Deformational Plagiocephaly and Craniosynostosis: Cranial Remolding - Professional Issues Lecture

Infant vs Adult Cranium

Growth of the neurocranium (skull) and viscerocranium (face) occurs at different rates. Important to note when addressing facial asymmetry.

• Neurocranium - most significant growth occurs before 1 year of age

• Viscerocranium - growth occurs very slowly over the first 10 years of life

At birth the skill comprises about 1/3 of body (big head)

The infant face is only about 1/8 of the skill, compared with ½ of the adult.

Fontanel

An opening in the skill where the boney plates come together. Incomplete ossification - Allows for rapid stretching and deformation of the cranium as the brain expands faster then the surrounding bone can grow.

Lateral fontanels (4) - obliterated within the first 2-3 months

Posterior fontanel (1) - within the 1st year

Anterior fontanel (1) - middle of the 2nd year

Posterior Fontanel

Commonly referred to as a divot, hole, flat spot, ridge, etc.

Sutures

Definition - Fibrous joints (Sharpey fibers) that bind the individual bones of the skill, allowing for movement (birth process) and flexibility (rapid and continued brain growth).

Time of suture closure varies widely (infancy to adulthood) can lead to delayed ossification.

Metopic suture union starts in the first year and is completed by the 8th year.

Other than metopic suture, all sutures should remain open during infancy and childhood

If closed prematurely that is called Craniosynostosis.

Intentional Cranial Deformation

• artificial deformation of infant craniums has been practiced of many years

• all of these cultures appreciated that early and persistent application of pressure to the cranium within the first year of life resulted in permanent alterations of skull shape.

Artificial Deformation

Head flattening or head binding is a form of body alteration in which the skull of a human is intentionally deformed.

• done by distorting the normal growth of a child’s skull by applying external forces.

Normal Growth Patterns

Unintentional Cranial Deformation - Deformational Plagiocephaly

Disease or physiological disruption that creates an imbalance in the static or dynamic modeling process, resulting in physical impairment of the individual.

Goal of Treatment for Unintentional Cranial Deformation

To correct unintentional cranial deformation - to achieve symmetry

Cranial Remolding Orthosis (CRO)

• intentional reformation

• the product of the passive control of the normal vectors of infantile neurocranial growth through external restrictions

• a redirection of growth

Will it Correct on its Own?

Basic Principles of deformation of reformation depends on -

• pliability of the tissues

• stage of development

• duration of deforming force

Not treating can see positive or negative effects.

Theory of Cranial Remolding

Restrain the growth of the normal shape of the cranium by only allowing growth in the deformed areas. Providing the skull a symmetrical and/or proportional environment during rapid periods of growth.

• Control the growth during the final stages of rapid cranial growth, 4-6 months of age.

Bone responds to

• mechanical demand

• force magnitude

• rate of application

• mode of load

• frequency of load

Modeling Considerations

• time of onset

• duration of deforming forces

• degree of severity

• diagnosis/etiology

• remaining growth

• health of physiologic structures

• developmental level

• associated conditions

Principles of Orthotic Intervention

• provide total contact in the areas where growth is to be curbed.

• allow space in the areas where growth is desired.

• passively controls the direction of cranial growth, not the overall magnitude.

• there is a critical window of opportunity, specifically between 4-6 months of age, when the skull is most actively growing.

Clinical Documentation - What Information to Collect?

Gather Accurate & Comprehensive Information -

• birth history

• developmental observations

• clinical evaluation of head deformity

• Congenital Muscular Torticollis (CMT)

• parent/caregiver education topics

• thorough craniofacial evaluation

• anthropometric cranial measurements

Clinical Documentation - Birth History

• weeks gestation

• NICU stay

• single vs multiple - where they twins, quadruplets, etc.?

• vaginal vs cesarean birth

• deformity noted by parent ‘at birth’

• other congenital anomalies or medical conditions

Clinical Documentation - Developmental Observations

Developmental Milestones

• what skills does the infant have?

• are delays present?

• currently, what’s your infant’s sleep preference

Does the child have appropriate head control, neck strength, and posture?

Can they sit independently at appropriate milestone?

What is the significance of the “Back to Sleep” Program?

