Refractory Chylothorax Research Articles

MIDODRINE: BREAKING NEW GROUND IN THE TREATMENT OF CHYLOTHORAX

SESSION TITLE: Disorders of the Pleura SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/21/2019 3:15 PM - 3:15 PM INTRODUCTION: Chylothorax is a rare condition associated with high morbidity and mortality when left untreated. Thoracic surgical procedures are the most common cause for traumatic chylothorax, presumably due to disruption of the thoracic duct or tears in the lymphatic tributaries. Pleural fluid triglyceride level of 110 mg/dL establishes the diagnosis of chylothorax. Most post-operative chyle leaks are low output, and have been managed with drainage for symptom relief, dietary modification and octreotide. When these measures fail, patients undergo surgical interventions. Midodrine, an α1-adrenergic agonist, causing lymphatic constriction is being explored as a novel therapeutic option for refractory chylothorax prior to surgical management. We present a patient with chylothorax after bilateral lung transplantation that was effectively managed with dietary modification, proton pump inhibitor, octreotide and midodrine. CASE PRESENTATION: 61-year-old female with past medical history of hypertension, hyperlipidemia, diabetes mellitus, chronic kidney disease, and pulmonary sarcoidosis presented to the hospital for bilateral lung transplantation. Her post-operative course was complicated with persistent exudative serosanguinous drainage from right chest tube with a triglyceride level of 143 mg/dL. She was treated with octreotide, proton pump inhibitor and midodrine 20 mg three times a day. She was given a fat-free supplement and PPN to meet protein needs. A specialty formula, Vivonex RTF, which contains minimal fat and MCT oil, was started 7 days later. Her fat intake was initially limited to 10 grams/day. Eventually, we increased intake to goal calories to prevent risk of essential fatty acid deficiency. Her chyle leak improved, and eventually her chest tube was discontinued. DISCUSSION: The management of chylothorax post-operatively is variable. The mainstay of treatment is a low-fat diet and octreotide. There are no studies evaluating the use of alpha-adrenergic agonists for the treatment of chylothorax. Alpha-agonistic activity may help decrease chyle flow, by causing smooth-muscle contraction in lymphatic vessels via alpha-1 receptor on lymphatic channels. Therefore, midodrine, an alpha-1 agonist, is being explored for use in the treatment of chylothorax. This case highlights the potential role of α1-adrenergic agonists in the management of chylothorax. CONCLUSIONS: The disruption of the chyle duct may be at multiple sites in a post-operative chyle leak, for which a systemic lymphatic constrictor may help stop the leak. Alpha-agonists may be helpful as an adjunctive medication for the treatment of chylothorax. Further studies are needed to determine the efficacy, appropriate dosage regimen and safety profile of midodrine in the treatment of chylothorax. It is readily available in oral forms and relatively inexpensive which makes it an ideal choice for widespread use for the treatment of chylothorax. Reference #1: McGrath, E. E., Blades, Z., & Anderson, P. B. (2010). Chylothorax: Aetiology, diagnosis and therapeutic options. Respiratory Medicine, 104(1), 1-8. https://doi.org/10.1016/j.rmed.2009.08.010 Reference #2: Sukumaran K. Nair, Matus Petko, Martin P. Hayward; Aetiology and management of chylothorax in adults, European Journal of Cardio-Thoracic Surgery, Volume 32, Issue 2, 1 August 2007, Pages 362–369, https://doi.org/10.1016/j.ejcts.2007.04.024 Reference #3: Liou, D., Warren, H., Maher, D., Soukiasian, H., Melo, N., Salim, A., & Ley, E. (2013). Midodrine. Chest, 144(3), 1055-1057. https://doi.org/10.1378/chest.12-3081 DISCLOSURES: No relevant relationships by Javed Iqbal, source=Web Response No relevant relationships by Joshua Lee, source=Web Response No relevant relationships by Dolly Patel, source=Web Response No relevant relationships by Marjan Rahmanian, source=Web Response No relevant relationships by Scott scheinin, source=Web Response No relevant relationships by Harish Seethamraju, source=Web Response

Evaluating the Use of Octreotide for Acquired Chylothorax in Pediatric Critically Ill Patients Following Cardiac Surgery.

