Version July 2025
INTRODUCTION —
Pleural effusion occurs as a result of an abnormal fluid collection within the pleural space. Once a pleural effusion has been diagnosed in the neonate, management decisions are based on the effusion's effect on the respiratory status of the patient, which is primarily based on the size of the effusion and the cause and chronicity of the condition.
The management of chronic neonatal pleural effusions will be reviewed here. The etiology, presentation, and acute management of neonatal pleural effusions are discussed separately:
●(See "Approach to the neonate with pleural effusions".)
Pediatric parapneumonic effusions and empyema are discussed elsewhere:
●(See "Management and prognosis of parapneumonic effusion and empyema in children".)
PLEURAL FLUID REMOVAL
Initial serial aspiration — The management of recurrent and chronic effusions depends primarily on the rapidity of resolution over time, and as confirmed by imaging with ultrasound and/or plain radiographs, when the underlying cause is treated. In symptomatic neonates with ongoing effusions, needle aspiration (thoracocentesis) is repeated two or three times. If the effusion persists or recurs despite serial needle aspirations, placement of a chest tube or catheter (thoracostomy) is warranted, as discussed below (algorithm 1). (See 'Chest tube/catheter placed for persistent effusion' below.)
Most cases of neonatal pleural effusions are transient and do not need further intervention. Recurrent effusions caused by hydrops or other nonchylous etiologies can usually be treated with serial needle aspirations over a relatively brief period of time as the underlying condition is treated or spontaneously resolves. In contrast, chylous effusions typically persist and require chest tube or catheter placement.
Initial acute management of pleural effusions and thoracocentesis are discussed in detail separately. (See "Approach to the neonate with pleural effusions", section on 'Initial evaluation and acute management' and "Approach to the neonate with pleural effusions", section on 'Thoracocentesis'.)
Chest tube/catheter placed for persistent effusion — In the neonate with persistent pleural effusion(s) that continue to cause respiratory distress despite serial needle aspirations, we place an indwelling catheter for slow continuous drainage of fluid using an underwater seal system. In our center, we typically use pigtail catheters of 8.5 French or greater.
Placement of a chest tube(s) or catheter(s) with an underwater seal system can slowly drain pleural fluid on an ongoing basis, resulting in improved respiratory function [1]. In neonates, chest tubes in a size range of 10 to 12 French, or pigtail catheters of 8.5 French or greater should be used. We prefer pigtail catheters as they tend to remain in place longer. Alternatively, a larger bore (eg, 10 to 12 French) chest tube may be used if patency is an issue. (See "Thoracostomy tubes and catheters: Indications and tube selection in adults and children", section on 'Tube sizing'.)
Prior to performing the procedure, chest ultrasound or plain radiographs are obtained to confirm the size and location of the effusion. Bilateral chest tube/catheter placement may be necessary if the effusions are large on both sides.
Similar to thoracentesis, thoracostomy should be performed under sterile conditions, and involves the following steps:
●The neonate is placed in a supine position.
●Local anesthesia is administered.
●The tube is inserted in the midaxillary line in the 5th or 6th intercostal space and directed posteriorly. Chest ultrasound can be used to guide tube placement.
●The tube is sutured in place and covered with an airtight occlusive dressing. The distal end is then connected to a closed system calibrated suction device with 10 to 20 cm water negative pressure.
●Proper position of the tube should be verified with chest radiographs, including both PA and lateral views to ensure posterior positioning of the tube's tip. After initial chest radiographs confirm appropriate chest tube/catheter placement, radiographs are repeated only if there is increased respiratory distress or if a sudden decrease in output occurs.
●After initial fluid drainage, the volume of drainage should be measured. Monitoring and replacement of fluid losses is discussed in more detail below. (See 'Monitoring and replacing fluid losses' below.)
Online videos demonstrating this procedure are available; an example is Insertion of a Neonatal Chest Tube.
In neonates, persistent effusions are rarely due to infectious causes. The management of pediatric parapneumonic effusions and empyema, including the indications for chest tube/catheter placement, are discussed separately. (See "Management and prognosis of parapneumonic effusion and empyema in children", section on 'Chest tubes'.)
