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Chylothorax
Chylothorax
Chylothorax
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Chylothorax
Three bottles of chyle drained from a chylothorax
SpecialtyRespiratory medicine
SymptomsNone, breathlessness
ComplicationsDehydration, malnutrition, abnormal electrolyte levels, weakened immune system
TypesLow output, high output
CausesComplication of surgery, trauma, cancer, infections, lymph vessel abnormalities
Diagnostic methodX-ray, CT scan, thoracic MRI, fluid sampling
TreatmentRemoving fat from the diet, decreasing lymph flow, chest tube, surgery
MedicationOctreotide, midodrine, and sirolimus
Prognosis~10% risk of death

A chylothorax is an abnormal accumulation of chyle, a type of lipid-rich lymph, in the pleural space surrounding the lung. The lymphatic vessels of the digestive system normally return lipids absorbed from the small bowel via the thoracic duct, which ascends behind the esophagus to drain into the left brachiocephalic vein. If normal thoracic duct drainage is disrupted, either due to obstruction or rupture, chyle can leak and accumulate within the negative-pressured pleural space. In people on a normal diet, this fluid collection can sometimes be identified by its turbid, milky white appearance, since chyle contains emulsified triglycerides.

Chylothorax is a rare but serious condition, as it signals leakage of the thoracic duct or one of its tributaries. There are many treatments, both surgical and conservative.[1] About 2–3% of all fluid collections surrounding the lungs (pleural effusions) are chylothoraces.[2] It is important to distinguish a chylothorax from a pseudochylothorax (a pleural effusion that happens to be high in cholesterol), which has a similar appearance visually but is caused by more chronic inflammatory processes and requires a different treatment.[3]

Signs and symptoms

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The symptoms of a chylothorax depend its size and the underlying cause. A small chylothorax may not cause any symptoms and only be detected on a chest X-ray performed for another reason. A large chylothorax may lead to breathlessness or a feeling of pressure in the chest, caused by fluid restricting the expansion of the lungs, although large chylothoraces may remain asymptomatic if the chylothorax has accumulated slowly, as the lungs may have had time to become used to the pressure. Fever or chest pain are not usually associated with chylothorax, as chyle does not generate inflammation by itself.[4]

On examination, chylothorax may lead to reduced breath sounds on the affected side, associated with a dull sound when the chest is tapped or percussed. In cases of postoperative chylothorax, the first sign may be persistent drainage from intercostal drains.[1] Large chylothoraces may cause signs related to the loss of nutrients, including features of malnutrition or decreased ability to fight infections.[5] Rapidly accumulating chylothoraces can cause a sudden drop in blood volume, leading to low blood pressure.[5]

Causes

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There are three main types of chylothorax: traumatic, non-traumatic, and idiopathic. Historically the most common form of chylothorax was non-traumatic, but traumatic chylothoraces now represent the majority of cases, with most arising as postoperative complications of surgery.[6][7] The most common cause of non-traumatic chylothoraces is cancer.[1] Chylothoraces can also be classified as low- or high-output based on the rate of chyle accumulation: low-output chylothoraces accumulate <500 mL of chyle per 24 hours, while high-output chylothoraces accumulate >1000 mL per 24 hours.[8]

Non-traumatic

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Malignancies are the most frequent cause of non-traumatic chylothorax. Cancers like chronic lymphocytic leukemia, lung cancer, lymphoma, Kaposi sarcoma, metastatic carcinoma or esophageal cancer are potential causes of chylothorax. Infectious causes are also observed, most often in developing countries. The most common cause of an infectious chylothorax is a complication of tuberculous lymphadenitis. Other possible causative infections include aortitis, histoplasmosis, and filariasis. Chylothorax can also be congenital, and may co-occur with other lymphatic malformations like lymphangiectasis and lymphangiomatosis. Other conditions like tuberous sclerosis, congenital heart disease, trisomy 21 (Down syndrome), Noonan syndrome, or Turner syndrome (missing X chromosome) are also possible causes of congenital chylothorax. Other, more rare causes of congenital chylothorax include Castleman's disease, yellow nail syndrome, Waldenström's macroglobulinemia, sarcoidosis, venous thrombosis, thoracic radiation, macroglobulinemia, amyloidosis, and a goiter. These diseases cause chylothorax by obstructing or destroying the thoracic duct. Also, parenteral nutrition has been a possible cause; a quick dose of total parenteral nutrition can overwhelm the thoracic duct, causing the chyle to leak into the surrounding pleural space.[1]

Traumatic

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Iatrogenic chylothorax after surgery is the most common variety of chylothorax.[1] It is a common and serious complication of a pneumonectomy.[9] It is especially common in surgeries requiring mediastinal dissection.[5] The probability of chylothorax depends on the type of surgery. The surgery with the highest risk of chylothorax is an esophagostomy, with a 5-10% risk of chylothorax. Lung resection and mediastinal node dissection have the second highest risk, with 3-7% risk. Other operations like mediastinal tumor resection, thoracic aneurysm repair, sympathectomy, and any other surgeries that take place in the lower neck or the mediastinum can lead to chylothorax. Chylotharax after trauma but not after surgery has also been described after central line placement, pacemaker implantation, and embolization of a pulmonary arteriovenous malformation. Blunt trauma to the chest region is another cause of chylothorax, which has occurred after blast injuries and even simple injuries from coughing or sneezing.[1]

Mechanism

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The main mechanism of chylothorax is the leaking of chyle from the thoracic duct, usually caused by a disturbance affecting the structural integrity of the thoracic duct.[5] For example, placement of a central venous catheter can potentially disrupt drainage of lymph into the subclavian veins, followed by the thoracic duct, resulting in chylothorax.[5] The disturbances cause the pressure in the thoracic duct to increase. Soon, collateral channels form, which eventually drain into the thorax.[10] Trauma affecting the thoracic duct is the most common disturbing mechanism.

