Figure 1: Near complete occlusion of distal TD.
Figure 2: Inguinal lymph nodes for TD access. Sample nodes indicated by red arrows; cannulation indicated by green arrow.
Figure 3: Cisterna chyli (green arrow) and proximal TD (red arrows).
Figure 4: TD localization in operative field relative to a microvascular clamp.
Figure 5: Surgical field after bypass.
Figure 6: Confirmation of patent anastomosis.
Interventional radiologists and plastic surgeons at the Penn Center for Lymphatic Disorders have developed a procedure to bypass occlusions in the lymphatic thoracic duct (TD), an advance with potentially life-saving consequences.
Although its role in well-being is substantial, the TD is only 3/16ths of an inch wide at its broadest point, and is complicated by a tortuous and deep set course, characteristics that heighten the risk for obstruction, injury, and occlusion. The cause of TD occlusion may be traumatic, iatrogenic, or disease-related (e.g., malignancy, sarcoidosis). Occlusion is associated with the accumulation of chyle, a triglyceride rich fluid, and its leakage into the thoracic cavity and surrounding tissues. Chief among the complications of TD occlusion are recalcitrant chylous pleural effusion, chylopericardium, metabolic derangement, chylous ascites, generalized lymphedema, and other morbidities. In affected individuals, untreated occlusion has been associated with a mortality of from 10%–50%.
Treatment
Definitive management of TD occlusion is difficult and rarely curative. Conservative management ranges from dietary adjustments to pharmacological treatment. Approaches for advanced disease have included angioplasty and minimally invasive procedures for ligation, embolization, and disruption. Although beneficial, these measures are limited in scope, application, and therapeutic success.
The treatment paradigm for TD occlusion is beginning to change, however, with recent advances in microsurgery and lymphatic visualization at the Penn Center for Lymphatic Disorders. Lymphangiography-guided TD lymphovenous bypass, an intervention developed at the Center, now permits IR specialists and plastic surgeons to identify the locus of occlusion and re-establish normal outflow of lymphatic fluid via a bypass between the TD and the jugular vein. The benefits of this new procedure are particularly relevant to the treatment of adults with refractory occlusion of the TD.
Case Study
Mr. W, a 61-year-old male with a history of Noonan’s syndrome, pulmonary lymphangiectasias, chylothorax, and oxygen dependency was referred to the Penn Center for Lymphatic Disorders following an acute episode of painful swelling and edema involving his left shoulder and axilla with neuropathy and weakness of the left arm. Lymphangiography was significant for distal TD occlusion at the level of the left subclavian vein (Figure 1). Mr. W was deemed a candidate for TD bypass performed jointly between IR and plastic surgery, and agreed to have the procedure after a consultation to explain its potential risks and benefits.
At surgery, a thoracic duct cannulation was performed via bilateral ultrasound-guided inguinal lymph node puncture (Figure 2). Lipiodol was then infused and intermittent fluoroscopy was used to identify intra-abdominal lymphatics and the cisterna chyli (Figure 3). Following cannulation of the TD with a microcatheter and guide-wire, selective lymphangiography was conducted to advance upon the occlusion.
A neck dissection took place at this point and fluoroscopy was employed to aid in identification of the TD in the operative field relative to nearby surgical retractors (Figure 4). Once identified, the TD was divided proximal to the obstruction, and a microvascular anastomosis performed between the TD and the external jugular vein (Figure 5). The patency of this procedure was then confirmed (Figure 6).
Following the bypass, Mr. W had total resolution of chylothorax and edema and was no longer oxygen dependent from a respiratory standpoint. He was discharged from the hospital on the day following his procedure, and had an uneventful recovery thereafter.
About the The Penn Center for Lymphatic Disorders at Penn Interventional Radiology
The Penn Center for Lymphatic Disorders, led by Director Maxim Itkin, MD, includes experts in the diagnosis, treatment and research of lymphatic disorders. Members of the team often collaborate to provide you with the highest level of patient care. A national leader in research, education and patient care, Penn Interventional Radiology is also one of the oldest and largest IR programs in the United States. In addition to performing more than 12,000 procedures annually, Penn Interventional Radiology has an inpatient admitting service, inpatient consult service and daily outpatient consultation and follow-up clinic.
Referring physicians, please use the information below to contact the Penn Center for Lymphatic Disorders:
Megan Asher
Lymphatics Nurse Practitioner
Email: PennLymphatics@uphs.upenn.edu
Fax number: 215-615-3545
Penn Medicine Faculty Team
Associated Resources
Clinical Briefing: Diagnosis and Treatment of Protein Losing Enteropathy
Patient Story: A mystery illness solved, a beloved colleague cured
Clinical Articles from Penn Interventional Radiology: Microsurgical Thoracic Duct Lymphovenous Bypass in the Adult Population