Chylothorax refers to the accumulation of chyle in the pleural cavity. Although rare, it is the most frequent cause of pleural effusion in the neonatal period. Its incidence is estimated at one in 15,000 neonates. The causes of chylothorax are multiple, and there are several major types of chylothorax defined by origin, ie, traumatic (and iatrogenic) chylothorax, medical (spontaneous) chylothorax, and congenital chylothorax. A case of neonatal chylothorax following surgery for esophageal atresia and our therapeutic approach to this entity are presented. Conservative therapy with total enteral nutrition and drainage were sufficient. Treatment for chylothorax is essentially medical, ie, pleural drainage, removal of dietary fats, treatment of any medical cause, and use of drugs to reduce production of chyle. In the event of failure or reappearance of a large effusion, surgical treatment is needed.
chylothorax; octreotide; somatostatin; children
Chylothorax is readily diagnosed from the characteristic qualities of the effusion. Treatment should initially be conservative, consisting of multiple aspirations followed, if necessary, by suction drainage.
Approximately half of the patients will not respond to these measures, and direct ligation and division of the duct is necessary for cure. This operation is most readily carried out through the right chest, the thoracic duct being ligated just above the diaphragm. In cases in which the duct is surrounded by tumor, radiotherapy to the mediastinum is often successful in controlling the reaccumulation of chyle, but irradiation is generally not recommended until after a tissue diagnosis has been made by thoracotomy.
Nutritional problems are often concomitants of chylothorax.
An unusual case of chylothorax is described in a 4-year-old child after repair of a ventricular septal defect and ligation of a patent ductus arteriosus through a median sternotomy. Left chylothorax developed after a latent period of six days and was treated initially with continuous drainage and parenteral supplementation of proteins and lipids. Operative intervention with oversewing of the site of the leak in the anterior mediastinum proved necessary after three weeks. The anatomical variations of the thoracic duct are outlined to explain the occurrence of chylothorax after diverse intrathoracic operations. The physiological effects of a thoracic duct fistula and various aspects of management are reviewed.
Chylothorax is a rare cause of pleural effusion. Here we present a case of chylous pleural effusion in which acid-fast bacilli (AFB) was demonstrated (by AFB direct smear examination). The patient had been suffering from chronic pancreatitis for one year and had undergone pancreatoduodenostomy nine months back. He presented with abdominal pain and dyspnea of six months duration and his chest skiagram showed right-sided pleural effusion. Thoracocentasis showed milky white pleural fluid with triglyceride content of 678 mg/dl, diagnostic of chylothorax. The patient clinically improved and his pleural effusion also completely resolved with anti-tuberculosis treatment.
Chylothorax; acid-fast bacilli; pleural effusion; tuberculosis
Chylothorax caused by chronic lymphocytic leukemia is very rare and the best therapeutic approach, especially the role of modern immunochemotherapy, is not yet defined.
We present the case of a 65-year-old male Caucasian patient with right-sided chylothorax caused by a concomitantly diagnosed chronic lymphocytic leukemia. As first-line treatment four cycles of an immunochemotherapy, consisting of fludarabine, cyclophosphamide and rituximab were administered. In addition, our patient received total parenteral nutrition for the first two weeks of treatment. Despite the very good clinical response of the lymphoma to treatment, the chylothorax persisted and percutaneous radiotherapy of the thoracic duct was applied. However, eight weeks after the radiotherapy the chylothorax still persisted and our patient agreed to a surgical intervention. A ligation of the thoracic duct via a muscle sparing thoracotomy was performed, resulting in a complete cessation of the pleural effusion. Apart from the first two weeks our patient was treated on an out-patient basis for nearly six months.
In this case of chylothorax caused by chronic lymphocytic leukemia, immunochemotherapy in combination with conservative treatment, and even consecutive radiotherapy, were not able to stop pleural effusion, despite the very good clinical response of the chronic lymphocytic leukemia to treatment.
Out-patient management using repetitive thoracocenteses can be safe as bridging until definitive surgical ligation of the thoracic duct.
Treatment for esophageal atresia has advanced over several decades due to improvements in surgical techniques and neonatal intensive care. Subsequent to increased survival, postoperative morbidity has become an important issue in this disease. The aim of our study was to analyze our experience regarding the treatment of esophageal atresia.
We reviewed and analyzed the clinical data of patients who underwent surgery for esophageal atresia at Severance Children's Hospital from 1995 to 2010 regarding demographics, surgical procedures, and postoperative outcomes.
