641. ALPORT SYNDROME WITH ESOPHAGEAL LEIOMYOMA TREATED WITH CONSERVATIVE DISTAL ROBOTIC MYOTOMY: AVOIDING ESOPHAGECTOMY?

Abstract Alport Syndrome manifests as dysmorphic hematuria associated or not with nephrotic syndrome, renal failure and visual and auditory impairment, in addition to family history. Leiomyoma is the most common benign neoplasm of the esophagus, but it is rare in childhood, and controversies persist as to the best option for its surgical treatment. We report the case of an 8-year-old female patient diagnosed with Alport syndrome and association with esophageal leiomyoma. An 8-year-old female had 3 episodes of pneumonia related to bronchoaspiration. During childhood, he presented pictures of retrosternal pain related to dysphagia, which had worsened in intensity in the last 2 years. She had a hypothesis of achalasia, she underwent an Endoscopic Poem Myotomy. During the procedure, a mass was observed in the distal esophagus and a biopsy showed that it was an esophageal leiomyoma. Given the close association of this tumor with Alport Syndrome, biopsy confirmed the diagnosis. She was then submitted to robotic surgery for possible esophagectomy, however, only partial resection of the Leiomyoma was performed avoiding esophagectomy. Due to young age and the morbidity of esophageactomy, only partial resection of the Leiomyoma was performed, with significant improvement in esophageal emptying for liquids and solids in the postoperative period and hospital discharge after 5 days. Considering leiomyomatosis a benign disease, partial resection was permitted since the histological aspects such as high rates of Ki67% and cellular morphological features were not found. The case in question presented a diagnosis due to the severe manifestations that were provided by the esophageal leiomyoma in a patient with atypical epidemiological characteristics, such as age group and sex. The conservative surgical strategy was chosen due to the patient's age and the surgical result was satisfactory, being suggested in similar cases.