To evaluate the impact of octreotide on time to resolution of chylothorax compared with conventional therapy. Secondary outcomes include the following: time to reduction of chest tube output by 20%, additional surgeries for chylothorax, hospital length of stay, in-hospital mortality, and adverse drug reactions. We retrospectively evaluated the efficacy of octreotide vs conventional therapy for treatment postoperative chylothorax in pediatric patients in the cardiac ICU following surgery for congenital heart disease between October 2008 and June 2017. Final analysis included 32 patients with chylothorax who met inclusion criteria. Patients who received octreotide had a longer duration of chest tube drainage than those who received conventional therapy (24 vs 9 days, p < 0.001). Resolution of chylothorax was achieved in 13 of 16 (81.3%) octreotide patients and 16 of 16 (100%) conventional patients (p = 0.178). There was a comparable time to reduction by 20% in drainage (6 vs 8 days, p = 0.337). There was no significant correlation between time after starting conventional management and reduction chylous output in either the octreotide or conventional therapy group (p = 0.809, p = 0.107, respectively). However, there was a significant and moderate correlation between octreotide and reduction in a chylous output following initiation of octreotide (R 2 = 0.464, p = 0.021). Octreotide is potentially a safe and effective therapy for treatment in pediatric patients with refractory chylothorax following surgery for congenital heart disease.

Interventional Therapies for Thoracic Duct Injury and Intractable Chylothorax.

Injury to the thoracic duct with resultant chylothorax can cause significant patient morbidity and mortality. Conservative treatment strategies often fail to address the problem. Open surgical and percutaneous approaches are often required to manage patients with refractory chylothorax. This review describes in detail the major role of minimally invasive interventional therapies for thoracic duct (TD) injury. The review emphasizes strategies for identifying the TD on preprocedural imaging and describes various techniques for percutaneous access to the TD. The advantages and disadvantages of several approaches for accessing the duct are discussed. The technique of duct embolization is highlighted. The role of the minimally invasive percutaneous approach over open surgical approaches is discussed with a review of clinical outcomes, as reported in the literature. This review will also briefly discuss the surgical approach to TD ligation.

Experiences in the treatment of refractory chylothorax associated with lymphoproliferative disorders

BackgroundChylothorax is a rare condition which can be associated with malignant lymphoproliferative disorders (LPDs). We retrospectively analyzed the results of the conservative treatment of 10 patients with persistent non-traumatic malignant chylothorax.ResultsConservative treatment lead to a decline of chylothorax after mean of 66 days and consisted of the treatment of the underlying disease and of simultaneous long-term supportive care (drainage of the thoracic cavity, dietary measures and nutrition management). In most cases (80%), chylothorax disappeared only after a successful therapeutic response of the underlying disease. Low-dose radiotherapy had very good effects in two patients.ConclusionConservative treatment of malignant chylothorax can be considered a suitable method. Based on our results, successful treatment of the lymphoproliferative disorder seems to be a very important factor for the disappearance of chylothorax.

Devastating cerebral Lipiodol® embolization related to therapeutic lymphangiography for refractory chylothorax in a patient with Behçet’s disease

Onset of neurological symptoms early after intranodal lymphangiography can occur due to Lipiodol droplet migration through intrapulmonary lymphovenous communication. Patients with Behçet's disease may be at higher risk of developing this devastating complication.

Management of Life-threatening Refractory Chylothorax and Chylopericardium due to Lymphangiomatosis

Lymphangiomatosis is a rare disorder. Chylous effusion and osteolytic lesions are diagnostic clues. Radiographic findings can be suggestive of the disease, but the final confirmatory diagnosis is made histologically. We report a case of an infant who presented with life-threatening refractory chylothorax and chylopericardium due to diffuse pulmonary lymphangiomatosis, leading to critical condition with challenging management. With multidisciplinary approach, the child improved and was discharged home with satisfactory outcome.

