|Year : 2017 | Volume
| Issue : 4 | Page : 691-694
Chylothorax in children with cancer: A milky predicament
Sidharth Totadri1, Amita Trehan1, Anish Bhattacharya2, Deepak Bansal1, Savita Verma Attri3, Radhika Srinivasan4
1 Department of Pediatrics, Pediatric Hematology-Oncology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Nuclear Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Pediatrics, Pediatric Biochemistry, Postgraduate Institute of Medical Education and Research, Chandigarh, India
4 Department of Cytology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||30-Jul-2018|
Dr. Amita Trehan
Department of Pediatrics, Pediatric Hematology-Oncology, Postgraduate Institute of Medical Education and Research, Chandigarh
Source of Support: None, Conflict of Interest: None
Chylothorax is an uncommon complication in children. Although surgery and trauma are the most common causes encountered, hematological as well as solid malignancies can present with chylothorax. This study aimed to describe the presentation and management of malignant chylothorax in children. This is a case series from a pediatric hematology-oncology unit. Chylothorax was diagnosed by demonstrating high triglyceride content in the pleural fluid and a low cholesterol concentration in relation to the serum cholesterol. Cytology for malignant cells and investigations for tuberculosis were performed in all patients. Initial management included placement of an intercostal tube and administration of a fat-free diet with the addition of medium-chain triglycerides. Appropriate treatment of the underlying malignancy was initiated simultaneously. Three children with diagnoses of Stage IV neuroblastoma, lymphoblastic lymphoma, and Hodgkin lymphoma developed chylothorax. Malignant cytology was positive in the patient with T-NHL. All patients were found to have associated hypoproteinemia and hypoalbuminemia. The chylothorax resolved with conservative measures in two patients. It remained intractable in the child with T-NHL, in whom the lymphoma was refractory to chemotherapy. Chylothorax is a rare but challenging complication that can accompany childhood malignancies. Surgical interventions, radiotherapy, and pleurodesis are alternatives if the chylothorax is refractory to medical management.
Keywords: Hypogammaglobulinemia, lymphoscintigraphy, massive pleural effusion, milky effusion, pediatric malignancy
|How to cite this article:|
Totadri S, Trehan A, Bhattacharya A, Bansal D, Attri SV, Srinivasan R. Chylothorax in children with cancer: A milky predicament. Indian J Cancer 2017;54:691-4
| » Introduction|| |
Chylothorax is an infrequent cause of pleural effusion in children. Chyle accumulates in the pleural cavity following injury to the thoracic duct., Although surgery and trauma constitute the most common causes, malignancies can lead to compression or infiltration of the thoracic duct., Lymphomas comprise the most common group of malignancies associated with chylothorax. Chylothorax contributes to significant respiratory morbidity, nutritional compromise, and secondary immunodeficiency. This entity poses an additional challenge in the management of children with cancer. We describe three children with different malignancies presenting with chylothorax.
| » Case Reports|| |
A 2-year-old boy presented with progressive abdominal distention for 6 weeks. A contrast-enhanced computerized tomography (CECT) of the chest and abdomen revealed a large suprarenal mass. An imaging guided tru-cut biopsy from the mass confirmed the diagnosis of neuroblastoma, which was Stage 4 on metastatic workup. Metastatic sites included mediastinal and supraclavicular lymph nodes. He received chemotherapy as a high-risk neuroblastoma based on the SIOPEN HR-NBL1 protocol. He developed acute respiratory distress following the administration of the second cycle of chemotherapy. Chest X-ray and CT chest showed the presence of a massive pleural effusion. Milky-white-colored fluid was aspirated on pleural tap, done to relieve respiratory distress. Simultaneous biochemical analysis of pleural fluid and serum corroborated with the diagnosis of chylothorax [Table 1]. An intercostal tube was placed which drained 500–750 ml of chyle per day [Figure 1]. The child was placed on a fat-free diet with the addition of medium-chain triglycerides (MCTs). Octreotide infusion was initiated which reduced the intercostal drain significantly. The chylous output gradually declined and ceased after 5 cycles of chemotherapy, following which the intercostal tube was removed. At the end of 8 cycles, the child underwent debulking surgery. The family abandoned therapy subsequent to surgery. The child succumbed to relapse of disease 1 year later.
|Table 1: Diagnosis, salient investigations, and outcome in the three children with malignant chylothorax|
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|Figure 1: Intercostal tube draining white, opalescent chylous fluid in a child with metastatic neuroblastoma|
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A 6-year-old boy presented with a short history of dyspnea. Examination was suggestive of a superior mediastinal syndrome. A chest X-ray and CECT of the chest showed a large anterior mediastinal mass with left-sided pleural effusion [Figure 2]. The child was diagnosed as a T-lymphoblastic lymphoma (T-NHL). Pleural tap demonstrated opalescent fluid, and the diagnosis of chylothorax was ratified by biochemical investigations [Table 1]. The respiratory distress resolved rapidly with glucocorticoid prephase. He completed induction chemotherapy uneventfully. However, a repeat imaging of the chest revealed persistence of the mediastinal mass and pleural effusion. He subsequently developed recurrence of respiratory distress necessitating placement of an intercostal drainage tube. The effusion was confirmed to be chylous. The patient progressed to develop bilateral chylothorax on medical management which included octreotide infusion and total parenteral nutrition. A single-photon emission CT/CT-based lymphoscintigraphy was performed, which suggested evidence of active lymphatic leak/abnormal lymphatic collection at the mid-thoracic region [Figure 3]. A surgical thoracic duct ligation was planned. Unfortunately, the pleural fluid remained persistently positive for malignant cells even after consolidation chemotherapy. In view of a refractory T-NHL, the patient opted for palliative care and surgery was not performed.
