Grade IV Esophageal Achalasia Complicated by Cicatricial Stricture and Perforation in a Teenager: A Clinical Case

Esophageal achalasia in children and adolescents is a rare neuromuscular disorder that is often diagnosed at advanced stages due to nonspecific clinical manifestations and low clinical suspicion. End-stage (grade IV) achalasia is characterized by severe esophageal dilation and deformation, loss of peristalsis, profound dysphagia, and significant nutritional impairment. The presence of a cicatricial esophageal stricture further aggravates the disease course by combining functional and organic obstruction and substantially increasing the risk of complications during invasive treatment. To present a clinical case of grade IV esophageal achalasia in an adolescent complicated by a cicatricial stricture and severe postoperative complications, and to discuss diagnostic and therapeutic considerations in such patients. We report the case of a 17-year-old male with a long history of progressive dysphagia, severe malnutrition, and complete inability to swallow solids and liquids. The diagnosis was established based on clinical findings and radiological imaging. A staged treatment strategy was chosen, including Heller cardiomyotomy, endoscopic bougienage of the cicatricial stricture, and subsequent esophageal stenting. Despite the staged approach, the early postoperative period was complicated by esophageal perforation, followed by pleural empyema, septic shock, and multiple organ failure. The patient required intensive care management, including broad-spectrum antibiotic therapy, pleural drainage, respiratory support, and aggressive hemodynamic stabilization. This clinical case highlights the substantial risks associated with invasive interventions in adolescents with end-stage esophageal achalasia and severe nutritional deficiency. Early diagnosis and timely referral to specialized centers are crucial to prevent disease progression, reduce the likelihood of life-threatening complications, and improve treatment outcomes.