Novel thoracoscopic navigation surgery for neonatal chylothorax using indocyanine-green fluorescent lymphography

BackgroundPostoperative chylothorax after surgery for esophageal atresia/tracheoesophageal fistula (TEF) is a rare but serious complication, especially in neonates. This study aimed to identify the thoracic duct and ligate chylous leakage sites, using thoracoscopic navigation of an indocyanine-green (ICG)-based near-infrared (NIR) fluorescence imaging system. MethodsFrom November 2014 to April 2017, thoracoscopic intraoperative ICG-NIR imaging was performed in 10 newborns (11 surgeries) with first TEF operation or with persistent postoperative chylothorax after TEF operation. NIR imaging was performed 1h after an inter-toe injection of ICG. Thoracoscopic ligations against the NIR-detected leakage sites were performed with sutures. ResultsThe thoracic duct or lymphatic leakage was directly visualized in each patient. In 8 surgeries with first thoracoscopic TEF operation, one case had suspected minor chylous leakage without postoperative chylothorax. Another case with no chylous leakage at the first operation resulted in chylothorax at postoperative day 11. In three neonates with postoperative chylothorax, leakage points were detected near the ablation site of the azygos vein during the first operation. These points were properly ligated, and postoperative chylous leakage ceased with no adverse events. ConclusionsThoracoscopic ICG-NIR imaging encourages the repair of refractory chylothorax and seems reliable. Level of EvidenceIV

Pulmonary Lymphangiectasia Complicating Adult Cardiac Surgery

A middle-aged female underwent uneventful mitral valve surgery for rheumatic mitral stenosis. Postoperatively, she developed chylothorax. She was initially managed conservatively with a low-fat and medium-chain triglceride diet, pleural space evacuation, enetric rest, hyperalimentation, and octreotride. On failure of medical management she underwent a lymphagiography, which revealed thoarcic duct agenesis. Surgical re-exploration was planned, which revealed no obvious leaking site. A parietal pleurectomy and ligation of overlying fat and thymic tissue was done. Drain output ceased and patient was asymptomatic at discharge and follow-up. Addition of lymphangiography to medical management in refractory chylothorax is therapeutic and diagnostic. In our patient we could detect the presence of pulmonary lymphangiectasia, which is a rare finding and the cause of chylothorax. If medical management fails aggressive surgical therapy should be considered.

Intractable Chylothorax Following Mitral Valve Replacement in an Adult

Chylothorax after an open-heart surgery has previously been mentioned in the literature. We report this association in a young female post-mitral valve replacement surgery due to abnormal lymphatic channels. Medical management was conducted for 4 weeks. On failure of the conservative treatment, she underwent pleurectomy with suture ligation of the thymic gland remnants and pericardium for the chylothorax, which resolved following the redo surgery. This rare incidental variation in lymphatic drainage was responsible for refractory chylothorax, which otherwise could have been managed medically.

Necrotizing Enterocolitis after Octreotide Treatment in a Preterm Newborn with Idiopathic Congenital Chylothorax

Octreotide, a somatostatin analogue, has been used for the management of patients with refractory chylothorax. Side effects related to the gastrointestinal system associated with octreotide are necrotizing enterocolitis (NEC) and focal intestinal perforation. NEC is the most common and dangerous gastrointestinal emergency in premature infants. We present the development of necrotizing enterocolitis after octreotide treatment in a preterm infant with idiopathic congenital chylothorax which settled after discontinuation of octreotide.