Whether a chylothorax occurs in the left or right pleural space is a consequence of the thoracic duct's anatomic location in the body and depends on the level where the duct was injured. If the thoracic duct is injured above the fifth thoracic vertebra, then a left-sided chylothorax results.[5] Conversely, a thoracic duct injury below that level will lead to the formation of a right-sided chylothorax.[5] Chylothoraces most commonly occur in the right pleural space (50% of cases).[5] Left-sided and bilateral chylothoraces are less common and occur in 33% and 17% of cases, respectively.[5]

In the case of cancer, invasion into the thoracic duct or collateral lymph channels can obstruct lymph. In the case of mediastinal lymphadenopathy, the enlarged lymph node causes compression of the lymphatic channels and thoracic duct. This impedes the centripetal drainage of the flow of lymph from the edges of the lung parenchyma and pleural surfaces. This causes the chyle to ooze extensively into the pleural cavity, leading to a chylothorax. In the case of yellow nail syndrome, or lymphedema, chylothorax is caused by hypoplasia or dilation of the lymph vessels. In rare cases, like in hepatic chylothorax, chylous ascites crosses the diaphragm into the pleural cavity. In idiopathic cases like genetic disorders, the mechanism is not known.[5] Up to three liters of chyle can easily drain into the pleural space daily.[10]

Diagnosis

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Bilateral chylothorax seen on a thoracic MRI
CT scan showing extensive chylothorax caused by leakage from the thoracic duct

Chest X-rays can detect a chylothorax. It appears as a dense, homogeneous area that obscures the costophrenic and cardiophrenic angles. Ultrasounds can also detect a chylothorax, which appears as an echoic region that is isodense with no septation or loculation. However, neither a normal chest x-ray nor an ultrasound can differentiate a chylothorax from any other type of pleural effusion.[1]

Chest X-ray showing bilateral chylothorax

The cisterna chyli can be found in a thoracic MRI, making it possible to confirm chylothorax. However, MRI is not the ideal method to scan the thorax, and so it is rarely used. Another diagnostic technique is conventional lymphangiography. It is rarely used since there are equally sensitive yet less invasive techniques available to identify a chylothorax. Lymphangiography procedures use the contrast dye agent lipiodol, which is injected into the lymphatic vessels. The chylothorax shows up on the images and identifies the source any leak in the thoracic duct.[1]

Another, more commonly used type of lymphogram is nuclear lymphoscintigraphy; this procedure requires human pentetic acid labeled Tc99m to be injected into the subcutaneous lesions of both sides of the dorsum of the foot. Then two images, anterior and posterior, are obtained using gamma-ray cameras. This test can be used with an integrated low-dose CT-scan with photon emission to get images that are more precise. Once pleural effusion is detected, a thoracentesis is recommended.[1]

The fluid of a chylothorax may appear milky, serous or serosanguineous. If the appearance of the fluid is not milky, that does not exclude a chylothorax from consideration. Since chyle is rich in triglycerides, a pleural effusion that is rich in triglycerides (>110 mg/dL) confirms the presence of a chylothorax; a pleural effusion that is low in triglyceride content (<50 mg/dL) virtually excludes the diagnosis.[11][12] If a pleural effusion contains triglycerides between 50 and 110 mg/dL, analysis of the lipoprotein content of the pleural effusion to evaluate for chylomicrons is recommended.[11] If that procedure detects chylomicrons in the fluid, that confirms a chylothorax. Chylothoraces are typically exudative and often contain a high number of lymphocytes and have low levels of the enzyme lactate dehydrogenase (LDH).[11] However, atypical chylothoraces can occur and are transudative in 14% of cases.[11] A milky appearance of pleural fluid is insufficient to confirm the diagnosis of chylothorax as pseudochylothoraces and empyemas can mimic this appearance.[11] Conversely, the absence of a milky appearance does not mean a chylothorax is not present as they may instead appear serous or bloody.[11]

Treatment

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The treatment for chylothorax depends on the underlying cause but may include dietary modification, medication to prevent chyle formation including somatostatin/octreotide, midodrine and sirolimus, pleurodesis, and surgical treatment including ligation of the thoracic duct, pleurovenous or pleuroperitoneal shunting or thoracic duct embolization.[1]

Initial

[edit]

The initial treatment of a chylothorax is usually drainage of the fluid from the pleural space. This may be necessary to restore lung function compromised by the pressure exerted by the chyle on the lungs.[1] Those with large chylothoraces may need nutritional support due to the nutrients lost, primarily to correct protein and electrolyte losses. Once the affected person is hemodynamically and nutritionally stable, then specific treatment can begin.[5]

Conservative

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A conservative treatment is changing diet to include fewer long-chain fatty acids, in particular free fatty acids. Since chyle is formed from these acids, chyle formation will reduce, allowing the defects to heal spontaneously. However, this can lead to fat deficiency and malnutrition over time. A possible response to this drawback is a venous fat hemorrhage, in which small and medium-chain fatty acids are given by diet, and long-chain fatty acids are given intravenously. Thoracentesis and an indwelling catheter for use at home is generally used to drain the chylothorax.[1] If a malignant neoplastic chylothorax is present, then treatment with radiotherapy and/or chemotherapy is warranted.

