Amir H Taghinia1, Joseph Upton2, Cameron C Trenor3, Ahmad I Alomari4, Anna P Lillis5, Raja Shaikh4, Patricia E Burrows6, Steven J Fishman7. 1. Department of Plastic and Oral Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115; Vascular Anomalies Center, Boston Children's Hospital, Boston, MA 02115. Electronic address: amir.taghinia@childrens.harvard.edu. 2. Department of Plastic and Oral Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115; Vascular Anomalies Center, Boston Children's Hospital, Boston, MA 02115. 3. Division of Hematology Oncology, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115; Vascular Anomalies Center, Boston Children's Hospital, Boston, MA 02115. 4. Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115; Vascular Anomalies Center, Boston Children's Hospital, Boston, MA 02115. 5. Department of Radiology, Nationwide Children's Hospital and Ohio State University College of Medicine, Columbus, OH 43205. 6. Department of Radiology, Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, WI 53226. 7. Department of Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115; Vascular Anomalies Center, Boston Children's Hospital, Boston, MA 02115.
Abstract
BACKGROUND: Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement. METHODS: We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct. Chylous leaks occurred in multiple different anatomic sites. All patients had CCLA and failure of thoracic duct emptying demonstrated by preoperative intranodal lymphangiography. RESULTS: Five patients had complete resolution of symptoms, and two patients had partial improvement. There were no major complications. Of 5 patients with PLE, only one improved after lymphaticovenous bypass. Repeat traditional lymphangiography was performed in 4 patients who did not improve, demonstrating patency of the bypass in all cases with persistent sluggish drainage. One patient had repeat MR lymphangiography that did not show the thoracic duct well. CONCLUSIONS: Bypass of the terminal thoracic duct is a novel procedure that offers improvement and a chance of cure for some patients with devastating manifestations of CCLA who lack other effective therapeutic options. LEVEL OF EVIDENCE: IV.
BACKGROUND: Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement. METHODS: We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct. Chylous leaks occurred in multiple different anatomic sites. All patients had CCLA and failure of thoracic duct emptying demonstrated by preoperative intranodal lymphangiography. RESULTS: Five patients had complete resolution of symptoms, and two patients had partial improvement. There were no major complications. Of 5 patients with PLE, only one improved after lymphaticovenous bypass. Repeat traditional lymphangiography was performed in 4 patients who did not improve, demonstrating patency of the bypass in all cases with persistent sluggish drainage. One patient had repeat MR lymphangiography that did not show the thoracic duct well. CONCLUSIONS: Bypass of the terminal thoracic duct is a novel procedure that offers improvement and a chance of cure for some patients with devastating manifestations of CCLA who lack other effective therapeutic options. LEVEL OF EVIDENCE: IV.