A case report of esophageal leiomyoma in Alport's syndrome treated with robotic-assisted distal myotomy: A surgical technique to avoid esophagectomy.

ABSTRACT:

Introduction

Alport's syndrome is the most common hereditary nephropathy, characterized by progressive renal failure, sensorineural deafness, and ocular abnormalities. It may rarely coexist with diffuse leiomyomatosis of the digestive tract, respiratory tract, or female genitalia, and in this setting, it is called Alport-leiomyomatosis syndrome. The leiomyomas most commonly affect the esophagus, and the symptoms have early onset. Treatment is usually esophagectomy.

Case presentation

We report the case of an 8 years-old girl in which we performed a novel strategy of an esophagus-sparing approach with a robotic-assisted myotomy. This conservative approach has never been described in the literature to our knowledge.

Discussion

The underpinning rationale was to resolve th

SUBMITTER: Takeda FR 

PROVIDER: S-EPMC10382766 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature