2. Rickets
Disease of the growing skeleton.
Characterised by failure of normal mineralisation.
Seen prominently at the growth plate.
Resulting in softening of the bones and deformities.
3. Causes
Mainly two types.
1. Type l
2. Type II
TYPE I - deficiency of vit D or defect in its metabolism.
*Due to deficiency of vit D.
-diminished intake eg: malnutrition
-diminished absorption
eg: lack of 25 hydroxylation of vit D
Increased degradation of vit D – anti convulsive therapy
Lack of 1 hydroxylation (autosomal recessive)
4. Type ll
. Defective absorption of phosphates through renal tubules
-hypophosphataemic rickets
-fanconi syndrome
- renal tubular acidosis
. Diminished intake or absorption of phosphate.
Nutritional deficiency continues to be the most common causes of rickets in developing
countries because of poor socio economic conditions.
5. Clinical features
Craniotabes – pressure over the soft membraneous bones of skull gives the
feeling of a ping pong ball being compressed and released.
Bossing of skull- bossing of the frontal and parietal bones becomes evident
after the age of 6 months.
Broadening of ends of long bones-prominently around wrists and knees.
Delayed teeth eruption
6. Harrison’s sulcus- a horizontal depression, along the lower part of the chest
corresponding to the insertion of diaphragm.
Pigeon chest-The sternum is prominent.
Rachitic rosary-The costochondral junctions on anterior chest wall becomes
prominent, giving rise to appearance of a rosary.
Muscular hypotonia- the child’s abdomen becomes protuberant (pot belly)
because of marked muscular hypotonia.Visceroptosis and lumbar lordosis
occurs.
Deformities –deformities of the long bones resulting in knock knees or bow
legs.
7. Radiological Features.
Delayed appearance of epiphyses.
Widening of the epiphyseal plates
Cupping of the metaphysis.
Splaying of the metaphysis
Rarefaction of the diaphyseal cortex occurs late.
Bone deformities: knock knees, bow legs, coxa vara.
10. Treatment
Medical and orthopaedic treatment.
Medical treatment:
. Administration of vit D 6,00,000 units as a single dose induces rapid
healing.
. If line of healing not seen on x ray within 3-4 weeks of therapy-same
dose is repeated.
. Child responds to therapy: maintenance dose of 400IU of vit D is given
per day.
. Child doesn’t respond even after 2nd dose: REFRACTORY RICKETS.
11. Orthopaedic Treatment
For correction of deformities by conservative or operative methods.
A. Conservative methods
A. Mild deformities correct spontaneously.
B. Designed split(mermaid splint)
C. Orthopaedic shoes
B. Operative methods
A. Moderate or severe deformities require surgery.
B. Done after 6 months of starting medical treatment
C. Corrective osteotomies done.
12. Osteomalacia
Adult counterpart of rickets.
Softening of bones.
Primarily due to deficiency of vit D.
Failure to replace the turnover of calcium and phosphorus in the organic
matrix of bone.
Bone content is demineralised and the bony substance is replaced by soft
osteoid tissue
13. Etiology
Women wearing purda- lack of exposure to sunlight.
Dietary deficiency
Under nutrition during pregnancy
Mal absorption syndrome
After partial gastrectomy.
14. Clinical features.
Bone pains-skeletal discomfort ranging from back ache to diffuse
bone pain. Bone tenderness is common.
Muscular weakness-patient feels weakness. Difficulty in climbing up
and down stairs. Waddling gait. Tetany manifests as carpopedal
spasm and facial twitching.
Spontaneous fractures- occurs in spine and results in kyphosis.
15. Investigations
Radiological examinations:
1. Diffuse rarefaction of bones
2. Looser’s zone- radiolucent zones occuring at site of stress. Common sites are
pubic rami, axillary border of scapula, ribs and medial cortex of
neck of femur. Caused by rapid resorption and slow mineralisation and may
be surrounded by a collar of callus.
3. Triradiate pelvis in females.
4. Protrusio-acetabuli i.e. the acetabulum protruding into pelvis.
Bone biopsy: Uncalcified osteoids
Serum: calcium- low, phosphates- low, alkaline phosphatase- high
16.
17. Treatment
Due to defective intake: vit D supplementation
Vit D daily maintenance dose of 400 IU.
In case of malabsorption: high dose or intramuscular dose.
In patients with renal disease: alfacalcidol may be used.
Calcium supplementation
Underlying cause treated.