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Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 233-237

Lymphatic Malformation Presenting as Recurrent Pleural Effusion and Ascites

Department of Pulmonology, Institute of Child Health and Hospitals for Children, Chennai, Tamil Nadu, India

Date of Submission12-Jul-2022
Date of Decision31-Oct-2022
Date of Acceptance01-Nov-2022
Date of Web Publication29-Nov-2022

Correspondence Address:
Dr. Sarath Balaji
Department of Pulmonology, Institute of Child Health and Hospitals for Children, Chennai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ipcares.ipcares_172_22

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Background: Chylothorax is defined as the accumulation of chyle in the pleural space and is characterized by elevated triglyceride content in pleural fluid >110 mg/dl. Chylous ascites is the accumulation of chyle in peritoneal fluid characterized by triglyceride levels >200 mg/dL. Causes can be traumatic or nontraumatic; portal or nonportal; and congenital or acquired (inflammatory, postoperative, malignant, or infectious). Lymph duct abnormalities are a rare cause of concurrent occurrence of chylothorax with chylous ascites. Clinical Description: We report a 4½-year-old girl who initially presented a fever and was diagnosed to have right-sided pleural effusion. Since no other focus was identified, she was treated as a case of tubercular pleural effusion, following which her symptoms settled transiently. She thereafter had a recurrence of her symptoms in association with ascites and was managed as a case of relapse of tuberculosis (TB) with category 2 anti-tubercular therapy. Since her symptoms did not resolve, she was referred to our hospital for further management. Management: Clinical reasoning based on history and examination coupled with investigations ruled out cardiac, renal, hepatic, malabsorption, or nutritional pathologies of recurrent effusions. TB was ruled out. A lymphatic malformation was suspected. Therapeutic cum diagnostic paracentesis was done in a fed state, which indicated a chylous nature by the milky appearance and suggestive cytology and biochemistry. Lymphoscintigraphy confirmed the presence of a lymphatic duct abnormality. The child was managed with diet modifications, following which she improved within a week. Conclusions: The approach to recurrent effusions without any focus should be logical and sequential, as described above, to exclude the aforementioned common conditions. If workup for TB is repeatedly negative other less likely causes should be considered.

Keywords: Chylothorax, chylous ascites, lymphangiectasia

How to cite this article:
Balaji S, Anuradha D, Shankaran S, Gunasekar V. Lymphatic Malformation Presenting as Recurrent Pleural Effusion and Ascites. Indian Pediatr Case Rep 2022;2:233-7

How to cite this URL:
Balaji S, Anuradha D, Shankaran S, Gunasekar V. Lymphatic Malformation Presenting as Recurrent Pleural Effusion and Ascites. Indian Pediatr Case Rep [serial online] 2022 [cited 2023 Jan 30];2:233-7. Available from: http://www.ipcares.org/text.asp?2022/2/4/233/362240

The concurrent occurrence of chylous ascites (CA) with chylothorax is rare in children, as well as the general population.[1] A tertiary care center reported an incidence of 1 in 20,000 admissions, inclusive of adults and children over a period of 20 years.[2] Congenital CA is associated with Klippel-Trenaunay and Yellow-Nail syndrome. The secondary causes of CA are heterogeneous and include lymphatic duct obstruction, chyle leakage through a lymphoperitoneal fistula, malignancy, postoperative complications, radiation, infections (i.e., tuberculosis (TB), filariasis), pancreatitis, sarcoidosis, pericarditis, blunt injury to the abdomen, hepatic cirrhosis, drugs (calcium channel blockers, sirolimus), portal vein thrombosis, and nephrotic syndrome.[3]

Loss of chyle results in significant loss of protein and fat with resultant manifestations such as edema (hypoproteinemia), steatorrhea (impaired lymphatic drainage leads to leakage of lymph into the intestine and malabsorption), asthenia, tetany (loss of calcium in the lymph fluid), and an immunosuppressed state (loss of lymphocytes and hypogammaglobinemia).[3]

We report a case of CA with chylothorax who presented with recurrent episodes of unexplained pleural effusion and abdominal distention. We hope to sensitize clinicians to consider the possibility of uncommon conditions like lymphatic abnormalities when working looking for the cause of unexplained recurrent pleural effusion in a child.

  Clinical Description Top

A 4.5-year-old girl was referred to us as a diagnostic dilemma with recurrent episodes of breathlessness and abdominal distention for a year. These episodes had not responded to treatment being given at the referral and other centers where she had sought medical attention.

