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Congenital chylothorax: lymphopenia and high risk of neonatal infections.

AIM: To describe the clinical course of patients with congenital chylothorax focusing on infectious complications. Congenital chylothorax is a common manifestation of non-immune hydrops fetalis (NIHF). The drainage of chyle leads to loss of cellular and plasmatic factors that influence the patient's immune response and increase the risk of infections. METHODS: In a retrospective analysis of 24 preterm infants with NIHF treated between 1998 and 2002, congenital chylothorax was diagnosed in 7 patients. RESULTS: All 7 patients were treated conservatively with pleural drainage over a median period of 22 d (range 10-36 d). Lymphopenia was found in all patients (median of minimal lymphocyte counts 285/microl, range 80-770). The nadir was on day 5 (2-6 d). Lymphopenia lasted for 12 d median (range 4-39 d) and was significantly correlated with the duration of lymph drainage (p = 0.001). Cell-surface analysis of peripheral blood lymphocytes was performed in two patients. Both patients had a decreased number of total T cells. Four out of seven (57%) patients developed nosocomial infections. This incidence of nosocomial infections in patients with congenital chylothorax is about three times higher than that in other neonatal patients. None of the children suffered from fungal or viral infection. Although there was a very high incidence of infections, no correlation between lymphopenia and the occurrence of infections could be shown. CONCLUSION: Drainage of congenital chylothorax results in the loss of lymphocytes and bears a high risk of infections.

Bacterial Infections↗

Intractable congenital chylous ascites.

UNLABELLED: A male newborn presented with congenital chylous ascites, the treatment of which is difficult and complicated due to persistent loss of chyle. The primary therapeutic goal is to reduce the lymph flow and to allow the leakage to heal naturally. Usually, conservative management--enteric rest with total parental feeding or an enteral diet with medium-chain triglycerides--can achieve this. A new treatment option is the use of octeotride, a somatostatin analogue. Both therapies failed in our patient, and surgical ligation of the leaking lymphatic duct was successful. CONCLUSION: The success of the surgical treatment depends on localizing the leaking lymphatic duct. Lymphatic imaging with lymphoscintigraphy and pre-operative administration of lipophilic dye can assist the surgeon.

Chylous Ascites↗

Intrapleural bleomycin in the treatment of chylothorax.

Chylothorax is an accumulation of thoracic lymph or chyle in the pleural cavity. It is a rare condition and is usually caused by trauma or malignant disease. We present three cases with chylothorax due to malignant non-Hodgkin's lymphoma [high grade malignant (1 case) and low grade malignant (2 cases)] treated with pleurodesis with bleomycin and systemic chemotherapy (CHOP, CNOP, trofosfamide). Complete remissions (CR) were achieved in all three cases. Two patients had a recurrent chylothorax 3 and 12 months after initial treatment. They were treated with a second intrapleural installation of bleomycin and continuing systemic chemotherapy (CNOP, trofosfamide) and are still alive in CR with a follow-up period of 28 and 30 months respectively. One patient died of relapsing non-Hodgkin's lymphoma after 23 months of follow-up. There was no sign of recurrent chylothorax. We conclude that chylothorax caused by lymphoma can be satisfactorily controlled by pleurodesis with bleomycin combined with systemic chemotherapy. Immediate action is necessary to prevent great loss of lipids and proteins. The underlying malignancy must be controlled to achieve a good prognosis.

Antineoplastic Agents↗

Chylothorax and chylous ascites due to malignant lymphoma.

Chyle is a fluid rich in triglycerides and is characterized by the presence of chylomicrons. Chylous effusions are unusual complications of malignant neoplasms, usually lymphomas. The combination of chyloperitoneum and chylothorax is very rare. When abdominal lymphatics are obstructed, chylous ascites results and eventually leads to a chylothorax. We present the case of a 68-year-old woman with a chyloperitoneum and a right-sided chylothorax due to an underlying malignant B-cell lymphoma. After thoracocentesis and replacement therapy with medium chain triglycerides, she was treated with a combination of cyclophosphamide, vincristine and prednisone. This has resulted in a regression of the chylous effusions. A short review of the literature describes causes, diagnosis and therapy of chylous effusions.

