Background: Lymphangiomyomatosis is a rare disease involving the lymph vessels, causing obstruction and cystic formation with an incidence of 3-8 per million women. The disease might be sporadic or inherited. Lymphangiomyomatosis mostly affects the pulmonary system, whereas extrapulmonary Lymphangiomyomatosis may present in various site, occasionally as a localized abdominal mass. The diagnostic process might entail surgical resection to obtain a specimen for pathology that may also help to achieve a long-term control of the disease. Methods: Herein, we present a case of a 45 years old female, who suffered from pulmonary symptoms, and during her workup an abdominal mass was found. The patient underwent exploratory laparotomy with resection of a left retroperitoneal bilobar mass. Results: Histopathological report revealed Lymphangiomyoma. She had a complication of a lymphatic leakage that required a second laparotomy with satisfactory clinical outcome. Conclusions: Surgeons should be aware of the pathological lymphatics and manage post-operative complications by a trial of conservative.
Keywords: excisional operations; extrapulmonary lymphangiomyomatosis; lymphatic; venography.