Hanoi Ngoc Minh, 22 years old, had difficulty breathing for 6 months, had pleural effusion and blood that had to be continuously aspirated. Doctors discovered a lymphatic malformation in the bone, a rare disease with only 39 cases recorded in world medical literature.
Previously, the patient went to 4 hospitals, had about 1-2 liters of fluid aspirated every week for about 6 months, and did many tests but could not detect the cause of the hemothorax.
On October 30, Associate Professor, Dr. Nguyen Xuan Hien, Director of the Center for Diagnostic Imaging and Interventional Radiology, Tam Anh General Hospital, Hanoi, said that the patient was admitted to the hospital with lung, bone, liver, and spleen damage; anemia, weight loss, pale skin, difficulty breathing, and chest pain. The patient's left pleura was full of fluid, and the doctor drained three liters of pink fluid, while the amount of pleural fluid in a normal person is 7-10 ml.
"If we continue to drain the fluid, the patient will become exhausted, but if we don't drain it, it will cause collapsed lungs and respiratory failure," Associate Professor Hien said.
Test results showed that the pleural fluid contained a lot of lipids, a substance found in lymphatic fluid. The entire thoracic duct was dilated and tortuous. The doctor concluded that the patient had lymphatic malformations in multiple organs such as the bones, liver, and spleen. The lymphatic malformation in the chest area ruptured, causing fluid and blood to overflow into the left pleura, causing the patient to lose blood and nutrients.
The patient's pleural fluid after being aspirated. Photo: Provided by the hospital
"Lymphangioma is rare, and bone-invasive lymphangioma is even rarer," Associate Professor Hien said, adding that so far, a 2022 Chinese study published in the US National Library of Medicine is the only retrospective study of 39 patients with bone-invasive vascular malformations, with the rest being just isolated reports of a few cases. The disease is very rare and there are no specific statistics. Therefore, the disease is rarely given attention, and doctors can easily miss or misdiagnose it.
The team plugged the thoracic duct system to seal the leak. The Artis Pheno robot took a picture of the entire lymphatic system, detecting the leak at the level of the left lung hilum. Associate Professor Hien sealed the leak with metal coils and biological glue. The doctor assessed this as a difficult technique because it punctured directly through the epigastric region, the lymphatic duct is very small with a diameter of about 1.5-2 mm, difficult to find and block, requiring an experienced doctor.
Doctors perform lymphatic fistula embolization for the patient. Photo: Provided by the hospital
After one hour of intervention, the doctor successfully blocked the leak. After one day, the pleural fluid decreased significantly, by 10% compared to before the intervention. The patient could breathe easily, eat well, and the pleural fluid was gone after 5 days and was discharged from the hospital.
Lymphatic malformations are usually harmless and have almost no symptoms, making them difficult to detect. Most cases are discovered when there is trauma or surgery that causes the tumor to rupture, causing fluid to spill into the pleural, peritoneal, and pericardial cavities. Some cases are accidentally discovered with enlarged liver, enlarged spleen, and spontaneous bone fractures...
According to Associate Professor Hien, normally, when a lymphangioma ruptures, doctors use an MRI to find the leak point, but this method takes a lot of time and is expensive. Currently, Tam Anh General Hospital in Hanoi uses CT scans of the lymphatic system, which helps to accurately detect the location of the lymphatic leak. The diagnostic technique is faster and cheaper than MRI.
Lymphangioma is an incurable disease because there is no specific treatment. One of the complications of the disease is lymphatic leakage, at which point the patient should undergo radiological intervention. With the support of the machine, the doctor can find and block the leakage point accurately, without causing pain or blood loss, and with a quick recovery.
Hoai Pham
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