
bracing (25 to 45 degree curves) and PT
surgical interventions for scoliosis
spinal fusion, growing rods, vertical expandable prosthetic titanium rib (VEPTER)
pre-operative considerations for the VEPTR
baseline level of function (6MWT), education, chest wall meausrements
post-operative considerations for the VEPTR/spinal fusion
maximize independence and educate about precautions
what is congenital muscular torticollis (CMT)?
tight SCM muscle causing ipsilateral lateral flexion and contralateral rotation
how is CMT named?
for the side that is tight
how would a child with left CMT present?
left side bend and right rotation
what are the 3 main subtypes of CMT?
postural, muscular, sternomastoid tumor
• Infants’ postural preference
• No SCM muscle tightness, no PROM
restrictions
• No mass, X-rays are normal
postural torticollis
• Tightness of SCM and PROM restrictions
• No mass, X-rays are normal
muscular torticollis
• Discrete mass or fibrotic thickening palpable in SCM
• PROM limitations
• X-rays are normal
sternomastoid tumor torticollis
theories for etiology for torticollis
restricted intrauterine environment ischemic injury based on abnormal vascular patterns, birth trauma
risk factors for CMT
large birth weight, male gender, breech, multiple birth, first pregnancy, difficult labor/delivery, use of vacuum/forceps, nuchal cord, maternal uterine abnormalities
what is the most common pediatric MSK condition?
CMT
what is the incidence of CMT?
3.9-16%
why is early referral important in CMT
reduces length of treatment time needed for resolution of symptoms
what domains should be included in the history of a child with CMT?
neuromuscular, cardiorespirtory, MSK, integumentary, GI
what 9 birth and health history factors should be documented in a child with CMT?
Chronological age at time of eval, age of symptom onset, pregnancy history, delivery history, use of assistance during delivery, head posture/preference and head/face asymmetries, family history, medical conditions, developmental milestones
other interview questions for a child with CMT
whio is spending the most time with the child, any previous interventions, preferred feeding positions/issues, sleeping position, positioning devices, time in prone
what should be involved in examination of a child with CMT?
posture, degree of resting head tilt, PROM (rotation and lateral flexion), AROM (rotation), strength, extremity ROM (UE, LE, spine), muscle tone, pain, oculomotor assessment, other asymmetries, compensations, palpation of cervical musculature, developmental status, craniofacial examination
normal PROM values for infants
70 degrees of lateral flexion and 110 degrees of rotation
how is neck strength assessed in infants?
muscle function scale (MFS)- tip horizontally in front of mirror and classify head righting on 6 point scale, compare sides
what should be screened for as there is an increased incidence in those with CMT?
DDH
other possible asymmetries/compensations you may observe in children with CMT
upper trap tightness, ipsilateral shoulder hiking, limb use, protective/righting reactions, scoliosis, trendelenburg sign
how can motor development be screened
through symmetry and milestones using a standardized tool (AIMS, TIMP, PDMS-2)
how common is plagiocephaly (flattening of one part of skull) in children w/ CMt?
67-90%
common findings with plagiocephaly
occipital flattening, frontal bossing, anterior ear displacement, mandibular asymmetries, smaller/elevated eye
how is plagiooephaly examined?
cranial vault asymmetry index (CVAI), compares 2 diagonals
treatment for plagiocephaly
conservative (repositioning program, supplemental devices), cranial remolding orthosis (helmet)
goals of treatment in children with CMT
symmetrical cervical AROM/PROM, midline head control in age appropriate positions, symmetric development and use of extremities, development/gross motor skills, symmetric cervical strength
first choice interventions for CMT
neck PROM, neck and trunk AROM, development of symmetrical movement, environmental adaptations, parent/caregiver education
recommendations for neck PROM for CMT
use low intensity, sustained, and pain free stretches. stop if patient resists or with change in breathing/circulation
how is neck/trunk AROM acheived?
positioning, carrying, feeding, exercises isolating the weaker muscles (rolling), righting reactions, developmental play
how can development of symmetrical movement be achieved?
interventions to promote symmetric movement and prevent compensatory movement patterns, prone positioning for >1 hour while awake to offset transient effects of supine sleep positions
environmental adaptations for CMT
adapt car seat to promote symmetry, alternate positioning, minimize time in car seats/infant carriers, place toys on affected side, alternate feeding sides, tummy time
inportant components of parent education for CMT
have parents return demonstrations of HEP, environmental adaptations, teach to encourage symmetric movement when playing with child
how is classification of CMT determined?
age of treatment initiation and type/severity- grades 1-8, early mild to very late
what factors may extend PT prognosis
age of treatment initiation, severity, intensity of intervention, comorbidities, rate of change, adherence with HEP
when should you refer out to physicia?
red flags (poor visual tracking, abnormal muscle tone, extra-muscular masses, other asymmetries inconsistent with CMT, side changes, late onset, seven months or older w/ mass) or after 4-6 weeks of intense intervention with little to no reduction in neck asymmetry
more invasive interventions for CMT
surgical lengthening or release of SCM, botox injection
what is the prognosis for needing surgery for CMT based on?
limitations in cervical ROM greater than 15 degrees, SCM mass, older age at treatment initiation, typically made after period of conservative treatment
when is it appropriate to discharge a patient with CMT?
resolved within accepted ranges (full AROM/PROM within 5 degrees of unaffected side and no visible head tilt), age-appropriate motor development/symmetry, documentation reflects expected outcomes of POC, caregivers understand what to monitor in child and when to monitor PCP/PT
when should a follow up screening be done after discharge of a CMT patient?
3-12 months typically when a child is gaining new developmental skills