Imaging in Rickets & Scurvy.pptx

Rickets is the softening and weakening of bones in children, usually because of an extreme and prolonged vitamin D deficiency. Rare inherited problems also can cause rickets.

Vitamin D helps your child's body absorb calcium and phosphorus from food. Not enough vitamin D makes it difficult to maintain proper calcium and phosphorus levels in bones, which can cause rickets.

Adding vitamin D or calcium to the diet generally corrects the bone problems associated with rickets. When rickets is due to another underlying medical problem, your child may need additional medications or other treatment. Some skeletal deformities caused by rickets may require corrective surgery.

Rare inherited disorders related to low levels of phosphorus, the other mineral component in bone, may require other medications.

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Book: Mayo Clinic Family Health Book, 5th Edition
Symptoms
Signs and symptoms of rickets can include:

Delayed growth
Delayed motor skills
Pain in the spine, pelvis and legs
Muscle weakness
Because rickets softens the areas of growing tissue at the ends of a child's bones (growth plates), it can cause skeletal deformities such as:

Bowed legs or knock knees
Thickened wrists and ankles
Breastbone projection

1. Imaging in Rickets & Scurvy Presented by- Moderator- Dr. Shivani Gupta Dr. Shalini Agarwal Junior Resident 1 Dept. of Radio-diagnosis PGIMS Rohtak

2. PHYSIS/ GROWTH PLATE It is the anatomical difference between growing and mature bone. It appears radiologically as a lucency between the epiphysis and the metaphysis . It represents the site where longitudinal bone growth occurs. It is the primary site for the effect of metabolic and endocrine bone disorder.

3. 3 zones of physis - Resting/ germinal zone Proliferating zone Hypertrophic zone : zone of maturation, degeneration and provisional calcification

4. RICKETS

5. INTRODUCTION It is a disease of childhood characterized by failure of mineralization of osteoid tissue in developing skeleton, particularly at the growth plate. Age: 6-12 months. At risk- Dark pigmented individuals, infants breast-fed for prolonged periods of time by a multiparous mother, at higher altitudes

6. Pathology of rickets v/s scurvy

7. PATHOLOGY Decrease in quantity of calcified osteoid & increase in uncalcified osteoid . Osteopenia Cartilage cells at physis grow normally but FAIL TO CALCIFY. Growth plate is widened due to overgrown & hypertrophied cartilage

8. CAUSES OF RICKETS 1.Vitamin-D deficiency 2.Abnormality in phosphate metabolism. 3.Calcium Deficiency

9. CLINICAL FEATURES First 6 months : Tetanic convulsions Irritability, weakness Delayed development Small stature Bony deformities and pain Rachitic rosary Swelling of wrist and costocartilage Thoracic kyphosis with pigeon chest Harrison’s sulcus Greenstick fractures are common

10. RADIOLOGICAL FEATURES MC and non specific finding : osteopenia Loss of provisional zone of calcification Changes are seen at open growth plate Especially visible at fast growing growth plates like costochondral junction of the middle ribs, knees, wrists & ankles. Earliest sign : distal ends of radius and ulna. Ulnar growth plate grows more rapidly so manifestations are seen earlier in ulnar growth plate

11. WIDENING OF GROWTH PLATE Earliest and specific radiological change Due to increase in cartilaginous cell mass and loss of normal zone of provisional calcification

12. METAPHYSEAL FRAYING Irregular metaphyseal margins Cause- Disorganisation of spongy bone in metaphyseal region

13. METAPHYSEAL CUPPING AND WIDENING Protrusion of bulky mass of cartilageneous cells in the zone of hypertrophy into the poorly mineralized metaphysis Cupping is common in both ends of fibula and distal end of ulna and tibia Not seen in bones of elbow Paint-brush metaphysis

14. SHAFT ABNORMALITIES Rarefaction of shaft due to loss of mineral content Cortex becomes thin with a coarse texture

15. SKELETAL DEFORMITIES Ribs : rachitic rosary Skull : Craniotabes Long bones : bowing deformities Spine : scoliosis and vertebral end plate deformities (when weight bearing becomes prominent) Pelvis : triradiate configuration

16. RACHITIC ROSARY Bulbous enlargement of costochondral junction especially middle ribs Cause- Widening of rib epiphyseal cartilage.

