Abstract
<title>Abstract</title> <p> <bold>Background:</bold> Triple A syndrome (Allgrove syndrome) is a rare autosomal recessive disorder caused by <italic>AAAS</italic> variants, classically defined by alacrima, achalasia, and adrenal insufficiency. Emerging evidence suggests a broader multisystem disease with progressive neurogastrointestinal involvement, although its longitudinal evolution remains poorly characterized. <bold>Case presentation:</bold> A 17-year-old female with genetically confirmed Triple A syndrome (AAAS c.1331+1G>A) evolved from surgically treated esophageal achalasia to refractory multisegmental upper gastrointestinal dysmotility, including severe gastroesophageal reflux and gastroparesis, requiring repeated endoscopic interventions despite prior surgical and medical management. <bold>Discussion:</bold> This case expands the gastrointestinal spectrum of Triple A syndrome, demonstrating progression from focal esophageal dysfunction to complex multisegmental involvement. The clinical course and limited therapeutic response support a diffuse enteric neuropathy underlying disease progression, with implications for treatment response and longitudinal management. <bold>Conclusion:</bold> Triple A syndrome should be considered a progressive neurogastrointestinal disorder rather than a triad-based entity. Persistent symptoms after Heller myotomy may reflect denervation-related pump failure and evolving multisegmental dysfunction, underscoring the need for early genetic diagnosis and dynamic long-term surveillance. </p>