Thoracocentesis for the initial acute management of pleural effusion is discussed in more detail elsewhere. (See "Approach to the neonate with pleural effusions", section on 'Thoracocentesis'.)
MONITORING AND REPLACING FLUID LOSSES —
The electrolyte content of pleural effusions is similar to that of plasma. Thus, significant amounts of water and electrolytes (especially sodium) can be lost with repeated needle aspiration or persistent chest tube/catheter drainage. In some cases (eg, chylous effusions), significant protein losses can also occur. These losses should be monitored and repleted as necessary.
Monitoring and indications for replacement — We monitor chest tube/catheter output and hydration status frequently and replace fluids, electrolytes, and colloids based on the composition and volume of the fluid drainage (algorithm 1). With continuous chest tube/catheter drainage, daily assessments of weight and electrolytes are essential early on in management. The need for replacement of losses will depend on the magnitude of the volume losses and changes in serum electrolytes. After initial fluid drainage, the volume of drainage should be measured every six to eight hours. As the volume decreases, the measurement interval can be increased (eg, every 8 to 12 hours). Replacement of lost volume also depends on the neonate's daily fluid and electrolyte intake and daily output. Fluid and electrolyte therapy in newborns is discussed in more detail elsewhere. (See "Fluid and electrolyte therapy in newborns".)
Protein loss may also be significant depending on the composition of the pleural fluid. We determine replacement needs by measuring albumin and total protein levels once daily to start; measurement frequency can then be decreased as fluid output and protein losses diminish. In particular, chylous effusions can result in significant losses of proteins and cellular components (eg, albumin, immunoglobulins and lymphocytes), which may cause clinically significant immunosuppression. Thus, management of chylous effusions requires additional monitoring and replacement of other components, as discussed below. (See 'Additional replacement therapy' below.)
Analysis of pleural fluid composition is discussed in more detail elsewhere. (See "Approach to the neonate with pleural effusions", section on 'Pleural fluid analysis' and "Epidemiology, clinical presentation, and evaluation of parapneumonic effusion and empyema in children", section on 'Pleural fluid analysis'.)
Choice of replacement fluid — In most neonates, normal saline can be used to replace fluid losses. We administer normal saline if the chest tube/catheter output is greater than 6 mL/kg per hour and replace volume over one to two hours, every six to twelve hours. We also provide appropriate amounts of potassium, depending on the composition of the drainage and the neonate's serum electrolyte levels (eg, transudate versus chylous effusion).
For neonates with significant ongoing protein losses, we also use protein-containing replacement fluid (eg, albumin, fresh frozen plasma [FFP]) so that the neonate does not become hypoproteinemic. For neonates with nonchylous effusions (eg, generalized hydrops), we generally prefer FFP to albumin as it contains additional proteins and as albumin is most likely to be depleted from leaky capillaries. The management of protein losses in neonates with chylous effusions is discussed below. (See 'Protein depletion' below.)
The evaluation and management of neonatal hydrops is discussed in more detail elsewhere. (See "Nonimmune hydrops fetalis in the neonate: Causes, presentation, and overview of neonatal management" and "Alloimmune hemolytic disease of the newborn: Postnatal diagnosis and management", section on 'Early life-threatening anemia (hydrops fetalis)'.)
ADDITIONAL MANAGEMENT FOR CHYLOUS EFFUSIONS
Site of care — Chylous pleural effusions are the most frequent cause of chronic neonatal pleural effusions and can lead to prolonged drainage. Because of the complexity of management, neonates with chylothorax require care in tertiary centers with staff who have expertise in caring for this condition.
Management of neonates with chylous effusions is challenging. Pleural fluid drainage may cause significant losses of fluid and electrolytes, proteins (eg, albumin, coagulation factors, and immunoglobulins), and, in addition to humoral components, cellular elements of immunity (particularly lymphocytes) [2,3]. However, retrospective data from large case series have indicated that comprehensive management with ventilatory support and dietary management focused on decreasing thoracic duct lymph flow (eg, chylous fluid production) improves the outcome of neonates with chylothorax [4,5].