|Figure 2: Chest X-ray posterior–anterior view demonstrates evidence of massive left pleural effusion in a child with T-non-Hodgkin lymphoma|
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|Figure 3: Lymphoscintigraphy images in a child with malignant chylothorax. (a) Early image obtained after 10 min showed tracer activity in the femoral and inguinal lymph nodes (arrow). There was diffuse tracer activity in the left hemithoracic region (arrow). (b and c) Hybrid single-photon emission computerized tomography/computerized tomography image revealed diffuse tracer activity in the left pleural cavity, predominantly in the apical region (arrow). A focus of mild tracer activity was noted in the posterior mediastinum at the level of D5/D6 vertebrae, suggesting a probable site of leak (arrow)|
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A 6-year-old boy presented with a painless, progressive swelling in the left cervical region for 1 year and respiratory distress for 10 days. He was diagnosed at a peripheral hospital to have massive left pleural effusion. He was referred with an intercostal drainage tube in situ which was draining chylous fluid. Biochemical investigations established the diagnosis of chylothorax [Table 1]. A tru-cut biopsy from the conglomerate lymph nodal mass in the left cervical region was consistent with the diagnosis of Hodgkin lymphoma, nodular sclerosis Type-II, being a Stage IV disease on further workup. He was treated based on the Euronet protocol for Hodgkin lymphoma. After the first cycle of chemotherapy, the chylothorax resolved. He is currently in remission after chemotherapy.
| » Discussion|| |
The lymphatic channels that carry chylomicrons absorbed from the intestine converge to form the thoracic duct. Disruption of the duct leads to accumulation of chyle in the pleural space. In children, the usual causes for chylothorax include congenital lymphatic malformations, injury to the thoracic duct as a consequence of surgery/trauma, increased central venous pressure, mediastinal tumors, and granulomatous infections such as tuberculosis., In a large study of over 200 patients with chylothorax, surgery/trauma constituted the most common cause. Malignancies such as lymphoma accounted for one-sixth of the cases, comprising the second-most common cause. Childhood malignancies which have been associated with chylothorax include lymphoma, neuroblastoma, germ cell tumor, Kaposi sarcoma, and Wilms tumor.,, The three patients in our report had chylothorax at presentation and had no prior invasive intervention/surgery or history of trauma. The postulated mechanisms for malignant chylothorax are thoracic duct infiltration or obstruction in the case of mediastinal tumors., While the patient with T-NHL had an isolated mediastinal site of disease, the patients with neuroblastoma and Hodgkin lymphoma had mediastinal lymphadenopathy as part of a disseminated disease. In a study of 18 patients with malignant chylothorax, 8/18 (44%) had mediastinal involvement. The authors proposed lymphatic obstruction, increased venous pressure, and transdiaphragmatic flow of chyle as possible mechanisms in abdominal tumors. Among our patients, the child with T-NHL had lymphoblasts visible on chylous fluid cytology, suggesting an infiltrative process. The patients with neuroblastoma and Hodgkin lymphoma had mediastinal adenopathy and no evidence of malignant cells in the chyle, possibly signifying a compressive process.
Confirmation of chylothorax is by measurement of pleural fluid and serum levels of cholesterol and triglyceride. A pleural fluid triglyceride exceeding 110 mg/dl in conjunction with a ratio of pleural fluid to serum cholesterol of <1 supports the diagnosis. Chronic pleural effusions may mimic chylothorax due to high levels of cholesterol (pseudochylothorax). In countries such as India with a high incidence of tuberculosis, it is essential to perform appropriate investigations to identify this potentially treatable cause. Sahoo et al. reported a case of tuberculous chylothorax in a patient with T-NHL. None of our patients tested positive for tuberculosis.
The occurrence of chylothorax adds to the adversities encountered during the management of children with cancer. Respiratory distress is a cardinal feature. Unless a rapid resolution is anticipated, placement of an intercostal drainage tube is required to allow the underlying collapsed lung to expand and improve lung function. Nutrition is severely compromised in these patients as a result of the necessary fat-free diet to reduce the flow of chyle and as a result of treatment and tumor-related factors., MCTs that are absorbed directly into the portal venous circulation are added to the diet., Hypogammaglobulinemia due to loss of proteins in the chylous fluid contributes to further immunodeficiency in children who are previously immunosuppressed by the malignancy and cytotoxic therapy. All the three patients described had significant hypoalbuminemia and hypoglobulinemia. Other complications which may follow chylothorax include fluid and electrolyte imbalance and a prothrombotic state.
While surgical repair is essential in postoperative/traumatic chylothorax, treatment of the underlying disease is vital in nontraumatic chylothorax. Chylothorax is likely to resolve if the malignancy responds to conventional therapy. Of the three patients we described, two achieved remission of disease with corresponding improvement in chylothorax. However, the chylothorax in the child with refractory lymphoma remained unresolved, probably secondary to a nonresponsive tumor. Pleurodesis and radiotherapy have been attempted previously in patients with refractory lymphoma-associated chylothorax.,, Teng et al. observed that an adverse effect of chylothorax on survival was more pronounced in patients with solid tumors as compared to those with lymphomas.
| » Conclusions|| |
Chylothorax is an unusual complication in children with cancer. It contributes to additional morbidity and mortality during management. Treatment involves initiation of fat-free diet, addition of MCT, and placement of intercostal tube. The chylothorax is likely to resolve if the underlying malignancy responds to chemotherapy.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patients has/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.
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