Enamel hypoplasia of the primary dentition in a 4-year-old with intestinal lymphangiectasia

Intestinal lymphangiectasia (IL) is a rare disorder, and its incidence and prevalence is unknown for either Australia or world-wide. It is characterized by diarrhoea, mild steatorrhoea, oedema, enteric loss of protein (protein-losing enteropathy) and abnormal dilated lymphatic channels in the small intestine. Whilst oedema and diarrhoea are the predominant clinical features, other observed features include hypoalbuminemia, hypogammaglobulinemia, trace metal deficiency, hypocalcemia and chylous pleural effusions. While medical presentation of the condition has been reported widely, few descriptions of oral findings have been published. A search of Medline found two reports of dental findings in the permanent dentition in patients with IL. To date, there have been no reports on dental findings in the primary dentition. The primary dentition of a 4-year-old boy with IL had teeth with enamel defects which reflected the timing of enamel development and the period in which the disease was active. The present report highlights the need for early involvement of the dental team in the dental management of children with IL.