1. Imaging the Misshapen Head David Nielsen, MD Pediatric Radiologist

2. Imaging the Misshapen Head Objective: –Better understand how to image the most common causes of a misshapen head

3. Imaging the Misshapen Head Common causes: –Macrocephaly –Microcephaly –Craniosynostosis –Posterior plagiocephaly

4. Imaging the Misshapen Head Common causes: –Macrocephaly –Microcephaly –Craniosynostosis –Posterior plagiocephaly

5. Macrocephaly Definition: –Macrocephaly = Macrocrania

6. Macrocephaly Definition: –Macrocephaly = Macrocrania –Head circumference > 2SD (> 95%) above the mean for age, sex, race, and gestation

7. What is the most common imaging finding in macrocephaly? A.Hydrocephalus B.Benign Enlarged Subarachnoid Spaces (BESS) C.Subdural Hematoma D.Intracranial Mass

8. Macrocephaly Ddx: –#1: Benign Enlarged Subarachnoid Spaces (BESS) –Also called: Benign macrocrania Benign extra-axial collections Benign external hydrocephalus Transient communicating hydrocephalus NL BESS

9. Macrocephaly Benign enlarged subarachnoid spaces –Clinical:

10. Macrocephaly Benign enlarged subarachnoid spaces –Clinical: Macrocephaly presents between 3-6 months and peaks at about 7 months

11. Macrocephaly Benign enlarged subarachnoid spaces –Clinical: Macrocephaly presents between 3-6 months and peaks at about 7 months May have family history of macrocephaly

12. Macrocephaly Benign enlarged subarachnoid spaces –Clinical: Macrocephaly presents between 3-6 months and peaks at about 7 months May have family history of macrocephaly Normal developmental/neurological exam

13. Macrocephaly Benign enlarged subarachnoid spaces –Clinical: Macrocephaly presents between 3-6 months and peaks at about 7 months May have family history of macrocephaly Normal developmental/neurological exam Stabilizes by 18 months along a curve paralleling the 95% curve

14. Macrocephaly Benign enlarged subarachnoid spaces –Clinical: Macrocephaly presents between 3-6 months and peaks at about 7 months May have family history of macrocephaly Normal developmental/neurological exam Stabilizes by 18 months along a curve paralleling the 95% curve Spontaneously resolves by 24-36 months

15. Macrocephaly Benign enlarged subarachnoid spaces –Imaging:

16. Macrocephaly Benign enlarged subarachnoid spaces –Imaging: Symmetrical enlargement over the frontoparietal convexities and within the interhemispheric fissure, cortical sulci, and sylvian fissures

17. Macrocephaly Benign enlarged subarachnoid spaces –Imaging: Symmetrical enlargement over the frontoparietal convexities and within the interhemispheric fissure, cortical sulci, and sylvian fissures No mass effect

18. Macrocephaly Benign enlarged subarachnoid spaces –Imaging: Symmetrical enlargement over the frontoparietal convexities and within the interhemispheric fissure, cortical sulci, and sylvian fissures No mass effect Same imaging characteristics as CSF

19. Macrocephaly Benign enlarged subarachnoid spaces –Imaging: Symmetrical enlargement over the frontoparietal convexities and within the interhemispheric fissure, cortical sulci, and sylvian fissures No mass effect Same imaging characteristics as CSF Cortical veins course through the fluid

20. Macrocephaly Benign enlarged subarachnoid spaces –Imaging: Symmetrical enlargement over the frontoparietal convexities and within the interhemispheric fissure, cortical sulci, and sylvian fissures No mass effect Same imaging characteristics as CSF Cortical veins course through the fluid Ventricles are normal or mildly enlarged

21. Cortical veins Benign enlarged subarachnoid spaces Macrocephaly

22. Benign enlarged subarachnoid spaces Cortical veins

23. Macrocephaly Ddx: –#1: Benign Enlarged Subarachnoid Spaces (BESS) –Other: Hydrocephalus (HC) Subdural hematoma Intracranial mass (rare) Congenital/syndromic/ metabolic (rare)

24. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI Imaging is based on development and fontanel/age:

25. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI

26. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Normal neurological exam with open fontanel

27. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Normal neurological exam with open fontanel –Short-term clinical follow-up with serial head circumference measurements with or without ultrasound

28. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Normal neurological exam with open fontanel –Short-term clinical follow-up with serial head circumference measurements with or without ultrasound If head stabilizes (i.e. measurements again parallel the normal curve), the likely diagnosis is BESS:

29. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Normal neurological exam with open fontanel –Short-term clinical follow-up with serial head circumference measurements with or without ultrasound If head stabilizes (i.e. measurements again parallel the normal curve), the likely diagnosis is BESS: –No imaging (or no additional imaging) is recommended

30. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Normal neurological exam with open fontanel –Short-term clinical follow-up with serial head circumference measurements with or without ultrasound If head stabilizes (i.e. measurements again parallel the normal curve), the likely diagnosis is BESS: –No imaging (or no additional imaging) is recommended If head continues to enlarge disproportionate to the child’s growth (i.e. measurements do not again parallel the normal curve) and clinical exam is still otherwise normal:

31. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Normal neurological exam with open fontanel –Short-term clinical follow-up with serial head circumference measurements with or without ultrasound If head stabilizes (i.e. measurements again parallel the normal curve), the likely diagnosis is BESS: –No imaging (or no additional imaging) is recommended If head continues to enlarge disproportionate to the child’s growth (i.e. measurements do not again parallel the normal curve) and clinical exam is still otherwise normal: –Ultrasound to screen for severe hydrocephalus or large mass

32. Benign enlarged subarachnoid spaces Macrocephaly

33. Choroid plexus papilloma

34. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI

35. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Normal neurological exam with closed fontanel

36. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Normal neurological exam with closed fontanel –Case-by-case risk/benefit assessment of short-term clinical follow-up with serial head circumference measurements versus imaging with CT (radiation risk) or MRI (sedation risk)

37. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Normal neurological exam with closed fontanel –Case-by-case risk/benefit assessment of short-term clinical follow-up with serial head circumference measurements versus imaging with CT (radiation risk) or MRI (sedation risk) –Each modality also has advantages for the clinical question to be answered (e.g. CT is preferred for bones)

38. Macrocephaly Benign enlarged subarachnoid spaces 6 mo 11 mo

39. Macrocephaly Pilocytic Astrocytoma

40. MRI - Benign enlarged subarachnoid spaces Macrocephaly

41. Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI

42. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT or MRI Developmentally abnormal with open or closed fontanelMRI Abnormal developmental/neurological exam with open or closed fontanel

43. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT or MRI Developmentally abnormal with open or closed fontanelMRI Abnormal developmental/neurological exam with open or closed fontanel –MRI to evaluate brain parenchyma, extra-axial spaces

44. Macrocephaly Non-communicating hydrocephalus

45. Macrocephaly Anaplastic medulloblastoma

46. Macrocephaly Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI This approach to imaging macrocephaly reduces both unnecessary imaging and radiation exposure References: Smith, MR, JC Leonidas, J Maytal. The Value of Head Ultrasound in Infants with Macrocephaly. Pediatric Radiology 1998; 28:143-146. Wilms G, Vanderschueren G, et al. CT and MR in infants with pericerebral collections and macrocephaly: benign enlargement of the subarachnoid spaces versus subdural collections. American Journal of Neuroradiology 1993; 14:855-860. Hudgins, R, Boydston WR. All Heads Great and Small, Macrocephaly. Children’s Healthcare of Atlanta. http://www.choa.org/default.aspx?id=921. Accessed June 15, 2008.

47. 12-month-old male with macrocephaly and developmental delay. What study is indicated? A.Ultrasound B.CT C.MRI D.Brain PET scan

48. Imaging the Misshapen Head Common causes: –Macrocephaly –Microcephaly –Craniosynostosis –Posterior plagiocephaly

49. Imaging the Misshapen Head Common causes: –Macrocephaly –Microcephaly –Craniosynostosis –Posterior plagiocephaly

50. Microcephaly Definition: –Head circumference < 2SD (< 5%) below the mean for age, sex, race, and gestation

51. Microcephaly Ddx: –Congenital malformation –Infection (TORCH) –Hypoxia-Ischemia –Old trauma –Toxic/Metabolic

52. Microcephaly Clinical: –Abnormal developmental or neurological exam Imaging: –MRI Polymicrogyria

53. Imaging the Misshapen Head Common causes: –Macrocephaly –Microcephaly –Craniosynostosis –Posterior plagiocephaly

54. Imaging the Misshapen Head Common causes: –Macrocephaly –Microcephaly –Craniosynostosis –Posterior plagiocephaly

55. Craniosynostosis Definition: –Premature fusion of cranial sutures Synonyms: –Craniostenosis, sutural synostosis, cranial dysostosis M:F = 3:1