This program aimed to reduce the risk of SIDS and other sleep related deaths by encouraging parents to place their babies on their backs for every sleep.

Succeeded in reduction of sleep related deaths and SIDS.

Important to note that the number of infants diagnosed with deformational plagiocephaly increased as the risk for SIDS and sleep related deaths decreased.

Clinical Documentation - Congenital Muscular Torticollis (CMT)

• 3rd most common congenital musculoskeletal anomaly

• may subsequently result in DP and facial asymmetry

• the sternocleidomastoid (SCM) muscle is affected

• cervical muscle imbalance -

  • affected side is tight and shortened

  • opposite side is elongated and weak

• identified 0-8 weeks of life

• Deformational Plagiocephaly (DP) -

  • 90% of DP cases have CMT

• Early, proper identification is critical

Clinical Presentation of Congenital Muscular Torticollis (CMT)

• head laterally flexes to the affected side

• chin rotates away from the affected side

• patient presents with limited ROM, both in flexion of head and neck

• Right SCM is more common than left.

Treatment of Congenital Muscular Torticollis (CMT)

• physical therapy is the first line of treatment -

  • positioning

  • handling

  • stretching

• surgery reserved only for severe, non-responsive cases

• multidisciplinary approach when couples with DP

  • treated as a separate condition

Evaluation of Head Deformity

This table shows the different possible abnormal head shapes and the characteristics of each.

CI - a width to length ratio to determine asymmetry of head shape

Cranial Vault Asymmetry (CVA) or Transcranial Diagonal Differential (TTD) - two diagonal measurements taken 30 degrees off of midline compared through subtracting the two.

Deformational Plagiocephaly (DP) - Evaluation

Asymmetric

• unilateral occipital flattening

• ipsilateral forehead bossing

• ipsilateral anterior ear shift

• contralateral occipital prominence

• contralateral forehead flattening

• associated with congenital muscular torticollis ~90%

What is the goal of treatment with Deformational Plagiocephaly (DP)?

improve overall symmetry

Deformational Brachycephaly - Evaluation

Disproportionate - width

• bilateral occipital flattening

• increased cranial width

• decreased cranial vault height

• bilateral frontal bossing

What is the goal of treatment of Deformational Brachycephaly?

reduce disproportion

Deformational Asymmetric Brachycephaly (DAB) - Evaluation

Asymmetric and disproportionate -

• combined asymmetric and disproportional deformity

• MOST COMMON TYPE

• hybrid of DP and DB

• bilateral occipital flattening, with increased flattening on one side crossing midline

• prominences at the posterior parietal and the lateral parietal

• frontal asymmetry and/or bossing may be present

• facial asymmetry may be present

What is the goal of treatment for Deformational Asymmetric Brachycephaly (DAB)?

reduce asymmetry then reduce disproportion

Deformational Scaphocephaly (DS)

Disproportionate - Length

• increased cranial length

• decreased cranial width

• occipital protuberance

• frontal protuberance/bossing

• asymmetry may be present

• least common infant skull deformation

What is the goal of the treatment of Deformational Scaphocephaly (DS)?

increase disproportion

Anthropometric Measurements - Circumference

• taken at eyebrow level

• head in neutral position

• horizontal to floor

Anthropometric Measurements - Cephalic Index (CI)

Cranial Width - at widest dimension

Cranial Length - at longest dimension

Cephalic Index (CI) = Width / Length x 100

Anthropometric Measurements - Transcranial Diagonal Differential (TDD)

Diagonal 1 - right anterior quadrant to left posterior quadrant

Diagonal 2 - left anterior quadrant to right posterior quadrant

30 degrees off of midline

TDD = D1-D2

Tool - M/L Caliper

Anthropometric Measurements - Cranial Base Asymmetry

• Tragions - point just anterior to ear

• Subnasion - point at the center of the tip of the nose

• length of right tragion to subnasion

• difference between right and left

• measures ear asymmetry

Tool - Facial Caliper or Tape Measure

Anthropometric Measurements - Orbitotragial Depth

• Tragion

• Excanthion - point just lateral to the eye

• length of right tragion to excanthion

• length of left tragion to excanthion

• difference between right and left

• measure orbit depth asymmetry

Tool - Facial Caliper or Tape Measure

Anthropometric Measurements

Analysis of measurements determines which head shape classification you are treating.

