Endocrine Abstracts

Introduction

Triple-A syndrome, also known as Allgrove syndrome, is a rare autosomal recessive disorder. It is a multisystemic disease with an estimated prevalence of 1 per 1 000 000 individuals The 3 features of this syndrome are achalasia, adrenal insufficiency, and alacrima. Recently, dental impairment has been the subject of several case reports and reviews. However, this abnormality remains under-diagnosed.

Purpose

This article reports a patient with multisystemic features of the syndrome with particular attention to premature loss of permanent teeth.

Case report

We represent the case of a 23-year-old male patient with no family medical history, born to consanguineous parents. He was diagnosed with adrenal insufficiency at the age of 3 after a hypoglycemic and hyponatremia seizure with low cortisol and elevated ACTH level. He was treated with oral hydrocortisone. Medical examination showed hyperpigmentation, microcephaly, delayed developmental milestones and dysmorphic facial features: narrow face, long philtrum, down-turned mouth. Further examination confirmed achalasia and dysphagia. The Allgrove syndrome was then suspected and confirmed when genetic analysis identified the mutation of the AAAS gene (IVS14 + 1G). He had esophageal dilatation on 2 occasions (2000 and 2011) with real improvement of dysphagia. At the age of 6, the patient started having gingivitis and dental caries. Due to tendency to eating sweet foods and poor oral hygiene, the teeth have decayed rapidly. Also, due to inadequate root length remaining after the removal of decay, all of them were extracted. Because of economic problem, he had implant treatment for only his upper teeth when he was 20-years-old. However, the implant was only successful for 2 years and then the patient started losing his teeth again.

Conclusion

Triple A syndrome is a multisystemic disease needing multidisciplinary management: endocrinology, gastrology, ophthalmology and dentists. The prevention and management of bucco-dental complications associated with xerostomia and achalasia are essential to minimize the loss of permanent teeth and improve the quality of life.