A51 PEDIATRIC CRITICAL CARE INCLUDING INTERVENTIONAL BRONCHOSCOPY / Thematic Poster Session

Multidisciplinary Management of a Large Lymphatic Malformation with

Recurrent Pleural Effusion, Response to Sildenafil and Sirolimus

I. Lee1, J. Teng2, M. Jeng3, E. E. Morales1; 1Pediatrics - Pulmonology, Stanford University, Palo Alto,
CA, United States, 2Dermatology, Stanford University, Palo Alto, CA, United States, 3Pediatrics -
Hematology/Oncology, Stanford University, Palo Alto, CA, United States.

Corresponding author's email: ilee33@stanford.edu

Introduction: Cystic lymphatic malformations are clinically heterogeneous with complications dependent
on location, size, and relation to adjacent structures. Often a multi-disciplinary approach is required. The
combination of sildenafil and sirolimus along with surgical and interventional radiology procedures for
treatment of lymphatic malformations has not been well described. We present a case of a large
lymphatic malformation with recurrent pleural effusions successfully treated with sildenafil, sirolimus,
surgical ligation of the malformation leak, and embolization of a thoracic duct defect. Case summary: A
nine-year-old male presented with symptoms of an upper respiratory infection and progressive shortness
of breath. Chest x-ray showed a large parapneumonic effusion. MRI revealed a macrocytic lymphatic
malformation extending from the spermatic cord and groin through the diaphragmatic hiatus to the lower
paraspinal region. A chest tube was placed, and sirolimus and octreotide were started. Daily chest tube
output was over two liters. During surgical exploration, leaking areas in the malformation were ligated.
After six weeks, the pleural effusion decreased, the chest tube was removed, and he was discharged on
oral sirolimus. Interval imaging demonstrated significant decrease in malformation size and pleural
effusion. One month after discharge, he was asymptomatic. Six months later, while on sirolimus, he
again developed respiratory distress and chest x-ray demonstrated large right pleural effusion. Chest
tubes were placed, and octreotide restarted. After two weeks, he was again discharged on sirolimus.
One month later, a third recurrence of the pleural effusion prompted placement of a home pleural drain
and addition of oral sildenafil. Lymphangiography demonstrated dilated pelvic and abdominal lymphatic
ducts as well as contrast in the thoracic duct with delayed extravasation into the right pleural effusion of
uncertain origin. Subsequent lymphangiogram found a leak from the mid-thoracic duct into the effusion
which was embolized, leading to decrease in pleural fluid output and removal of chest tube. To date, he
has been on sildenafil and sirolimus for over nine months without evidence of pleural fluid re-
accumulation. Discussion: Complex, severe lymphatic malformations can be difficult to manage. In many
cases, surgical and radiologic approaches are often temporary in effect. Sildenafil and sirolimus are
individually promising therapies. This complex lymphatic malformation clinically responded to a
combination of sildenafil and sirolimus with surgical and interventional radiology techniques highlighting
the continued importance of multiple specialties in addressing these lesions.
This abstract is funded by: None

Am J Respir Crit Care Med 2020;201:A1937

Internet address: www.atsjournals.org Online Abstracts Issue