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?
Scurvy, or vitamin C deficiency, is a disease of malnutrition. It is one of the oldest nutritional deficiencies identified, and the source of one the world’s first randomized control trials. In 1753 James Lind published A Treatise of the Scurvy. Like a lot of research, this paper was widely ignored. It wasn’t until about 10 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.
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 the breast milk. Vitamin C has important roles in the body including wound healing, bone, cartilage and blood vessel maintenance and helps with the absorption of iron. It is also involved in fatty acid transport, neurotransmitter synthesis, prostaglandin metabolism and nitric oxide synthesis.
Dietary doses of up to 100mg/day can be absorbed in the distal small intestine. It is renally excreted and therefore the kidneys are important 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 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. Consider the infant with fatigue, anorexia, weight loss and low grade fever – these are all early signs of scurvy but 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, 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.
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, as well as the extremely fussy child.
Scurvy is a clinical diagnosis based on presentation of typical signs and symptoms alongside a dietary history of restrictive vitamin C intake for at least 1-3 months. Most laboratories cannot process ascorbic acid levels. If they can be measured then a level less than 11 umol/L would be considered deficient.
The diagnosis is, in the main, a clinical one. When a signs and symptoms respond to dietary changes or supplementation with Vitamin C then 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.
- 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 in 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 being met. The child continued to have a restricted diet and will therefore need a multivitamin supplement long term.
Scurvy is a preventable, easily treatable disease which due to its non-specific symptoms is often misdiagnosed or carries a delay in diagnosis, with patients presenting to healthcare professionals on multiple occasions. A good nutritional history is key 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