Additional replacement therapy
Protein depletion — Because chyle contains significant amounts of protein, ongoing drainage can lead to significant loss of (algorithm 1):
●Albumin – We follow serum albumin concentrations once or twice weekly and replace losses with 20 to 25 percent albumin 1 to 2 grams/kg to maintain levels above 2 to 2.5 g/dL.
●Coagulation factors – Coagulation factors, especially fibrinogen and factor VII, can be lost in clinically significant amounts. We measure PT/INR and PTT once or twice weekly. Although there are no specific guidelines, for neonates with significantly prolonged PT/INR or PTT (eg, >1.5 times the upper limit of normal), we provide replacement therapy with an intravenous (IV) infusion of fresh frozen plasma. We also monitor fibrinogen weekly and administer cryoprecipitate to replace abnormally low levels.
●Immunoglobulin – We monitor serum immunoglobulin G (IgG) levels on a weekly basis and begin replacement dosing with IVIG in neonates with severe hypogammaglobulinemia (eg, IgG <200 mg/dL). Initial dosing of IVIG for immune replacement is 400 to 600 mg/kg; we aim to maintain a target level of 500 mg/dL. The normal half-life of IgG is approximately three weeks; however, neonates with high rates of drainage may require additional dosing at shorter intervals to maintain the IgG level at or above the target level.
Ongoing chyle drainage results in a significant loss of immunoglobulins, which may result in severe hypogammaglobulinemia and risk for infection [6-8]. There are no published guidelines regarding management of secondary hypogammaglobulinemia due to chylous effusions, and retrospective data from small cases series have failed to establish the beneficial effects of intravenous immune globulin (IVIG) in patients with chylothorax [6-9]. Guidelines from the American Academy of Allergy, Asthma, and Immunology (AAAAI) on the management of immunoglobulin loss due to similar processes, including protein-losing enteropathies (PLE) following the Fontan procedure in patients with congenital heart disease, cite the lack of randomized trials evaluating IgG replacement therapy and note that infections in such patients are rare [10]. Others advise the use of IgG replacement only in patients who are actively fighting infections [11].
However, patients with post-Fontan PLE are usually at least two to five years of age and would be expected to have established some degree of immunity to common pathogens, and studies have reported that these patients are at increased risk for infections following heart transplant [12]. Additionally, older studies in animals have demonstrated that thoracic duct cannulation and drainage for as little as five days can produce a significant humoral and cellular immune defect [13].
Thus, we continue to provide IVIG to patients with persistent hypogammaglobulinemia until there is more definitive evidence of the lack of efficacy or harm. We target a trough level of 500 mg/dL, as this is the mean IgG level for a one-month-old infant and the replacement trough level used in the care of patients with primary immune deficiencies [14]. Additional details regarding the management of lymphocyte depletion and administration of IVIG is discussed elsewhere. (See 'Lymphocyte depletion' below and "Immune globulin therapy in inborn errors of immunity", section on 'Trough levels'.)
Lymphocyte depletion — Significant lymphocyte loss may occur in neonates with chronic chylous effusion and can result in profound cellular deficiency. Thus, we initiate measures (eg, dietary measures) to reduce thoracic lymph flow and reduce lymphocyte depletion, as discussed below (see 'Interventions to reduce chyle production' below). Additionally, we obtain a complete blood count (CBC) and T cell lymphocyte (CD4) levels on a weekly basis at minimum to assess for the degree of lymphopenia.
Culture-positive sepsis is a potential complication of lymphocyte depletion, as illustrated in a large case series of 178 infants with chylothorax [5]. In a smaller study of seven infants with congenital chylothorax, all infants had lymphopenia (median 12 days, range 4 to 39 days), and four infants developed bacterial nosocomial infections; none had fungal or viral infections [15]. In another report, one neonate with postsurgical chylothorax developed T cell lymphopenia severe enough to trigger the newborn screen for severe combined immunodeficiency [16]. Although use of antimicrobial prophylaxis with trimethoprim/sulfamethoxazole has been reported [15], we do not recommend routine antibiotic prophylaxis for neonates with chylothorax as risks outweigh potential benefits. Use of trimethoprim/sulfamethoxazole for prophylaxis against Pneumocystis pneumonia (PCP) may be indicated in patients with severe lymphopenia (eg, CD4 count <200 cells/microL).
