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Funny Shaped Heads

Funny Shaped Heads. Ronald L. Young II, MD. Funny shaped heads. Etiologies Congenital deformities Positional molding Cephalohematomas Mass lesions. Molded Heads. Plagiocephaly Brachycephaly Scaphocephaly. Positional Plagiocephaly. Occipital flattening Anterior displacement ear

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Funny Shaped Heads

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

  2. Funny shaped heads • Etiologies • Congenital deformities • Positional molding • Cephalohematomas • Mass lesions

  3. Molded Heads • Plagiocephaly • Brachycephaly • Scaphocephaly

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

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

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

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

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

  9. Pathology • Soft skull • Weight of brain able to flatten skull • Rapidly growing skull • Self sustaining • Flat tire

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

  11. Time Course • Improvement of the flat spot 6 m/o • Posterior bulge • Dent in inion 6 m/o • Bulge takes 12-16 months 2 y/o • Brain molds from inside

  12. Time Course • Ear asymmetry 3 y/o • Not cosmetic problem • Rare residualdeformity

  13. Why So Long? Head Growth

  14. 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

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

  16. TreatmentMolding Helmet • Act like dental braces • Slowly push in secondary deformity • Keep off the primary deformity

  17. TreatmentMolding 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

  18. TreatmentMolding Helmet • Cost • Cranialtech $3000 • Advanced Orthopro $1400 • Complications • Compliance • Skin breakdown

  19. TreatmentMolding 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

  20. Craniosynostosis

  21. 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

  22. 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

  23. 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

  24. Calvarial Development

  25. Metabolic disorders Rickets Hyerthyroidism Mucopolysaccharidoses Hurler’s, Morquio’s Hematologic disorders Thalassemias, sickle cell anemia, polycythemia vera Teratogens Valproic acid, aminopterin, retinoic acid Malformation Microcephaly Encephalocele shunted hydrocephalus holoprosencephaly Pathobiology Known causes of craniosynostosis

  26. Pathobiology • Syndromic • Crouzon • Apert’s • Pfeiffer • Jackson-Weiss

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

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

  29. Diagnosis • Characteristic skull shape • Lack of movement at suture • Palpable ridging of fused suture • CT scan • Fusion, sclerosis, skull base deformities

  30. Metopic

  31. Metopic

  32. Unicoronal

  33. Bicoronal

  34. Sagittal

  35. Lamdoid

  36. Cloverleaf deformity

  37. 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

  38. Surgery

  39. Surgery

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

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