Esophageal leiomyomatosis in children: Report of a case and review of the literature

The authors describe a case of a Ei-years-old girl affected by diffuse oeso phageal leiomyomatosis presenting with progressive dysphagia, cyanosis, rec urrent pneumonia and retrosternal pain. She also suffered from occasional c onstipation due to perineal involvement by the disease and had a past histo ry of recurrent microscopic hematuria, suggesting an association with an Al port-like syndrome. Only 24 cases of esophageal leiomyomatosis could be fou nd in the medical literature in children aged less than 14 years, confirmin g the rarity of the disease. A clinical analysis of all cases reviewed from the literature is made, stressing the importance of an accurate preoperati ve diagnosis for the choice of proper surgical treatment to avoid recurrenc e.