SCURVY - ORTHOPAEDIC MANIFESTATIONS
Vitamin C Deficiency | Collagen Synthesis Failure | Paediatric Metabolic Bone Disease
RADIOGRAPHIC SIGNS OF SCURVY
Critical Must-Knows
- Scurvy = Vitamin C deficiency causing failure of collagen cross-linking (hydroxylation of proline and lysine)
- Subperiosteal hemorrhage is the hallmark orthopaedic manifestation - causes severe pain and pseudoparalysis
- Radiographic triad: Frankel line, Trummerfeld zone, Wimberger ring - pathognomonic for scurvy
- Corner sign and Pelkan spurs mimic metaphyseal fractures of NAI - CRITICAL differential diagnosis
- Treatment: Vitamin C 200-500 mg daily - dramatic improvement within 1-2 weeks
Examiner's Pearls
- "Scurvy causes failure of collagen TYPE I synthesis (requires vitamin C for proline/lysine hydroxylation)
- "Pseudoparalysis in scurvy = painful subperiosteal hemorrhage, NOT neurological deficit
- "Unlike rickets (growth plate disorder), scurvy affects OSTEOID formation and vessel integrity
- "Frankel line is DENSE (hypermineralized) because cartilage calcifies but cannot be resorbed - opposite to rachitic changes
Clinical Imaging
Imaging Gallery
Critical Scurvy Exam Points
Collagen Synthesis Failure
Vitamin C required for hydroxylation of proline and lysine in collagen synthesis. Without hydroxylation, collagen triple helix is unstable. Affects Type I collagen predominantly - bone matrix, blood vessels, tendons. Results in defective osteoid formation and capillary fragility.
Subperiosteal Hemorrhage
Hallmark orthopaedic finding. Capillary fragility causes bleeding under periosteum. Presents as painful swelling, tenderness, and pseudoparalysis (child refuses to move limb). Most common at distal femur and proximal tibia. Can mimic infection or malignancy.
Radiographic Triad
Frankel line (dense white metaphyseal line), Trummerfeld zone (lucent debris zone), Wimberger ring (epiphyseal ring sign). Also see corner sign/Pelkan spurs at metaphyseal margins. Generalized osteopenia with ground-glass appearance.
NAI Differential
Corner sign mimics classic metaphyseal lesions of child abuse. Key differentiators: scurvy is SYMMETRIC, associated with dietary history, gingival bleeding, and responds to vitamin C. NAI has asymmetric injuries, multiple ages, soft tissue injuries, and inconsistent history.
Scurvy vs Rickets vs NAI - Critical Differential Diagnosis
| Feature | Scurvy | Rickets | Non-Accidental Injury |
|---|---|---|---|
| Pathophysiology | Vitamin C deficiency - collagen synthesis failure | Vitamin D/calcium deficiency - mineralization failure | Trauma from abuse |
| Primary Defect | Osteoid/collagen production, vessel fragility | Cartilage mineralization at growth plate | Mechanical injury |
| Metaphyseal Changes | Dense Frankel line, corner sign/spurs | Widened, frayed, cupped metaphyses | Classic metaphyseal lesions, bucket handle |
| Epiphyseal Changes | Wimberger ring (calcified rim, osteopenic centre) | Delayed ossification, irregular | Usually normal |
| Distribution | Symmetric, bilateral, lower limb predominant | Symmetric, bilateral at all growth plates | Asymmetric, variable, often multiple ages |
| Associated Findings | Gingival bleeding, petechiae, poor wound healing | Rachitic rosary, frontal bossing, delayed fontanelle | Bruising, burns, retinal hemorrhages, inconsistent history |
| Biochemistry | Low vitamin C (ascorbic acid), normal Ca/PO4/ALP | Low vitamin D, high ALP, abnormal Ca/PO4 | Normal biochemistry |
SCURVYRadiographic Signs of Scurvy
Memory Hook:SCURVY causes subperiosteal bleeding and characteristic radiographic signs - remember the disease name spells out the X-ray features!
VITAMIN CClinical Features of Scurvy
Memory Hook:Without VITAMIN C, you get all these systemic and musculoskeletal problems - the disease tells you what nutrient is missing!
ABSENTCauses of Paediatric Scurvy
Memory Hook:Vitamin C is ABSENT from the diet in these high-risk scenarios - look for dietary history clues!
Overview and Definition
Definition
Scurvy is a systemic disease caused by severe vitamin C (ascorbic acid) deficiency, resulting in defective collagen synthesis with widespread musculoskeletal, vascular, and connective tissue manifestations. In children, the orthopaedic manifestations are particularly striking and include subperiosteal hemorrhage, pseudoparalysis, and pathognomonic radiographic changes that must be distinguished from non-accidental injury.
Vitamin C is an essential cofactor for prolyl hydroxylase and lysyl hydroxylase, enzymes required for hydroxylation of proline and lysine residues in collagen. Without hydroxylation, collagen molecules cannot form stable triple helices, leading to weak, defective connective tissue.
Epidemiology
Scurvy in developed countries is rare but not extinct:
High-Risk Populations:
- Autism spectrum disorder - restrictive eating patterns (most common cause in modern practice)
- Developmental delay with sensory processing disorders
- Neglected or abused children
- Prolonged exclusive breastfeeding without supplementation beyond 6 months
- Bottle-fed infants with boiled or pasteurized milk (vitamin C heat-labile)
- Children with gastrointestinal disorders - malabsorption, short gut syndrome
- Children with cerebral palsy on restricted diets or tube feeds
- Food fad diets imposed by parents (alternative medicine, extreme veganism without supplementation)
Historical Context:
- Described by James Lind in 1753 in sailors (citrus fruit curative)
- Barlow disease (infantile scurvy) described by Sir Thomas Barlow in 1883
- Peak incidence historically at 6-12 months (weaning period)
Modern Epidemiology:
- Incidence approximately 2-5 per 100,000 children in developed countries
- Rising incidence due to autism spectrum disorders and restrictive eating
- Usually requires greater than 3 months of severe vitamin C deficiency before symptoms appear
Pathophysiology
Vitamin C in Collagen Synthesis
Normal collagen biosynthesis:
- Procollagen synthesis in ribosome - pro-alpha chains with proline and lysine
- Hydroxylation of proline → hydroxyproline (by prolyl hydroxylase)
- Hydroxylation of lysine → hydroxylysine (by lysyl hydroxylase)
- Triple helix formation - stabilized by hydrogen bonds involving hydroxyproline
- Secretion and cleavage of propeptides
- Cross-linking via lysyl oxidase (copper-dependent, not vitamin C dependent)
Vitamin C (ascorbic acid) role:
- Essential cofactor for prolyl hydroxylase and lysyl hydroxylase
- Maintains iron in ferrous (Fe2+) state at enzyme active site
- Without vitamin C, iron oxidizes to Fe3+ and enzymes become inactive
- Result: underhydroxylated collagen that cannot form stable triple helices
Consequences of defective collagen:
- Osteoid matrix defects - impaired bone formation
- Vascular fragility - capillary basement membrane weakness
- Impaired wound healing - fibroblasts cannot produce functional collagen
- Connective tissue weakness - tendons, ligaments, gingiva
Type I Collagen
Most affected by scurvy:
- Bone matrix (90% of organic bone)
- Tendons and ligaments
- Skin (dermis)
- Blood vessel walls
- Dentin
Defective Type I collagen explains musculoskeletal and vascular manifestations.
Iron Metabolism
Additional vitamin C roles:
- Enhances non-heme iron absorption (Fe3+ → Fe2+)
- Mobilizes iron from stores
- Scurvy causes iron deficiency anemia (macrocytic/normocytic)
- Explains concurrent anemia in scurvy patients
Clinical Presentation
Systemic Features
Orthopaedic Manifestations
Subperiosteal Hemorrhage (Hallmark Finding):
- Location: Lower limbs most common (distal femur, proximal tibia)
- Presentation: Painful swelling, exquisite tenderness
- Pseudoparalysis: Child refuses to move limb, holds in frog-leg position
- Mimics: Infection (osteomyelitis), malignancy (neuroblastoma metastases), trauma
- Physical findings: Firm swelling over metaphysis, warmth, may be mistaken for cellulitis
Bone Pain:
- Severe, diffuse, especially lower limbs
- Child screams when handled ("scorbutic cry")
- Reluctance to walk or crawl
- May assume frog-leg position (hips flexed and externally rotated)
Fractures:
- Metaphyseal corner fractures (corner sign)
- Epiphyseal separation through weakened zone
- Rib fractures (costochondral junction fragility)
- Fractures with minimal or no trauma
Muscle Involvement:
- Intramuscular hematomas
- Hemarthrosis (joint bleeding)
- Muscle weakness and atrophy
Pseudoparalysis - Not Neurological
Pseudoparalysis in scurvy is due to pain avoidance, not neurological deficit. The child has full motor function but refuses to move due to severe pain from subperiosteal hemorrhage. Deep tendon reflexes are preserved. This distinguishes scurvy from polio, Guillain-Barre, and spinal pathology.