Aged↗

The identification of chylomicra and lipoproteins in tissue sections and their passage into jejunal lacteals.

The electron microscopic appearances of chylomicra and lipoproteins have been investigated. The particles were isolated from rat chyle by differential flotation in an ultracentrifuge. Various fixing and embedding media were used. The two kinds of particles were then identified in thin sections of the jejunum of rats. The chylomicra had diameters of from 1,000 A to 1 micro; the lipoproteins ranged from 100 to 1,000 A. They were identified by their sizes and their similarities to the isolated particles after the various fixing and embedding procedures. In addition, the relative amounts of the two kinds of particle varied greatly under different dietary conditions. The chylomicra had a thin rim, probably of phospholipid. Section B records the passage of the two kinds of particle into the lacteals in the villi of the jejunum. Both chylomicra and lipoproteins were seen passing through many open junctions. From permeability considerations it would seem that this is the most important route. These open junctions appear to act as "inlet valves" which prevent backflow as the contractions of the villi pump material out of the lacteals. Both chylomicra and lipoproteins were also seen entering the endothelial cells and lying inside them. The lipoproteins entered via "normal" caveolae and were seen in "normal" vesicles ( approximately 500 A); the chylomicra necessarily occupied much larger organelles. Both kinds of particles were also seen in caveolae on the luminal surface of the endothelium, but it was impossible to be certain that these were not just particles entering the cells from the lumen. The chylomicra often seemed to be washed out of these caveolae as many large, empty ones were seen on the luminal sides of the cells. Frequently, these caveolae had dark membranes.

Animals↗

Thoracoscopic management of chylothorax complicating esophagectomy.

BACKGROUND: Chylothorax is a relatively uncommon complication of esophageal surgery that may lead to severe respiratory, nutritional, and immunologic deficiencies. PATIENTS AND METHODS: Between 1992 and 2000, 3 of 316 patients (0.9%) undergoing transthoracic esophagectomy for carcinoma developed postoperative chylothorax. Two of them had previously been treated with neoadjuvant chemoradiation, and one had been submitted to esophagogastric resection through a left thoracotomy. After a 2-week trial of total parenteral nutrition and drainage, two patients underwent thoracic duct ligation via thoracotomy. In the last patient, the operation was completed by thoracoscopy. The azygos vein and the periaortic tissue above the diaphragm were encircled en bloc by a right-angled clamp, and a roticulating endostapler was applied. RESULTS: Reoperation was successful in all patients. The postoperative hospital stay was 4 days. CONCLUSION: Thoracoscopy is a safe and effective procedure for the treatment of chylothorax complicating esophagectomy. Given the minimal trauma to the patient, early thoracoscopic reoperation can be advocated in patients with high-output chyle loss in order to reduce the hospital stay.

Adenocarcinoma↗

Laparoscopic ligation of the thoracic duct in management of chylothorax.

Laparoscopic ligation of the cisterna chyli at the level of the aortic hiatus was performed in a 69-year-old woman with post-lobectomy chylothorax refractory to 3 weeks of conservative therapy and one repeat thoracotomy with attempted ligation of a leaking lymphatic channel. This laparoscopic procedure was successful, and resolution of the chylothorax was achieved. We feel that this technique offers surgeons a valid, minimally invasive treatment option for a persistent chylothorax in which conservative management or more direct thoracic procedures have failed to control the chyle leak.

Aged↗

Net-targeted mutant mice develop a vascular phenotype and up-regulate egr-1.

