1. You: Welcome to the Peds Clinic ! How can I help you? Mom: Destiny’s face is all screwed up. She was rejected from her fourth Gerber photo-shoot in the last 2 weeks!

2. Normal Infant Skull Flexible enough to get through vagina –Molding Expansile enough to accommodate rapid brain growth

4. Infant Skull Anatomy

5. Suture Growth Sutures allow growth perpendicular to them –Growth at suture lines related to brain growth

6. Suture Closure Bone Union By Age 20 Ossification By Age 8

7. Early Closure Causes Growth Parallel to the Suture

8. Craniosynostosis: Early Fusion of a Suture

10. Sagittal Synostosis “Boat-Head” (Scaphocephaly)

13. Coronal Synostosis “Bent-Head” (Plagiocepahly)

14. Right Coronal Synostosis

16. Metopic Synostosis “Triangle-Head” (Trigonocephaly)

17. Metopic Synostosis

19. Lamboid Synostosis “Slant-Head” (Occipital Plagiocephaly) MATTRESS

20. Isolated Primary Craniosynostosis Sutures Involved

21. Clinical Exam OFC Head shape (from above, side) Ear and facial symmetry Palpate suture lines & fontanelles –Look for ridging Look for associated anomalies Skull X-ray or CT

22. Craniosynostosis Microcephaly Prematurity VP Shunting Positioning SecondaryPrimary Isolated Abnormal Suture Syndromic

23. Deformational Scaphocephaly Impaired mobility & prolonged positioning Persists until adulthood Prevention: –Donut-shaped head supports – waterbed mattresses Does not warrant intervention Prematurity

24. Former Preemie’s Head

25. VP Shunting Scaphocephaly Chronic hydrocephalus thickens the skull Once decompression with shunt, the suture fuses Surgery Indications: –OFC > 50 cm (4-5+ STDs) –When VPS performed during when VLBW

26. Microcephaly Surgical correction not indicated Abnormal OFC –in primary craniosynostosis, OFC remains normal yet oddly shaped Rare cases of multisutural craniosynostosis restricting head growth, but manifests with increased ICP

27. Most common cause Usually forehead asymmetry Sometimes associated with torticollis Usually acts on coronal or lamboidal suture 40% of newborns Positional Deformation

28. An Epidemic of Lamboidal Plagiocephaly 1992: Back to Sleep Campaign 1996: Tertiary Care Centers report rise in lamboidal plagiocephaly from 3% to 20%

29. Sorting out the “Epidemic” 102 Patients with occipital plagiocephaly over 4 year period Only 4 (3%) had true lamboidal synostosis The rest were deformational –Only 3 were progressive (required surgery) – Other responded to positioning or helmets

32. Syndromic Craniosynostosis 10-20 % of cases Autosomal Dominant –Linked to Chromosome 10q Multi-sutural, complex cases If a suture is fused, check hands, feet, big toe and thumb

33. Distinguishing Clinical Features in the Craniosynostosis Syndromes MuenkeCrouzon Jackson- Weiss ApertPfeiffer Bear- Stevenson ThumbsNormal Fused to fingers Broad, deviated Normal Hands± Carpal fusion NormalVariableBone syndactyly Variable brachyda ctyly Normal Great Toes ± BroadNormalBroad, deviated Fused to toes Broad, deviated Normal Feet± Tarsal fusion NormalAbnormal tarsals Bone syndactyly Variable brachyda ctyly Normal

34. Crouzon’s Normal intellect Normal extremities 5 % have acanthosis nigricans 30 % have progressive hydrocephalus

35. Apert’s “Crouzon’s with Hand Involvement” Varying intellect (50 % with MR) Mitten Glove Syndactyly Cervical vertebral anomalies Rare hydrocephalus

36. Apert Extremity Findings

37. True Craniosynostosis & Surgery Single Suture Synostosis: Confirm by exam and skull x-rays Complex cases: CT or 3D CT X-Ray: Fused sutures have a broad ridge of overgrowth of solid bone along a previous suture, or suture is completely obliterated Ridge is especially characteristic of fused sagittal suture

38. Management Surgery vs. Conservative Management

39. The Decision to Operate Raised ICP in 1/3 of cases, but no neuro impairment Cosmetic considerations usually most important –affects peer acceptance, parent-child bonding, self-image and coping

40. Imaging Skull X-ray CT 3-D CT

42. Surgery If not part of syndrome, the earlier the operation the better –At the latest 6-12 months (by 12 months, skull is 85% of adult size) –For coronal suture, operate before 2 months because of facial symmetry and visual system development –Procedure depends on continuing skull growth –Hospitalization for 3-10 days

43. Surgery Syndromic cases may need special airway support Blood loss significant due to scalp vascularity –transfusion rates 20-500 % of infant estimated blood volume PICU stay (facial edema) Results on xray within several days

47. Metopic Synostosis

48. Surgery Unilateral coronal suture: difficult. Orbital relocation as well. Syndromic or multi-suture cases: staged repairs.

49. Apert: Post-Op

50. Crouzon Surgical Pics

51. Conservative Therapy for Deformational Plagiocephaly Re-positioning If no improvement by 6 months…. Helmet Molding

52. Custom Made for each head 24/7 wear for 4 months

53. Ocular Morbidity Pre- and post-op impairments seen with unilateral coronal and metopic synostoses –dossociated movements –amblyopia –refractive errors Ophtho involvement in work-up and follow- up

54. Long Term Follow-Up Speech Genetic Counseling Feeding / Swallowing Ophtho