Surgical

[edit]

Surgery is indicated if the case is post-traumatic, iatrogenic, or refractory to other treatments, in which cases surgery reduces mortality by 40%. One invasive surgical intervention called a thoracic duct ligation involves closing off the thoracic ducts.[1] Surgical pleurodesis is another option and can be undertaken if the affected person fails to respond to conservative treatment and is not a candidate for surgical intervention.[13]

Another treatment option is pleuroperitoneal shunting (creating a communication channel between the pleural space and peritoneal cavity). Since surgery to close the leak is not reliable, talc pleurodesis is recommended; in a case study of 19 people with refractory malignant chylothorax due to lymphoma, it resulted in success for all affected individuals.[5] Chemical pleurodesis is an option, since the leaking of lymphatic fluids is stopped by irritating the lungs and chest wall, resulting in a sterile inflammation. This causes the lung and the chest wall to fuse together, thus preventing lymphatic fluids from leaking into the pleural space.[14]

Prognosis

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The morbidity and mortality rates associated with chylothorax have declined as treatments have improved. Malignant, bilateral, and chronic chylothoraces have an inferior prognosis to other types.[5] Currently, the mortality and morbidity rates are about 10% if treated surgically.[1] If cases are post-operative and treated conservatively, mortality rates approach 50%.[5]

Complications

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Complications of chylothorax include malnutrition, immunosuppression, dehydration, and respiratory distress.[6] The severity of the complications depends on how quickly the chylothorax accumulated, its size, and its chronicity.[13]

Epidemiology

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Chylothoraces are rare and usually occur as a complication of surgeries in the neck and mediastinum. It has no gender or age predisposition. A chylothorax occurs in 0.2-1% of cardiothoracic surgeries, 5-10% of esophagostomies, and 3-7% of lung resections.[1]

Other animals

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Horses

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Chylothorax is uncommon in horses. Clinical signs and symptoms in foals include difficulty breathing, fast breathing, cough, fever, and lethargy. The fluid generally appears opalescent and milky without any odor. A line of fluid is observed on percussion and there are reduced lung sounds. To differentiate between chyle is pseudochyle, which does not clear after centrifugation. There is not much information on the treatment of chylothorax in horses. Supportive care, antimicrobials, drainage of the thorax, and dietary management have been used with success. Surgery has been done in other animals with limited success, but has not yet been reported in horses. Although success has been reported, the prognosis is relatively unknown due to the lack of data.[15]

References

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Chylothorax

View on Grokipedia
from Grokipedia
Chylothorax is a rare but serious medical condition characterized by the accumulation of —a , lipid-rich lymphatic fluid produced in the intestinal lacteals—in the pleural space surrounding the lungs, typically due to disruption or leakage from the . This accumulation, which accounts for approximately 3% of all pleural effusions, can lead to respiratory compromise, , and if untreated, with reported 90-day mortality rates as high as 82% in severe cases. The condition arises from either traumatic or nontraumatic causes, with iatrogenic injury during thoracic or neck surgery being the most common trigger, occurring in up to 5-10% of esophagectomy procedures. Nontraumatic etiologies include malignancies such as (responsible for 70-75% of malignant cases), congenital lymphatic malformations like , infections (e.g., ), and idiopathic factors in about 10% of instances. Pathophysiologically, the , which drains roughly 75% of the body's including at an average daily production of 2.4 liters, breaches, allowing —rich in chylomicrons, lymphocytes (400-6800 cells/μL), proteins, and fat-soluble vitamins—to enter the , often unilaterally on the right side in two-thirds of cases. Clinically, small chylothoraces may be , but progressive accumulation typically presents with , chest pressure, , and unintended due to compression of the lungs and loss of nutritional elements. is confirmed through , where pleural fluid analysis reveals triglycerides exceeding 110 mg/dL (with below 200 mg/dL) and the presence of chylomicrons, often appearing milky in 22-44% of samples; supportive imaging includes chest CT, , or lymphangiography to identify leaks or underlying causes. Management employs a stepwise, multidisciplinary approach prioritizing conservative measures such as low-fat diets supplemented with medium-chain triglycerides to reduce flow, total , and pharmacological agents like to decrease production, often resolving 50-70% of cases without intervention. For persistent leaks, options escalate to repeated drainage via or chest tubes, chemical , percutaneous (with near 100% success in select nontraumatic cases), or surgical ligation. Early intervention is critical, particularly in neonates where congenital incidence ranges from 1:10,000 to 1:24,000 live births, to prevent long-term complications like growth impairment.

Background

Definition

Chylothorax is defined as the accumulation of in the pleural space, resulting from disruption or obstruction of the or its major tributaries. is a milky lymphatic fluid produced in the during fat digestion, characterized by its high content of triglycerides, chylomicrons ( particles that transport dietary ), and lymphocytes, along with proteins, electrolytes, and fat-soluble vitamins. The condition is classified into chylous and pseudochylous effusions based on the nature of the fluid. True chylothorax, or chylous effusion, is confirmed by pleural fluid levels exceeding 110 mg/dL and the presence of chylomicrons, distinguishing it from pseudochylous effusions, which arise from chronic inflammation or and feature high content without chylomicrons. The term "chylothorax" derives from the Greek words chylos (meaning "juice," referring to the fluid's appearance) and (meaning "chest"). The serves as the primary conduit for chyle transport from the to the venous system.

Epidemiology

Chylothorax is a rare condition, accounting for less than 3% of all pleural effusions. As of 2024, post-surgical incidence rates remain consistent with earlier reports, ranging from 0.5-1% following adult and 1-4% after esophagectomy due to the 's anatomical proximity to operative sites. Demographic patterns of chylothorax exhibit a bimodal distribution, with peaks in neonates due to congenital forms and in adults over 50 years often linked to . In neonatal cases, congenital chylothorax has an incidence of 1 in 10,000 to 24,000 live births, making it the most common type of in newborns. In pediatric cases, congenital chylothorax accounts for about 50% of instances, frequently associated with lymphatic malformations or syndromes. Among adults, traumatic chylothorax shows a predominance, reflecting higher rates of in this group, while non-traumatic cases demonstrate more equal gender distribution. remains a leading cause in adults, comprising up to 68% of cases in international registries. Geographic variations in chylothorax incidence correlate with regional differences in trauma prevalence and rates, with higher occurrences reported in areas of elevated surgical volumes or cancer burden, though no significant seasonal patterns have been identified.

