Skull deformities in pediatrics
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Skull Deformities in Pediatrics. Case 1. 7wk female with parental concern over head shape PMHx: 35wk twin A; NICU x 6days (FEN, ID, bili) Normal feeding/wets/stools Progressive “flattening” of head since birth No hx IVH; no neurological symptoms No flattening in twin. Case 1.

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Skull Deformities in Pediatrics

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Skull deformities in pediatrics

Skull Deformitiesin Pediatrics


Case 1

Case 1

  • 7wk female with parental concern over head shape

  • PMHx:

    • 35wk twin A; NICU x 6days (FEN, ID, bili)

    • Normal feeding/wets/stools

    • Progressive “flattening” of head since birth

    • No hx IVH; no neurological symptoms

    • No flattening in twin


Case 11

Case 1

  • Physical Exam:

    • Ht, Wt, HC tracking

    • Alert, NAD

    • AFOSF, RR present

    • Flattened R occiput, with protrusion of R forehead and R ear farther forward

    • Mild flattening of L face

    • O/W unremarkable


What s normal

What’s normal?

  • Skull growth mainly secondary to brain growth

    • 40% adult size at term

    • 90% adult size by 1yo

    • 95% adult size by 6yo

  • Suture closure

    • PF 3-6mo

    • AF 9-18mo

    • C,S,L sutures 40years


What s most likely

What’s most likely?

  • Deformational Plagiocephaly:

    • Asymmetric head

    • Up to 48% healthy infants

    • Most common referral to Peds NSGY

    • Risk Factors:

      • Intrauterine crowding/positioning

      • Decreased mobility (dev.delay, MR, prematurity)

      • Posturing (torticollis, C-spine defect)

      • “Back to Sleep” campaign

      • Increased physician awareness


What s the worry

What’s the worry?

  • Craniosynostosis:

    • Premature closure of sutures

    • 1 in 2,100-3,000 live births

    • Isolated (80%) vs. Syndromic (20%)

    • Risk Factors:

      • Single-gene mutations (FGFR, TWIST, MSX2)

      • Rickets, hyperthyroidism

      • Phenytoin, retinoids, VA, MTX, fluconazole


What s a pediatrician to do the h p of course

What’s a Pediatrician to do?The H&P, of course!

  • Molding

    • Pressure in AP dimension (labor)

    • Resolves in hours-weeks

  • Deformation

    • Pressure in local region (occipital)

      • No sutural ridging; bones mobile

  • Synostosis

    • Premature closure of sutures

      • Palpable ridges over suture; bones immobile


What s normal1

What’s normal?

PARALLELOGRAM

TRAPEZOID

Deformational Plagiocephaly

Lambdoid Synostosis


What s not

What’s not?

Metopic Synostosis

Bicoronal Synostosis


What s not1

What’s not?

Sagittal Synostosis


Deformational plagiocephaly

Deformational Plagiocephaly

  • AKA: Positional, Posterior, Occipital, Plagiocephaly without synostosis

  • Diagnosis:

    • Usually clear based on H&P

    • Head shape (parallelogram)

    • Xray if unsure – sutures patent


Deformational plagiocephaly1

Deformational Plagiocephaly

  • Treatment:

    • Repositioning

      • Effective in 85% mild cases

      • Alternate sleeping sides

      • Encourage “tummy time”

      • Discourage carseats

    • Helmet

      • 23 hrs/day

      • Frequent adjustments

  • Typically does not require NSGY referral


When to refer

When to refer?

  • True craniosynostosis

    • 1/5-1/6 syndromic

    • Increased risk ICP, hydrocephalus, Chiari

  • Multi-D Team:

    • NSGY, Plastics, Ophtho, Neuro, ENT, Orthodontics, Psych, Genetics, Social Work

  • Neurosurgery: 3-9 months old


Case 2

Case 2

  • Newborn female, term SVD

  • No maternal hx; no family hx

  • Physical Exam:

    • Large, low AF

    • ?Fused coronal/lambdoid sutures?

    • Significant caput

    • Hypertelorism

    • Syndactyly on 3 extremities; clubfoot

    • Broad, flat hallux


Case 21

Case 2

  • Craniofrontonasal Dysostosis

    • X-linked

    • Variable expression (F>M)

  • Genetics eval

  • Orthopaedics eval

  • Ophthalmology eval

  • Neurosurgery/OMFS eval


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