Dynamic Contrast MR Lymphangiography with percutaneous lymphatic intervention of pulmonary lymphatic leak in the setting of a VSD and partial anomalous pulmonary venous return

Description of Clinical Presentation: This is a case of a 3 year old presenting with abnormal pulmonary lymphatic perfusion. This patient has a history of a large perimembranous ventricular septal defect, tricuspid regurgitation, s/p VSD patch repair and tricuspid valvuloplasty, PDA ligation complicated by recurrent chylous pleural and pericardial effusions, also with partial anomalous pulmonary venous return of the right upper pulmonary vein to the R-SVC (unrepaired)

Diagnostic Techniques and Their Most Important Findings: The patient underwent a combined lymphatic intervention with lymphatic MRI imaging in a hybrid suite (Siemens 1.5T Sola MRI scanner and Artis Icono X-ray system) with a sliding door and Nexaris Interventional table transfer system, allowing for seamless intermodality transfer. He was intubated, his inguinal lymph nodes were accessed bilaterally with a 24-gauge LNX needle along with 25-gauge spinal needle was advanced into the echogenic tissue adjacent to the left portal vein. He was then transferred across the suite to the MRI scanner. Diluted Gadavist contrast was injected into the bilateral inguinal lymph nodes (diluted 1:2, total dose 0.2ml/kg). Pre-contrast coronal T2 weighted 3D turbo spin echo sequence (T2 prep) images were obtained of the chest abdomen and pelvis. During injection of the periportal liver access needle and groin lymph node access needles with dilute gadolinium, coronal dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) with TWIST sequence images were acquired through the chest abdomen and pelvis. Conventional contrast enhanced MR angiogram (MRA) and a delayed contrast coronal Nav IR TrueFISP sequences were obtained post DCMRL.

Pre-contrast T2 images demonstrate increased signal in the paravertebral region in the right upper chest as well as left upper chest and supraclavicular regions. Following periportal injection, on DCMRL there is progressive enhancement of periportal and paravertebral abdominal lymphatic channels and lymph nodes. There is also progressive opacification of iliac chain lymph nodes and channels as well as the thoracic duct following contrast injection into the groin lymph nodes along with progressive enhancement of right upper chest paravertebral tissues and left upper chest and supraclavicular tissues that had been seen on pre-contrast T2. The MR angiographic images were utilized as overlay on the fluoroscopic image guidance during lymphatic intervention.

Learning Points from this Case: The abnormal network of collateral lymphatic vessels in the upper chest were occluded with sclerotherapy/embolization using sodium tetradecyl sulfate. This method was very successful, and the patient tolerated the procedure well with no adverse events. To verify this success, contast was injected post lymphatic intervention in the Xray Cath Lab, through a microcatheter which demonstrated filling of the patent thoracic duct in the chest and left upper neck, with increased flow through the thoracic duct terminus and flow into the subclavian vein, with no contrast seen entering the treated lymphatic region.

This case illustrates the utility of MRI for evaluation of lymphatic abnormalities in a patient with complex congenital anatomy, chronic chylous pleural and pericardial effusions, and partial anomalous pulmonary venous return.  Specifically, MRI in this case helped delineate lymphatic anatomy and was used as an image road map for a difficult lymphatic intervention. Significant planning and inter-disciplinary collaboration were necessary to ensure patient safety and a successful procedure.

T2Prep image depicting increased signal intensity in the paravertebral tissues of the right upper chest as well as left upper chest and supraclavicular regions suggestive of abnormal lymphatic perfusion.

DCMRL Twist sequence image showing progressive enhancement of right upper chest paravertebral tissues and left upper chest and supraclavicular tissues

Xray fluoro image pre intervention depicting abnormal lymphatic vessels seen on MRI