Intrapleural Infection Causing Pleurodesis in a Patient With Refractory Chylothorax Related to Yellow Nail Syndrome

SESSION TITLE: Disorders of the Pleura SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Yellow nail syndrome (YNS) is a rare cause of chylothorax, and empyema is an unusual complication in these patients. While tunneled intrapleural catheter (IPC) related infections are uncommon, they may lead to post-infectious pleurodesis. Herein we report a case of refractory chylothorax due to YNS with empyema leading to post-infectious pleurodesis. CASE PRESENTATION: A 66 year old man with YNS, manifested by chronic chylous pleural effusions managed by placement of tunneled IPCs 4 years prior, was admitted to the hospital with increasing dyspnea and oxygen requirement. Imaging revealed patchy opacities in the bases of the lungs bilaterally, and broad antibiotic coverage was initiated. Ultrasound demonstrated increased septations within the pleural fluid compared to previous imaging, and bilateral thoracenteses were performed with Enterococcus faecalis growth noted from the left effusion. Evacuation of the pleural space was accomplished via instillation of fibrinolytic agents via the left IPC, and was later repeated on the right following decrease in output with noted increase in septations on ultrasound. Antibiotic coverage was continued for a total of three weeks. On follow-up, the catheters had both stopped draining for three weeks, and ultrasound revealed small residual effusions allowing removal of both catheters. There was no recurrence of the effusions at 6 months, demonstrating pleurodesis. DISCUSSION: Yellow nail syndrome is a rare disorder characterized by thickened and yellow nails with lymphedema and multiple respiratory manifestations. The cause is unknown, but the mechanism of the clinical manifestations is thought to be acquired lymphatic dysfunction. A recent review of the literature has identified less than 400 cases with an estimated prevalence in the general population of < 1:1,000,000. While parapneumonic effusions and empyemas are quite common complications of bacterial pneumonia, empyemas appear to be uncommon within YNS, with one study documenting empyema in only 3.5% of patients (3/85). A 2013 study of over 1000 patients with malignant pleural effusions by Fysh et al. demonstrated that IPC related infections were uncommon, occurring in <5% of cases, but of these, 62% (31/50) achieved post-infectious pleurodesis. CONCLUSIONS: Our patient required long term IPC management for chronic chylothoraces; however, following treatment for empyema, achieved bilateral pleurodesis with durable response. Though infections are undesirable, pleurodesis is a positive outcome that is commonly noted in IPC related infections. Reference #1: Fysh, E. T., et al. (2013). “Clinical outcomes of indwelling pleural catheter-related pleural infections: an international multicenter study.” Chest 144(5): 1597-1602. Reference #2: Valdes, L., et al. (2014). “Characteristics of patients with yellow nail syndrome and pleural effusion.” Respirology 19(7): 985-992. Reference #3: Vignes, S. and R. Baran (2017). “Yellow nail syndrome: a review.” Orphanet J Rare Dis 12(1): 42 DISCLOSURE: The following authors have nothing to disclose: Bryan Kelly, Ryan Kern No Product/Research Disclosure Information

Laparoscopic ligation of cisterna chyli for refractory chylothorax: A case series and review of the literature

ObjectivesWe describe an alternative surgical technique for the treatment of chylothorax in patients who have had failure of or are not candidates for transthoracic ligation or embolization by interventional radiology. MethodsWe describe our experience with laparoscopic ligation of the cisterna chyli in 3 such patients and compare our results with published literature. We used a 5-port approach as for foregut surgery. We retracted the liver, transected the gastrohepatic ligament, and retracted the stomach to the left. We exposed the right lateral aspect of the aorta at the level of the celiac trunk and clipped fatty tissue between the aorta and the right crus. We skeletonized the right crus and dissected from the right crus to the inferior vena cava. We then retracted the inferior vena cava laterally, exposed all soft tissue posteriorly, and identified the cisterna chyli posteromedially to the inferior vena cava. Finally, we ligated and clipped all fatty tissue between the right crus and the inferior vena cava. ResultsSuccess rate was 67%; 1 patient with idiopathic chylothorax did not have resolution and eventually died of multisystem organ failure. There were no procedure-related complications. ConclusionsLaparoscopic ligation of cisterna chyli is an available therapeutic option for patients with chylothorax unresponsive to medical management, embolization, and transthoracic ligation of the thoracic duct. Our series is comparable with other reports of transabdominal approach to chylothorax.