On eliciting history and reviewing whatever medical records she had, we learned that she had been apparently well until 3.5 years of age when she had experienced the first episode of respiratory symptoms. This had developed after a fever for 2 weeks' duration. The child had minimal breathing difficulty that did not interfere with her activities of daily living. There was no history of cough, coryza, or chest retractions. There were no other localizing or constitutional symptoms. The child was immunized for age, and there was no history of contact with TB.

We were unable to find any medical records of earlier examination findings during that period. However, some basic investigations for the workup of fever were available. Salient results were minimal leukocytosis (total Leucocyte count 14,200 cells/cu.mm) with Equal predominance of neutrophils and lymphocytes (45%). Platelet counts were normal (3.5 Lakhs/cumm). The C-reactive protein was elevated (42 mg/dL), and the blood culture was sterile. The chest X-ray showed right-sided pleural effusion. The Mantoux test was negative, as were the cartridge-based nucleic acid amplification tests (CB-NAAT) performed on pleural fluid and gastric aspirates (both of which were exudative as per cytology and biochemistry). She was initially managed with a short course of drugs (nature unknown), following which she was empirically started on anti-tubercular therapy (ATT) and discharged. Defervescence and resolution of right-sided effusion on a repeat X-ray were taken as a successful therapeutic trial, and she completed a course of category 1 ATT.

Regarding the current episode, she had been symptomatic for around 3 months. Abdominal distention developed 3–4 months after completing the course of ATT, followed by a recurrence of breathlessness for a month. The abdominal distention was insidious in onset and had progressively increased over 3 months. It was painless and not associated with fever, jaundice, increasing pallor, bleeding from any site, vomiting, recurrent diarrhea, greasy stools, or change in bowel habits, oliguria, or other urinary complaints. There was no history of edema of her feet or elsewhere. Fast breathing had appeared for the past month that increased in the supine and left lateral positions but displayed minimal change with physical activity or exercise. There was no history of chest pain, productive cough, or syncopal attacks. There had not been any loss of weight, though her appetite had decreased (daily intake of 1200 calories and 20 g of protein). There was no history of oral ulcers, skin rashes, or joint swellings. The child had not been exposed to any abdominal trauma or surgery in the past. She was developmentally normal and the second-born issue of nonconsanguineous parents. There was no family history of similar problems. The referral center had repeated investigations more or less on the lines of the previous approach, but was unable to find anything conclusive. A provisional diagnosis of relapse of abdominal TB was made, and she was started on category 2 ATT. The child was referred to us when she remained symptomatic despite medication.

We proceeded with the examination. She was alert and active. Vital parameters were stable; afebrile, heart rate 104/min, respiratory rate 32/min, and blood pressure 94/70 mm Hg (below 90th percentile). The weight was 16 kg (Z score 0 to −2), height 102 cm (Z score 0 to −2), and body mass index (BMI) was 15.38 (z score between 0 to +1). Pallor was present. No edema was noted in the periorbital region, sacrum, or feet. There was no icterus, clubbing, cyanosis, lymphadenopathy, petechiae, purpura, rashes, or signs of vitamin deficiencies. A Bacillus Calmette–Guérin scar was present. No abnormalities were noted in the oral cavity, skin, or joints. The respiratory system examination showed decreased air entry over the right hemithorax with stony dullness indicative of pleural effusion. Air entry was equal and vesicular in character in other areas. Abdomen examination showed a distended abdomen. There was no tenderness, guarding, or rigidity. Shifting dullness was present. The liver was mildly enlarged with a span of 10 cm. There was no other organomegaly. The cardiovascular and neurological examinations were within the normal limits.

Management and outcome

We reviewed the clinical details to ascertain the etiology of the pleural and ascitic fluid collection. The points in favor of disseminated TB (abdominal and pleural) were the prolonged but relatively indolent course of illness, initial symptomatic relief on starting ATT, presence of straw-colored pleural and ascitic fluid with an exudative nature but the absence of frank pus. However, the absence of prolonged fever, respiratory findings other than that of effusion, loss of weight, and history of contact, in the setting of negative Mantoux test (normal BMI) and CB-NAAT were against this diagnosis. Other less likely differentials were an underlying anicteric hitherto undetected liver disease, nephrotic syndrome, congestive cardiac failure, or malabsorption. However, there were no strong clinical indicators for any of these. Autoimmune disorders were also considered, but the absence of fever, joint involvement, or rashes made this possibility unlikely.

