A three-year-old boy with speech and language delay presents to the Emergency Department with a unilateral atraumatic limp. He is initially managed as per the ‘Limping Child Pathway’. At his follow up review, he is now non-ambulatory, resistant to examination and holds his legs in a flexed position.
He has an extensive array of normal investigations including basic blood tests, pelvis and bilateral leg x-rays and abdominal ultrasound. He subsequently has an MRI which shows florid symmetrical increased signalling in his long bones with a wide non-specific differential. He develops a small patch of petechiae on his ear. Reviewing his history, it appears that his diet is extremely restrictive and made up of only lactose-free milk and cheesy biscuits.
Could this be scurvy?
Definition
Scurvy, or vitamin C deficiency, is a disease of malnutrition. It is one of the oldest nutritional deficiencies identified and the source of one of the world’s first randomised control trials. In 1753 James Lind published A Treatise of the Scurvy. This paper should have been more widely accepted, like a lot of research. It wasn’t until about ten years later, when sailors got to try sauerkraut, wort, or syrup of oranges and lemons (all contain vitamin C) did the higher-ups at the Admiralty begin to take notice.
Scurvy is rare in higher income countries and therefore often forgotten as a potential differential diagnosis.
Pathophysiology
Vitamin C, also known as ascorbic acid, is a water-soluble essential vitamin. It is found in citrus fruits, peppers, potatoes and broccoli. In babies, vitamin C is provided in breast milk. Vitamin C has essential roles in the body, including wound healing, bone, cartilage and blood vessel maintenance, and helps with iron absorption. It is also involved in fatty acid transport, neurotransmitter synthesis, prostaglandin metabolism and nitric oxide synthesis.Â
Dietary doses of up to 100mg/day can be absorbed in the distal small intestine. It is renally excreted; therefore, the kidneys are essential in regulating vitamin C concentration in the blood. The World Health Organisation recommended daily allowance for infants and children is 25mg per day.
The clinical manifestations of scurvy result from disordered pathways that utilise vitamin C, such as collagen and connective tissue synthesis. Symptoms can occur after just one to three months of inadequate vitamin C intake.
Signs and symptoms
Early manifestations of scurvy are often non-specific. For example, consider the infant with fatigue, anorexia, weight loss and low-grade fever – these are all early signs of scurvy. Still, scurvy was unlikely to have made it into your differentials list. Petechiae can also be present – you probably ran an FBC and CRP. But petechiae, follicular hyperkeratosis and perifollicular haemorrhage are all cutaneous manifestations of scurvy, while gingival signs include swelling, bleeding and loss of teeth.
And consider the limping child. Arthralgia, limb and joint swellings, limp, and inability to weight-bear are often the presenting features of children being brought to medical attention. Although rare, scurvy should be considered in the differential of the limping child once the more common causes have been ruled out.
Risk Factors
In children of the developed world, risk factors for developing scurvy are severe dietary restriction of fruit and vegetables. These can be secondary to autism, developmental delay and psychiatric disorders, and the extremely fussy child.
Diagnosis
Scurvy is a clinical diagnosis based on typical signs and symptoms alongside a dietary history of restrictive vitamin C intake for at least 1-3 months. Unfortunately, most laboratories cannot process ascorbic acid levels. However, if they can be measured, a level less than 11 umol/L would be considered deficient.
The diagnosis is, in the main, a clinical one. When signs and symptoms respond to dietary changes or supplementation with Vitamin C, you know you are on the right track.
Classic bony signs on imaging include periosteal oedema, sub-periosteal collections and sub-periosteal haematomas, lucent bands through long bone metaphyses, osteopenia and widening of the distal extremity of the femur.
Management
- Vitamin C supplementation orally for children with 100-300mg for one month or until full recovery.
- All children under five years of age are recommended to take a multivitamin (unless formula feeding as this is already fortified).
- Referral to a dietician for dietary education is imperative.
- Resolution of symptoms can start within 24 hours but may require a few weeks of treatment to fully resolve.
Two months later, the child was seen by paediatric outpatients running and jumping in the consulting room. They subsequently had an appointment with the dieticians, who discussed dietary changes with the family and ensured that all calorie and nutrient requirements were met. The child continued to have a restricted diet and will need a long-term multivitamin supplement.
Scurvy is a preventable, easily treatable disease that, due to its non-specific symptoms, is often misdiagnosed or carries a delay in diagnosis, with patients presenting to healthcare professionals on multiple occasions. An excellent nutritional history is critical to diagnosis.
Selected references on scurvy
Agarwal A, Shaharyar A, Kumar A et al. Scurvy in pediatric age group- A disease often forgotten? Journal of clinical orthopaedics and trauma. 2015; 6(2): 101-7 https://doi:10.1016/j.jcot.2014.12.003
Alqanatish JT, Alqahtani F, Alsewairi WM, Al-Kenaizan S. Childhood Scurvy: an unusual case of refusal to walk in a child. Pediatric Rheumatology 2015; 13(1): 23 https://doi:10.1186/s12969-015-0020-1
Chalouhi C, Nicolas N, Vegas N et al. Scurvy: A New Old Cause of Skeletal Pain in Young Children. Frontiers in Pediatrics. 2020; 8:8 doi:10.3389/fped.2020.00008
De Ioris MA, Geremia C, Diamanti A et al. Risks of inadequate nutrition in disabled children: four cases of scurvy. Archives of Disease in Childhood. 2016; 101(9): 871 https://doi:10.1136/archdischild-2016-310911, https://doi:10.1136/archdischild-2016-310911
Kitcharoensakkul M, Schulz CG, Kassel R et al. Scurvy revealed by difficulty walking: three cases in young children. Journal of Clinical Rheumatology: practical report on rheumatic and musculoskeletal disease. 2014; 20(4): 224-228 https://doi: 10.1097/RHU.0000000000000101
Noble JM, Mandel A, Patterson MC, Scurvy and rickets masked by chronic neurologic illness. Pediatrics. 2007; 119(3): e783-90 https://doi:10.1542/peds.2006-107
Pazirandeh S, Burns D. Overview of water-soluble vitamins. In: D, Seres, L, Kunins eds. UpToDate. 2020. Waltham, UpToDate [ Accessed 28th February 2021 ] Available from https://www.uptodate.com/contents/overview-of-water-solube-vitamins
Ratanachy EK, Sukswai P, Jeerathanyasakun Y, Wngtapradit L. Scurvy in pediatric patients: a review of 28 cases. Journal of the Medical Association of Thailand. 2003; 86(3): S734-S740
Weinstein M, Babyn P, Zlotkin S. An orange a day keeps the doctor away: scurvy in the year 2000. Pediatrics. 2001; 108 (3): E55. https://doi:10.1542/peds.108.3.e55
World Health Organization. Scurvy and its prevention and control in major emergencies [online]. World Health Organisation, 1999 [Viewed 28th February 2021]. Available from: https://apps.who.int/iris/bitstream/handle/10665/66962/WHO_NHD_99.11.pdf?ua=1
Good article. Would be worth adding that one classic symptom is ‘corkscrewing’ of hairs at the follicle which is practically pathognomonic of Scurvy.
I once had a case confirmed by a dermatologist who reviewed an inpatient (where scurvy was suspected) & saw this under magnification. Vit C level came back undetectable 3 weeks later.
That’s great – I’ll have to look more carefully next time I’m looking for nits