Seventy-two patients had surgery for esophageal atresia. The most common gross type was C (81.9%), followed by type A (15.3%). Primary repair was performed in 52 patients. Staged operation was performed in 17 patients. Postoperative esophageal strictures developed in 43.1% of patients. Anastomotic leakages occurred in 23.6% of patients, and recurrence of tracheoesophageal fistula was reported in 8.3% of patients. Esophageal stricture was significantly associated with long-gap (≥3 cm or three vertebral bodies) atresia (P = 0.042). The overall mortality rate was 15.3%. The mortality in patients weighing less than 2.5 kg was higher than in patients weighing at least 2.5 kg (P = 0.001). During the later period of this study, anastomotic leakage and mortality both significantly decreased compared to the earlier study period (P = 0.009 and 0.023, respectively).
The survival of patients with esophageal atresia has improved over the years and the rate of anastomotic leakage has been significantly reduced. However, overall morbidities related to surgical treatment of esophageal atresia still exists with high incidence.
Esophageal atresia; Tracheoesophageal fistula; Prognosis; Survival
Chylothorax is characterized by the presence of chyle in the pleural cavity. The healing rate of non-operative treatment varies enormously; the maximum success rate in series is 70%. We investigate the efficacy and outcomes of radiotherapy for postoperative chylothorax.
Chylothorax was identified based on the quantity and quality of the drainage fluid. Radiation was indicated if the daily chyle flow exceeded 450 ml after complete cessation of oral intake. Radiotherapy consisted of opposed isocentric portals to the mediastinum using 15 MV photon beams from a linear accelerator, a single dose of 1–1.5 Gy, and a maximum of five fractions per week. The radiation target area was the anatomical region between TH3 and TH10 depending on the localization of the resected lobe. The mean doses of the ionizing energy was 8.5 Gy ± 3.5 Gy.
The median start date of the radiation was the fourth day after chylothorax diagnosis. The patients’ mediastinum was radiated an average of six times. Radiotherapy, in combination with dietary restrictions, was successful in all patients. The median time between the end of the radiation and the removal of the chest tube was one day. One patient underwent wound healing by secondary intention. The median time between the end of radiation and discharge was three days, and the overall hospital stay between the chylothorax diagnosis and discharge was 18 days (range: 11–30 days). After a follow-up of six months, no patient experienced chylothorax recurrence.
Our results suggest that radiotherapy in combination with dietary restriction in the treatment of postoperative chylothorax is very safe, rapid and successful. This novel interventional procedure can obviate repeat major thoracic surgery and shorten hospital stays and could be the first choice in the treatment of postthoracotomy chylothorax.
Chylothorax; Radiotherapy; Postoperative complication
Loss of integrity of the thoracic duct will produce a chylous thoracic effusion. latrogenic chylothorax occurring during an intrathoracic surgical procedure in the vicinity of the thoracic duct is an omnipresent reality. The fluid is characteristically milky in appearance, sterile, and high in fat content.
There seems to be a general agreement that initial management be conservative, namely, dietary control and adequate drainage. The success of conservative management will depend on collateral lymphatic channels developing. If conservative measures fail, thoracotomy for ligation of the duct is indicated. When to terminate nonoperative therapy and opt for operative is controversial.
A significant number of esophageal atresia and tracheoesophageal fistula patients have long gaps and a high propensity to leak. Anastomotic leak in esophageal atresia is associated with a significant morbidity and mortality.
In a prospective randomized trial, we analyzed the risk factors leading to anastomotic dehiscence and studied the effect of pleural wrap as an additional vascular cover around the esophageal anastomosis.
Materials and Methods:
Forty patients were divided into two groups A and B randomly. In 20 patients of group A, pleural wrap was utilized for covering the anastomosis and in 20 patients of group B, no such wrap was utilized.
Both the groups were comparable regarding age, sex, weight, gap length, tension at anastomosis and the hospital stay. The overall leak rate was 25% (10/40) in both the groups. The leak rate was not significantly different in two groups whenever a gap length was less than 2 cm or more than 3 cm. However, for a gap length of 2–3 cm, the leak rate in group A was 18% (2/11) and in group B was 50% (4/8) (P = 0.05). Thirty percent (3/10) of patients, whose anastomosis was under tension, leaked in group A as compared to 75% (6/8) in group B patients (P = 0.001).
Use of pleural wrap was associated with less anastomotic dehiscence in patients with moderate gap esophageal atresia (2–3 cm) especially when the anastomosis was under tension.