56. Craniosynostosis Incidence: 3-5 cases per 10,000 live births –Sagittal – 56% (1/3600) Scaphocephaly

57. Craniosynostosis Incidence: 3-5 cases per 10,000 live births –Sagittal – 56% (1/3600) Scaphocephaly

58. Craniosynostosis Incidence: 3-5 cases per 10,000 live births –Sagittal – 56% (1/3600) Scaphocephaly –Coronal – 26% (1/7700) Brachycephaly

59. Craniosynostosis Incidence: 3-5 cases per 10,000 live births –Sagittal – 56% (1/3600) Scaphocephaly –Coronal – 26% (1/7700) Brachycephaly

60. Craniosynostosis Incidence: 3-5 cases per 10,000 live births –Sagittal – 56% (1/3600) Scaphocephaly –Coronal – 26% (1/7700) Brachycephaly –Metopic – 8% (1/25,000) Trigonocephaly

61. Craniosynostosis Incidence: 3-5 cases per 10,000 live births –Sagittal – 56% (1/3600) Scaphocephaly –Coronal – 26% (1/7700) Brachycephaly –Metopic – 8% (1/25,000) Trigonocephaly

62. Craniosynostosis Incidence: 3-5 cases per 10,000 live births –Sagittal – 56% (1/3600) Scaphocephaly –Coronal – 26% (1/7700) Brachycephaly –Metopic – 8% (1/25,000) Trigonocephaly –Lambdoid – 5% (1/40,000) Brachycephaly (bilateral) or Trapezoid skull (unilateral) 1

63. Craniosynostosis Incidence: 3-5 cases per 10,000 live births –Sagittal – 56% (1/3600) Scaphocephaly –Coronal – 26% (1/7700) Brachycephaly –Metopic – 8% (1/25,000) Trigonocephaly –Lambdoid – 5% (1/40,000) Brachycephaly (bilateral) or Trapezoid skull (unilateral) –Other /syndromic – 5% 1

64. Incidence: 3-5 cases per 10,000 live births –Sagittal – 56% (1/3600) Scaphocephaly –Coronal – 26% (1/7700) Brachycephaly –Metopic – 8% (1/25,000) Trigonocephaly –Lambdoid – 5% (1/40,000) Brachycephaly (bilateral) or Trapezoid skull (unilateral) –Other /syndromic – 5% 1 Craniosynostosis

65. Posterior Plagiocephaly Posterior plagiocephaly:

66. Posterior Plagiocephaly Posterior plagiocephaly: –Synonyms: positional plagiocephaly, deformational plagiocephaly, positional molding, postural flattening

67. Posterior Plagiocephaly Posterior plagiocephaly: –Synonyms: positional plagiocephaly, deformational plagiocephaly, positional molding, postural flattening –Commonly seen since “Back to Sleep” began in the 1990’s

68. Posterior Plagiocephaly Posterior plagiocephaly: –Synonyms: positional plagiocephaly, deformational plagiocephaly, positional molding, postural flattening –Commonly seen since “Back to Sleep” began in the 1990’s –Asymmetrical flattening of the posterior skull due to recumbent/sleep position

69. Posterior Plagiocephaly Posterior plagiocephaly: –Synonyms: positional plagiocephaly, deformational plagiocephaly, positional molding, postural flattening –Commonly seen since “Back to Sleep” began in the 1990’s –Asymmetrical flattening of the posterior skull due to recumbent/sleep position –Does not usually require imaging

70. Posterior Plagiocephaly Posterior plagiocephaly: –Synonyms: positional plagiocephaly, deformational plagiocephaly, positional molding, postural flattening –Commonly seen since “Back to Sleep” began in the 1990’s –Asymmetrical flattening of the posterior skull due to recumbent/sleep position –Does not usually require imaging –Must distinguish from lambdoid synostosis