Interventions to reduce chyle production
Dietary management for all patients — Dietary management is focused on decreasing thoracic duct lymph flow and includes the following (algorithm 1):
●Initial trial of enteral feeds – Although the optimal approach is controversial, most centers, including our own, advocate an initial noninvasive trial of a formula with a high concentration of medium-chain triglycerides. Most neonates respond with a decrease in chyle drainage (defined as a 25 percent reduction in drainage) and resolution of the effusion within one to two weeks.
The use of formula with a high concentration of MCT and a low concentration of long-chain fatty acids has been shown to be effective in decreasing chyle flow with resolution of chylous effusions in several observational studies [5,17-20]. Fat-free human milk produced by centrifugation and supplemented with MCT or total parenteral nutrition (TPN) has also been reported to be successful in the management of both congenital and postoperative chylothorax [21]. The mechanism of decreased chyle flow appears to be due to MCT being directly absorbed into the portal vein system, thereby bypassing the lymphatic system and resulting in a reduction in the volume and lipid concentration of the pleural fluid [22].
●Nonresponse to enteral feeds – In neonates who cannot tolerate enteral feeds or who continue to have chyle drainage for more than a week while on enteral feeds, we withhold feeds (nothing by mouth) and initiate total parenteral nutrition (TPN) [18,23]. We resume enteral feeds once chyle drainage decreases by 25 percent based on daily assessments or once the neonate can tolerate feeds, as discussed above.
Enteral feeds stimulate thoracic duct lymph flow; thus withholding feeds (nothing by mouth) and administering TPN may decrease chylous flow in patients who fail to respond to MCT [18,19]. However, we prefer to resume enteral feeds due to the long-term complications of TPN, including cholestasis and central line infections, if possible.
●Dietary management after resolution of effusion – For neonates responsive to dietary measures, chest tube(s)/catheter(s) are removed after the drainage ceases. Once the chest tube/catheter is removed, we continue the same dietary management and monitor the neonate for an additional one to two weeks. We then initiate feeds with maternal or donor human milk. If human milk is not available, a standard formula is used. Indications for and monitoring after chest tube/catheter removal are discussed in more detail below. (See 'Chest tube/catheter removal' below.)
●Persistent effusion after dietary management – For neonates with ongoing chylous drainage despite two or more weeks of withholding feeds, pharmacotherapy may be considered, as discussed below. (See 'Octreotide for selected cases' below.)
Although the optimal approach is controversial, most centers advocate for enteral formula feeds with a high concentration of medium-chain triglycerides. Based on retrospective data from large case series, comprehensive management with ventilatory support, and dietary management focused on decreasing thoracic duct lymph flow (parenteral nutrition and the use of medium-chain triglycerides [MCT]) have improved the outcome of neonates with chylothorax [4,5].
Octreotide for selected cases — Octreotide is a synthetic analogue of somatostatin, a regulatory hormone that reduces intestinal blood flow and can decrease the production rate of chyle. We do not routinely use octreotide in neonates with chylothorax because it has significant adverse effects, and data remain uncertain about its benefit for chronic neonatal chylothorax. However, for neonates with chylothorax refractory to dietary management, we cautiously use octreotide as follows (algorithm 1):
●Initiation – For neonates who fail to respond to dietary measures within one to two weeks and have continued chest tube/catheter output, we administer octreotide after a thorough discussion with the neonate's parent(s) or legal guardian regarding the risks and uncertainty of benefits. If given, octreotide can be administered either subcutaneously or intravenously. We prefer a continuous intravenous infusion of octreotide starting at an initial dose of 1 mcg/kg per hour. During octreotide administration, we continue to withhold feeds and administer TPN because of concerns with splanchnic ischemia and necrotizing enterocolitis [24].
●Dose titration and maintenance in responders – The dose is increased by 1 mcg/kg per hour once daily until a response is noted or the infusion rate reaches a maximum of 10 mcg/kg per hour. A positive response is considered to be at least a 25 percent reduction in chest tube/catheter output.