The ternary complex factors (TCFs) Net, Elk-1 and Sap-1 regulate immediate early genes through serum response elements (SREs) in vitro, but, surprisingly, their in vivo roles are unknown. Net is a repressor that is expressed in sites of vasculogenesis during mouse development. We have made gene-targeted mice that express a hypomorphic mutant of Net, Net delta, which lacks the Ets DNA-binding domain. Strikingly, homozygous mutant mice develop a vascular defect and up-regulate an immediate early gene implicated in vascular disease, egr-1. They die after birth due to respiratory failure, resulting from the accumulation of chyle in the thoracic cage (chylothorax). The mice have dilated lymphatic vessels (lymphangiectasis) as early as E16.5. Interestingly, they express more egr-1 in heart and pulmonary arteries at E18.5. Net negatively regulates the egr-1 promoter and binds specifically to SRE-5. Egr-1 has been associated with pathologies involving vascular stenosis (e.g. atherosclerosis), and here egr-1 dysfunction could possibly be associated with obstructions that ultimately affect the lymphatics. These results show that Net is involved in vascular biology and egr-1 regulation in vivo.

Animals↗

The lymphedema chaos: a lancet.

Pathophysiology of lymphedema is not an enigma. It is caused by a low-output failure of the lymph vascular system in combination with an inadequate scavenging of stagnating plasma protein by macrophages. Axillary venous diseases alone never cause chronic postmastectomy edema. In the diagnosis of lymphedema, invasive methods (i.e., direct lymphography and venography) are not only unnecessary but are potentially harmful and do not give any information of therapeutic relevance. Lymphedema of the limbs without reflux of lymph or chyle is not a surgical disease. It can be treated successfully by the skillful application of specific physiotherapeutic measures free of any side effect. The results of this therapy can be maintained if the patient's compliance is good.

Blood Proteins↗

Chylous peritonitis in children: case report and literature review.

Sixty-one cases of acute chylous peritonitis have been reported in all age groups in the surgical literature. Only seven of these cases have been noted in children. We report an additional case of a 7-year-old child who presented with acute peritoneal irritation and, at laparotomy, was found to have chyle in the peritoneal cavity and retroperitoneum. Proposed etiologies and management of this surgical entity are reviewed.

Acute Disease↗

Safety of modified radical neck dissection for differentiated thyroid carcinoma.

OBJECTIVES/HYPOTHESIS: The management of cervical metastases from differentiated thyroid carcinoma (DTC) remains controversial. Most surgeons perform a neck dissection (ND) for clinically apparent disease. The extent of nodal dissection varies from regional to comprehensive. Morbidity from ND in the setting of DTC remains high, particularly when performed in the setting of a thyroidectomy (TT). To determine complications from ND for DTC, we retrospectively reviewed our surgical experience of modified radical neck dissection for nodal metastases. STUDY DESIGN: Retrospective chart review. METHODS: Between 1997 and 2002, 39 consecutive patients (31 females and 8 males) underwent 44 comprehensive NDs of levels II-V for DTC. Central compartment dissection (CCD) (levels VI and VII) was also performed during 23 of these procedures. Twenty (45.5%) patients had prior treatment elsewhere. Preoperative pathology revealed papillary carcinoma in 22 patients (56.4%), tall cell variant in 11 (28.2%), and follicular variant in 6 (15.4%). RESULTS: Ten patients (20%) underwent ND alone, whereas 6 (14%) underwent simultaneous ND and TT. Fifteen patients underwent simultaneous ND, TT, and CCD (30%). Temporary hypocalcemia occurred after 21% of NDs that were performed in the setting of either TT or CCD or both. There were no cases of permanent hypoparathyroidism. Transient regional lymph node (RLN) paresis occurred in two patients and was associated with a concomitant central compartment nodal dissection; there were no permanent RLN palsies. Transient spinal accessory nerve paresis developed after 27% of NDs performed. Two patients developed chyle leaks. CONCLUSIONS: When ND is necessary for the treatment of thyroid malignancies, the procedure can be performed safely with acceptable morbidity.

