Case Report
An 8-year-old girl born in the Yunnan Province of China was admitted to the department of internal medicine-cardiovascular, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine in June 2020 with chief complaints of 7 years after the operation of congenital heart disease (atrial septal defect, pulmonary valve stenosis and patent ductus arteriosus), lower limb edema for more than 4 years. She was the first-born child to her parents and was born at 38 weeks and 4 days of gestation after an unremarkable pregnancy. No obvious abnormalities were found during her neonatal period. The patient had no family history of NS or chylothorax.
More than 7 years ago, the repair of atrial septal defect, correction of pulmonary valve stenosis, aortic widening and patent ductus arteriosus occlusion was performed (details unknown). More than 4 years ago, there was no obvious inducement for left lower limb edema. At the beginning of the disease, the knee was obvious, and then the swelling gradually spread to the right lower limb and perineum. There was no skin flush, lower limb pain, eyelid edema, and groundless sitting breathing. After standing and walking for a long time, the lower limb edema was obvious. She once visited the outpatient department of local hospital, and was suggested to wear elastic stockings, but the lower limbs still had repeated edema after wearing elastic stockings. The patients were treated with continuous right thoracic closed drainage, chylous meal. Because the cause of chylothorax was not clear, there were still lower extremity edema.
General physical examination revealed the heart rate was 100 beats/min, the heart rate was even and the heart sound was strong. 2/6 systolic murmur was heard between 2-3 intercostal of left sternum. The abdomen was flat and soft. The lower edge of the liver was 2cm below the ribs, soft in texture and clear in boundary. There was no depression edema in the left lower limb, obvious in leg and ankle joint, no tenderness. Edema of perineum. After admission, the thoracic drainage fluid was 610 ml at most and 110 ml at least every day, which was light chylous. The results of laboratory tests are shown in Tab.1.
During the operation in other hospitals, it was found that the right lymphatic vessel and thoracic duct were compressed by the surrounding tissue, resulting in obstruction. After releasing the surrounding tissue, the obstruction was relieved and chylothorax disappeared.