Allgrove syndrome (AS), or Triple-A syndrome, is a multi-system disorder characterized by alacrima (a decrease or absence of tear production), adrenal insufficiency and achalasia (absence of esophageal muscle peristalsis and failure to relax the lower esophageal sphincter). This syndrome may affect the autonomic nervous system, in which case it is called a 4A syndrome. It is a rare autosomal recessive inheritance, and early identification is difficult due to the rarity and wide phenotypic variation even among members of the same family. Endocrinologists, gastroenterologists, ophthalmologists, neurologists and surgeons are needed to coordinate care for these patients. We describe a case of AS that took several years to complete the diagnosis. She was diagnosed with alacrima at the age of 1-year-old, adrenal insufficiency at the age of 9 and achalasia at the age of 16. This case demonstrates the difficulty and delay in the diagnosis of AS.
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