Once a response is obtained, we usually continue infusion at the same rate for 7 to 10 days before beginning a slow taper over another week. If chest tube/catheter drainage increases after the initial response (eg, during the taper), we increase the octreotide infusion rate and maintain it for at least another week before tapering and discontinuation.
If intravenous access is lost, octreotide can be given subcutaneously at the same dose at six- to eight-hour intervals until intravenous access is restored.
Following resolution of effusion and discontinuation of octreotide, we monitor the neonate for an additional two days before removing the chest tube/catheter and resuming enteral feeds with maternal or donor human milk or a standard formula (if human milk is not available). Indications for and monitoring after chest tube/catheter removal are discussed in more detail below. (See 'Chest tube/catheter removal' below.)
●Glucose monitoring – Because octreotide blunts insulin release, close monitoring of serum glucose values should be performed during the induction phase with spacing of measurements as a stable infusion rate is achieved. We obtain point of care glucose measurements every six hours to start and measure less frequently once glucose levels stabilize.
●Discontinuation for nonresponse – If maximal octreotide dosing does not reduce chest tube/catheter output after two or three days, we rapidly taper octreotide over a day or two and assess other options for intervention (eg, chemical pleurodesis, surgical or other intervention). (See 'Limited role for other interventions' below and 'Options for refractory cases' below.)
Evidence on the benefits of octreotide for chronic neonatal chylothorax that is resistant to dietary management is limited. In neonates, there are no clinical trials studying the effect of octreotide in the treatment of chylothorax. Evidence is limited to case reports, and data from observational studies are contradictory [5,25-27].
In a systematic review of 138 infants with congenital chylothorax who were treated with octreotide, approximately 78 percent had resolution of chylothorax without the need for surgical intervention [26]. An earlier systematic review reported that octreotide resulted in a decrease or cessation of chylous drainage in approximately 50 percent of cases [25]. A small case series reported similar benefit in pediatric patients with refractory chylothorax following surgery for congenital heart disease [27]. Octreotide has also been reported to be beneficial in the treatment of chronic chylothorax in adults. (See "Management of chylothorax", section on 'Somatostatin and octreotide'.)
In contrast, in a large case series that included 172 infants who were initially managed medically with chest tube drainage and nutritional measures, the addition of octreotide (in 45 infants) was not associated with additional benefit [5].
Adverse effects of octreotide have been reported in approximately 14 percent of neonates treated for chylothorax [25]. Significant complications of octreotide for patients with chylothorax and other conditions (eg, congenital hyperinsulinism and enterocutaneous fistulas) have been reported, including transient hypothyroidism, persistent pulmonary hypertension of the newborn (PPHN), and necrotizing enterocolitis (NEC) [15,28-33].
Limited role for other interventions — Other interventions have been used in neonates with chylothorax; however, these interventions should not be used routinely, as the evidence of demonstrated benefit is inadequate. They should only be considered on a case-by-case basis for neonates that fail to respond to routine management or octreotide as potential alternatives to pleurodesis or surgery. These include:
●Procedural interventions
•High-frequency ventilation and high end-expiratory pressure – High-frequency ventilation [34] and high end-expiratory pressure [35] have been reported in small case series to be beneficial in the management of infants with chylothorax who are receiving mechanical ventilation.
•Interventional cardiac catheterization – Chylothorax resulting from occlusion or stenosis of the left innominate vein may be successfully remedied by balloon dilation in the cardiac catheterization laboratory [36].
•Embolization – Case reports suggest that congenital lymphatic diseases may be treated by embolization via interventional radiology [37-39].
●Pharmacologic interventions
•Propranolol – A limited number of case reports suggest that propranolol may be effective in treating chylothorax and lymphangiectasia [40-43]. There are also case reports of prenatally administered propranolol for fetal chylothorax [43]. Evidence is insufficient to recommend its use.
•Glucocorticoids – Comparative studies and case reports suggest that glucocorticoid therapy may be helpful in the management of postoperative chylothorax [44,45].
•Inhaled nitric oxide – A single case report noted a reduction in chyle flow with inhaled nitric oxide (iNO) in an infant with postoperative chylothorax and pulmonary hypertension [46].
