Dataset Information

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.

SUBMITTER: Takeda FR

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

REPOSITORIES: biostudies-literature

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Publications

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

Takeda Flavio Roberto FR de Meira Junior Jose Donizeti JD Sallum Rubens Antonio Aissar RAA

International journal of surgery case reports 20230621

<h4>Introduction</h4>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.<h4>Case presentation</ ...[more]

PMID: 37352772

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Dataset Information

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

Introduction

Case presentation

Discussion

Conclusion

Publications

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

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