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
Clinical 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
| S | Subperiosteal hemorrhage Calcified subperiosteal bleeding - lifting of periosteum |
| C | Corner sign (Pelkan spurs) Lateral metaphyseal corner defects - mimics NAI |
| U | Undermineralized bone Generalized osteopenia with ground-glass appearance |
| R | Ring of Wimberger Dense epiphyseal ring around osteoporotic centre |
| V | Very dense Frankel line White line at metaphysis - zone of provisional calcification |
| Y | Yellow marrow zone (Trummerfeld) Lucent debris zone beneath Frankel line |
| S | Subperiosteal hemorrhage Calcified subperiosteal bleeding - lifting of periosteum | U | Undermineralized bone Generalized osteopenia with ground-glass appearance | V | Very dense Frankel line White line at metaphysis - zone of provisional calcification |
| C | Corner sign (Pelkan spurs) Lateral metaphyseal corner defects - mimics NAI | R | Ring of Wimberger Dense epiphyseal ring around osteoporotic centre | Y | Yellow marrow zone (Trummerfeld) Lucent debris zone beneath Frankel line |
Hook:SCURVY causes subperiosteal bleeding and characteristic radiographic signs - remember the disease name spells out the X-ray features!
VITAMIN CClinical Features of Scurvy
| V | Vascular fragility Petechiae, purpura, easy bruising from capillary weakness |
| I | Irritability Marked irritability and pain - child screams when touched |
| T | Tenderness of extremities Severe pain over long bones from subperiosteal hemorrhage |
| A | Anemia Macrocytic anemia from impaired iron absorption |
| M | Mouth bleeding Gingival swelling, bleeding, and tooth loosening |
| I | Impaired wound healing Poor collagen cross-linking prevents healing |
| N | Not moving limbs Pseudoparalysis - painful immobility, frog-leg position |
| C | Corkscrew hairs Coiled fragile hairs from abnormal keratin (pathognomonic) |
| V | Vascular fragility Petechiae, purpura, easy bruising from capillary weakness | A | Anemia Macrocytic anemia from impaired iron absorption | N | Not moving limbs Pseudoparalysis - painful immobility, frog-leg position |
| I | Irritability Marked irritability and pain - child screams when touched | M | Mouth bleeding Gingival swelling, bleeding, and tooth loosening | C | Corkscrew hairs Coiled fragile hairs from abnormal keratin (pathognomonic) |
| T | Tenderness of extremities Severe pain over long bones from subperiosteal hemorrhage | I | Impaired wound healing Poor collagen cross-linking prevents healing |
Hook:Without VITAMIN C, you get all these systemic and musculoskeletal problems - the disease tells you what nutrient is missing!
ABSENTCauses of Paediatric Scurvy
| A | Autism spectrum disorder Restrictive eating patterns, food texture aversions |
| B | Bottle-fed with boiled milk Vitamin C destroyed by prolonged boiling |
| S | Sensory processing disorders Limited food acceptance in developmental disorders |
| E | Extreme dietary restriction Parents with unusual diets, food fads, neglect |
| N | Neurological disability Cerebral palsy, tube feeding without vitamin C |
| T | Toddler diet - no fruits/vegetables Prolonged exclusive milk feeding, junk food diet |
| A | Autism spectrum disorder Restrictive eating patterns, food texture aversions | S | Sensory processing disorders Limited food acceptance in developmental disorders | N | Neurological disability Cerebral palsy, tube feeding without vitamin C |
| B | Bottle-fed with boiled milk Vitamin C destroyed by prolonged boiling | E | Extreme dietary restriction Parents with unusual diets, food fads, neglect | T | Toddler diet - no fruits/vegetables Prolonged exclusive milk feeding, junk food diet |
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 reflects tissue stores better but is rarely available
- Normal calcium, phosphate and vitamin D distinguish scurvy from rickets; ALP normal-to-mildly raised (very high in rickets)
- Inflammatory markers (CRP/ESR) may be elevated - this does NOT exclude scurvy and can mislead toward sepsis or rheumatic disease
- Screen for concurrent deficiencies (iron, folate) in restricted diets
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.
