Presentations

Abstract

Introduction:

While rare in the United States, malnutrition of at-risk populations needs to be considered in a differential diagnosis. X-ray findings of scurvy are well established. However, detecting MRI findings of scurvy can spare pediatric patients unnecessary exposure to radiation and invasive procedures such as a bone marrow biopsy to rule out leukemia. This case presents a 7-year old patient suffering from scurvy.

Case Information:

7-year-old male patient presents with one month of progressively worsening lower extremity weakness & pain (L worse than R) now with an inability to walk. He also presents with left calf tenderness with overlying bruising.MRI findings in this patient include symmetrical bone marrow edema in all visualized metaphyses bilaterally on STIR images. On T1 weighted images, there is symmetric hypointensity in all visualized metaphyses. In the calf on the T1 fat suppressed image there is intrinsic hyperintense signal consistent with blood products indicative of hemorrhage. An axial view of the distal femurs demonstrates bilateral hyperintense signal in the femoral bone marrow and mild surrounding periostitis.

Laboratory testing demonstrates vitamin C deficiency <0.1 (Reference range: 0.2 to 1.5 mg/dL), vitamin A, D deficiency, and an MCV of 68.

The child has been improving well following a prescription of vitamin C 250mg daily, vitamin D 50,000 units, and iron supplements. Discussion:

This 7-year-old male patient presents with one month of progressively worsening lower extremity weakness & pain (L worse than R) now with an inability to walk. He also presents with left calf tenderness with overlying bruising. MRI findings in this patient include symmetrical bone marrow edema in all visualized metaphyses bilaterally. In the calf on the T1 fat suppressed image there is intrinsic hyperintense signal consistent with blood products indicative of hemorrhage. An axial view of the distal femurs demonstrates bilateral hyperintense signal in the femoral bone marrow and mild surrounding periostitis.

This patient's history coupled with the MRI findings, as in similar cases, gives rise to the differential diagnoses of osteomyelitis, chronic recurrent multifocal osteomyelitis (CRMO), leukemia, and scurvy. Scurvy is more likely than CRMO as MRI findings are more symmetrical. Similarly, osteomyelitis findings on MRI are typically focal. Leukemia is less likely as it is typically a diffuse bone marrow signal abnormality. Recognizing these MRI findings in correspondence with clinical presentation are indicative of scurvy, serum vitamin C level are checked revealing vitamin C deficiency <0.1 (Reference range 0.2-1.5 mg/dL), Common clinical presentations of scurvy include easy bruising, swollen gums, poor wound healing, and anemia. Symptoms present within one-to-three months after initiation of a vitamin- deficient diet. This child also has an MCV of 68 and vitamin A and D deficiency.While x-ray findings of scurvy are well established, this pediatric patient has been spared exposure to radiation upon recognition of signs of scurvy on MRI. Such x-ray findings of scurvy include thin epiphyseal cortex, dense band of provisional metaphyseal calcifications (Frankel line) with an adjacent radiolucent line (Trummerfeld zone), and metaphyseal beaking.

Scurvy results from a defect of collagen hydroxylation caused by a deficiency of the co-factor ascorbic acid (vitamin C). Risk factors include food avoidant behavior in patients with neurological illnesses such as autism, restricted diet lacking fruits and vegetables, and nutrition neglect. The unstable collagen predisposes to weakened bones, increased susceptibility of fractures, and fragile vascular walls in areas of greatest growth such as distal femur and proximal tibia and fibula.

The child has been improving well following a prescription of vitamin C 250mg daily, vitamin D 50,000 units, and iron supplements.

Conclusion:

Scurvy results from vitamin C deficiency leading to defect of collagen hydroxylation. This results in poor wound healing, anemia, swollen gums, and hemorrhage. While x-ray findings of scurvy are well established, detecting MRI findings of scurvy can spare pediatric patients unnecessary exposure to radiation and invasive procedures. MRI findings of scurvy include bilateral symmetrical bone marrow edema in the metaphysis in the long bones with overlying soft tissue edema.