Scurvy is less common in the pediatric population, but case reports still appear [1
]. A review of the literature by Noble et al. reveals twenty three case reports of scurvy in children with behaviourally restricted diets including autistic children, mentaly retarded children, and children with cerebral palsy [4
Musculoskeletal manifestations are present in 80% of patients with scurvy and are prominent in pediatric population [3
]. Musculoskeletal manifestations include subperiosteal hemorrhages leading to bone pain and musculoskeletal complaints such as limb pain, limping, swelling over long bones, and progressive leg weakness and fractures [6
]. Dermatological manifestations include petechiae, ecchymoses, hyperkeratosis, and perifollicular hemorrhage [3
]. Oral symptoms include gingival disease characterized by swelling, ecchymoses, bleeding gums, and loosening of teeth [3
]. Systemic symptoms of scurvy in children include lassitude and fatigue, failure to gain weight, loss of appetite, and irritability [6
]. In addition to these symptoms, deficiency of ascorbic acid may lead to an hypochromic microcytic anemia because of decreased absorption of iron, bleeding, and dietary deficiencies [3
]. Our two cases had severe anemia and received transfusions of packed red cells.
The radiographic findings of infantile scurvy are multiple [9
]: the most common is osteopenia but this sign is non specific; more specific signs are less common: a transverse metaphyseal line of increased density called white line or Frankel sign; a transverse metaphyseal bands of decreased density next to the Frankel sign called scurvy lines; osteoporosis of the epiphysis which is surrounded by a white line of calcification called ring sign or Wimberger; lateral metaphyseal excrescences of the beaks secondary to infarction subperiosteal hemorrhages irregular calcification and widening of the costochondral rib junction; and epiphyseal separations. These radiographic findings only become manifest after three to six months of nutritional vitamin C deficiency [9
The scintigraphic findings in scurvy are as follows: in the early stage there is a generalized increased uptake along the shaft, of the femur without widening of the shaft and when the hematoma becomes organized and calcified, there is a markedly increased club shaped uptake [11
Computed tomography shows osteopenia and joint effusion and reveals the presence of multiple subperiosteal hematomas along the shaft of the femur, tibia, and humeri.
MRI shows heterogeneous signal intensities along nearly the entire femoral shaft on both T1 and T2 weighted images, and a large collection of subperiosteal fluid with rim enhancement and surrounding soft tissue edema was enhanced. The followup MRI showed a much more notable increase in the amount of subperiosteal hematoma on both femoral shafts; hence the recurrent subperiosteal hematoma was an important clue for the diagnosis of scurvy [9
Anemia, low serum cholesterol, and albumin levels are found in most patients with scurvy. A low vitamin C concentration in the plasma is specific for the diagnosis of scurvy; however, the result is more heavily dependent on the recent ascorbic acid intake than on the body pool [3
]. A serum concentration of vitamin C (<11
micro-mole/l) suggests scurvy.
The best evidence of the presence of scurvy is the resolution of the manifestations of the disease after ascorbic acid treatment [3
]. Weinstein et al. [3
] recommend oral doses of 100 to 300
mg of vitamin C daily until body stores are replenished per serum levels. Daily fruit and vegetable intakes should include a good source of vitamin C such as citrus, berries, cruciferous vegetables, or peppers. Once a regimen of vitamin C is begun, improvement of symptoms usually begins in 24 hours, with pain diminishing in two to four days, and gingival lesions recovering in two to three weeks [6
]. With vitamin C supplementation, metaphyseal abnormalities of scurvy will completely resolve [9
]. The large shells of periosteal bone are common radiographic findings particularly during the healing phase of disease [12
Various factors contribute to nutritional deficiencies in non ambulant children with severe spastic cerebral palsy like poor intake, oral motor dysfunction, feeding problems, and use of antiepileptic drugs [13
In conclusion, scurvy is rare in children. Musculoskeletal manifestations are prominent in pediatric scurvy. The diagnosis of scurvy is made by clinical and radiographic findings and may be supported by reduced concentration of vitamin C in the serum. In children with eating difficulties, it is essential to prevent scurvy by systematic dietary supplementation of vitamin C.