Complications
Scurvy is highly reversible, so most complications stem from delayed or missed diagnosis rather than the deficiency itself. With timely vitamin C the orthopaedic outcome is excellent.
Disease-Related
- Epiphyseal separation / displacement through the weakened Trummerfeld zone (usually heals without deformity once treated)
- Growth disturbance - rare; possible after significant physeal injury or very prolonged untreated disease
- Severe anaemia from capillary bleeding and impaired iron/folate handling
- Persistent pain and immobility with disuse muscle atrophy during the untreated phase
Diagnosis / Iatrogenic
- Invasive workup harms - biopsy, bone-marrow sampling, prolonged antibiotics or empiric chemotherapy from misdiagnosis as infection, malignancy, JIA or vasculitis
- Wrongful safeguarding action if scorbutic corner lesions are misread as non-accidental injury
- Missed coexisting pathology if an empiric vitamin C trial is relied on alone
- Recurrence if the underlying restrictive diet is not addressed
The Real Complication is the Workup
The most consequential "complication" of scurvy in modern practice is the harm and cost of an extensive, invasive diagnostic workup before a simple ascorbic acid level is checked. Diagnose early and the skeletal complications largely vanish.
Evidence Base
Musculoskeletal Manifestations of Scurvy
- Musculoskeletal symptoms occur in approximately 80% of cases - arthralgia, myalgia, hemarthrosis, muscular hematoma
- Vitamin C depletion causes defective osteoid matrix formation and increased bone resorption
- Imaging may show osteopenia, periosteal proliferation, osteolysis and osteonecrosis; children develop lower-limb pain from subperiosteal bleeding
- Serum ascorbic acid below 2.5 mg/L confirms diagnosis; supplementation gives prompt resolution
Scurvy in the Paediatric Age Group - A Disease Often Forgotten
- Children with abnormal dietary habits, mental illness or physical disability are most at risk
- Common radiological findings: subperiosteal haematoma, ring epiphysis, dense metaphyseal (Frankel) white line and the lucent rarefaction zone, with epiphyseal slips
- Rarity means scurvy is seldom suspected, frequently causing delayed recognition
- High index of suspicion plus bilateral limb radiographs aid diagnosis of this 'eternal masquerader'
Modern American Scurvy - Vitamin C Deficiency at a Children's Hospital
- At-risk groups were iron overload from transfusion (sickle cell/thalassaemia), neurologic disorders, and chemotherapy/bone-marrow transplant - NOT dietary deficiency in otherwise-well children
- Deficiency is frequently missed on clinical evaluation and imaging despite extensive workup
- Radiographs showed ill-defined sclerotic and lucent metaphyseal bands, mainly at the knee
- MRI showed diffuse T2 hyperintensity in lower-limb metaphyses, periosteal reaction and soft-tissue oedema
Scurvy as a Manifestation of Food Selectivity in Children with Autism
- All children had a developmental disorder, most commonly autism, with long-standing food selectivity (diets devoid of fruit and vegetables)
- None had been receiving a multivitamin supplement
- All presented with limp, prompting an elaborate panel of tests and procedures before scurvy was recognised
- Vitamin C led to rapid recovery, underscoring nutritional causes of musculoskeletal symptoms in autism
Guidelines, Registries & Global Practice
Global Epidemiology
Scurvy persists worldwide across both high- and low-resource settings, and is best understood as a disease of dietary pattern rather than geography.
| Setting | Typical at-risk group | Driver |
|---|---|---|
| High-income countries | Children with autism / developmental delay, restrictive eaters, transfusion-dependent iron overload, post-chemotherapy/BMT | Sensory food selectivity; processed diets; impaired absorption |
| Limited-resource / humanitarian settings | Refugees, famine and conflict populations, the institutionalised elderly | Restricted access to fresh fruit and vegetables |
| All settings | Exclusive boiled/over-processed milk feeding, food-fad diets, alcohol dependence, isolated/elderly poor | Ascorbic acid is heat-labile and not stored long-term |
US NHANES population data confirm measurable vitamin C deficiency even in food-abundant countries, concentrated in low-income, smoking and restricted-diet groups. Pooled paediatric series consistently identify autism spectrum disorder and developmental delay as the dominant modern risk factors, with diagnosis frequently delayed because scurvy is "off the radar."
