Presenter: Dr.

Arwa Al-saggaf

Chylothorax refers to the presence of lymphatic fluid (chyle) in the pleural space secondary to leakage from the thoracic duct or one of its main tributaries.

Chylothorax is the most common cause of pleural effusion during the first days of neonatal life. often associated with other malformations. Frequency o Congenital: Prevalence 1:10,000 deliveries o The incidence of postoperative chylothorax is between 0.25% and 5.3% (much lower as age increase) Mortality/Morbidity o Perinatal mortality is between 15 and 30 percent Sex o Male to Female ratio is 2:1.

Accumulation of lymph within the pleural cavity can result from: overproduction impaired re absorption/obstruction

Disruption of the lymphatic vessels.

Usually this condition is associated with birth trauma ( thoracic duct tear)

Respiratory distress may develop because of either insufficient lung expansion, persistent fetal circulation or lung hypoplasia

Chylothorax occurs usually as a unilateral pleural effusion involving the right side of the lung in most instances. In rare cases, Chylothorax can be bilateral

Tension Chylothorax can also shift the mediastinum, impair venous return, and lead to congestive heart failure and hydrops

May be associated with trisomy 21, Turner syndrome, Noonan syndrome . Congenital pulmonary lymphangiectasis, Tracheoesophageal fistula, Extralobar lung sequestration Multiple malformation complex (anemia, tracheoesophageal fistula). Other pathologies inclued: Hydrops fetalis, congenital goiter, lung tumors, , congenital cytomegalovirus and adenoviral infections, right diaphragmatic hernia and group B streptococcal infections.

A pseudochylous milky fluid may be present in chronic serous effusion, where fatty material arises from degenerative changes in the fluid and not from lymph

Pseudochylous results from accumulation of cholesterol crystals in a chronic serous effusion.

The signs and symptoms are the same as those

due to pleural effusion of similar size

In 50% of cases, congenital chylothorax is present

with respiratory distress in the 1st day of life, but

they can also develop during the 1st week of life.

in the other 50%, onset is often gradual; however, after trauma to the thoracic duct, chyle may accumulate in the posterior mediastinum for days and then rupture into the pleural space with sudden onset of dyspnea, hypotension, and hypoxemia.

Symptoms from chylothorax first arise from fluid accumulation and lung compression. E.g SOB, poor feeding

Symptoms may progress rapidly a tension chylothorax Because chyle is bacteriostatic and not irritative, dyspnea is seen rather than fever or pleurisy.

Hx suggestive of congenital anomalies: eg consanguinity,

Surgical Hx (for non-congenital cases).

Respiratory. distress. Absent/decreased breath sounds Traumatic or surgical chylothorax may appear rapidly with respiratory embarrassment and shock. Usually, however, surgical chylothorax appears over many days because the postoperative patient is not fed and, hence, has minimal chyle flow Look for dysmorphic features: low-set auricles, micrognathia, high palate, short neck, bilateral flexion contractures of the interphalangeal joints, and limitation of hip adduction

Chylothorax in a neonate with Fetal Hydrops

.. Three years later !

Thoracentesis and pleural fluid analysis are the criterion standards to establish a diagnosis of chylothorax. Appearance: milky, opalescent. In neonates who have not yet been fed, the fluid may be clear. Triglyceride level is >110 mg/dL Pleural fluid to serum triglyceride ratio is >1.0 Pleural fluid to serum cholesterol ratio is <1.0 Lipoprotein analysis reveals chylomicrons. Cells : primarily T lymphocytes.

Pleural fluid for triglyceride content helps to confirm a diagnosis of chylothorax.

A level greater than 110 mg/dL reflects a 99% chance that the fluid is chyle.

A level less than 50 mg/dL reflects only a 5% chance that the fluid is chyle.
If the level is 50-110 mg/dL, use other parameters for diagnosis.

If a nonchylous effusion is present for several weeks, it may be milky and have a high triglyceride level (>110 mg/dL) and very high levels of cholesterols. Chylothorax can be distinguished from pseudochylothorax by fluid analysis. In pseudochylothorax, the cholesterol level is greater than 200 mg/dL, no chylomicrons are present, and cholesterol crystals are seen at microscopy.