Preliminary routine tests on admission were normal [Table 1]. Routine urinalysis (including urine protein – creatinine ratio 0.2) was within the normal limits. The blood and urine cultures were sterile. The repeat Mantoux test was negative. The X-ray chest and abdomen [Figure 1] did not show any new findings. Computed tomography (CT) of the chest showed massive right-sided pleural effusion with minimal left-sided effusion and basal atelectasis. The echocardiogram was normal. Hence, cardiac, renal, hepatic, malabsorption, or nutritional pathologies were ruled out.
Table 1: Summary of preliminary investigations done at our centre (Institute of Child Health and Research Centre)

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Figure 1: Right-sided pleural effusion with ascites on chest X-ray

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Since the child was afebrile, not sick, and without any other systemic manifestations despite recurrent effusions, we reviewed other possible, more uncommon causes and suspected a lymphatic malformation. Therapeutic cum diagnostic thoracic and ascitic paracentesis was performed; however, this time, it was performed in a fed state. The pleural fluid had a milky appearance. Its cytology revealed total cell counts of 2000 cells/cu.mm, of which 80% were lymphocytes. Biochemistry showed high protein (3.5 g/dL, normal 1–2 g/dL) and normal lactate dehydrogenase levels (235 IU/L, normal 100–333 IU/L). The presence of elevated pleural fluid triglyceride levels (180 mg/dL, normal <110 mg/dL) confirmed chylothorax. Ascitic fluid analysis was also suggestive of CA with high protein (3 g/dL; serum albumin: ascitic gradient <1.1 g/dL) and triglycerides (245 mg/dL, normal <200 mg/dL) levels. The cultures of Pleural and Ascitic fluid cultures were sterile. The samples were also re-investigated for TB (Tuberculosis) via CBNAAT procedure and were found to be negative: Adenosine deaminase (ADA) levels normal (pleural fluid 28 IU/L, >40 U/L indicative of TB; ascitic Fluid 30 IU/L, >36 U/L indicative of TB) and CB-NAAT negative.

A diagnostic lymphoscintigraphy was planned to rule out lymphatic abnormalities, the most common cause of chylous effusions. This showed no evidence of lymph flow from the site of injection through superficial lymphatics and diversion through deep lymphatics [Figure 2]. Tracer uptake was seen in the abdomen and urinary bladder, suggestive of microscopic lympho-venal shunts at multiple levels giving rise to CA. The urine microscopy was repeated but did not show any abnormality, indicative that the extravasation was minimal. We decided against testing it for triglyceride and chylomicron levels (that would have been confirmatory) as these specialized tests would have increased the cost without changing the management. The child was diagnosed with a case of CA with chylothorax due to lymph duct abnormalities, probably of congenital origin. She was started on a low-fat and medium chain triglycerides (MCT) rich diet.
Figure 2: Lymphoscintigraphy showing no tracer uptake from right lower limb, normal uptake from left lower limb

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Symptomatic improvement began gradually, and by 2 weeks, the pleural effusion and ascites had resolved. During the hospital stay, the serum albumin showed a declining trend (from 3.9 g/dL at admission to 3.6 and 3.2 g/dL over a week), probably due to minimal continuity of chylous leak. However, since she was otherwise asymptomatic, no specific treatment was planned. At discharge, we planned to consider injection of octreotide in case of recurrence of severe ascites or severe breathlessness, as per standard protocol. However, 3 months have elapsed in follow-up, and she remains symptom-free.

  Discussion Top

Chylous ascites and chylothorax is noninfectious extravasation of a milky fluid into the respective body cavities, draining ≥200 mL/day, and has triglyceride levels of ≥110 mg/dL.[1] The etiology of CA can be classified as follows:[4],[5],[6],[7],[8] traumatic or nontraumatic; portal or nonportal (based on the presence or absence of diseases affecting the portal system pressure); and congenital or acquired (inflammatory, postoperative, malignant, or infectious). Abdominal malignancy and cirrhosis are the common causes in developed countries, whereas the most causes are infectious diseases such as TB and filariasis in developing countries. A review of causes of CA listed lymphatic anomalies as the most common (32%), followed by cirrhosis (11%), mycobacterial infections (10%), and malignancy (7%).[5] The simultaneous occurrence of CA with chylothorax is a rare association above infancy. Similar cases are summarized in [Table 2].[9],[10],[11]
Table 2: Literature review and their follow up