• Using Cephalic Index (CI)

• Using Cranial Vault Asymmetry (CVA) or (TDD)

Circumference, M/L, A/P, transcranial distance A and transcranial distance B, MUST be taken at each appointment.

Measurements help to direct clinical decisions.

Positive feedback for parents/caregivers.

Cranial Base and Orbitotragial Asymmetry measurements to be taken at initial, mid-treatment, and final evaluation.

Cephalic Index Measurement Scale

Cranial Vault Asymmetry (CVA) or (TDD) Scale

Guidelines used for Classifying Infants requiring treatment with a cranial remolding orthosis (CRO)

Determining Severity using Scales

Rating scale based on visual assessment criteria - Argenta Scale

Rating scale based on quantitative assessments - Hutchinson Scale

Rating scale based on visual and quantitative criteria - Plagiocephaly Severity Scale by Children’s Healthcare of Atlanta (CHOA)

Argenta Scale for Plagiocephaly

Rating scale based on visual assessment criteria.

Posterior Lateral Cranial Asymmetry

• Type I - Posterior Asymmetry

• Type II - Ear Malpositions

• Type III - Frontal Asymmetry

• Type IV - Facial Asymmetry

• Type V - Temporal Bossing or Posterior Vertical Growth

Argenta Scale for Brachycephaly

Rating scale based on visual assessment criteria

Posterior only Cranial Asymmetry

• Type I - Central posterior flattening

• Type II - Widening of posterior skull

• Type III - Temporal bossing or posterior vertical growth

Hutchinson Scale

Rating scale based on quantitative assessments -

• SVA for lateral Plagiocephalic shape

• CI for posterior only Brachiocephalic shape

Standards remains to be established across disciplines.

Children’s Healthcare of Atlanta Plagiocephaly Severity Scale

This is the most commonly used scale for assessing asymmetry.

Cranial Technologies - DOC Band

FIRST company to be cleared by FDA in 1998.

• fabricates remolding and post-op cranial orthoses

• low profile design

• thermoplastic, side-opening design

• fabricates from a scan

Digital Surface Imaging (DSI) - acquires the image in a fraction of a second, allowing for movement.

• no lasers are involved in the process

Orthomerica - STARband

SECOND company to be cleared by FDA in 2000.

STARband (thermoplastic)

• remolding cranial orthosis

• side opening or bi-valve

STARlight (Surlyn)

• remolding cranial orthosis

• side opening or bi-valve

STARlight PRO (Surlyn)

• post-op cranial orthosis

• bi-valve

STARband 3D

STARScanner

SmartSoc

Orthomerica - SmartSoc

SmartSoc 3D Capturing System

• used to manufacture the STARband and STARlight

• works off of a smartphone Iphone or Android

• allows movement

• portable system

MCU Report

FDA Regulation

1998 - cranial remolding orthoses were reclassified as a Class II Medical Device

• all cranial remolding orthoses must be manufactured by a 510(k) approved manufacturer

• post manufacture modifications must be limited to minor adjustments in order to remain compliant

• this made it illegal for anyone who was not granted FDA clearance to fabricate the orthoses

The Two Kinds of Family/Parent Personalities

Well-Informed and educated

• want to start the process right away

• need additional research to prove efficacy of treatment

Lack of previous research or education on process

• be clear and concise in the way you provide information

• provide written information for them to refer back to

• be aware of what parents may see or read on the internet, specifically in Facebook groups or research articles.

Distractions in the Treatment Room

Distraction Gadgets and Toys

• KEY is movement and lights

• this keeps the baby distracted for measuring and scanning

• have disinfectant wipes or spray available to clean your toys and gadgets!

Scanner in the Treatment Room

• Be thoughtful when choosing a spot for your scanner unit so that family can see

• live feed if possible

Baby Seat in the Treatment Room

• Bumbo Floor Seat

• Mamas and Papas Baby Snug

• A device WITH a seat strap

Treatment Tools

ML Gauge

Flexible tape measure

Distraction gadgets

Sharpies

Lipstick or chalk

Pen/paper

Anatomy of infant skull

Craniosynostosis

Condition where the bones of a baby’s skull fuse together too early, before the brain is fully developed.

