Alacrymia revealing allgrove disease (SD Triple-A)

Abstract

Allgrove's disease (Triple-A syndrome) is characterized by a symptomatic triad composed of alacrymia, cardiac achalasia and adrenal insufficiency. To this clinical picture are added peripheral neuropathic lesions. These are very diverse among patients but all induce a disabling long-term attack. The diagnosis is then clinical. We report the case of a child from consanguineous marriage, in which the ophthalmological examination revealed a severe dryness with keratitis invading the visual axis. Radiological assessment: Oesogastroduodenal transit (TOGD) revealed a mega esophagus. A treatment based on artificial tears and then scleral lenses is instituted immediately, as well as a laparoscopic surgical treatment. The knowledge of this pathology will allow an awareness of the severity of this disease in addition to suggesting its management.

Keywords

• allgrove´s disease
• alacrymia
• cardiac achalasia
• adrenal insufficiency