Anastomotic dehiscence; esophageal atresia; pleural wrap; tracheoesophageal fistula
A review of the experience with esophageal atresia and tracheoesophageal fistula over a 25-year period appears to lead to the advisability of the following procedures in surgical management:
• Emergency gastrostomy under local anesthesia in all patients.
• Extrapleural interruption of tracheo-esophageal fistula and end-to-end esophago-esophagostomy in patients who have the common type of upper esophageal atresia with distal tracheo-esophageal fistula.
• Upper esophageal stretching and eventual esophago-esophagostomy in patients with proximal and distal esophageal atresia with or without proximal tracheo-esophageal fistula.
Tracheal agenesis (TA) is an extremely rare malformation. We report here autopsy findings in a case of TA with bronchoesophageal fistula of Floyd type III. The other malformations present included laryngeal atresia, Right lung hypolobulation, ventricular septal defect in membranous portion, bilateral cystic renal dysplasia, spleninculus, Meckel's diverticulum, and imperforate anus. The constellations of malformations present in our case have overlapping features with Vertebral anomalies, Anal atresia, Cardiovascular anomalies, Tracheo-esophageal fistula, Esophageal atresia, Renal anomalies, Limb anomalies and Tracheal atresia or laryngo tracheal atresia, Cardiac anomalies, Renal anomalies, Duodenal atresia association described previously in the literature.
Tracheal atresia or laryngo tracheal atresia; Cardiac anomalies; Renal anomalies; Duodenal atresia association; Tracheal agenesis; Vertebral anomalies; Anal atresia; Cardiovascular anomalies; Tracheo-esophageal fistula; Esophageal atresia; Renal anomalies; Limb anomalies association
AIM: To assess whether the use of fibrin sealant shortens the closure time of postoperative enterocutaneous fistulas (ECFs).
METHODS: The prospective case-control study included 70 patients with postoperative ECFs with an output of < 500 mL/d, a fistulous tract of > 2 cm and without any local complication. They were divided into study (n = 23) and control groups (n = 47). Esophageal, gastric and colocutaneous fistulas were monitored under endoscopic visualization, which also allowed fibrin glue application directly through the external hole. Outcome variables included closure time, time to resume oral feeding and morbidity related to nutritional support.
RESULTS: There were no differences in mean age, fistula output, and follow-up. Closure-time for all patients of the study group was 12.5 ± 14.2 d and 32.5 ± 17.9 d for the control group (P < 0.001), and morbidity related to nutritional support was 8.6% and 42.5%, respectively (P < 0.01). In patients with colonic fistulas, complete closure occurred 23.5 ± 19.5 d after the first application of fibrin glue, and spontaneous closure was observed after 36.2 ± 22.8 d in the control group (P = 0.36). Recurrences were observed in 2 patients because of residual disease. One patient of each group died during follow-up as a consequence of septic complications related to parenteral nutrition.
CONCLUSION: Closure time was significantly reduced with the use of fibrin sealant, and oral feeding was resumed faster. We suggest the use of fibrin sealant for the management of stable enterocutaneous fistulas.
Enterocutaneous fistulas; Fibrin sealant; Spontaneous closure
BACKGROUND: Respiratory complications are common after neonatal repair of oesophageal atresia and tracheo-oesophageal fistula. The prevalence of lung function abnormalities and the relation between gastrointestinal complications and lung function has not been studied in a large number of patients. METHODS: Lung volumes and flow-volume loops were measured in 155 patients without spinal curvature aged 6-37 years who had undergone surgery for oesophageal atresia and tracheo-oesophageal fistula. RESULTS: Sixty four of the 155 patients had evidence of mild lower airways disease, with values for FEV1 more than two standardised scores below the predicted value in 39 (25%) and above 2 standardised scores for the residual volume (RV)/total lung capacity (TLC) ratio in 64 (41%). Restrictive lung disease (TLC more than 2 standardised scores below predicted) was present in 28 (18%). Severe lung function abnormalities were present in under 10% of the 155. Half the subjects had some evidence of extra-thoracic tracheal obstruction, with a high ratio of expiratory to inspiratory flow for peak flow in 76 (50%) and at 50% of vital capacity in 59 (38%). Patients with radiological gastro-oesophageal reflux in early childhood had more airways obstruction and smaller lung volumes. Patients with current gastrointestinal symptoms were similar in their lung function to symptom free patients. CONCLUSIONS: Minor lung function abnormalities are common in patients after repair of oesophageal atresia. Early diagnosis and management of gastro-oesophageal reflux may help to minimise these lung function abnormalities.