Cranial Vault Reconstruction

• typically performed by plastic surgeon at 9 months old

• removal of the skull at the equator, reshaping each individual piece

Endoscopic Craniectomy

• typically preformed prior to 4 months of age, by a neurosurgeon with assistance from a plastic surgeon

• removal of the premature suture(s)

• Post operative Cranial Remolding Orthosis

Timeline of Endoscopic Craniectomy

• Receive Rx and notes for physician

• Day 1 - pre-operative consult, evaluation, scan

• Day 2 - surgery

• Day 3 - discharged

• Day 4 - post-operative scan

• Day 6-10 - patient seen for fit and delivery

Timeline of a Smaller Segment of Bone (Endoscopic Craniectomy)

• receive Rx and notes for physician

• Day 1 - pre-operative consult, evaluation, pre-op scan

• Day 2 - surgery

• Day 3 - discharged

• Day 2-5 - patient for fit and delivery

CRO Designs

Traditional Surlyn

• 1/4” Surlyn

• Fishmouth design, with lay over Velcro

• Correction within orthosis

Bivalve Design

• Copolymer

• Bi-valve design, with lay over Velcro

• Lined

• Total Contact, minimal correction

ALL correction principles are the same despite what design is being used.

Clinical Appearance of Craniosynostosis- Sagittal Suture

• ridged ossification of sagittal suture and anterior fontanel

• long head - boat or football shaped

• bitemporal and biparietal narrowing

• frontal bossing and sloping occiput - occipital cupping

Synostotic Sagittal vs Deformational Scaphocephaly

Sagittal Synostosis is the most common synostosis

• premature fusion of the sagittal suture

• severe (bilateral) frontal and occipital bossing

• posterior cranium narrows

• no facial asymmetry

Clinical Appearance of Craniosynostosis - Unicoronal Suture

• ridged ossification of affected coronal suture

• frontal bone asymmetry

• superior orbit/sphenoid asymmetry, eye appears larger on effected side

• frontal bossing of non-affected side

• root of nose deviates to non-affected side

Right Unilateral Coronal Synostosis vs. Right Deformational Plagiocephaly

Clinical Appearance of Craniosynostosis - Bicoronal Suture

• Ridged ossification of both coronal sutures

• tall forehead

• bi-parietal widening, brachycephalic

Clinical Appearance of Craniosynostosis - Metopic Suture

• ridged ossification of metopic suture

• midline frontal bossing

• bitemporal narrowing

• trigonalcephaly - triangular shape of the head

Clinical Appearance of Craniosynostosis - Lambdoid Suture

• impressive unilateral posterior flattening without anterior bossing

• ear is positioned close to the area of flattening

• mastoid prominence on the same side of closed suture

• opposite side parietal bossing

Lambdoid = Trapezoid

Unilateral Lambdoid Synostosis

• impressive unilateral posterior flattening without anterior bossing

• ear is positioned close to the area of flattening

• mastoid prominence on the same side of closed suture

• opposite side parietal bossing

Deformational Plagiocephaly

• unilateral posterior flattening with anterior frontal bossing on the same side as the posterior flattening

• ear move away from the area of flattening

• from a proximal view - “parallelogram-shaped” head

Craniosynostosis must be ______ ______.

ruled out

Is imaging necessary for Craniosynostosis?

nope, you can see or feel the abnormalities of the bony structures

Follow-up

Following Fit/Delivery

• 1 week post delivery

• 4 two week follow ups

• 3 week follow ups until completion

Evaluate fit, are principles of correction being followed

Review anthropometric measurements, every appointment.

Rescan for new orthosis, with physician’s orders.

Utilize the Cranial Report.

2-3 CRO’s until a year old.

