Oral manifestations of systemic disease — branching viva

Opening — the pale tired child with swollen bleeding gums

Examiner: A six-year-old boy has two weeks of fatigue, easy bruising and progressively swollen, painful, bleeding gums, with palatal petechiae and a fever of 38.6 degrees Celsius. Talk me through your immediate thoughts and examination. [1]

Candidate should cover: the recognition of acute leukaemia from the pale, tired child with boggy enlarged bleeding gums out of proportion to plaque, mucosal petechiae and bruising indicating thrombocytopenia; the differentiation from simple gingivitis on the basis of severity, systemic company and cytopenic signs; and a focused assessment of hydration, sepsis and bleeding risk. [1]

Branch 1 — the urgent work-up and neutropenic-sepsis safety

Examiner: What is your immediate management? [1]

Candidate should cover: securing intravenous access and sending an urgent full blood count and film to confirm leukaemia and the cytopenias; giving empiric broad-spectrum antibiotics for presumed neutropenic sepsis given the fever; arranging urgent paediatric haematology transfer; and the principle that the gingival infiltration resolves only with treatment of the leukaemia, not with mouthwash or oral hygiene alone. [1]

Branch 2 — the child with chalky teeth and poor growth

Examiner: Now a different child: a nine-year-old with chalky symmetrical dental enamel defects on the permanent incisors and first molars, recurrent aphthae, angular cheilitis and faltering growth. What is the diagnosis and the work-up? [2]

Candidate should cover: the diagnosis of coeliac disease from the symmetrical, datable enamel defects with aphthae, angular cheilitis and poor growth; the work-up of coeliac serology (tissue transglutaminase IgA) with a total IgA to exclude IgA deficiency, confirmed by small-bowel biopsy where serology is positive; and the two-handed management of a strict gluten-free diet with paediatric dental referral, explaining that existing defects are permanent but the diet prevents new defects and heals the gut. [2] [3]

Branch 3 — the child losing primary teeth too early

Examiner: Finally, a four-year-old whose primary incisors have been shed years too early with minimal root resorption, and no caries. What do you consider, and what single blood test is most revealing? [3]

Candidate should cover: the differential of premature loss of primary teeth as a symptom of systemic disease — hypophosphatasia (with a low alkaline phosphatase and rickets-like bone change), Papillon-Lefevre syndrome (with palmoplantar keratoderma and aggressive periodontitis), cyclic and congenital neutropenia (with recurrent ulcers and periodontitis), and leukaemia; the single most revealing test of an alkaline phosphatase with calcium and phosphate for hypophosphatasia; and the involvement of metabolic medicine, dentistry and clinical genetics, with enzyme replacement (asfotase alfa) considered for the severe skeletal forms. [3] [4]