Recurrent chylous effusion is rare in children and carries significant morbidities and even mortality. We report a case of 13 months old boy who had a surgical repair of PDA in early infancy, subsequently developed SVC syndrome and delayed recurrent bilateral chylothorax and needed a surgical management.
A 13 month old male, ex 28 week premie presented with brief viral illness with bilateral large effusions and respiratory distress. He had a Broviac placed in right internal jugular vein in neonatal period and had septic and thrombotic complications of central venous line placement. PDA ligation was done at four months of age with no complications. After being placed on mechanical ventilation, chylous fluid was drained from both pleural spaces. Fluid analysis showed WBCs 29,500/mm3 - 98% lymphocytes and triglyceride 352 mg/dL. Gram stain smear was negative and bacterial culture was sterile. Pertinent physical examination was mild facial plethora and distended external jugular veins and scalp veins. Echocardiogram showed good biventricular systolic function with no evidence of pulmonary hypertension. Chest CT showed upper SVC, right IJ and bilateral brachiocephalic vein thromboses (Fig-1). The remains of the left brahiocephalic vein were replaced with multiple collaterals. Angiography demonstrated that the SVC was completely occluded above the azygous vein. The right subclavian vein was patent but became completely occluded right at the thoracic inlet. There was no evidence of a patent right internal jugular vein (Fig-2). Catheter mediated attempt for recanalisation to relieve increased systemic venous pressure was unsuccessful. Patient was placed on portagen and low molecular weight heparin and chylothorax subsided after 2 weeks. The patient returned to the hospital despite on low fat diet four months later again with brief viral illness and massive right pleural effusion which was chylous in nature. Conservative management over next 4 weeks, including portagen, total parenteral nutrition and chest tube drainage was unsuccessful. Subsequently, he underwnt a right thoracotomy with ligation of the thoracic duct. He was subsequently followed up at 3 monthly intervals for one year and did not show a recurrence of chylothorax. Repeat chest CT with contrast after prolong low molecular weight heparin therapy showed marginal improvement in venous thrombosis (Fig-3). His weight has increased from six to 10 kilograms, 8 months after surgery.
Thoracic duct injury has been shown to be associated with catheterization of both the left internal jugular/ subclavian vein and thoracotomy/open heart surgery in which disruption of lymphatic channels can occur during removal of thymus as a part of operative procedure. Delayed chylothorax in present case is related with progression of clot from right internal jugular vein to surrounding venous structure with subsequent increased venous pressure leading to leakage of lymphatic channels in both pleural spaces. Angiography demonstrated venous drainage from head and upper extremities diverted to IVC through bypass venous channels. Patient is doing well in short term follow-up after thoracic duct ligation, without gastrointestinal complications.
This report describes the unusal delayed presentation of recurrent chylous effusion after PDA ligation secondary to iatrogenic complication related with use of central line in neonatal period. Based upon underlying etio-pathogenesis. aggressive management with thoracic duct ligation should be considered in children.
M.E. Dumas, None.