Diaphragmatic fenestration for refractory chylothorax after congenital cardiac surgery in infants

ObjectiveMedically refractory chylous pleural effusion after congenital heart surgery is associated with significant morbidity and mortality, especially in infants. We reviewed our experience with diaphragmatic fenestration procedure in this group of patients. MethodsA retrospective chart review of all patients who had diaphragmatic fenestrations for chylous effusion at our institution over a 2-year period was performed. ResultsA total of 9 diaphragmatic fenestration procedures were performed in 8 patients who had failed medical management of chylous pleural effusions. All procedures except 1 were performed on the right side. The median age at time of procedure was 4.6 months (range, 3 weeks to 14 months). The average time between primary congenital cardiac surgery and fenestration was 26 days (range, 4-53 days). Three patients had single ventricle repair. Average time of chest tube removal after procedure was 4 days. Average time to extubation was 3 days. All patients but 1 were able to advance to full feedings without reaccumulation of chylous effusion within 12 days. No complications developed in the patients. Recurrent effusion over a median follow-up period of 19 months did not develop in the patients. ConclusionsDiaphragmatic fenestration is an effective and safe strategy for management of persistent chylous effusions after congenital cardiac surgery.

Intravascular Stent Implantation as Effective Therapy for Refractory Chylothorax Secondary to Congenital Superior Vena Cava Stenosis in an Infant

Intravascular Stent Implantation as Effective Therapy for Refractory Chylothorax Secondary to Congenital Superior Vena Cava Stenosis in an Infant

Successful Treatment With Lymphovenous Anastomosis for Lower Extremity Edema Secondary to Lipiodol Lymphangiography

Background: Chylothorax is one of the complications after thoracic surgery and treated by conservative or surgical means. Lipiodol lymphangiography is one of the options and it causes obliteration of chylous leak by inflammatory manner. In this article, we describe a case of lymphedema of the bilateral lower extremities occurs after lipiodol lymphangiography and it is treated successfully by lymphovenous anastomosis. Case presentation: A 67-year-old man presented with refractory chylothorax after subtotal esophagectomy and thoracic lymph node dissection. His chylothorax developed 4-month later of subtotal esophagectomy and was refractory to the conservative treatment (i.e. tube thoracostomy). He was referred to our department to treat chylothorax. We chose lipiodol lymphangiography as the treatment. Lymphatic duct of left foot was detected with indocyanine green and exposed to inject lipiodol into lymphatic duct directly. Chylothorax improved immediately after lipiodol lymphangiography and his edema of right lower extremity emerged 22-month later of lipiodol lymphangiography. We considered that his lower extremity edema was caused by lipiodol lymphangiography and performed lymphovenous anastomosis. Lymphovenous anastomosis was performed at the proximal of right thigh and the dorsum of the foot. At six-month later of lymphovenous anastomosis, we revealed that his right lower extremity had become thinner significantly, nevertheless laterality remained. Conclusion: To our best knowledge, this is the first report of lymphedema of the bilateral lower extremities after lipiodol lymphangiography for chylothorax. Lymphovenous anastomosis is a treatment option for such condition.

