Comprehensive management of diffuse leiomyomatosis in a patient with Alport syndrome.

Roberto Burgos, Eduardo Muñiz, Edgardo Rivera Rosa, Carlos J Olivares, Josefina Romaguera

Index: P. R. Health Sci. J. 32(4) , 200-2, (2013)

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Abstract

Alport syndrome with diffuse leiomyomatosis (ASDL) is a complex combination that doesn't have a specific course of treatment. In this case report, we present a 44-year-old woman with ASDL and detail her treatment. The patient presented at the emergency room (ER) with symptoms of anemia, bronchial asthma, and abnormal uterine bleeding (AUB). The patient had diffuse myomas in different areas of her body, including the esophagus and genital tract. She was treated by a multidisciplinary team that included members from the hematology/oncology, pulmonary, interventional radiology, anesthesia, surgery, and gynecology services. A physician from interventional radiology performed an embolization of the uterine arteries to treat the patient's AUB. Surgery was done in May 2011 to remove the esophageal leiomyomas to improve her pulmonary function. Surgery included a distal esophagectomy, a proximal gastrostomy, and the resection of the leiomyomatous mass. In order to shrink the tumor in her genito pelvic region so that it could be extirpated with the highest likelihood of success, the patient was treated with gonadotropin-releasing hormone agonist (leuprolide acetate, 3.75 mg/month for 4 months). In May 2012, the patient had a total abdominal hysterectomy (TAH), with a bilateral salpingo-ophorectomy (BSO), the excision of a leiomyoma, and a posterior colporrhaphy.