Chylothorax: Causes, Symptoms & Treatment

The accumulation of lymph fluid in the chest cavity is called chylothorax. The lymph fluid accumulates between the lungs and the chest wall. This happens as a result after various other diseases and triggers breathing problems.

What is chylothorax?

Chylothorax is not a disease in its own right. It occurs rather rarely. It is a sequela following an injury to the thoracic duct. Causes of damage generally include accidental injury, medical intervention, or tumors of the lymphatic vessels. In addition, chylothorax can also occur after birth trauma. Chylothorax is considered a special form of pleural effusion. In this case, the lymphatic vessel, the thoracic duct, is damaged and suffers one or more leaks. This injury to the so-called thoracic duct leads to the fact that the return flow of lymphatic fluid into the blood system is no longer possible. As a result, the fluid accumulates unabated in the area of the pleural cavity. The pleural cavity is the slit-shaped body cavity between the two leaves of the pleura. The lymphatic fluid is chyle, a fatty lymph from the human digestive tract. Under normal circumstances, it flows independently through the thoracic duct into the venous blood. Chylothorax results in dyspnea. This is difficulty breathing due to a shortness of breath or shortness of breath.

Causes

There are many causes of chylothorax. Most commonly, it occurs with cancer. This may be due to a malignant tumor of the lymphatic vessels or surgical obstruction of the thoracic duct as a result of a tumor. Furthermore, chylothorax can be caused by injury during surgery. Unintentional damage can occur during a surgical procedure such as to the esophagus, chest, mediastinum, aorta, or heart. The mediastinum is a tissue space in the chest cavity. Tubing from a chest drain or placement of a central venous catheter can also cause damage to the thoracic duct in the patient. In addition, chylothorax may occur suddenly as a spontaneous rupture of the thoracic duct. Furthermore, trauma such as blunt thoracic trauma may be the cause of the damage. In some cases, chylothorax has been diagnosed in patients who have Gorham-Stout syndrome. If the pleural effusion occurs in the first few days of life, it is most often due to malformations of the thoracic duct or trauma from birth. In rare cases, liver cirrhosis or the infectious and worm diseases filariasis can cause chylothorax.

Symptoms, complaints, and signs

The accumulated lymph fluid causes problems with the patient’s breathing. In adults, significant symptoms occur at about a fluid volume of two liters. Coughing and chest pain are considered the first signs. The increase in fluid displaces the lungs and mediastinum. Symptoms of chylothorax include indicators such as shortness of breath and increased heart rate. At the same time, respiratory insufficiency and increased respiratory rate may occur. Additionally, the patient is noted to have decreased or absent breath sounds. A test by performing and determining the tapping sound leads to attenuation at the affected site. Due to the loss of lymph, there is an overall decrease in the lymphocyte count in the blood. Care should be taken not to feed breast milk to infants in the presence of a chylothorax. This is because fatty lymph fluid accumulates in the thoracic cavity, and fatty breast milk further increases this process.

Diagnosis and course

Diagnosis of chylothorax is made by several routes. Clinically, breath sounds and lung sounds are observed and measured. In addition, the body surface is palpated. On chest x-ray, a shadow is seen in the pleural cavity. This is particularly the case in the deeper layers of the chest. The lymphatic fluid can be removed by puncture and examined in the laboratory for its components. The fluid is milky turbid and sterile. An increased fat content and a high protein content are measured. Since the body is unable to remove the accumulation of lymph fluid in a chylothorax on its own, there is a steady deterioration in breathing.Spontaneous regression does not occur and medical intervention takes place.

When should you go to the doctor?

When breathing difficulties occur, as well as coughing and chest pain, a doctor should always be consulted. Although these complaints do not always indicate a chylothorax, they must be clarified in any case. If there is a concrete suspicion of the condition, it is best to consult a specialist in lymphology directly. In case of anxiety or panic attacks, the nearest clinic should be consulted. Parents who suspect their child has chylothorax or another condition should talk to their pediatrician. Because the condition can be particularly severe in infants, medical evaluation and treatment are essential. Patients who have had cancer or surgery on the esophagus, chest, aorta or heart are also at particular risk. If the aforementioned warning signs occur shortly after a surgical intervention or a tumor condition that has been overcome, the physician in charge must be informed. A chylothorax must always be treated in hospital. Affected individuals are advised to undergo regular examinations after therapy to rule out any complications.

