Girls with persistent cough persist because of extremely rare disease, the world has less than 20 cases

On October 15, according to information from Children's Hospital 2, Ho Chi Minh City, doctors successfully rescued a patient from a rare disease, the world only recorded less than 20 cases.

TTQN girl is a child (10 years old, residing in Dong Nai) who was taken to hospital by her family in a state of prolonged cough, difficulty breathing when exertion. Through a CT scan of the chest, doctors discovered a giant lung lung invasion of the left obstructed left lung , while the left and left pulmonary bronchus left the child with only one lung lung. right.

The surgical crew decided to only biopsy the needle with the hope that if it was a malignant tumor, chemotherapy to help reduce the size so that the operation was feasible and safer. However, the biopsy results show that this is a congenital peribronchial myofibroblastic tumor (congenital peribronchial myofibroblastic tumor) in the bronchial fibroblast tumor .

This is an extremely rare tumor and belongs to the malignant border type that does not respond to chemotherapy.

According to MSc Vũ Vũ Nhân Nhân - Deputy General Surgery, this is an extremely rare tumor and belongs to the malignant border type that does not respond to chemotherapy. According to WHO documents, as of 2015, only 16 cases have been reported in the world and are often detected in the newborn age due to prenatal diagnosis.

However, in the case of this girl due to lack of clear symptoms and no prenatal diagnosis, she had had a tumor for 10 years. That's why the tumor has grown so loudly that it invades the entire left lung of the baby. This may be the first case in the world recorded at this age with such a large size.

Doctor Nguyen Tran Viet Tanh - one of the doctors who participated in the operation said: 'This is the operation that 10 doctors encounter and 7-8 people who want to give up. The reason is that the tumor is so large that it invades the pericardial cavity, the left pulmonary and bronchial veins make the surgery very difficult and prolonged, only a small mistake during surgery also causes the patient to die immediately. on the operating table.

Besides, after removing the tumor, the pericardium must be reconstructed to prevent heart hernia, an extremely dangerous complication. In addition, complications after left-lung removal such as medial swaying, bronchial twisting and large blood vessels and scoliosis, although rare, can occur after this major surgery. In addition, the patient needs to be monitored closely every day after a major lung left surgery.

However, due to the dangerous nature of the tumor, if the surgery is not carried out, the tumor may block the artery and bronchus in the side, causing the child to die suddenly. After 8 hours, the surgeon skillfully removed and completely removed the tumor weighing 1.5kg. The results of 6-month post-operative follow-up showed that the baby recovered completely and did not suffer from complications.