71. Otherwise normal child with posterolateral flattening Normal sutures/positional plagiocephaly SagittalLambdoidCoronal Posterior Plagiocephaly

72. Otherwise normal child with posterolateral flattening Posterior Plagiocephaly NL Normal sutures/ positional plagiocephaly

73. Craniosynostosis Lambdoid synostosis

74. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull x-ray study Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations When imaging is required, it depends on the risk category as determined by history/physical:

75. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull x-ray study Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations When imaging is required, it depends on the risk category as determined by history/physical:

76. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull x-ray study Plain films:

77. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull x-ray study Plain films: Lowest radiation dose

78. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull x-ray study Plain films: Lowest radiation dose Adequate screening for all craniosynostosis

79. Otherwise normal child with posterolateral flattening Normal sutures/positional plagiocephaly SagittalLambdoidCoronal Posterior Plagiocephaly

80. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull x-ray study Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations

81. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull x-ray study Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations

82. Craniosynostosis Risk CategoryImaging Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT Low-dose head CT:

83. Craniosynostosis Risk CategoryImaging Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT Low-dose head CT: ~80% less radiation than standard head CT

84. Craniosynostosis Risk CategoryImaging Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT Low-dose head CT: ~80% less radiation than standard head CT Optimized for evaluation of the bones/sutures

85. Craniosynostosis – Intermediate Risk Standard CTLow Dose CT

86. Standard CTLow Dose CT Craniosynostosis – Intermediate Risk

87. Child with mild developmental delay and right parieto- occiptal flattening NL Normal sutures/ positional plagiocephaly Craniosynostosis – Intermediate Risk

88. Child with developmental delay and left posterior flattening Normal sutures/ posterior plagiocephaly Craniosynostosis – Intermediate Risk NL

89. Craniosynostosis Risk CategoryImaging Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT Low-dose head CT: ~80% less radiation than standard head CT Optimized for evaluation of the bones/sutures Only at CMH Why you send patients here!

90. Low Radiation CT at CMH Dedicated low-dose pediatric protocols for: –Paranasal sinuses –Scoliosis spines –Cranial dermoid cysts –Facial bones –Cleft palate –Etc.

91. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull x-ray study Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations

92. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull x-ray study Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations

93. Craniosynostosis Risk CategoryImaging High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations Standard head CT:

94. Craniosynostosis Risk CategoryImaging High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations Standard head CT: Higher radiation dose

95. Craniosynostosis Risk CategoryImaging High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations Standard head CT: Higher radiation dose Infants are significantly more affected by radiation (cancer risk) Infants have a longer lifespan to manifest the effects (cancer risk)

96. Craniosynostosis Risk CategoryImaging High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations Standard head CT: Higher radiation dose Infants are significantly more affected by radiation (cancer risk) Infants have a longer lifespan to manifest the effects (cancer risk) Use to evaluate skull and brain

97. Craniosynostosis Risk CategoryImaging High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations Standard head CT: Higher radiation dose Infants are significantly more affected by radiation (cancer risk) Infants have a longer lifespan to manifest the effects (cancer risk) Use to evaluate skull and brain Used by the surgeon for pre-surgical planning This is NOT a prerequisite for a plastic surgery consultation

98. Craniosynostosis Risk CategoryImaging High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations Standard head CT: Higher radiation dose Infants are significantly more affected by radiation (cancer risk) Infants have a longer lifespan to manifest the effects (cancer risk) Use to evaluate skull and brain Used by the surgeon for pre-surgical planning This is NOT a prerequisite for a plastic surgery consultation Do not use as a screening exam for craniosynostosis

99. Craniosynostosis – High Risk History: clinical exam suggesting coronal synostosis NL

100. Craniosynostosis – High Risk History: pre-operative coronal synostosis repair

101. Craniosynostosis – High Risk History: pre-operative sagittal synostosis repair

102. Craniosynostosis – High Risk History: pre-operative sagittal synostosis repair 3D Surface Rendering Max Intensity Projection Intracranial Superior View

103. Craniosynostosis – High Risk History: pre-operative sagittal synostosis repair 11111111 11111111 11111111 11111111 11111111 11111111 11111111 11111111 11