Evidence Based Care of Child with Deformational Plagiocephaly: Part 1 Flannery & Looman

• importance of repositioning and education prior to 4 months when patient gains head control

• torticollis needs to be identified and treated as it limits repositioning

• classification of head shape deformities - determining brachycephalic

• crucial to be clinically trained in ruling out craniosynostosis

Evidence Based Care of Child with Deformational Plagiocephaly: Part 2 Flannery & Looman

• systematic review of literature to identify best practice in management of plagiocephaly

• American Academic of Pediatrics recommends at least 6-8 weeks of repositioning prior to considering CRO treatment

• multidisciplinary approach provides best outcomes for patients and families

• no evidence that CRO treatment causes harm and is most effective if begun by 6 months of age

Orthotic Management of Deformational Plagiocephaly - Lin et.al.

• Clinical Practice Guidelines are based on this systematic review

• statements taken from the literature and rated on a Likert scale by 30 high volume cranial orthotists

• total of 54 consensus statements are present in Appendix A of the article

• created algorithm to aide clinicians in knowing when to initiate treatment based on severity and age

RCT: Helmet Therapy in Infants with Positional Skull Deformation - Van wijk et.al.

• frequently referred to as the BMJ study or the Dutch study

• findings suggested no difference between treatment with a CRO and natural course (no treatment)

• patients with torticollis were excluded from this study

• cannot be reproduced due to lack of standards, consistency between treating orthotists, and unknown education level

CHINSTRAP IS NOT COOL

Clinical Practice Guidelines

Standard Definition - systematically developed statements to assist practitioner and patient decisions about appropriate health care for specific circumstances

• provide guidance to novice clinicians

• respect the expertise of established clinicians

• establishing boundaries (stay within these lines)

• ensure comprehensive considerations

Findings in Research

Fifty four best practice statements were identified, along with several clinical algorithms.

• defined in 4 categories

  • diagnosis

  • presentation and severity

  • initiating treatment

  • management principles

Clinical Practice Guidelines - Diagnosis

Recommendation - Craniosynostosis should be ruled out during the initial evaluation of a patient with an atypical head shape.

• radiologic imaging is generally unnecessary

• craniofacial specialists (neurosurgeon, cranial orthotist, pediatric physical therapist, etc.) should be consulted when moderate to severe deformation is observed

• lambdoid synostosis is rare, but should be ruled out

• when in doubt, refer to craniofacial clinic and/or neurosurgeon

Clinical Practice Guidelines - Presentation and Severity of Plagiocephaly

Recommendation - The severity of plagiocephaly is a product of anthropometric measurements, ear malposition, and forehead symmetry

• Objective - Transcranial Diagonal Differential (TDD)

• Subjective - Ear Malposition and forehead symmetry

• Collectively - Mild, Moderate, and Severe definitions

Plagiocephaly - Presentation and Severity

Clinical Practice Guidelines - Presentation and Severity of Brachycephaly

Recommendation - the severity of brachycephaly is the product of anthropometric measurement, skull widening and temporal bossing

• Objective - Cephalic Index (CI)

• Subjective - Posterior skull widening, temporal bossing & vertical head shape (cranial sloping)

Brachycephaly - Presentation and Severity

Clinical Practice Guidelines - Initiating Treatment

Recommendation - Ideally, the treatment should begin between 4 and 6 months, depending upon the severity of the presentation.

When to start treatment :

• Severe - at 4 months old

• Moderate - at 5 months old

• Mild - 6 months old at the family’s discretion

Basic Orthotic Strategies

Directed growth of the cranial sutures -

• total contact over areas of prominence

• void/space over areas of flattening

structured follow-up program

monitored cranial growth

ongoing adjustments as needed

monitor post-treatment to prevent regression

Initial Fitting Considerations - Before seeing pt

ALWAYS REMOVE CRO FROM BAG PRIOR TO FAMILY COMING IN.

• review order form

  • correct color

  • seam on correct side

  • were my directions follows

• utilize the CDC Provided Overlays

• what type of head shape

• what movement should i expect

• where should redness be

• where should the void be

Initial Fitting Considerations - Fitting the helmet

• the CRO should NOT fit intimately at initial visit

• verify sufficient contact over prominent areas (with slight blanching)

• try not to apply and remove the CRO more than 3 times during the initial fitting

• allow the infant to wear the CRO for at least 30 minutes during the initial fitting

• allow the infant to wear the CRO for at least 30 minutes to check skin tolerance and overall fit

• allow the infant to play and lay down with CRO donned to verify the security of the initial fit

Fitting Process - Pre-Fit

• room/tool readiness

  • sharpie, tape measure, ML stick, distraction devices, etc.

