Bone and Soft-Tissue Disorders
= BARLOW DISEASE = HYPOVITAMINOSIS C
= vitamin C (= ascorbic acid) deficiency with defective osteogenesis from abnormal osteoblast function
Infantile Scurvy
Age: 69 months (maternal vitamin C protects for first 6 months)
Predisposed: feeding with pasteurized / boiled milk
Pathogenesis: abnormal collagen formation
- irritability
- tenderness + weakness of lower limbs
- scorbutic rosary of ribs
- bleeding of gums ← teething
- legs drawn up + widely spread = pseudoparalysis
Location: distal femur (esp. medial side), proximal and distal tibia + fibula, distal radius + ulna, proximal humerus, sternal end of ribs
- ground-glass osteoporosis (CHARACTERISTIC)
- cortical thinning
- soft-tissue edema (rare)
- Metaphysis - white line = metaphyseal zone of preparatory calcification (DDx: lead / phosphorus poisoning, bismuth treatment, healing rickets)
- Trümmerfeld zone = radiolucent zone on shaft side of Fränkel white line (site of subepiphyseal infraction)
- Pelkan spurs = metaphyseal spurs projecting at right angles to shaft axis
 [Karl Francis Pel(i)kan (1890????), University of California]
- Park corner sign = subepiphyseal infraction / comminution resulting in mushrooming / cupping of epiphysis (DDx: syphilis, rickets)
 [E. Park, 1935, pathologist]
 
- Epiphysis - Wimberger ring = sclerotic ring around low-density epiphysis ← osteopenia of epiphysis
 
- Diaphysis - subperiosteal hematoma with calcification of elevated periosteum (sure sign of healing)
 
- Teeth - cyst formation + hemorrhage in enamel
 
DDx: TORCH infections, leukemia, neuroblastoma
Adult Scurvy
Frequency: rare
- hemarthrosis + bleeding at synchondrosis
Outline