Age-Related Presentation
Infants (6-12 months) - Barlow Disease:
- Peak presentation age
- Irritability, failure to thrive
- Frog-leg posture (pithed frog position)
- Lower limb swelling (subperiosteal hemorrhage)
- Pseudoparalysis - refuses to move legs
- Rosary (costochondral swelling) - similar to rickets
Toddlers (1-3 years):
- Refusal to walk (was previously walking)
- Limping, refusing to bear weight
- Leg pain, swelling
- Easy bruising
- Gingival changes (if teeth erupted)
Older Children:
- Similar to adult presentation
- Joint pain and swelling
- Gingival bleeding
- Poor wound healing
- May have underlying restrictive eating disorder
Investigations
Laboratory Findings
Biochemical Findings in Scurvy vs Related Conditions
| Investigation | Scurvy | Rickets | NAI |
|---|---|---|---|
| Serum Vitamin C (ascorbic acid) | Very low (less than 11 micromol/L) | Normal | Normal |
| 25-OH Vitamin D | Normal | Low | Normal |
| Calcium | Normal | Low-normal | Normal |
| Phosphate | Normal | Low | Normal |
| Alkaline Phosphatase | Normal or mildly elevated | Very high | Normal |
| PTH | Normal | Elevated | Normal |
| Full Blood Count | Anemia (normocytic or macrocytic) | May have anemia | Normal |
Key Laboratory Points:
- Serum ascorbic acid less than 11 micromol/L (0.2 mg/dL) confirms deficiency
- Leukocyte vitamin C more accurate (reflects tissue stores) but not widely available
- Normal calcium, phosphate, and vitamin D distinguishes from rickets
- Check for concurrent nutritional deficiencies (iron, folate, other vitamins)
Radiographic Features
Frankel Line (White Line of Frankel)
Pathognomonic dense white line at metaphysis
- Zone of provisional calcification becomes hyperdense
- Cartilage calcifies normally but cannot be resorbed
- Appears as bright white transverse line at metaphysis
- Best seen at knee (distal femur, proximal tibia)
- Opposite to rickets (which has loss of this line)
Trummerfeld Zone (Debris Zone)
Lucent band beneath Frankel line
- "Debris" or "rubble" zone (German: Trummerfeld = rubble field)
- Represents microfractures and cartilage debris
- Area of structural weakness at cartilage-bone junction
- Predisposes to epiphyseal separation
- Horizontal lucency parallel to Frankel line
Wimberger Ring
Dense ring around epiphysis with lucent centre
- Calcified rim of epiphysis (normal peripheral mineralization)
- Central epiphysis is osteoporotic (deficient osteoid)
- Creates "ring" or "halo" appearance
- Best seen at knee epiphyses
- Also called "pencil thin cortex" or "ring epiphysis"
Corner Sign / Pelkan Spurs
Critical NAI Differential
- Lateral metaphyseal corner defects or spurs
- Represent microfractures at weak metaphyseal corners
- Pelkan spurs = healing callus at corner fractures
- Appear similar to classic metaphyseal lesions of abuse
- Key difference: symmetric, bilateral in scurvy
Additional Radiographic Features:
- Generalized osteopenia - ground-glass appearance of bones
- Thin cortices - pencil-thin cortical bone
- Subperiosteal new bone - calcified subperiosteal hematoma (healing phase)
- Metaphyseal spurs - exuberant periosteal reaction from hemorrhage
- Epiphyseal displacement - through weakened Trummerfeld zone
Best Radiographs:
- Knees (AP and lateral) - most sensitive location
- Wrists (AP) - distal radius and ulna
- Chest - rib changes, costochondral junction
Differential Diagnosis
Scurvy vs Non-Accidental Injury - Medicolegal Importance
Scurvy can mimic child abuse and vice versa. Failure to diagnose scurvy may lead to wrongful accusation of parents. Failure to diagnose NAI may leave a child in danger. Key differentiating features:
Favors Scurvy:
- Symmetric bilateral involvement
- Dietary history of restrictive eating
- Systemic signs (gingival bleeding, petechiae, corkscrew hairs)
- Low serum vitamin C
- Rapid response to vitamin C treatment
Favors NAI:
- Asymmetric injuries of varying ages
- Inconsistent or changing history
- Delay in seeking care
- Retinal hemorrhages, subdural hematoma
- Normal vitamin C levels
- Soft tissue injuries (bruising in unusual locations)
When in doubt: Test vitamin C levels, involve child protection team, and treat with vitamin C while investigation proceeds. The two conditions can coexist.