Clinical Presentation

Symptoms

The primary symptom of chylothorax is progressive dyspnea, or , which develops as chyle accumulates in the pleural space and compresses the lungs, often worsening with or over time. Patients may also experience a non-productive , though in massive cases, it can occasionally produce milky known as chyloptysis. Systemic symptoms arise from the nutritional losses associated with chyle leakage, including , unintended , and anorexia due to the depletion of fats, proteins, and lymphocytes. In chronic untreated cases, these can lead to significant and muscle wasting. Less common symptoms include , particularly in traumatic etiologies, and or fever if secondary to or . may occur in prolonged cases due to from ongoing loss. Small-volume chylothoraces may remain until effusion volume increases.

Signs

In moderate-to-severe cases of chylothorax, vital signs often reflect respiratory compromise from pleural effusion compression, including tachypnea and hypoxemia. Tachycardia may occur if significant chyle loss leads to hypovolemia, particularly in high-output effusions exceeding 1000 mL per day. Physical examination typically reveals decreased breath sounds and dullness to percussion over the affected side, with effusions being unilateral in approximately 80% of cases and more commonly right-sided. In chronic chylothorax, signs of malnutrition such as cachexia and muscle wasting can be evident due to ongoing loss of proteins, fats, and lymphocytes in the chyle. If the condition is malignancy-related, such as lymphoma, peripheral lymphadenopathy may be palpable on examination. In neonates with congenital chylothorax, objective findings include respiratory distress manifesting as , grunting, and retractions, often appearing within the first 24 hours of life in half of cases. may accompany severe respiratory compromise, and can occur if associated with congenital lymphatic malformations leading to chylous .

Causes

Traumatic Causes

Traumatic chylothorax arises primarily from physical injuries that disrupt the , with iatrogenic causes being the most prevalent form. Surgical interventions in the carry a notable risk of thoracic duct injury, leading to chyle leakage into the pleural space. For instance, esophagectomy is associated with an incidence of postoperative chylothorax ranging from 0.5% to 3%. Similarly, repair has a reported incidence of 0.4% to 1.25% for chylothorax following the procedure, often due to inadvertent during or manipulation near the duct. Mediastinal , commonly performed during resections for cancer, follows with an incidence of approximately 1% to 2%, exacerbated by the proximity of the to mediastinal structures. These iatrogenic injuries typically manifest within days to weeks post-surgery, highlighting the importance of vigilant monitoring in high-risk procedures. Non-surgical trauma, including blunt and penetrating injuries, accounts for a smaller but significant subset of cases, often resulting from high-velocity impacts or direct wounds. , such as that from accidents, can or vertebrae, indirectly shearing the despite its protected mediastinal location; such events are rare, with an incidence of 0.2% to 3% among blunt thoracic injuries. , exemplified by stab wounds or gunshot injuries to the chest, more directly lacerates the duct, leading to immediate or rapid accumulation. A characteristic feature of these traumatic etiologies is delayed presentation, where symptoms may emerge up to several weeks after the initial injury, potentially triggered by resumption of oral intake or increased intrathoracic pressure. Radiation therapy to the represents another iatrogenic contributor under the traumatic umbrella, inducing chylothorax through progressive and obstruction of the . This complication arises from inflammatory responses and scarring following high-dose , particularly in treatments for or lymphomas. Cases often develop insidiously over months, distinguishing them from acute surgical disruptions.

Non-Traumatic Causes

Non-traumatic chylothorax results from systemic diseases, congenital anomalies, or idiopathic processes that compromise the or lymphatic drainage without acute injury, leading to accumulation in the pleural space. represents the predominant , accounting for approximately one-third of all chylothorax cases and over 50% of non-traumatic cases. Among neoplastic causes, is the most frequent, responsible for 50-70% of malignant chylothoraces in adults, with being more common than due to its propensity for mediastinal involvement. These tumors typically cause chylothorax through direct invasion, extrinsic compression, or obstruction of the , resulting in elevated lymphatic pressure and leakage. Other malignancies, such as , esophageal carcinoma, and metastatic tumors from distant sites (e.g., or ), contribute less frequently by similar mechanisms of ductal obstruction or lymphatic permeation. Congenital and idiopathic etiologies account for a notable portion of non-traumatic cases, particularly in pediatric populations but also in adults. In neonates, or represents a primary congenital cause, often presenting as isolated chylothorax with an incidence of 1 in 10,000 to 24,000 live births, stemming from developmental lymphatic malformations that prevent normal chyle flow; it is often associated with genetic syndromes such as , , and trisomy 21. Lymphangiomatosis, a rare disorder characterized by diffuse lymphatic malformations, leads to chylothorax via hyperpermeability and multiple lymphatic leaks, affecting up to 40% of patients with advanced (a related condition). Idiopathic chylothorax, diagnosed after exclusion of all other causes, comprises 6-10% of non-traumatic cases and is presumed to involve subtle, undetected lymphatic defects or transient pressure imbalances. Additional non-traumatic causes include a spectrum of infectious, inflammatory, vascular, and endocrine disorders that indirectly disrupt lymphatic integrity. Infections such as and predominate in endemic regions, causing chylothorax through granulomatous inflammation, lymphatic obstruction, or parasitic invasion of tributaries. induces rare cases via non-caseating granulomas that compress mediastinal lymphatics, mimicking neoplastic obstruction. , particularly from underlying malignancy or hypercoagulability, elevates and backflows into the , promoting chyle extravasation. has been infrequently associated, potentially through myxedema-related lymphatic congestion or in conjunction with substernal goiter compressing the duct, though it remains an uncommon and poorly understood contributor.