A pleural effusion containing chylomicrons is termed chylothorax and results from leakage of lymph fluid into the pleural cavity. We report on the case of a 59-year-old woman with severe dyspnea due to a large chylothorax. She was known to have liver cirrhosis but no ascites. There was no history of trauma, cardiac function was normal and thorough diagnostic work-up did not reveal any signs of malignancy. In summary, no other etiology of the chylothorax than portal hypertension could be found. Therapy with diuretics as well as parenteral feeding failed to relieve symptoms. After a transjugular intrahepatic portosystemic shunt (TIPS) had successfully been placed, pleural effusion decreased considerably. Eight months later, TIPS revision had to be performed because of stenosis, resulting in remission from chylothorax. This case shows that even in the absence of ascites, chylothorax might be caused by portal hypertension and that TIPS can be an effective treatment option.
Chylothorax; Cirrhosis; Liver; Portal hypertension; Transjugular intrahepatic portosystemic shunt
Fetal chylothorax (FC) is a rare condition characterized by lymphocyte-rich pleural effusion. Although its pathogenesis remains elusive, it may involve inflammation, since there are increased concentrations of proinflammatory mediators in pleural fluids. Only a few hereditary lymphedema-associated gene loci, e.g. VEGFR3, ITGA9 and PTPN11, were detected in human fetuses with this condition; these cases had a poorer prognosis, due to defective lymphangiogenesis. In the present study, genome-wide gene expression analysis was conducted, comparing pleural and ascitic fluids in three hydropic fetuses, one with and two without the ITGA9 mutation. One fetus (the index case), from a dizygotic pregnancy (the cotwin was unaffected), received antenatal OK-432 pleurodesis and survived beyond the neonatal stage, despite having the ITGA9 mutation. Genes and pathways involved in the immune response were universally up-regulated in fetal pleural fluids compared to those in ascitic fluids. Furthermore, genes involved in the lymphangiogenesis pathway were down-regulated in fetal pleural fluids (compared to ascitic fluid), but following OK-432 pleurodesis, they were up-regulated. Expression of ITGA9 was concordant with overall trends of lymphangiogenesis. In conclusion, we inferred that both the immune response and lymphangiogenesis were implicated in the pathogenesis of fetal chylothorax. Furthermore, genome-wide gene expression microarray analysis may facilitate personalized medicine by selecting the most appropriate treatment, according to the specific circumstances of the patient, for this rare, but heterogeneous disease.
Yellow nail syndrome is a type of lymphatic dysplasia syndrome characterised by the triad of yellow nails, lymphoedema, and pleural effusions. The case history is presented of a 70 year old patient with yellow nail syndrome who complained of dyspnoea caused by massive chylothorax. The patient underwent insertion of a pleuroperitoneal shunt which resulted in abdominal distension and deterioration of leg oedema. The pleuroperitoneal shunt was replaced by a pleurovenous shunt on the right side which led to an improvement in the bilateral pleural effusions, abdominal distension, and leg oedema. A pleurovenous shunt may be an alternative rescue therapy for yellow nail syndrome.
Lymphangioleiomyomatosis with tuberous sclerosis complex is a rare disease. One of the most frequent complications of lymphangioleiomyomatosis is pleural effusion (chylothorax) wich can be treated with the use of VATS. Authors report a case of pulmonary lymphangioleiomyomatosis in a 56-year-old female patient with tuberous sclerosis complex with an 8-week history of recurrent chylothorax, dyspnea and debilitating weakness. By CT scan a flat tissue proliferation was seen in the site of the thoracic duct and it was supposed to be the reason for the pleural effusion. A VATS resection of this laesion and ligation of the thoracic duct was performed successfully. Chylothorax is often associated with pulmonary lymphangioleiomyomatosis. Lymphangioleiomyomatosis combined with tuberous sclerosis complex is extremely rare. In case of chylothorax VATS treatment is successful and may be the first choice.
Videothoracoscopy; lymphangioleiomyomatosis; tuberous sclerosis complex
Pulmonary lymphangiectasia is an uncommon congenital anomaly, and familial occurrence has rarely been reported. We report on two sibs with bilateral pleural effusion/chylothorax and hydrops who died neonatally. One sib required prenatal intrauterine hemithoracic drainage. Autopsy confirmed congenital pulmonary lymphangiectiasia (CPL) histologically in the first case.