•Etilefrine – A report of two cases noted a significant reduction of chyle output after starting treatment with continuous infusion of etilefrine, a sympathomimetic agent with both alpha and beta adrenergic stimulation [47]. Both heart rate and blood pressure increased during the infusion but returned to basal values after etilefrine was discontinued.
•Sildenafil – A single case report indicated successful treatment of an octreotide-resistant congenital chylothorax with the phosphodiesterase inhibitor sildenafil [48].
•Sirolimus – Sirolimus has been successfully tested in a phase II trial for complicated lymphatic and vascular anomalies in infants, children, and young adults [49]. A case series reported successful treatment of infants with lymphatic malformations and chylothorax by using sirolimus [50].
CHEST TUBE/CATHETER REMOVAL —
We remove the chest tube/catheter once drainage has ceased and chest radiographs confirm resolution of effusion. We continue to monitor the neonate for at least one week for recurrence of a pleural effusion. For neonates with resolved chylous drainage, we initiate feedings with human milk or standard formula, as discussed above. (See 'Dietary management for all patients' above and 'Octreotide for selected cases' above.)
OPTIONS FOR REFRACTORY CASES
Surgical management — Neonates who continue to have chronic pleural effusions despite all attempts at medical therapy may require surgical intervention. Discussion of these options, which include mechanical pleurodesis, pleuroperitoneal shunt [51-53], and ligation of the thoracic duct for those with chylothorax [17,18,54-56], occur in conjunction with pediatric surgery consultation and is beyond the scope of this review.
Pleurodesis — Pleurodesis is a procedure that obliterates the pleural space to prevent a recurrent pleural effusion following pleural drainage. After draining the effusion, a chemical irritant that induces inflammation and fibrosis is instilled into the pleural space (ie, chemical pleurodesis). Various agents have been used for chemical pleurodesis. Many of these, especially chemotherapeutic agents, are contraindicated in neonates and young infants because of potential toxicities. (See "Chemical pleurodesis for the prevention of recurrent pleural effusion".)
The decision to use pleurodesis should be made by knowledgeable clinicians (eg, pediatric surgeons) on a case-by-case basis as use of these agents requires further study to determine their efficacy and safety in the treatment of neonatal pleural effusions. As a result, these interventions cannot be recommended for routine use. However, an infant with persistent unilateral chylous effusion was successfully managed with pleurodesis using doxycycline at our center.
Data in infants are limited and include case reports, primarily in patients with persistent chylous effusions, using the following agents:
●Talc [57]
●Iodopovidone [58-61]
●Tetracycline derivatives (doxycycline) [60,62]
●Fibrin glue [54,56,63-67]
●Streptococcus pyogenes A3 (OK-432) [68,69]
DISCHARGE FOLLOW-UP —
Neonates with resolved pleural effusion require close follow-up after discharge to monitor clinical symptoms and for other care needs. Signs of respiratory distress during the outpatient follow-up visit should prompt evaluation with chest radiographs to assess for recurrent effusion.
In neonates with chylothorax, long-term follow-up depends on how rapidly T cell lymphocyte and immunoglobulin levels recover. These levels typically normalize over a period of months after thoracic duct leakage has ceased. Neonates whose effusion has resolved and who have recovered T cell lymphocytes and immunoglobulins to normal or near normal concentrations by the time of discharge do not require further follow-up beyond the usual discharge management based on their neonatal intensive care hospitalization. Those with persistent abnormalities in their T cell lymphocyte and immunoglobulin levels should be referred to an immunology specialist for ongoing evaluation until their laboratory results are sufficiently reassuring.
Discharge planning and outpatient management of high-risk neonates are discussed elsewhere. (See "Discharge planning for high-risk newborns" and "Care of the neonatal intensive care unit graduate".)
SUMMARY AND RECOMMENDATIONS
●Pleural fluid removal – For neonates with symptomatic pleural effusions, needle aspiration (thoracocentesis) is the initial approach to fluid drainage. (See "Approach to the neonate with pleural effusions", section on 'Thoracocentesis'.)