Pathophysiology

Thoracic Duct Anatomy

The is the principal in the , responsible for returning and from the majority of the body to the systemic circulation. It originates from the , a dilated lymphatic sac located at the L1-L2 vertebral level in the , posterior to the and anterior to the vertebral column. From there, the duct ascends through the of the diaphragm into the , initially positioned to the right of the midline between the and the . It then courses posterior to the , crosses the midline at approximately the T5 vertebral level, and continues superiorly along the left side of the esophagus before arching laterally at the level of the second rib. The duct typically terminates by emptying into the junction of the left internal jugular and subclavian veins, about 2-3 cm superior to the . Its total length measures approximately 38-45 cm, with a of 2-5 mm, and it receives variable tributaries including the intestinal trunk, lumbar trunks, left jugular trunk, left subclavian trunk, and left bronchomediastinal trunk, draining from the lower extremities, , , and left , head, and neck. Physiologically, the functions to transport —a fat-laden produced postprandially in the intestines and collected via the mesenteric (intestinal) nodes—along with from other regions back to the venous system. In a typical adult, it conveys 2-3 liters per day of this , with flow rates averaging 1.38 mL/kg/hour under basal conditions but increasing significantly after meals due to heightened intestinal absorption. The unidirectional flow is facilitated by intrinsic pressure gradients, extrinsic compression from surrounding structures such as respiratory movements and arterial pulsations, and a series of bicuspid valves that prevent , maintaining intraductal pressures between 0-22 mmHg that vary with the respiratory cycle. Anatomical variations of the are common, occurring in approximately 50% of individuals and potentially increasing the risk of lymphatic leaks due to aberrant pathways. These include duplication or plexiform arrangements of the lower thoracic segment, absence of the in up to 50% of cases (with direct continuity from lumbar trunks), and multiple terminal branches. The standard configuration features a separate right lymphatic duct draining the right upper quadrant (head, neck, right arm, and right ) into the right , present in most individuals; however, variations such as right-sided termination of the occur in 1-6% of cases, while bilateral drainage systems or complete drainage by the alone are reported in 5-10% of anatomies.

Chyle Leak Mechanisms

Chylothorax primarily arises from direct leakage of into the due to laceration or rupture of the , often facilitated by anatomical defects in the that allow to seep from the disrupted vessel. This breach in ductal integrity, commonly occurring along its mediastinal course, results in the accumulation of lipid-rich lymphatic fluid within the , as the serves as the main conduit for transport from the intestines to the venous system. Such direct disruptions are frequently iatrogenic, stemming from thoracic surgeries like esophagectomy, where the incidence ranges from 4% to 10%. In obstructive cases, chyle leaks develop secondary to elevated lymphatic pressure caused by extrinsic compression or intrinsic blockage of the , such as from malignant tumors like , leading to backflow and eventual transudation of across pleural or diaphragmatic barriers. Tumor or enlarged nodes compress the duct, impeding chyle flow and causing upstream hydrostatic pressure buildup, which, combined with the negative intrathoracic pressure, promotes retrograde leakage through collateral vessels or small defects. is the leading cause of non-traumatic chylothorax, accounting for approximately 25-50% of cases, with responsible for 70-75% of malignant instances. Prolonged chyle leakage triggers secondary pathophysiological effects, including due to substantial loss of lymphocytes and immunoglobulins, which depletes the body's immune defenses and increases susceptibility to infections. Additionally, the ongoing depletion of proteins, fats, and fat-soluble vitamins in the leaked leads to , manifesting as , , and metabolic disturbances. Chronic inflammation from persistent pleural irritation can further culminate in , characterized by pleural and adhesions that restrict lung expansion.

Diagnosis

Fluid Analysis

Diagnosis of chylothorax begins with , the aspiration of pleural fluid for analysis, which often reveals a milky-white appearance due to the emulsified fat content of . This gross characteristic is present in approximately 44% of cases, though the fluid may also appear serous, serosanguinous, or bloody in others. The key biochemical markers for confirmation include a pleural fluid level greater than 110 mg/dL, which is diagnostic in over 98% of cases, while levels below 50 mg/dL virtually exclude the condition. Additionally, a level less than 200 mg/dL supports the and helps differentiate chylothorax from pseudochylothorax, where is typically elevated. The gold standard for definitive is the detection of chylomicrons in the pleural fluid via lipoprotein electrophoresis. Cellular analysis of the aspirated fluid typically shows a lymphocyte-predominant , with lymphocytes comprising more than 80% of the nucleated cells and low counts. The ranges from 7.4 to 7.8, reflecting the alkaline nature of , and the specific gravity is greater than 1.012. Additional tests include measurement of (LDH) and protein levels, which often follow an exudative pattern per Light's criteria, with protein exceeding 3 g/dL in many cases despite relatively low LDH (median around 96.5 U/L). Pleural fluid cultures for , fungi, and acid-fast are routinely performed to rule out concurrent infection.

Imaging Studies

Initial imaging for chylothorax typically begins with a chest , which reveals a as a homogeneous opacity obliterating the costophrenic and cardiophrenic angles, often unilateral in approximately 80% of cases with the right side affected in about two-thirds. This modality cannot distinguish chylothorax from other types of effusions but is essential for confirming the presence of fluid and screening for underlying causes such as masses or . Thoracic ultrasound complements chest by visualizing the effusion as an isodense, echoic region without septations and is particularly valuable for guiding to safely sample the fluid. Advanced imaging techniques provide greater anatomical detail to identify the chylothorax, locate lymphatic leaks, and evaluate potential etiologies. Contrast-enhanced computed tomography (CT) of the chest is more sensitive than plain radiography or , depicting the along with mediastinal masses, , or abnormalities; in rare cases (about 2%), it visualizes the as a low-attenuation tubular structure. (MRI) or MR lymphangiography excels in soft tissue resolution, reliably showing the in nearly all cases and assessing lymphatic flow without radiation exposure, though it is less commonly used due to challenges in thoracic . For precise mapping of leak sites, lymphoscintigraphy using technetium-99m-labeled agents, often combined with (SPECT)/CT, or intranodal lymphangiography with oil-based contrast offers high diagnostic accuracy, with reported sensitivities of 80-90% for detecting disruptions. Recent advances include (ICG) , which enhances intraoperative detection of chylous leaks by illuminating the and leakage sites under near-infrared light, improving localization during surgical interventions with detection rates up to 74%. This technique has proven particularly useful in refractory cases following conservative management, allowing for targeted ligation or repair.