Hydrops, characterized as subcutaneous edema and effusions in two or more body cavities, may be due to a variety of factors, but the co-occurrence of CPL in one of these sibs, although rare, supports the notion that chylothorax and hydrops may be caused by structural lesions of lymph channels. Although most cases of CPL are sporadic, the reported sibs support autosomal recessive inheritance, with intrafamilial variability of a lymphatic disorder on a genetic basis. Mutations in VEGFR3 in families with Milroy disease, mutations of FOXC2 in the lymphedema-distichiasis syndrome, and fatal chylothorax in α9-deficient mice are potential candidate genes.
chylothorax; congenital pulmonary lymphangiectasia; familial; hydrops
An 18 year-old-male sustained a gunshot wound to the abdomen which required an uneventful hepatorrhaphy. He later returned with a large right effusion and was diagnosed with a chylothorax. The output was persistent despite conservative measures. Thoracotomy with attempted thoracic duct ligation was unsuccessful at decreasing the output. Re-exploration and ligation of the thoracic duct was required thru an abdominal approach.
During the course of the disease a patient with systemic lupus erythematosus (SLE) may develop inflammation of one or more serous membranes, resulting in pleural, peritoneal, or pericardial effusion. Chylous ascites and chylothorax have rarely been described in patients with SLE. Therefore, in parallel with the analysis of blood samples, detailed analysis of the effusions should be carried out. Supportive measures are often needed to relieve the symptoms of chylothorax or chylous ascites together with the treatment of the primary disease. The available literature had reported just 4 cases of chylous ascites and/or chylothorax in association with SLE, and this patient presented here is one of the rare cases apart from the reported ones.
Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.
Esophageal atresia; Tracheoesophageal fistula; Esophageal stenosis; Long-gap; Gastro-esophageal reflux
A 25-yr-old woman presented with a right pleural effusion. Destruction of 9th through 12th ribs, adjacent vertebral bodies, and transverse processes was noted on plain radiograph and a large low-attenuated, irregular shaped mass lesion with peripheral rim enhancement, destroying vertebral body and transverse process, was revealed on the computed tomographic scan. Magnetic resonance imaging showed high signal on T1- weighted image and iso- and low signal on T2-weighted image for the mass lesion replacing the vertebral bony cortex and marrow space. An open rib biopsy revealed the histopathological changes of Gorham's disease (essential osteolysis), even though only bloody fluid filling the empty space and rib and vertebral transverse process destruction were grossly observed on operation. Even though there was no definite response to radiotherapy and pleurodesis, the patient showed stable condition up to 20 months after diagnosis.
Thoracoscopic repair of esophageal atresia is considered to be one of the more advanced pediatric surgical procedures, and it undoubtedly has a learning curve. This is a single-center study that was designed to determine the learning curve of thoracoscopic repair of esophageal atresia.
The study involved comparison of the first and second five-year outcomes of thoracoscopic esophageal atresia repair.
The demographics of the two groups were comparable. There was a remarkable reduction of postoperative leakage or stenosis, and recurrence of fistulae, in spite of the fact that nowadays the procedure is mainly performed by young staff members and fellows.
There is a considerable learning curve for thoracoscopic repair of esophageal atresia. Centers with the ambition to start up a program for thoracoscopic repair of esophageal atresia should do so with the guidance of experienced centers.
Chylothorax is a well-documented complication of thoracic trauma and is associated with mortality rates of up to 75%. The conservative treatment of chylothorax includes pleural drainage and a low-fat diet rich in medium-chain fatty acids, followed by total parenteral nutrition and nothing by mouth. If these measures fail and drainage continues to exceed 1 L/d, surgical thoracic duct ligation is usually recommended. However, many patients are unable to undergo this surgical procedure and require an alternative treatment. We present the cases of 2 adult patients, one of whom developed chylothorax after an elective surgical procedure, and the other after a traffic accident that caused multiple injuries. In both patients, conservative management with the addition of octreotide was successful and negated the need for surgical intervention.
Chylothorax; octreotide; pleural effusion; thoracic duct/surgery; trauma
During a high speed road traffic accident, a 26-year-old man suffered multiple fractures of his thoracic vertebrae and bilateral pneumothoraces. The day after admission and commencement of nasogastric feeding, milky fluid was noted in his right chest drain. Feeding was stopped and a contrast oesophogram and oesophagoscopy were performed, which were normal. The chylothorax quickly resolved and both drains were removed on day 6. Initial treatment of chylothorax aims to decompress the pleural space and minimise chyle production by stopping enteral feeding. Most authors recommend conservative management for 2 weeks or more unless certain parameters are met: average daily chyle loss of >1.5 litres for a 5 day period, or imminent nutritional complications. In this case, surgical management of the chylothorax and spinal fractures was planned. However, conservative management was successful, highlighting the fact that early aggressive surgical intervention for chyle leaks in blunt trauma is not necessary.