Pleurodesis with povidone–iodine for refractory chylothorax in newborns: Personal experience and literature review

IntroductionRefractory chylothorax is a severe clinical issue, particularly in neonates. Conventional primary approach is based on diet with medium-chain fatty acids and/or total parenteral nutrition. In nonresponders, proposed second line treatments include chemical or surgical pleurodesis, thoracic duct ligation, pleuroperitoneal shunting and pleurectomy but none of these have been shown to be superior to other in terms of resolution rate and safety. Our aim is to report our experience on povidone–iodine use for chemical pleurodesis in newborn infants with chylothorax unresponsive to conservative treatment.Our aim is to report our experience on povidone–iodine use for chemical pleurodesis in newborn infants with chylothorax unresponsive to conservative treatment. MethodsSince 2013, povidone–iodine pleurodesis was attempted in all patients with persistent chylothorax who failed conservative treatment (no response to at least 10days of total parenteral nutrition and maximum dosage of intravenous octreotide). Pleurodesis consisted in the injection of 2ml/kg of a 4% povidone–iodine solution inside the pleural space, leaving the pleural tube clamped for the subsequent 4hours. ResultsFive patients were treated with chemical pleurodesis of persistent chylothorax. Four of 5 patients had their pleural effusion treated by one single povidone–iodine infusion. Median time for resolution was 4days. A patient with massive superior vena cava thrombosis did not benefit from pleurodesis. None of the patients experienced long term side effects of the treatment. ConclusionOur data suggest that povidone–iodine pleurodesis may be considered a safe and effective option to treat refractory chylothorax in newborns.

Clinical and molecular characterization of an infant with a tandem duplication and deletion of 19p13

Copy number variations (CNVs) on the short arm of chromosome 19 are relatively rare. We present a patient with a tandem de novo 3.9 Mb duplication of 19p13.12p13.2 and an adjacent 288 kb deletion of 19p13.12. The CNVs were detected by genome wide SNP-array and confirmed by fluorescence in situ hybridization. Mate-pair sequencing revealed two breakpoint junctions leading to a germline tandem inverted duplication and an adjacent deletion. The patient had a major congenital heart defect and refractory edema leading to metabolic and endocrinological disturbances. Further complications occurred due to refractory chylothorax, severe inflammatory response syndrome, and repeating sepsis. After 2 months, the child died due to intractable respiratory failure. The phenotype of this patient was compared with reported patients with overlapping deletions or duplications. We conclude that the congenital heart defect, respiratory insufficiency, and abnormal neurologic examination are most likely due the contiguous gene deletion/duplication.

治療に難渋した成人特発性乳糜胸の一例

治療に難渋した成人特発性乳糜胸の一例

Twin Infant with Lymphatic Dysplasia Diagnosed with Noonan Syndrome by Molecular Genetic Testing

Noonan Syndrome is an autosomal dominant disorder characterized by short stature, congenital heart defects, developmental delay, dysmorphic facial features and occasional lymphatic dysplasias. The features of Noonan Syndrome change with age and have variable expression. The diagnosis has historically been based on clinical grounds. We describe a child that was born with congenital refractory chylothorax and subcutaneous edema suspected to be secondary to pulmonary lymphangiectasis. The infant died of respiratory failure and anasarca at 80 days. The autopsy confirmed lymphatic dysplasia in lungs and mesentery. The baby had no dysmorphic facial features and was diagnosed postmortem with Noonan syndrome by genomic DNA sequence analysis as he had a heterozygous mutation for G503R in the PTPN11 gene.

Successful treatment of refractory chylothorax by pedicled omentoplasty

A 52-year-old female with a history of past surgery for arteriovenous malformation of the thoracic spinal cord presented with left chylothorax. Lymphangiography identified diffuse lymphatic vessel (LV) growth with ectasia at the left supradiaphragmatic level. On the right side, the LVs were absent due to previous surgery. Given the ineffectiveness of conservative management, the patient required surgery. Thoracotomy showed extensive pleural fibrosis, lung atelectasis, and diffuse chyle defluxion on the parietal pleura. The diffuse chyle defluxion was not resolved by ligation. Therefore, we performed pedicled omentoplasty to fill the pleural space and to utilize its drainage and angiogenic capacity. Two years after the procedure, there was no sign of relapse. There are very few reports of refractory chylothorax successfully treated by pedicled omentoplasty. The procedure would be useful for the treatment of chylothorax when non-surgical or typical surgical management fails.