Treatment

Initial Management

The initial management of chylothorax prioritizes rapid stabilization of the patient, alleviation of respiratory compromise, and minimization of chyle production to prevent complications such as and . This approach is particularly urgent in cases of acute presentation, where can lead to and hemodynamic instability, especially following trauma. Drainage of the pleural space is the cornerstone of immediate intervention to relieve dyspnea and facilitate re-expansion. Therapeutic is indicated for initial symptomatic relief in patients with slowly accumulating s, particularly in non-traumatic cases, allowing for diagnostic fluid analysis while avoiding invasive procedures. For more rapid or high- accumulations, such as post-traumatic or postoperative chylothorax, insertion of a (thoracostomy) is preferred to provide continuous drainage and monitor output . Output exceeding 1000 mL per day is classified as high-flow, signaling the need for aggressive measures, while lower volumes may respond to conservative strategies. placement should be limited to under two weeks to minimize risk, with serial to assess resolution. Nutritional support aims to reduce thoracic duct flow by eliminating enteral fat intake, thereby decreasing chyle leakage. Patients are placed on nil per os (NPO) status, with total parenteral nutrition (TPN) initiated to meet caloric and protein requirements intravenously, bypassing the . TPN formulations should include adequate , electrolytes, and vitamins to counteract losses from drainage, with close monitoring of , counts, and overall nutritional status to detect early depletion. In high-output leaks, TPN is particularly essential, as it can lead to significant fluid shifts if not managed promptly. Supportive measures address immediate physiological derangements and enhance comfort during stabilization. is administered if is present due to ventilation-perfusion mismatch from the . Adequate pain control is provided using analgesics, as procedural interventions like insertion can cause discomfort, though itself is non-irritating. In traumatic chylothorax, hemodynamic stabilization takes precedence, involving intravenous fluid resuscitation (e.g., crystalloids) and vasopressors if shock is evident, alongside addressing associated injuries. Throughout, , volume status, and balance are vigilantly monitored to guide fluid replacement and prevent complications like or .

Conservative Management

Conservative management of chylothorax focuses on non-invasive strategies to decrease production and facilitate spontaneous closure of the leak, particularly in cases of low-output (typically <500-1000 mL/day). This approach is often the initial step after stabilization, aiming to avoid more invasive interventions by minimizing lymphatic flow through the thoracic duct. Success depends on factors such as etiology, output volume, and patient nutrition status, with overall resolution rates varying widely but generally higher in low-output scenarios. Dietary modification forms the cornerstone of conservative therapy, primarily through the use of a medium-chain triglyceride (MCT)-based diet. Unlike long-chain triglycerides, which are absorbed via the lymphatic system and contribute to chyle formation, MCTs are directly taken up by the portal vein into the bloodstream, thereby reducing intestinal chyle production and thoracic duct flow. This intervention is typically implemented for 2-6 weeks, often combined with fat restriction or total parenteral nutrition (TPN) if oral intake is poorly tolerated. In low-output chylothorax, MCT diets achieve success rates of approximately 20-50%, though palatability issues and gastrointestinal side effects like steatorrhea can limit adherence. Pharmacologic agents, such as octreotide or somatostatin analogs, are frequently added to enhance dietary measures by further suppressing chyle output. These somatostatin analogs inhibit gastrointestinal hormone release, reduce splanchnic blood flow, and decrease intestinal fat absorption, leading to a substantial reduction in chyle production—often by 50% or more within days of initiation. Octreotide is administered subcutaneously at doses of 50-100 mcg every 8 hours or intravenously at 6 mg/day, typically for 1-2 weeks, with monitoring for side effects including abdominal cramps, nausea, and hyperglycemia. As an adjunct to conservative management, octreotide yields success rates of 80-90% in select pediatric and postoperative cases, though efficacy is lower (around 47%) in neonatal congenital chylothorax. Ongoing monitoring involves serial pleural fluid drainage via thoracentesis or chest tube to alleviate respiratory symptoms and assess output trends, with daily volumes tracked to guide therapy duration. If low-output chylothorax persists despite dietary and pharmacologic interventions (e.g., <500 mL/day after 1-2 weeks), chemical pleurodesis may be considered as a non-surgical adjunct to promote pleural adhesion and seal the leak. Agents such as talc slurry or doxycycline (administered intrapleurally at 500 mg diluted in saline) have shown efficacy in refractory low-output cases, with success rates up to 75-80% when used via existing drainage tubes, though pain management is essential during instillation.

