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  • Title: Alport-leiomyomatosis syndrome requiring subtotal esophagectomy for refractory gastroesophageal reflux disease after childhood partial esophagogastrectomy: a case report.
    Author: Aoyama J, Miyawaki Y, Kato T, Fujiwara N, Sugita H, Sato H, Yasuda M, Sakuramoto S, Yamaguchi S.
    Journal: Gen Thorac Cardiovasc Surg; 2020 Feb; 68(2):199-203. PubMed ID: 31758390.
    Abstract:
    Alport-leiomyomatosis syndrome is an extremely rare condition occurring at a young age in which Alport syndrome coexists with diffuse leiomyomatosis of the digestive tract (primarily the esophagus). Most patients with diffuse esophageal leiomyomatosis require esophagectomy of variable extents. A 20-year-old man with Alport-leiomyomatosis syndrome was diagnosed with dysphasia and hematuria in childhood. Although he underwent partial esophagogastrectomy at 8 years of age, extremely severe gastroesophageal reflux symptoms were noted postoperatively. He was diagnosed with refractory severe reflux esophagitis associated with diffuse leiomyomatosis and esophagogastric anastomosis, for which he underwent subtotal esophagectomy, gastric tube reconstruction, and esophagogastric anastomosis in the left neck. The postoperative course was generally good, and he had no postoperative reflux symptoms. To achieve long-term control of symptoms, the lesion must be removed completely; nevertheless, unnecessarily extensive esophagectomy should be avoided.
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