Differential Diagnosis of Paediatric Limb Swelling and Pseudoparalysis
| Condition | Key Features | Investigations |
|---|---|---|
| Scurvy | Symmetric, dietary history, gingival bleeding, Frankel line on X-ray | Low vitamin C, normal Ca/PO4/ALP |
| Non-Accidental Injury | Asymmetric, inconsistent history, varying age fractures, soft tissue injury | Normal biochemistry, skeletal survey shows multiple fractures |
| Osteomyelitis | Fever, single bone involvement, elevated inflammatory markers | Raised CRP/ESR, blood culture positive, MRI shows abscess |
| Neuroblastoma Metastases | Abdominal mass, periorbital ecchymoses, systemic illness | Elevated catecholamines, bone marrow involvement, MIBG scan positive |
| Leukemia | Pallor, hepatosplenomegaly, lymphadenopathy, bleeding | Abnormal blood film, bone marrow confirms diagnosis |
| Rickets | Widened wrists/ankles, rachitic rosary, bowing, frayed metaphyses | Low vitamin D, high ALP, widened growth plates on X-ray |
Management
Vitamin C Replacement
Treatment Protocol:
- Dose: 200-500 mg vitamin C daily (oral or IV)
- Duration: Until clinical and biochemical resolution (usually 1-3 months)
- Maintenance: Ensure adequate dietary vitamin C long-term
Expected Response:
- Pain relief: Within 24-48 hours
- Bleeding stops: Within 1 week
- Bone tenderness improves: 1-2 weeks
- Radiographic healing: 2-4 weeks (subperiosteal calcification visible)
- Complete bone remodeling: 3-6 months
Route of Administration:
- Oral: Preferred if tolerated (ascorbic acid tablets or liquid)
- IV: For severe cases, vomiting, or malabsorption
- No need for loading dose - standard doses effective
Concurrent Nutritional Support:
- Address other nutritional deficiencies (common in restrictive diets)
- Iron supplementation if anemic
- Folate if macrocytic anemia
- Consider dietitian referral
- Address underlying eating disorder (autism, sensory issues)
Rapid Response = Diagnostic Confirmation
Dramatic improvement within 24-48 hours after vitamin C administration is virtually diagnostic of scurvy. Pain relief occurs before radiographic changes. If no improvement by 1 week, reconsider the diagnosis.
Evidence Base
Scurvy in Developed Countries - Modern Epidemiology
- Autism spectrum disorder is the most common risk factor in modern pediatric scurvy
- Mean age of presentation 6-8 years (older than historical Barlow disease)
- 90% had restrictive eating patterns or developmental disorders
- Delay in diagnosis common due to low clinical suspicion
Radiographic Features Distinguishing Scurvy from NAI
- Frankel line sensitivity 95% for scurvy diagnosis
- Bilateral symmetry present in 100% of scurvy vs 15% of NAI
- Wimberger ring highly specific for scurvy (not seen in NAI or rickets)
- Corner sign alone insufficient to differentiate - need full radiographic assessment
Response to Treatment in Pediatric Scurvy
- Clinical improvement (pain relief) within 24-48 hours in all cases
- Radiographic healing visible by 2 weeks (subperiosteal calcification)
- Complete bone remodeling by 3-6 months
- No surgical intervention required in any case
Scurvy vs Rickets - Distinguishing Radiographic Features
- Frankel line (dense) in scurvy vs absent/irregular ZPC in rickets
- Metaphyseal fraying absent in scurvy (present in 100% rickets)
- Generalized osteopenia more pronounced in scurvy
- Epiphyseal changes opposite: Wimberger ring (scurvy) vs irregular epiphysis (rickets)
Australian Context
Epidemiology in Australia
Scurvy in Australia is rare but continues to be reported, particularly in children with restrictive eating patterns. The Australian Paediatric Surveillance Unit (APSU) has documented cases associated with autism spectrum disorder, developmental delay, and food selectivity. Indigenous Australian children may be at increased risk in remote communities with limited access to fresh fruit and vegetables, though systematic data are limited.
Management Considerations
Australian eTG guidelines recommend vitamin C supplementation for confirmed deficiency at doses of 100-300 mg daily in children, with higher doses (up to 500 mg daily) for severe deficiency with skeletal manifestations. Most vitamin C supplements are readily available over-the-counter and are PBS-subsidized for documented deficiency states. Referral to paediatric dietitians through public hospital outpatient services is recommended for children with restrictive eating disorders to prevent recurrence. Multidisciplinary involvement including developmental paediatricians, occupational therapists, and speech pathologists is often necessary for children with autism spectrum disorder or sensory processing difficulties.