Complications

In most cases, chylothorax results in breathing difficulties. These can sometimes lead to death. However, the possible complications and other symptoms depend greatly on the cause of the disease. Often, there is severe chest pain or coughing. The cough may also be bloody in some cases. Patients suffer from shortness of breath, which can also cause a panic attack in many sufferers. This increases the heart rate, which in the worst case can lead to respiratory failure. Often there are also abnormal or absent breath sounds. If chylothorax is not treated, death usually results, although many patients also die as a result of the disease. The mortality rate is particularly high in young children and infants. The treatment itself is aimed at reducing the lyhphatic flow. However, surgical intervention may also be necessary if the symptoms do not improve. If there are no errors during the procedure, there are usually no further complications. With early and proper treatment, life expectancy is also not altered.

Treatment and therapy

Medical treatment of chylothorax is essential. In addition, because untreated patients can die from chylothorax, it is a very serious condition. About 50 percent of sufferers die as a result of complications. The rate is significantly higher in infants. The form of therapy depends on the cause of the chylothorax. There is no single form of treatment. As a rule, a special diet is prescribed to reduce the possibility of fat accumulation. If possible, the chest as a whole is relieved and the progression of the accumulation is monitored. Targeted reduction of lymphatic flow can lead to closure of the lymphatic leak. In this way, the chylothorax can heal without further measures. However, if regeneration does not occur, surgical intervention follows after approximately one to two weeks. Surgery is also performed if the patient’s health deteriorates significantly. Here, the thoracic duct is repaired and the leaks are closed again by suturing. Further leakage of lymphatic fluid is thus prevented and it can be directed by the body back into the blood system on its own.

Outlook and prognosis

If chylothorax is recognized and diagnosed in a timely manner, there is a good prognosis with prompt treatment. Since chylothorax is mostly a secondary symptom as a result of another disease, the patient is often already undergoing inpatient or outpatient treatment. Therefore, a good prerequisite is that chylothorax is detected and competently treated shortly after its onset. In addition, the cooperation of the patient in the recovery process is also decisive for the healing success. Within the treatment many patients have to follow a special diet. If this is not successful, the chances of recovery deteriorate. The patient’s general state of health is also decisive.Since surgical intervention is often required to treat chylothorax, the patient needs physical resources to be able to overcome all the stresses well. Overall, the prospects of recovery diminish considerably once the first complications have occurred. Approximately half of all patients die due to further sequelae. In addition, the mortality rate is significantly higher in infants and young children. Patients who have already been cured may also develop psychological disorders as a result of the experience. Anxiety disorder, personality disorder, behavioral problems, or a panic lead to years of impairment and significantly reduce the patient’s quality of life despite physical improvements.

Prevention

Preventive measures cannot be taken for chylothorax because it is a secondary condition.

What you can do yourself

The condition gives the affected person little room for maneuver to bring about relief or cure of the symptoms himself. Nevertheless, he has the opportunity to promote the healing process and, if he has good self-awareness, to pass on changes to the doctor in good time. As soon as the affected person feels that something is wrong or that he has unusually little air available, he should trust in himself and seek medical help. Care should be taken during all physical movements to avoid overexertion and shortness of breath. In particular, playing intense sports or strenuous physical activity should be avoided. In addition, physical activities should be reduced in hot outdoor temperatures. For living with the disease, it is helpful for the affected person to have a stable social environment. Furthermore, a strong immune system is necessary in the healing process. A balanced and healthy diet, maintaining the ideal weight and participating in social life, taking into account the available opportunities, are important. This promotes well-being and, in addition, it strengthens mental health. Self-help groups or conversations with other sufferers can also help to get tips and advice on how to live with the diagnosis in an optimized and confident way.