Interventional Procedures

Interventional procedures for chylothorax primarily involve minimally invasive, image-guided techniques aimed at localizing and sealing lymphatic leaks, offering alternatives to more invasive surgical options, particularly in high-risk patients. These approaches leverage advancements in interventional radiology to access the lymphatic system percutaneously, reducing recovery time and morbidity compared to traditional methods. Common procedures include thoracic duct embolization and targeted sclerotherapy, which have demonstrated clinical success rates ranging from 70% to 90% in appropriately selected cases, with low complication profiles. Thoracic duct embolization (TDE) is a cornerstone interventional technique, involving percutaneous access to the thoracic duct via pedal or intranodal lymphangiography to opacify the lymphatic system and identify the leak site. Once visualized, the duct is cannulated using a needle or catheter, followed by occlusion with embolic agents such as coils, glue, or vascular plugs to block chyle flow. This procedure is particularly effective for post-traumatic chylothorax, achieving technical success in approximately 63% of cases and clinical success in 79%, with major complications occurring in less than 3% of patients, including minor issues like wound infection or transient leg edema. For localized lymphatic leaks contributing to chylothorax, needle aspiration combined with sclerotherapy provides a targeted option, often guided by lymphangiography to ensure precision. In this approach, a needle is percutaneously inserted under imaging (e.g., CT or fluoroscopy) to aspirate chylous fluid and inject a sclerosing agent, such as ethanol or doxycycline, to induce fibrosis and seal the leak. Clinical success rates exceed 88% in postoperative lymphatic leaks, including chylothorax, with no reported complications in small series, making it suitable for refractory or isolated collections. Recent advances in interventional management emphasize refined embolic materials and hybrid strategies for refractory cases. N-butyl cyanoacrylate (NBCA) glue has emerged as a highly effective agent for thoracic duct occlusion, enabling rapid polymerization and sealing even in challenging anatomies, with successful embolization reported in up to 88% of iatrogenic chylothorax cases. Vascular plugs offer deployable alternatives for precise duct closure, enhancing outcomes in complex leaks. For persistent high-output chylothorax unresponsive to embolization, pleuroperitoneal shunting can be combined as a salvage intervention, diverting chyle from the pleural space to the peritoneum via a subcutaneous catheter, achieving resolution in over 80% of refractory pediatric and adult cases with minimal invasiveness.

Surgical Interventions

Surgical interventions for chylothorax are typically reserved for cases where conservative management, such as dietary modification and drainage, fails to resolve the effusion after 1-2 weeks or when high-output leaks (>1000 mL/day) persist, necessitating definitive repair of the underlying defect. These procedures aim to ligate the leaking lymphatic structures, address causative pathology, or redirect flow, with overall success rates ranging from 67% to 100% depending on and approach. Thoracic duct ligation remains the cornerstone surgical treatment for non-neoplastic chylothorax, particularly traumatic or postoperative variants, performed via open or minimally invasive (VATS). In VATS, the is identified in the right posterior (typically at the level of the or T5-T6 vertebrae) and ligated with clips or sutures after preoperative enhancement via oily contrast or injection to facilitate visualization. Success rates exceed 90% in traumatic cases, with VATS offering reduced morbidity, shorter hospital stays, and comparable efficacy to open surgery. For chylothorax secondary to neoplastic causes, such as or metastatic disease obstructing lymphatic flow, surgical options include pleurectomy to promote of pleural surfaces or resection of the causative mass to alleviate compression on the . Pleurectomy involves mechanical abrasion or partial removal of the parietal pleura, often combined with or instillation for , achieving resolution in cases where duct ligation is not feasible due to widespread involvement. Mass resection targets identifiable tumors, as seen in isolated reports of thymic or mediastinal neoplasms, with outcomes tied to the underlying control. In instances of chronic or loculated chylothorax leading to —a fibrotic constriction of the — is employed to peel away the restrictive pleural peel, restoring lung expansion and allowing drainage of trapped . This procedure, typically via , is indicated when reveals trapped and is particularly relevant in complicated, non-resolving effusions, though it carries higher risks in patients with from prolonged chyle loss. For high-output, chylothorax unresponsive to ligation, pleurovenous or pleuroperitoneal shunts provide palliation by diverting from the pleural space to the venous or peritoneal circulation, respectively. The shunt, a historical valved system, is inserted surgically and pumped intermittently; success rates approach 80-85% in selected cases, though complications like occlusion or limit its use in the favoring less invasive alternatives.

Prognosis

Short-Term Outcomes

Short-term outcomes for chylothorax vary based on , output volume, and approach, with conservative strategies achieving resolution in approximately 50-80% of cases, particularly for low-output leaks (<500-1,000 mL/day). Interventional procedures, such as thoracic duct embolization, and surgical interventions, including thoracic duct ligation, yield higher success rates of 80-95%, especially in traumatic cases where early aggressive is feasible. In neonates with congenital chylothorax, untreated cases carry a mortality risk of 20-50%, driven by respiratory compromise and nutritional deficits, though overall neonatal mortality with treatment ranges from 28-32%. Resolution timelines depend on chyle output and treatment modality, with low-output chylothorax often resolving within 2-4 weeks under conservative management involving dietary modification and drainage, while high-output cases (>1,000 mL/day) may require 4-6 weeks or longer, prompting escalation to interventions if no improvement occurs by 14 days. Recurrence rates following initial resolution are estimated at 10-20%, more common in non-traumatic etiologies due to persistent underlying pathology. Etiologic factors significantly influence short-term , with traumatic chylothorax demonstrating superior outcomes compared to malignant cases; mortality is typically under 5% in traumatic instances with prompt intervention, versus up to 40-50% in malignant chylothorax due to progression and treatment resistance. Overall 90-day mortality for chylothorax can reach 82% if nutritional and immunologic complications are not addressed early.

