For the paediatric patient who presents with multiple bone and joint pains, the diagnosis usually rests among rickets, juvenile rheumatoid arthritis, and reactive arthritis [
A four-and-half-year-old male child was brought to the outpatient clinic with a history of pain and swelling of joints for three months and inability to walk and sit for two months.
Child was well three months before, when he had fever for a few days documented at 100-101°F, which responded to antipyretics. Subsequently his parents first noticed swelling at right wrist joint followed by left knee and left ankle joint without pain or restriction of joint motion. Gradually, pain started in these joints, and was mild in intensity, dull in nature, and aggravated by movement of limbs. After one month of onset of symptoms, the child developed difficulty in walking due to painful restriction of knee motion, such that he became bed- and chair-bound.
Past history was significant for lower limb weakness one year back which was diagnosed as Guillian Barre’ Syndrome in another hospital. He was then treated with intravenous immunoglobulin for five days, and returned to normal health and activity within a month. Birth and vaccination history were insignificant. He was a student of class one.
Dietary history was significant in that the child was exclusively eating meat. There was no intake of vegetables, fruits, fresh juices, dairy products, or eggs. The family had moved from Pakistan to Dubai two years before, and the child’s current dietary habits were established when they had settled in Dubai. The child had received treatment of various kinds in Dubai without improvement, and was then brought to the Aga Khan University Hospital in Karachi, Pakistan, for further management.
On general physical examination the child was sick looking, irritable, and pale.
His weight was below the 5th percentile, though height was above the 50th. Heart rate was 130 beats per minute, respiratory rate 32 breaths per minute, temperature 36.4°C, blood pressure 110/60 mmHg, and O2 saturation 98% on room air.
On musculoskeletal examination, the child had swelling of wrist (Figure
Clinical photographs showing swelling of the right wrist (a) and left knee (b).
There were no abnormal findings on respiratory, cardiovascular, and abdominal examination.
Laboratory studies performed at the time of admission revealed severe microcytic hypochromic anemia and vitamin D insufficiency (Table
Laboratory results.
Test | Result |
---|---|
Hemoglobin | 5.4 (13.7–16.3 g/dL) |
Total leucocyte count | 15 (4–10 × 109/L), |
Platelet counts | 535 (150–400 × 109/L) |
SGPT | 8 (0–55 IU/L) |
Alkaline phosphatase | 144 (110–341 IU/L) |
Calcium | 9.3 (8.6–10.5 mg/dL) |
Phosphorus | 4.8 (2.7–4.8 mg/dL) |
Uric acid | 4.7 (<8.0 mg/dL) |
Ferritin level | 88 (7–140 ng/dL) |
Vitamin D level | 22 (30–100 ng/dL) |
Antinuclear antibody | Negative |
Rheumatoid factor | Negative |
X-rays of the knees (Figures
Anteroposterior (a) and lateral (b) X-rays of the knees showing varus deformity. Visible abnormalities include ground glass osteoporosis, thinning of the cortices, lucencies at metaphysis (Trummerfeld zone, black arrow), small corner fractures (Pelkan’s spur, solid arrow), and dense zone of provisional calcification in the physis (White line of Frankel, dotted arrow).
X-rays of the wrist (Figures
Anteroposterior (a) and lateral (b) X-rays of the hands and wrists showing severe osteopenia of the epiphyses of the phalanges (dotted arrow), metacarpals and the carpal bones, epiphyseal separation of the distal radius and ring sign (arrow).
Findings were suggestive of Vitamin C deficiency (scurvy).
The child was admitted in the hospital and after work up he was diagnosed to be suffering from scurvy. He was started on oral vitamin C replacement therapy in a dose of 250 mg daily. He was transfused packed red cells which brought hemoglobin level to 9.4 gm/dL. Oral supplementation of folic acid, iron, and multivitamins was added and the child was subsequently discharged.