17. CRANIOTABES Excess osteoid deposition in frontal and parietal regions with posterior flattening of skull due to supine posture of infant Squared configuration of skull Demineralisation of skull

18. BOWING OF LONG BONES Cause-Displacement of growth centres owing to asymmetrical m u sc u l o te n d i nous pull on the weakened growth plate

19. TRIRADIATE PELVIS Protrusion of hip and spine into the soft pelvis with protrussio acetabuli

20. PA radiograph of both hands shows diffuse osteopenia , cupping, fraying & splaying of metaphyses of b/l distal radius and ulna.

21. AP Radiograph of both lower limbs obtained 2 years after active disease phase shows bowing of tibia and fibula and transverse sclerotic bands at the metaphysis parallel to the growth plate (Harris growth arrest lines or park lines)

22. SIGNS OF HEALING RICKETS Seen within 2-3 weeks of adequate therapy Total calcification is usually complete in 2 months Signs : Reappearance of dense zone of provisional calcification : first evidence Increase in cupping of healing metaphysis Recalcification of subperiosteal osteoid resulting in thick cortex surrounding the shaft Sharply defined ossification centres

23. FIRST E/O HEALING RICKETS: REAPPEARANCE OF DENSE ZONE OF PROVISIONAL CALCIFICATION Seen as a transverse line of increased density which appears beyond the visible end of shaft with metaphysis interposed between two radiolucent areas

24. Complete healing and restoration of normal structure is the rule in rickets even if severe changes are present during the active stage !

25. SCURVY A.k.a. Barlow’s disease or hypovitaminosis C

26. Introduction Scurvy is a nutritional bone disorder which occurs due to long term deficiency of Vitamin C. Infantile Scurvy: due to pasteurised or boiled milk preparations Age: 8-14 months Latent period- 4 months before symptoms and skeletal changes become apparent.

27. PA THOLOGY Vitamin C is necessary for hydroxylation of proline to hydroxyproline which is vital for collagen synthesis. Vitamin C is also necessary for endothelial lining Deficiency causes increased vascular fragility Osteoblasts require vit C to form mature osteoid tissue. Decreased osteoblastic activity Generalised osteopenia and osteoporosis Cartilage proliferation is decreased but mineralisation is normal.

28. Clinical features Clinical hallmark : Spontaneous haemorrhage i.e. cutaneous petechiae,bleeding gums,melena & hematuria . Progressive irritability with tender edematous limbs and a tendency to lie supine & motionless with the thighs abducted (frog-leg position, pseudoparalysis ) Bulging at costochondral junction (Scorbutic rosary-Sharp pain & tender)

29. WHITE LINE OF FRENKEL Dense zone of provisional calcification. Radiodense line in the zone of provisional calcification at the growing metaphysis Cause- Cartilage proliferation decreased with normal mineralisation . Conversion into bone is delayed.

30. TRUMMERFELD ZONE ( Scorbutic zone ) Transverse radiolucent band direc t ly beneath the zone of provisonal calcification . Cause- S uppressed osteoblastic activity with normal mineralisation leading to disordered osteoid formation. Trabecular bone mass is decreased .

31. PELKAN’S SPUR Bony protuberances at the metaphyseal margins at right angles to the shaft. Cause- Zone of provisional calcification extends beyond the margins of the metaphysis resulting in periosteal elevation and marginal spur formation

32. WIMBERGER 'S SIGN (Ring epiphysis) Epiphysis is small & sharply marginated by sclerotic rim with central portion more radiolucent. Cause- D ecrease d cartilage proliferation and unimpaired mineralization ( sclerosis ) Differentiates healing rickets and scurvy

33. CORNER (ANGLE) SIGN Irregularity of the metaphyseal margins . Cause- I nf ar ctions of the epiphyseal- metaphyseal junction

34. SUBP E RIOSTEAL HEMORRAGES Seen in ends of long bones(femur,tibia, humerus) May cause periosteal elevation and new bone formation Cause-Increased capillary fragility

35. Coronal T2-weighted fat-suppressed MR image of both distal femoral metadiaphyses shows heterogeneously increased T2 signal intensity in the marrow (*) and around the bone (arrows)

36. HEALING SCURVY On vitamin C therapy, all changes are reversible though a single growth arrest line may remain in metaphysis as residual frenkel’s line. Following therapy, subperiosteal hematomas rapidly calcify and demarcate.

37. Thank you !