Chronic pleural effusions in the neonate are defined as those that require repeated needle aspirations to relieve respiratory distress. For neonates with persistent effusion and respiratory distress despite serial needle aspirations, an indwelling pleural catheter or tube (thoracostomy) is generally necessary for drainage. (See 'Pleural fluid removal' above.)
●Monitoring and replacing losses – We closely monitor total input and output (including from the pleural catheter/tube), weight, hydration status, and serum electrolyte, albumin, and protein levels. These inform the need for fluid, electrolyte, and protein replacement (algorithm 1). (See 'Monitoring and replacing fluid losses' above and "Fluid and electrolyte therapy in newborns".)
●Additional management of chylous effusion – Chylous drainage typically persists and requires additional management of losses. Neonates with chylothorax require care in tertiary centers with staff who have expertise in caring for this condition. Additional management includes (algorithm 1) (see 'Additional management for chylous effusions' above):
•Replacing additional losses – We also check albumin and coagulation studies (eg, PT/INR, PTT, fibrinogen) once or twice weekly, and immunoglobulins and complete cell counts (ie, complete blood count [CBC], T cell lymphocyte [CD4] levels) once weekly. We replace deficits as follows:
-Albumin – For neonates with hypoalbuminemia, we suggest intravenous (IV) albumin infusion rather than other protein containing fluid (Grade 2C). We administer 20 to 25 percent albumin at a dose of 1 to 2 g/kg to maintain serum albumin concentrations above 2 to 2.5 g/dL. (See 'Protein depletion' above.)
-Coagulation factors – For neonates with prolonged PT/INR or PTT (eg, >1.5 times the upper limit of normal), we suggest administering an IV infusion of fresh frozen plasma (Grade 2C). Cryoprecipitate may also be used to replace abnormally low fibrinogen levels. (See 'Protein depletion' above.)
-Immunoglobulins – For neonates with severe hypogammaglobulinemia (eg, IgG <200 mg/dL), we suggest replacement with intravenous immune globulin (IVIG) (Grade 2C). The initial dosing of IVIG for immune replacement is 400 to 600 mg/kg; we aim to maintain a target level of 500 mg/dL. (See 'Protein depletion' above.)
-Lymphocytes – Lymphocyte depletion is managed by reducing chylous drainage, as discussed below.
•Reducing chylous drainage – Our approach is as follows (see 'Interventions to reduce chyle production' above):
-Dietary management – We suggest an initial trial of enteral feeds with formula containing a high concentration of medium-chain triglycerides (MCT) and a low concentration of long-chain fatty acids (Grade 2C). (See 'Dietary management for all patients' above.)
For neonates with continued chylous drainage despite a trial of MCT enriched formula or those who cannot tolerate enteral feeds, we suggest withholding feeds and initiating total parenteral nutrition (TPN) (Grade 2C). (See 'Dietary management for all patients' above.)
-Selective use of octreotide – For neonates who do not respond to a one- to two-week trial of withholding feeds, we suggest octreotide rather than other pharmacologic or procedural interventions (Grade 2C). The decision to use octreotide should be made on a case-by-case basis because of the risk of adverse events and uncertain benefit. (See 'Octreotide for selected cases' above and 'Limited role for other interventions' above.)
●Options for refractory cases – Neonates with persistent pleural effusions despite maximal medical therapy may require chemical pleurodesis or surgical interventions (ie, mechanical pleurodesis, pleuroperitoneal shunt, ligation of the thoracic duct for those with chylothorax). For refractory chylothorax, other pharmacologic and procedural interventions have been used for neonates as potential alternatives to pleurodesis or surgery but have a more limited role. (See 'Options for refractory cases' above and 'Limited role for other interventions' above.)
●Follow-up – After chest tube/catheter removal, we continue to monitor the neonate for at least one week for recurrence of a pleural effusion. Neonates with resolved pleural effusion require close follow-up after discharge to monitor clinical symptoms and for other care needs. Signs of respiratory distress during the outpatient follow-up visit should prompt evaluation with chest radiographs to assess for recurrent effusion. Neonates with chylothorax and persistent abnormalities in their T cell lymphocyte and immunoglobulin levels should be referred to an immunology specialist for ongoing evaluation. (See 'Chest tube/catheter removal' above and 'Discharge Follow-Up' above.)