Long-Term Complications

Long-term complications of chylothorax arise primarily from the persistent loss of , which is rich in nutrients, proteins, lymphocytes, and immunoglobulins, leading to systemic effects that can persist even after initial resolution of the . These complications are more pronounced in cases of prolonged drainage or high-output effusions and can significantly impact , particularly in pediatric patients. Nutritional and immunologic deficits represent major long-term concerns, as the ongoing drainage of depletes essential fats, proteins, electrolytes, and fat-soluble vitamins, resulting in , , and a protein-losing enteropathy-like state that mimics gastrointestinal losses. This nutritional depletion can manifest as , muscle wasting, and electrolyte imbalances such as , exacerbating overall morbidity. Immunologically, chylothorax induces and lymphopenia due to the loss of lymphocytes (typically 400–6,800 cells per mL of ) and immunoglobulins, leading to and a threefold increased risk of nosocomial infections, including and . These immunologic changes, particularly lymphopenia, correlate directly with the duration of pleural drainage, with B-cell and T-cell depletion (including proportional declines in + and + subsets) observed in persistent cases. Pleural complications from chronic or recurrent effusions include the development of , where organized pleural restricts expansion and contributes to , potentially requiring for management. If the effusion becomes infected due to immunologic compromise, it can progress to , further complicating respiratory function and necessitating aggressive antimicrobial therapy or drainage. Other long-term issues encompass recurrence after treatment, occurring in approximately 10–25% of cases depending on the underlying and approach, often necessitating repeated interventions such as or ligation. In children, from chylothorax is associated with and growth delays, with affected infants at heightened risk for suboptimal height and weight gains that may persist into later childhood. Prolonged hospitalization for can also impose psychological burdens, including developmental delays or behavioral issues, though neurodevelopmental outcomes are generally favorable in isolated congenital cases without comorbidities.

Chylothorax in Animals

In Small Animals

Chylothorax in small animals, primarily dogs and cats, is characterized by the accumulation of in the pleural due to disruption or leakage from the or its tributaries. This condition is relatively uncommon, with cats affected approximately four times more frequently than dogs, and certain breeds such as Siamese and Himalayan cats, Afghan hounds, and Shiba Inus showing predisposition. The is often idiopathic, accounting for the majority of cases after exclusion of underlying causes, though specific rates for idiopathic forms vary across studies and are estimated around 50-60% in both species. In cats, the most common cause is idiopathic, accounting for over 50% of cases, particularly in Siamese and Himalayan breeds. Other main causes in cats include heart diseases such as cardiomyopathy, congestive heart failure, and pericardial thickening; tumors including thymoma, lymphoma, and masses compressing the thoracic duct; and trauma from accidents or falls from height. Rarer causes encompass fungal infections, heartworm disease, venous thrombi, congenital lymphatic defects, and complications following heart surgery. Common identifiable causes include trauma (such as blunt or penetrating injuries leading to thoracic duct rupture), neoplasia (particularly ), heartworm disease, cardiac disorders (e.g., congestive or ), and less frequently fungal infections or cranial vena cava . In contrast to cases, idiopathic chylothorax predominates in small animals, with secondary causes like neoplasia or trauma being more readily identifiable when present. Clinically, affected animals typically present with respiratory distress, including dyspnea manifested as rapid and labored , along with , reduced exercise tolerance, anorexia, and due to nutrient from chronic chyle loss. often reveals muffled heart and lung sounds, and in advanced cases, or collapse may occur. Diagnosis is confirmed through thoracocentesis, yielding a milky white, triglyceride-rich ; cytological analysis shows a lymphocytic predominance with high triglycerides (> serum levels) and low , distinguishing it from other pleural effusions. Complementary imaging, such as thoracic radiographs, , or CT lymphangiography, helps rule out underlying etiologies. Initial management involves therapeutic thoracocentesis to alleviate respiratory compromise, often repeated as needed, alongside supportive care like . Conservative treatments include a (MCT)-based to reduce production and supplements like (though efficacy is unproven). Pharmacologic options, such as (a analog), may be trialed to decrease lymphatic flow, particularly in or neonatal cases, but evidence in small animals remains limited. For persistent effusions unresponsive to medical therapy (typically after 5-7 days), surgical intervention is recommended, including ligation (TDL), often combined with partial pericardectomy to address potential lymphaticovenous anastomoses; success rates improve to 80-100% in dogs with this approach, though outcomes in cats are more variable at around 50%. Minimally invasive techniques like video-assisted thoracoscopic surgery (VATS) for TDL and ablation are increasingly utilized to enhance visualization and reduce complications. The prognosis is guarded, with overall mortality approaching 50% due to complications like progressive fibrosing pleuritis (a from chronic inflammation), recurrence of , or metabolic derangements. Short-term survival improves with prompt drainage and etiology-specific (e.g., anti-parasitics for heartworm), but long-term success depends on early surgical intervention in idiopathic cases, where medical management alone resolves only about 26% of instances. Cats generally fare worse than dogs, with higher rates of chronic pleural adhesions leading to persistent respiratory issues.

In Large Animals

Chylothorax is a rare condition in horses, characterized by the accumulation of in the pleural space, often resulting from disruption of the . While the is frequently idiopathic or unknown, traumatic causes are reported, including thoracic trauma from blunt injury or iatrogenic complications such as those associated with jugular or catheterization. Idiopathic cases are less common compared to traumatic presentations in this . Clinical signs typically include respiratory distress with rapid, and labored inhalation, weakness, and subcutaneous edema secondary to from chronic chyle loss. In other large animals, such as ruminants like , chylothorax is similarly uncommon and primarily idiopathic, though neoplastic causes including lymphosarcoma are documented, alongside traumatic etiologies like rupture during dystocia leading to rib fractures or vessel occlusion. Symptoms manifest as marked dyspnea with forceful inspiration, , coughing in chronic cases, and reduced lung sounds ventrally. involves thoracocentesis to confirm chylous , supported by and ultrasonography. Treatment emphasizes drainage via repeated thoracocentesis for symptomatic relief and supportive care including low-fat diets, to reduce production, antimicrobials, and anti-inflammatories; however, surgical interventions like ligation are challenging due to the complex, multi-branched anatomy of the in ruminants. The prognosis for chylothorax in large animals is generally guarded to poor, particularly in where chronic cases often necessitate due to progressive respiratory compromise, fibrosing pleuritis, and debilitation from nutrient losses. In ruminants, outcomes depend on addressing the underlying cause, but long-term survival is limited by anatomical constraints on and risks of secondary complications like . Recent advances, such as ablation combined with ligation, have shown promise in resolving chylothorax in veterinary patients, though application in large animals remains emerging and primarily extrapolated from small animal success.

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