We describe a patient with a chylous pleural effusion associated with primary lymphedema of his right leg and abdominal wall. On evaluation a generalized, severe hypoplasia of the lymphatic system turned out to be associated with hyperplastic, lymphangioma-like malformations.

BACKGROUND Lymphangioleiomyomatosis (LAM) is a rare disease caused by dysregulated activation of the mammalian target of rapamycin (mTOR). Sirolimus, an inhibitor of mTOR, has been reported to decrease the size of angiomyolipomas and stabilize pulmonary function in patients with LAM. However, the optimal dose for the treatment of LAM remains unclear. METHODS We conducted a retrospective, obse...

Background/objectives: Chylous fistula is an uncommon but serious complication of radical neck dissections and is a complication which can be better avoided than treated. Presence of amber colored lymph in the postoperative closed wound suction drainage is defined as chylous fistula. Materials and methods: The study was done over 18 months analyzing neck dissections done for 245 cases for compl...

A 34-year-old woman with primary chylous ascites due to lymphangiectasias was treated with sclerotherapy of dilated lymphatics and a lymphovenous shunt. She was referred to our institution after a thorough diagnostic and therapeutic approach in her community hospital. After four weeks of intensive diagnostic study, no secondary etiology for her chylous disorder was established. Conservative tre...

Severe ascites is a hallmark of advanced ovarian cancer (OVCA), yet the underlying mechanism that creates an imbalance between peritoneal vascular leakage and lymphatic drainage is unknown. Here, we identified and characterized peritoneal lymphatic vessels in OVCA mice, a model generated by implantation of human OVCA cells into athymic nude mice. The OVCA mice displayed substantial lymphangioge...

Chylous ascites occurring after abdominal surgery is rare. Despite being potentially critical, there is no definite treatment guideline because of its rarity. Here we present a case of massive chylous ascites occurring after rectal surgery which was successfully treated with an oral fat-free elemental diet (ED). A 67-year-old man underwent low anterior resection with para-aortic lymphadenectomy...

OBJECTIVE Chylous fistulas can occur after neck surgery. Both nonoperative measures and direct fistula ligation may lead to fistula resolution. However, a refractory fistula requires upstream thoracic duct ligation. This can be accomplished minimally invasively. Success depends on lymphatic flow interruption where the duct enters the thorax. We report on the utility of frozen section confirmati...

Three months after esophagectomy for esophageal cancer, a 58-year-old man presented with fluid trapped in his upper mediastinum due to chylous leakage from a duplicated left-sided thoracic duct that remained after excision of the main thoracic duct. Classical lymphangiography using lipiodol confirmed the presence of duplicated thoracic ducts. Conservative treatments were not effective, and then...

We reported a 14 months old boy referred for evaluation of chylous ascites to our nuclear medicine department. Tc-99m Phytate was injected subcutaneously in his calves and whole body images as well as abdominal SPECT were performed. The SPECT images could localize an area of several intestinal lymphangiomata in the abdominal region. The abnormal region was surgically resected and the patient as...