On follow-up visits he showed progressive improvement in the joint swellings and pain. The range of motion of the knee joints also improved, and he started walking after about four weeks of therapy. Follow-up X-rays (Figures
Follow-up X-rays of the knees (a) and wrist (b) at 6 weeks after initiation of vitamin C replacement therapy, showing improvement in osteopenia, healing of the epiphyseal fractures, and reduction in metaphyseal lucencies.
The patient returned to Dubai after 10 weeks.
In children, bone and joint pain and refusal to walk generally alerts the physician to possible osteomyelitis, septic arthritis, and rheumatic disorders [
Radiological changes in the long bones, particularly around the knee, are most diagnostic of scurvy. The bones of the child with scurvy demonstrate osteopenia [
Cortical bone in vitamin C deficiency is characterized by thinning, which is sometimes described as a “pencil-point” cortex. Metaphyseal bone exhibits decreased trabeculae resulting in a decrease in radio-opacity similar to ground glass appearance, as seen in radiographs of our patient.
The physis exhibits thickening and sclerosis (Frankel line) which may be accompanied by a subjacent zone of lucency. This physeal thickening is known as a Frankel line, and the adjacent lucent zone on its diaphyseal side (secondary to poorly formed trabeculae) is known as the Trümmerfeld (German for “field of rubble”) zone or scurvy line [
The zone of proliferating cartilage cells is distorted, producing spicules from the metaphysis into the epiphyseal plate region. The zone of temporary calcification broadens, producing a wide, radiopaque metaphyseal band. Subjacent to this is a zone of poor-quality trabeculae, which appears radiolucent. A step-like lateral projection is found at the epiphyseal line in patients who are severely affected. Scorbutic skeletal changes are radiologically more severe in the lower extremities as evident in radiographs of our patient, whereas skeletal changes seen in rickets are allegedly more severe in the upper extremities.
Metaphyseal “beaks” and transverse lines of increased or decreased opacity may be seen in scurvy. The “beaks,” known as Pelkan spurs, are associated with healing fractures of the Trümmerfeld zone, at the periphery of the zone of calcification [
Costochondral junctions of the first 6 or 8 thoracic ribs may be expanded; this change may be related to fracturing of the zone of provisional calcification during normal respiration. The costochondral junctions are rounded and appear smooth, knobby, and steplike. The enlargement of the costochondral junctions simulates that seen in rickets.
Skull changes may produce a “hair-on-end” or crew-cut appearance secondary to marrow hyperplasia in response to anemia. Examination of individuals with known scurvy reveals other skull changes (e.g., porotic hyperostosis, also described as a hair-on-end appearance, and marrow hyperplasia); however, no sphenoid changes are reported. Sphenoid porosity has not been shown to be caused by scurvy.
Subperiosteal hemorrhages are visualized only in the healing phase of scurvy, and these are almost invariably paraepiphyseal in distribution. Epiphyseal separation often results. Healing scurvy also appears with the loss of the scurvy line, in which the only residual manifestation is a double line of ossification at the original active site.
The diagnosis of scurvy is based on a combination of clinical and radiographic findings. A dietary history compatible with insufficient intake of vitamin C for at least 1–3 months should be present for signs and symptoms of scurvy to appear [
Healing occurs rapidly with the oral administration of 100 to 200 mg/d of vitamin C. As healing occurs, the intake of vitamin C may be reduced to 50 mg/d until complete clinical and radiologic resolution has taken place [
Scurvy may occur in the modern era despite ready availability of fruits and vegetables, owing to peculiar dietary habits especially in the paediatric population. Physical signs of scurvy are often confused with rickets, a much more common condition in lower socioeconomic status regions. A high index of suspicion with a detailed dietary history is thus required for prompt diagnosis of scurvy in children with bone and joint symptoms in order to avoid unnecessary and expensive testing for rheumatological and connective tissue disorders.
Written informed consent was obtained from the patient’s father for publication of this paper and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
The authors declare that they have no conflict of interests.
All authors did a major contribution in literature search and writing the paper. All authors read and approved the final paper.