Child Protection Considerations
When scurvy is suspected, clinicians must consider both nutritional neglect and the possibility of the presentation mimicking non-accidental injury. Australian child protection guidelines require mandatory reporting of suspected abuse or neglect. However, scurvy in the context of a child with known developmental disorder and restricted eating is usually not neglect but reflects the significant challenges families face. A collaborative, supportive approach with families is recommended while ensuring appropriate safeguards. Vitamin C levels should be checked before escalating child protection concerns, and the rapid response to vitamin C treatment can help clarify the diagnosis.
Exam Viva Scenarios
Practice these scenarios to excel in your viva examination
Viva Scenario 1: Limping Toddler with Refusal to Walk
"A 2-year-old boy is referred with a 2-week history of refusing to walk. He was previously walking independently. He cries when his legs are touched. There is no history of trauma. He is an extremely selective eater, accepting only white bread and milk. On examination, he is irritable, has swelling over both distal thighs, and assumes a frog-leg position. His gums appear swollen and bleed when touched."
Viva Scenario 2: Radiographic Findings Mimicking Child Abuse
"A 15-month-old girl presents with irritability and leg swelling. The parents report she has been refusing to eat anything except formula for months. X-rays show bilateral metaphyseal corner lesions at the knees and calcified subperiosteal elevation along the femurs. The treating team is concerned about non-accidental injury."
Viva Scenario 3: Child with Autism and Bone Pain
"An 8-year-old boy with autism spectrum disorder is referred for severe bilateral leg pain. His parents report he has an extremely restricted diet, eating only chicken nuggets and chips for the past 2 years. He has stopped walking over the past month. Examination shows painful swelling over both tibias, petechiae on his legs, and bleeding from his gums when he opens his mouth. His pediatrician has checked basic bloods showing normocytic anemia but normal calcium, phosphate, and alkaline phosphatase."
Scurvy - Orthopaedic Exam Quick Reference
High-Yield Exam Summary
Pathophysiology - One Liner
- •Vitamin C deficiency → failure of proline/lysine hydroxylation → defective collagen triple helix → weak osteoid + capillary fragility
- •Affects Type I collagen (bone, vessels, tendons) - NOT cartilage (so growth plate calcifies normally)
- •Frankel line = dense because cartilage calcifies but cannot be resorbed (opposite to rickets which has widened/frayed metaphysis)
Clinical Presentation - Key Features
- •Subperiosteal hemorrhage → painful swelling, pseudoparalysis, frog-leg position
- •Gingival bleeding and swelling (only if teeth erupted)
- •Petechiae, ecchymoses, corkscrew hairs
- •Peak age: 6-24 months (historical) or older in autism/restricted eating (modern)
Radiographic Signs - The Classic Triad Plus
- •FRANKEL LINE - dense white line at metaphysis (hypermineralized ZPC)
- •TRUMMERFELD ZONE - lucent debris band beneath Frankel line
- •WIMBERGER RING - dense epiphyseal rim with osteoporotic centre
- •CORNER SIGN / PELKAN SPURS - lateral metaphyseal corner defects (mimics NAI!)
- •Generalized osteopenia with ground-glass appearance
Differential from NAI - Critical Points
- •SCURVY: Bilateral symmetric, dietary history, gingival/skin bleeding, low vitamin C, rapid response to treatment
- •NAI: Asymmetric, inconsistent history, varying fracture ages, soft tissue injury, normal vitamin C
- •Both can coexist - nutritional neglect may accompany physical abuse
Biochemistry Pattern
- •Vitamin C: VERY LOW (less than 11 micromol/L)
- •Calcium, phosphate, vitamin D: NORMAL
- •ALP: NORMAL or slightly elevated (vs very high in rickets)
- •FBC: Normocytic or macrocytic anemia
Treatment - Exam Answer
- •Vitamin C 200-500 mg daily (oral or IV)
- •Pain relief within 24-48 hours (diagnostic!)
- •Radiographic healing by 2-4 weeks
- •Complete remodeling by 3-6 months
- •No surgery needed - fractures heal with medical treatment
Modern Risk Factors
- •Autism spectrum disorder with restrictive eating (MOST COMMON modern cause)
- •Developmental delay with sensory food aversions
- •Neglect or extreme parental dietary beliefs
- •Prolonged formula without supplementation
- •Processed food diet (nuggets, chips, white bread = no vitamin C)
References
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