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 the patient's symptoms with partial resection of the benign tumor, avoiding an esophagectomy. Although it is likely related to a higher relapsing rate, it is more tolerable by an 8 years-old patient, and was highly effective in resolving her symptoms.

Conclusion: The video of a successful minimally invasive conservative approach to esophageal leiomyomatosis is presented.

Keywords: Case report; Dysphagia; Esophagectomy; Leiomyomatosis; Robotic surgical procedures.