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Chirurgie 1. Dezember 2011

Esophageal leiomyomatosis 25 years after myotomy for achalasia

BACKGROUND: Esophageal leiomyomatosis is a rare hamartomatous disorder with cumulative proliferation of the smooth muscle layer of the esophagus causing stenosis and slowly progressive dysphagia. Children and young adults are predominantly affected by this condition. METHODS: Case report. RESULTS: We report about an unusual case of esophageal leiomyomatosis in a 30-year-old female patient with a history of achalasia with Heller myotomy and partial posterior fundoplication as a child. The radiological and endoscopic findings revealed a big circumferential tumor of the gastroesophageal junction with prestenotic dilation. The diagnosis was complicated by the large extension of the tumor and the unusual medical history of the patient mimicking a malign neoplasm. The surgical treatment consisted of esophageal resection with gastric pull-up. To the best of our knowledge, this case report is the first description of the unusual coincidence of an achalasia as a child and an esophageal leiomyomatosis as an adult. CONCLUSIONS: Esophageal leiomyomatosis is an interesting differential diagnosis for all those dealing with patients with diseases of the esophagus.

A. Perathoner, E. Laimer, L. Veits, W. Vogel, J. Pratschke, Th. Schmid, R. Margreiter, H. Wykypiel, European Surgery 6/2011

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