The leiomyoma of the esophagus is very rare in children, contrary to what has been observed in adults, with only 26 cases reported up to now. In this paper we present the case of a 9 year-old child in which the tumor was located in the abdominal esophagus, being progressive dysphagia its most important manifestation. Diagnosis was done during the surgical procedure, and the treatment involved sub-total esophagotomy and pyloroplasty, with ulterior esophageal substitution for a colon segment. The only post-surgical complication was dumping which resulted from the pyloroplasty. Its symptoms receded with appropriate diet. The review of the literature shows that leiomyomas in children may be diffuse or located, the former being the more common case (90%). Twelve children presented association with Alport's syndrome (nephropathy with hematuria, deafness and cataract) while others had hiatus hernia, esophageal peptic ulcer and cholelithiasis. In two children the tumor affected the tracheo-bronchial tree, and in four girls there was vulvar or perianal hypertrophy. Finally, there is a rarer kind of tumor in the esophageal muscular layer called benign idiopathic hypertrophy, which has been described for six children. Its basic histological characteristic is non neoplastic hyperplasia of the cells of the internal muscular layer. We conclude that the leiomyomas of the esophagus, although rare, must be considered in the differential diagnosis of the mediastinal mass and the esophageal stenosis in children.
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