Cases in pediatric radiology

1. Cases in Pediatric Radiology Brian Wells, MSM, MPH

3. Overview of imaging

4. Imaging statistics

5. Evolving guidelines

7. In the United States in 2006:

8. 377 million diagnostic and interventional radiology exams

9. 18 million nuclear medicine exams

10. Usage in the U.S. alone accounted for:

11. ~12% of the world’s radiologic procedures

12. ~50% of the world’s nuclear medicine proceduresRadiologic and Nuclear Medicine Studies in the United Statesand Worldwide: Frequency, Radiation Dose and Comparisonwith other Radiation Sources 1950-2007. Metler, FA ea al.Radiology 2009; 253:520-531

14. Society of Nuclear Medicine and the Society for Pediatric Radiology have expanded their pediatric radiation protection initiative by standardizing doses (based on body weight) for 11 nuclear medicine procedures commonly performed in children.

15. “Children may be more sensitive to radiation from medical imaging scans than adults. A radiopharmaceutical dose which is too low may risk poor diagnostic image quality. Doses too high may expose the child to unnecessary radiation exposure without benefit”

16. ”Child-size” the amount of radiopharmaceutical used by following the new guidelines*Treves ST, Davis RT, Fahey FH. Administered radiopharmaceutical doses in children: a survey of 13 pediatric hospitals in North America. J Nucl Med 2008; 49(6):1024-7.

17. Average Exposure Amounts by Procedure 1 rad = 0.01 gray (Gy) = 0.01 Sievert (Sv) = 1 rem (roentgen-equivalent man)and 0.001 rad = 1 mrad = 0.01 mGy

23. 5% - 8% of all congenital heart defects

24. Bicuspid aortic valve seen in 75%-80% of cases

25. Two types

26. Infantile (pre-ductal) and adult (post-ductal)

27. Infantile characterized by diffuse hypoplasia or narrowing of the aorta from just distal to brachiocephalic artery to level of ductus arteriosus, typically with discrete area of constriction just proximal to the ductus but distal to the origin of the subclavian artery.

29. 15%-20% of girls with Turner syndrome

30. VSD

31. Cyanotic congenital lesions (truncus arteriosus, transposition of the great vessels

32. Mitral valve defects

33. Various syndromes

34. All angiography capable of deliniating the coarctation and collaterals (CTA / MRA / DSA)

40. Caused by raised ICP from any cause, e.g. craniosynostosis, obstructive hydrocephalus, intracranial masses, etc.

41. Convolutional marking are similar in appearance but a normal finding

42. These are shallower and usually confined to the posterior skull

49. Most common genetic disease affecting European population

50. 1 in 2000 to 3500 live births

51. Pulmonary manifestations are among the best known

52. Clinical presentation is with expected recurrent bacterial infection (Pseudomonas, S. aureus/H. influenze in first 6 months, Burkholderia cepacia) and hemoptysis.

53. Chronic cough and expectorate of copious sputum

54. Later in disease – larger volume hemoptysis and pneumothoraces

55. Can be suspected antenatally due to echogenic bowel or genetic testing

56. Diagnosis with sweat test after birth (>60 mEq/L)

57. Treatment with antibiotics, corticosteroids, pancreatic enzyme supplementation (~85%), physiotherapy, lung transplant and others

62. Inheritance can be AD, AD with new mutation or AR depending on type

63. Incidence: 1 in 12,000 to 15,000 births

64. Types I to VIII

65. Hallmark feature is fragile bones that fracture easily. Affects both bone quality and quantity.

66. Clinical presentation is highly variable, ranging from mild with no deformity, normal stature and a few fractures (I) to a form that is lethal during the perinatal period (II)

68. Osteoporosis-pseudoglioma syndrome – severe form of OI that also causes blindness

69. Cole-Carpenter syndrome – OI with craniosynotosis and ocular proptosis

70. Bruck syndrome – OI with congenital joint contractures

75. Accounts for 10% of all congenital heart disease

76. Associated with a deletion on chromosome 21q11

77. Components: VSD, overriding aorta, right ventricular outflow obstruction, right ventricular hypertrophy

78. Pink tetralogy of Fallot

82. Rare severe developmental disorder.

83. Results from X-linked dominant defect that is fatal in males and thus is only seen in females (except for rare 47-XXY cases)

84. Typical presentation is

85. Infantile spasms (salaam seizures with typical bowing of the head)

86. Corpus callosum dysgenesis (most consistent feature)

87. Distinctive chorioretinal lacunae (pathognomonic)

88. Mental retardation

89. Most also have severe impediment related to speech

90. No cure. No defined standard course of treatment.

92. Case 6

95. Guidelines

96. Clinical casesAortic coarctation Copper beaten skull Cystic fibrosis Osteogensis imperfecta Tetralogy of Fallot Aicardi Syndrome

97. Thank you! Slides available online at http://www.slideshare.net/brianwells/newsfeed http://db.tt/Q9g4y2Z