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The diagnosis of chylothorax is made by abdominal paracentesis/pleural analysis. Pleural fluid analysis in the fed state was the game changer in this case. If the patient is fed orally, the fluid has a milky appearance that otherwise appears straw colored (the probable reason for it being mistaken as tubercular in this case). Sometimes, the fluid may appear cloudy on enteral feeding. Chylous fluid is characterized by cell count of more than 500/μL (mostly lymphocytes), proteins between 2.5 and 4 g/dL, and triglycerides >110 mg/dL, with the predominance of chylomicrons. Ultrasonogram, CT, or magnetic resonance imaging of the thorax is indicated to rule out malignant (lymphoma, chronic lymphoid leukemia, and mediastinal tumors) or pre-operatively in surgical causes (i.e., tumor resection, thoracic aneurysm repair, or sympathectomy). If these investigations are not contributory, malformations of the lymphatics should be considered. The investigative modality of choice is lymphoscintigraphy[6] which involves the injection of 99mTc-dextran, sulfur colloid or human albumin into the interdigital web spaces of the feet. Laparoscopy allows to explore a magnified image of the abdominal cavity in its natural state.[6]

The treatment is primarily conservative when no surgical cause is identified. The protocol depicted in [Figure 3] may be adapted for the clinical approach of such patients.[12] Chronic loss of chyle leads to anemia, hypoproteinemia, hypocalcemia, hypolipidemia, malnutrition, and an immunocompromised state, all of which are managed symptomatically as per individual protocols, keeping the underlying cause in mind. Repeated paracentesis is required to relieve symptoms. Our patient did not require repeated paracentesis since she became asymptomatic and remained the same after a single paracentesis. The primary goal is to maintain nutrition by initiating a low-fat, high-protein diet with MCT; and decreasing the production and flow of chyle. Around 50% of cases improve by this management.[7] In refractory or persistent chylothorax (absence of defervescence and resolution of symptoms within 2 weeks of starting treatment), administration of somatostatin or its analog octreotide may be considered.[7] These drugs decrease the gastric, pancreatic, and biliary secretions and reduce the total flow of gastric lymphatics, thereby reducing the effusions.[8] If there is no improvement within a year, surgical treatment is preferred. This includes thoracentesis, pleurocentesis, thoracic duct ligation, or embolization.[8]
Figure 3: Algorithm for management and follow-up of patients with chylous effusions

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To conclude, clinicians must proactively consider and exclude causes other than TB in cases of pleural effusion or ascites (even if it straw colored), if the clinical phenotype is not supported by investigations.

Informed consent

An informed consent for publication was obtained from the child's parents and has been attached to the manuscript.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kaas R, Rustman LD, Zoetmulder FA. Chylous ascites after oncological abdominal surgery: Incidence and treatment. Eur J Surg Oncol 2001;27:187-9.  Back to cited text no. 1
Press OW, Press NO, Kaufman SD. Evaluation and management of chylous ascites. Ann Intern Med 1982;96:358-64.  Back to cited text no. 2
Al-Busafi SA, Ghali P, Deschênes M, et al. Chylous ascites: Evaluation and management. ISRN Hepatol 2014;2014:240473.  Back to cited text no. 3
Browse NL, Wilson NM, Russo F, et al. Aetiology and treatment of chylous ascites. Br J Surg 1992;79:1145-50.  Back to cited text no. 4
Steinemann DC, Dindo D, Clavien PA, et al. Atraumatic chylous ascites: Systematic review on symptoms and causes. J Am Coll Surg 2011;212:899-905.e1.  Back to cited text no. 5
Noel AA, Gloviczki P, Bender CE, et al. Treatment of symptomatic primary chylous disorders. J Vasc Surg 2001;34:785-91.  Back to cited text no. 6
Campisi C, Bellini C, Eretta C, et al. Diagnosis and management of primary chylous ascites. J Vasc Surg 2006;43:1244-8.  Back to cited text no. 7
Huang Q, Jiang ZW, Jiang J, et al. Chylous ascites: Treated with total parenteral nutrition and somatostatin. World J Gastroenterol 2004;10:2588-91.  Back to cited text no. 8
Rützel S, Gebauer C, Pulzer F, Steinhoff KG, Thome U, Knupfer M. Cessation of severe chylothorax and chylous ascites in a newborn with trisomy 21 after whole blood pleurodesis: A case report. Pediatr Dimens 2017;2:1-3. [doi: 10.15761/PD.1000148].  Back to cited text no. 9
Bellini C, Ergaz Z, Radicioni M, et al. Congenital fetal and neonatal visceral chylous effusions: Neonatal chylothorax and chylous ascites revisited. A multicenter retrospective study. Lymphology 2012;45:91-102.  Back to cited text no. 10
George J, Kanaparthi S, Aroor SA, et al. Primary chylous ascites in children: Rare cause of a common presentation. Sri Lanka J Child Health 2019;48:359-60.  Back to cited text no. 11
Bhardwaj R, Vaziri H, Gautam A, et al. Chylous ascites: A review of pathogenesis, diagnosis and treatment. J Clin Transl Hepatol 2018;6:105-13.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1], [Table 2]


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