• patient positioning

  • on parent’s lap, faced outward, extended out to knee, holding infant’s torso

• position the orthosis

  • kneel in front of patient

  • spread opening from plastic as wide as possible (never Velcro/chafe)

  • center the CRO on infant’s head using the midline of the forehead as a guide

  • do not secure Velcro strap

• assess and mark initial trim lines

Fitting Process - Establishing Initial Trim Lines

Lateral Edges -

• lateral trims should extend to the end of the infant’s earlobe

• clear infant’s peripheral vision

• check to make sure all lateral extensions are making total contact

• ear trims should be very close to the ears (<1cm away)

  • keep them smaller than you think

Posterior Edge -

• it should capture the entire occipital area, extending inferior to the occiput bone and end just proximal to C7

• the infant should be able to extend their head, even in a prone position, without any interference from the posterior-distal/lateral edges

Anterior Edge -

• the trimlines around the face are verified and trimmed LAST

• anterior trim lines should extend just proximal to the brow (< 1 cm)

• ‘Square-off’ around radius of anterior trim (around eyes)

Fitting Process - Evaluate the Side Opening

• The side opening should be fully come together and close

  • if there is gapping, the infant may have had a cranial growth spurt since the scan (see troubleshooting)

  • The Velcro strap is used merely to secure the CRO

  • verify that the opening is on the correct side

Fitting Process - Evaluate the Proximal Opening

• roughly 50-60% of the width and length of the head

• should not extend below the crown of the head

• a larger proximal opening will ease the donning process, but must be considered relative to the overall skull deformity

Fitting Process - Evaluate Areas of Total Contact/Relief

• slight blanching is observed over areas of total contact where skull growth is unwanted

• voids are observed over the flattened areas where skull growth is encourage

• there design modifications promote more symmetrical and/or proportional skull growth

Fitting Process - Finalize the Initial Fitting

• refit the CRO on the infant and demonstrate proper donning and doffing techniques to the parent

• parents turn! they must also practice

• re-assess the final fit and all trim lines

  • anterior, posterior, laterals, and superior

• review the wearing schedule

• review the cleaning schedule

• schedule follow up appointment

Hanger Wear Schedule

Hanger Cleaning Instructions

Caregiver Education at Delivery

• redness or discoloration

  • blanching or pink areas are normal and expected in areas of total contact

  • you should never see any discoloration in areas of void/space and will need to remove material immediately if it occurs

• when redness occurs, if it does not dissipate within 60 minutes after removing orthosis - stop wearing CRO and call office asap

• because the CRO does not fit intimately, there may be some slight movement initially (especially when laying against a surface)

• clean daily, but do not clean 24 hrs before each visit

• initial infant reactions and adjustment periods may vary for each patient

• increased head perspiration is common the first 2-3 weeks

  • will eventually subside as infant acclimates

• subsequent itching of head due to increased moisture

• perspiration may persist in summer months or for infant’s who are ‘sweaters’ (clothe lightly and corn starch use)

• dress accordingly

• remove CRO if infant has a fever

  • can re-apply once fever has reduced and normal body temperature maintained (without Tylenol management)

Recommended Tools

• urethane arbor

• Tycro wheel

• conical sanding arbor

• sanding drum

• keep cranial tools separate from your day to day tools

  • prevents harsh materials from coming into contact with the sensitive skin of the infant

Follow-up Guidelines

The purpose of a structured follow-up schedule

• stay ahead of growth in desired areas

• prevent unnecessary skin irritation

• maximize improvements to cranial symmetry and/or proportion

Ongoing adjustments

• strategically remove material to encourage growth

• strategically relieve areas to reduce pressure

• strategically add padding to resist rotation/translation

Patient follow-up with a cranial remolding orthosis is more critical than ANY other orthosis you will fit.

Cranial remolding orthoses are the ONLY orthosis that is classified as a Class II medical device by the FDA.

When to Remove Foam?

• when voids have been filled

• when max head circumference has been reached

• areas of redness