104. Craniosynostosis – High Risk History: pre-operative lambdoid synostosis repair 111 111 111 111 111 111 111 NL

105. Craniosynostosis Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull (plain films) Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations This approach to imaging craniosynostosis and posterior plagiocephaly reduces both unnecessary imaging and radiation exposure

106. 6-month-old infant with flat posterior skull & normal development. Which study is indicated? A.3D CT B.MRI C.Ultrasound D.No imaging

107. Imaging the Misshapen Head Common causes: –Macrocephaly –Microcephaly –Craniosynostosis –Posterior plagiocephaly

108. Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI How to Image Macrocephaly: How to Image Craniosynostosis/Posterior Plagiocephaly: Imaging the Misshapen Head Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull (plain films) Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations

109. Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI How to Image Macrocephaly: How to Image Craniosynostosis/Posterior Plagiocephaly: Imaging the Misshapen Head Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull (plain films) Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations

110. Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI How to Image Macrocephaly: How to Image Craniosynostosis/Posterior Plagiocephaly: Imaging the Misshapen Head Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull (plain films) Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations

111. Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI How to Image Macrocephaly: ( How to Image Craniosynostosis/Posterior Plagiocephaly: Imaging the Misshapen Head Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull (plain films) Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations

112. Clinical Presentation & Fontanel/AgeImaging Developmentally normal with open fontanel (<6 mo)Ultrasound Developmentally normal with closed fontanel (>6 mo)CT (or MRI) Developmentally abnormal with open or closed fontanelMRI How to Image Macrocephaly: How to Image Craniosynostosis/Posterior Plagiocephaly: Imaging the Misshapen Head Risk CategoryImaging Low risk – developmentally normal and posterior or posterolateral flattening only No imaging, or 4-view skull (plain films) Intermediate risk – children who don’t clearly fit into the low or high risk group Low-dose head CT High risk – developmentally abnormal and/or obvious head deformity almost certainly needing surgery Standard head CT with 3D reformations How to Image Children in KC!

113. References 1.Arch, Michael and Donald P. Frush. “Pediatric Body MDCT: A 5-year follow up survey of scanning parameters used by Pediatric Radiologists.” AJR 2008; 191: 611-617. 2.Brenner DJ, Hall EJ. Computed tomography: an increasing source of radiation exposure. N Engl J Med 2007; 357:2277-2284. 3.Brenner, DJ Estimating cancer risks from pediatric CT: going from the qualitative to the quantitative. Pediatric Radiology 2002: 32: 228-231 4.Brenner DJ, Elliston CD, Hall EJ, and WE Berdon. Estimated risks of radiation-induced fatal cancer from pediatric CT. AJR 2001;176: 289-296 5.Cohen, MM Jr. Epidemiology of Craniosynostosis. In: Cohen, MM Jr, ed Craniosynostosis: diagnosis, evaluation, and management, 2nd ed. New York: Oxford University Press, 2000: 112-118. 6.Goske MJ, et. al. The ‘Image Gently’ campaign: increasing CT radiation dose awareness through a national education and awareness program. Pediatr Radiol 2008 38:265-269. 7.The Image Gently Campaign: Working Together to Change Practice. AJR February 2007; 100:273-274. 8.Lajeunie, E, Le Merrer, et al. Genetic study of nonsyndromic coronal craniosynostosis. Am J Med Genet 1995; 55: 500-504 9.Lee, CI, Haim, AH, Monico, EP et al. Diagnostic CT scans: assessment of patient, physician, and radiologist awareness of radiation dose and possible risks. Radiology 2004; 231: 393-398. 10.Medina, LS, R Richardson, and K Crone. Children with Suspected Craniosynostosis: A Cost Effectiveness Analysis of Diagnostic Strategies. AJR 2002; 179: 215-221. 11.“One size does not fit all: Reducing Risks from Pediatric CT” ACR Bulletin February 2001 57(2): 20- 23. 12.Slovis, Thomas L. Introduction to Seminar in Radiation Dose Reduction. Pediatric Radiology (2002) 32: 707-708 13.Silvio Podda Craniosynostosis Management. E-medicine. Accessed 3/18/11

114. Thanks/Contributed: Julianne Dean, MD Tiffany Lewis, DO Lisa Lowe, MD Trent Phan, DO Cindy Taylor, MD