Side-by-Side Guidance
There is no high-level interventional trial base for scurvy; management rests on consistent nutritional and paediatric guidance rather than competing society protocols.
| Source | Position |
|---|---|
| WHO (nutrition guidance) | Defines deficiency thresholds and emphasises dietary adequacy / fortification in at-risk populations; treatment-dose ascorbic acid restores stores rapidly |
| BNF for Children / paediatric formularies (UK) | Therapeutic vitamin C for established deficiency, then maintenance via diet/supplement |
| US dietary reference intakes (IOM/NAM) | Define RDA and the intake gap that precedes clinical scurvy |
| Paediatric nutrition consensus (ESPGHAN-aligned practice) | Screen restrictive eaters and tube-fed/neurodisabled children; supplement and involve dietetics |
The practical message is uniform across bodies: confirm with serum ascorbic acid where available, give therapeutic vitamin C, correct the underlying dietary cause, and expect rapid response.
Registry and Resource Notes
Scurvy is not tracked in arthroplasty/implant registries; surveillance comes from paediatric and nutrition reporting networks and published case series. In high-resource settings, serum ascorbic acid and MRI are available to confirm the diagnosis and avoid invasive workup. In limited-resource settings, diagnosis is necessarily clinical and therapeutic (a trial of vitamin C with documented rapid response), and prevention via fresh produce, fortification, or supplementation is the priority.
Safeguarding Considerations (Global Principle)
When scurvy is suspected, clinicians must weigh both nutritional neglect and the possibility of the picture mimicking (or coexisting with) non-accidental injury. Most jurisdictions mandate reporting of suspected child abuse or neglect. Scurvy in a child with a known developmental disorder and restricted eating usually reflects family difficulty rather than neglect; a collaborative, supportive approach is appropriate alongside appropriate safeguards. Check vitamin C levels before escalating safeguarding concerns - the rapid response to treatment helps clarify the diagnosis.
Controversies and Areas of Uncertainty
- Diagnostic threshold and assay reliability. Reported "deficiency" cut-offs vary between series (commonly under 11 micromol/L, but some cohorts use under 23 micromol/L). Plasma ascorbic acid reflects recent intake and is degraded by sample handling; leukocyte vitamin C better reflects tissue stores but is rarely available. A normal-borderline plasma level after recent dietary change can therefore mask true tissue depletion.
- Diagnosis versus child abuse. Metaphyseal corner changes overlap with the classic metaphyseal lesions of non-accidental injury, and the two can coexist. There is no validated radiographic scoring system to separate them with certainty, so practice relies on bilateral symmetry, systemic scorbutic signs, dietary history and treatment response rather than imaging alone.
- Empirical vitamin C trial as a diagnostic test. Rapid response (24-48 h) to vitamin C is widely cited as confirmatory, but a therapeutic trial is not a controlled test and can delay recognition of coexisting pathology if relied on in isolation.
- Optimal dose and route. Reported regimens range widely (roughly 100-500 mg daily, oral or IV). No comparative trials define an optimal paediatric dose, duration, or route; recovery appears rapid across the range used.
- True incidence is unknown. Scurvy is not registry-tracked; published figures derive from case series and are likely underestimates given frequent misdiagnosis as infection, malignancy, vasculitis or arthritis.
Clinical Decision Scenarios
Use these scenarios to practise clinical reasoning and management decisions
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
Clinical 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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