1. Funny Shaped Heads
Ronald L. Young II, MD

3. Funny shaped heads Etiologies Congenital deformities
Positional molding
Cephalohematomas
Mass lesions
Congenital: craniosynostosis, intrauterine molding, encephalocele,
Mass lesions: tumors, arachnoid cysts

4. Molded Heads Plagiocephaly Brachycephaly Scaphocephaly
The most prevalent head shape of the American infant has gone from normocephalic to brachycephalic in one generation because of the back to sleep initiative.

5. Positional Plagiocephaly
Occipital flattening
Anterior displacement ear
Bulge forehead
Bulge malar eminence
Parallelogram
Local bald spot

7. Positional Plagiocephaly
Associated with “torticollis”
Short sternocleidomastoid on flat side
Not stretched out
Decreased rotation away from flat side
Uterine constraint

8. Positional brachycephaly
Symmetric flat occiput
Increased bi-parietal diameter
No ridging
Focal bald spot
Severe cases have ears pointing down

9. Positional brachycephaly
Deep sleepers
Don’t move after falling asleep
Don’t sleep in bed
Infant seat
Bouncy seat
Hypotonia
Cause not effect

10. Positional Scaphocephaly
“Premie head”
Common in severe premies
Can evolve into craniostenosis
VP Shunt

11. Pathology Soft skull Weight of brain able to flatten skull
Rapidly growing skull
Self sustaining
Flat tire
Flat tire: the head tends to end up on the flat spot.

12. Time Course Flat spot noticeable 1 m/o
Deformity peaks 4 m/o (Corrected)
Head control
Primary deformity
Flat spot
Secondary deformity
Compensatory bulge

13. Time Course Improvement of the flat spot 6 m/o Dent in inion 6 m/o
Posterior bulge
Dent in inion 6 m/o
Bulge takes months 2 y/o
Brain molds from inside

14. Time Course Ear asymmetry 3 y/o Rare residual deformity
Not cosmetic problem
Rare residual deformity

16. Why So Long? Head Growth

18. Treatment Position Therapy
Keep off the flat spot
Sleep
<4 m/o Wedges and blankets
Roll on side
>4 m/o Fetal position
Let baby fall asleep in usual position
Then rotate onto side in fetal position
Start at naps

20. Treatment Position Therapy
Sit up in swing
Get out of bouncy seat and infant seat
Exer-saucer
Walker
“Tummy Time”

21. Treatment Molding Helmet
Act like dental braces
Slowly push in secondary deformity
Keep off the primary deformity

22. Treatment Molding Helmet
Patients with helmets look better faster
Need to be worn to work
Shoot for 23 hours / day
Need to be custom fit
Earlier the better
Won’t work as well after 12 mo

23. Treatment Molding Helmet
Cost
Cranialtech $3000
Advanced Orthopro $1400
Complications
Compliance
Skin breakdown

24. Treatment Molding Helmet
My indications
Borderline parents
Can’t get concept of keeping off flat spot
Hypotonic children
Won’t have head control by 4 months
Hydrocephalus post shunt
Will not have brain growth to mold skull
Significant facial bulge
Just not getting better
Typically make this decision around 7-8 months of age.
Babies that cant’t be repositioned, severe deformities.

25. Craniosynostosis

26. What is it? Premature fusion of the cranial sutures
Sutures allow progressive enlargement of the skull with brain growth
Normal sutural fusion is complete at 6 to 8 years of age
Premature fusion produces progressive skull deformity

29. Epidemiology Occurs in 1 in 2100 children
Primary non-syndromic is most common
Lambdoid synostosis is very rare
Multiple sutures involved in 8% of non-syndromic cases
Calvarial bones grow perpendicular to the sutures. Growth: 50% at 2 months, 100% at 6 months, and 200% at 10 months.

30. Calvarial Development
Intramembranous ossification
Margins of calvarial bones form osteogenic front
Sutures form at sites of near contact
New bone is laid down by osteoblasts in spicules at the sutural margins
Overall bone growth is driven by the expanding brain
Calvarial vault develops in a fibrous membrane over the brain called the ectomeninx.
Bone then divides ectomeninx into outer periosteum and inner dura. Osteogenic front: specialized proliferative cells
Sutures form at areas of major dural reflections.
Interaction between dura and osteogenic fronts maintains sutures Brain growth is exponential during the first year.

31. Calvarial Development

32. Known causes of craniosynostosis
Pathobiology
Known causes of craniosynostosis
Teratogens
Valproic acid, aminopterin, retinoic acid
Malformation
Microcephaly
Encephalocele
shunted hydrocephalus
holoprosencephaly
Metabolic disorders
Rickets
Hyerthyroidism
Mucopolysaccharidoses
Hurler’s, Morquio’s
Hematologic disorders
Thalassemias, sickle cell anemia, polycythemia vera
Hyperthyroidism: premature osseous fusion, shunted hydrocephalus: true bony fusion does not occur
2-6% of isolated sagittal and 8-14% of cases of coronal synostosis are familial. Likely autosomal dominant with 0.6% penetrance by segregation analysis/

33. Pathobiology Syndromic Crouzon Apert’s Pfeiffer Jackson-Weiss
Mutation in gene coding for fibroblast growth factor receptors

34. Pathobiology <10% inherited or syndromic 90% spontaneous
Uterine constraint
Early drop into cervical canal
Multiple births
Severe back pain last month of pregnancy

35. Pathobiology
Premature closure of calvarial sutures also affects the skull base
Coronal and metopic
Effect extends to nasoethmoid complex, orbital roofs, and supraorbital ridges

36. Diagnosis Characteristic skull shape Lack of movement at suture
Palpable ridging of fused suture
CT scan
Fusion, sclerosis, skull base deformities
Deformational skull anomalies are much more common

37. Metopic
1 frontal keel 2 bitemporal narrowing 3 coronal suture displaced anteriorly 4 parieto-occipital bulging 5 bizygomatic narrowing 6 posterior displacement of supraorbital rim

38. Metopic

40. Unicoronal
1 fused suture 2 ipsilateral frontal flattening 3 bulging of temporal squama 4 contralateral frontal bulging 5 ipsilateral nasal root deviation 6 anterior displacement of ipsilateral ear 7 elevation of sphenoid wing 8 shortening of ipsilateral anterior cranial fossa 10 reduced mediolateral dimension of ipsilateral orbit

41. Bicoronal
1 fused suture 2 recessed supraorbital rim 3 prominent, elongated frontal bone 4 flattening of the occiput 5 bitemporal protrusion 6 anteriorly displace vertex 7 harlequin deformities

42. Sagittal

44. Lamdoid

45. Cloverleaf deformity

46. The case for surgery Aesthetic and functional
Elevated ICP in 14% of single and 47% of children with multiple sutural synostosis
Uncorrected children often socially isolated and stigmatized
Low risk with modern craniofacial surgery

48. Surgery

49. Surgery

50. Complications Blood loss Infection CSF leak Post surgical trauma
Poor cosmetic result
Persistent cranial defects

51. Results

52. Results

53. Results

54. ?

55. Thank You!