Chest radiographic findings are nonspecific for chylothorax and indistinguishable from other causes of pleural effusion. Determine if effusion is bilateral. Look for a mediastinal shift. If the etiology of the chylothorax is unknown, obtain CT scans of the chest and abdomen to rule out malignancy. Lymphangiography is useful when the anatomy of the thoracic duct needs to be defined preoperatively or when the site of the leak is not clinically obvious.

Rt. Congenital chylothorax in a Jordanian girl, note the drained chyle is clear yellow b/c the baby is not breast-fed yet

The workup of a fetus with hydrothorax should include: fetal karyotype determination maternal antibody screen viral studies fetal echocardiography A thorough ultrasound examination to look for associated anomalies.

The following laboratory studies are not required for diagnosis but are useful to determine the metabolic and nutritional status of the patient:
Serum electrolyte tests
Serum albumin test

CBC count with differential to look for lymphocyte

depletion

 Isolated

pleural effusions non immune hydrops. Empyema, Pleuropulmonary Hemothorax CHF

Spontaneous recovery occurs in >50% of cases of neonatal chylothorax probably due to development collateral lymphatic channels. Treatment is either conservative or surgical.

Principles of Treatment:
Treating the underlying cause Instituting necessary respiratory care.

Providing appropriate fluid and nutritional replacement

Decreasing chyle production Draining and obliterating the pleural space

When a diagnosis is made before viability, the option of TOP can be offered. After viability, the management depends on the gestational age and the development of signs of hydrops or mediastinal shift. In term infants, prevent severe asphyxia by: thoracentesis may be considered before delivery to avoid respiratory failure due to a large pleural effusion. Anticipation for neonatal resuscitation.

The first option of treatment because of its high success rate (70-80%) Repeated thoracentesis and/or is tube thoracostomy done as needed to relieve pressure symptoms & maintain full lung expansion;

In cases with respiratory distress supportive mechanical ventilation is needed

Nutritional management:
Aim of nutritional management is: (1) Replacement of nutritional losses from the chyle leak: Chyle contains electrolytes & nutrients, If chyle is drained from the pleural space in appreciable quantities, these nutrients should be replaced

(2) Reduction of the volume of thoracic duct lymph flow: by using a formula made of compounds are absorbed by means of portal system blood flow rather than through the lymphatic system

Initial therapy includes enteral feedings with a low-fat or medium-chain triglyceride high-protein diet (MCT diet). If there is no resolution in 12 wk, total parenteral nutrition is instituted , Loss of electrolytes and protein in the pleural drainage may lead to decreased blood serum levels and the resultant hyponatremia or metabolic acidosis must be corrected.

Somatostatin, or its analogue octreotide, acts on

somatostatin receptors in the splanchnic area to reduce lymph fluid production.

Care must be taken to watch for adverse effects of somatostatin therapy, including diarrhea, hypoglycemia, and hypotension.

Indications :
Failure of conservative treatment (leak persisted > 2wks) Nutritional or metabolic complications, including electrolyte depletion and immunosuppression Loculated chylothorax, fibrin clots, or trapped lung Postesophagectomy chylothorax (Patients with this carry a high mortality rate if treated conservatively.)

Surgical options
Thoracic duct ligation is the criterion standard. is done either via open thoracotomy or video-assisted thoracoscopy. A pleuroperitoneal shunt can be successful for refractory chylothorax but can be complicated by infection and obstruction. Chemical pleurodesis or radiation is often used for malignant chylothorax or in inoperable patients. Surgical pleurectomy at age of 6 wks is a treatment option.

Thoracic duct ligation

Slooomothorax

The prognosis of congenital chylothorax is good. Perinatal mortality is between 15 and 30 percent. Mortality is increased in case of: Prematurity, accompanying pulmonary hypoplasia and development of nonimmune hydrops increase

mortality

Malnutrition: Repeated aspirations causes significant loss of calories, protein, and electrolytes Immunosuppression: repeated and chronic thoracenteses - hypogammaglobulinemia & loss of T lymphocytes increased risk of infection in neonates; patients should not receive live virus vaccines. Lack of resolution of chylothorax can lead to inanition, infection, and death.

Massive chylothorax respiratory embarrassment.

Co-morbidities.

The prognosis of congenital chylothorax is good. Perinatal mortality is between 15 and 30 percent. Mortality is increased in case of: Prematurity, accompanying pulmonary hypoplasia and development of nonimmune hydrops increase

mortality

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