Adolescent↗

Lymphatic malformation causing intractable chylorrhagia.

Lymphatic malformation is a developmental error usually noticed at birth or in early childhood. Lesions of the upper leg and lower trunk are the most difficult to remove, because they are often the largest ones encountered and also because they tend to extend proximally into the retroperitoneal tissues. Chyle reflux, usually associated with lymphedema of the extremity, has not been reported to be caused by lymphatic malformation. We report a case of intrapelvic retroperitoneal lymphatic malformation with an extension of gluteal-thigh soft-tissue involvement causing intractable chylorrhagia. The tumor was subtotally excised, and the defect was closed by a distally based, peninsular latissimus dorsi myocutaneous flap. The flap served both as a filling material and as a "bridge" between the residual tumor, including abnormal lymphatics, and normal lymph flow.

Adolescent↗

Planned neck dissection after concomitant radiochemotherapy for advanced head and neck cancer.

OBJECTIVES/HYPOTHESIS: Since 1998, at our academic, multidisciplinary head and neck cancer treatment center, it has been our policy to treat appropriate patients with locoregionally advanced squamous cell carcinoma of the head and neck (SCCHN) with concomitant radiochemotherapy followed within 6 weeks by planned neck dissection(s). Our objective was to investigate the oncologic efficacy of planned neck dissection, to date, in this patient population with a focus on outcomes in the neck. STUDY DESIGN: Retrospective analysis of a cumulative patient database. METHODS: The medical records of all patients who underwent planned neck dissection(s) after concomitant radiochemotherapy for locoregionally advanced SCCHN at Beth Israel Medical Center and The Institute for Head and Neck Cancer in New York City were reviewed. For each patient, preradiochemotherapy primary and neck stage, postradiochemotherapy/preneck dissection clinical and radiographic neck status, type of neck dissection(s) performed, pathologic status of the neck dissection specimen(s), length of follow-up (after planned neck dissection), disease status at last follow-up, and site(s) of recurrence were recorded. Local, regional, and distant disease control rates were calculated by the Kaplan-Meier method. RESULTS: Fifty-one planned neck dissections were performed on 39 radiochemotherapy patients (12 patients had bilateral operations) between early 1998 and October, 2003. Thirty-two (82%) patients had N2 or greater neck disease, with 29 (74%) having T3/T4 disease at various upper aerodigestive tract primary sites. Patients received an average of 6,700 cGy and 6,000 cGy external beam radiation therapy to primary disease sites and involved cervical lymphatics respectively, concomitant with one of three platinum-based chemotherapy schedules. At a mean follow-up time of 24 (range 8-57) months for the entire study population, there has been only one neck recurrence (N2A neck). No patient with N2B (n = 11), N2C (n = 13, with majority of heminecks staged N2B), or N3 (n = 5) disease has recurred in the neck. No recurrences have occurred in the 41 heminecks (in 33 patients) where modified neck dissection (including 24 selective procedures) was performed despite the presence of residual carcinoma in 13 (32%) of these heminecks on pathologic review. Among all heminecks with residual carcinoma present (n = 18) in the neck dissection specimen, there has been only one neck recurrence. There have been no recurrences in the 26 heminecks (in 19 patients) with incomplete clinical response after radiochemotherapy despite the presence of residual carcinoma in 14 (54%) of these necks on pathologic review. The clinical and radiographic absence of residual disease after radiochemotherapy did not always predict a complete pathologic response. Surgical complications have been limited (1 chyle leak, 1 wound breakdown). CONCLUSIONS: The integration of planned neck dissection into the multidisciplinary management of patients with locoregionally advanced SCCHN is highly effective in controlling cervical metastatic disease. Modified and selective neck dissection procedures can be performed in the majority of patients, regardless of the response in the neck subsequent to concomitant radiochemotherapy. We recommend a planned neck dissection(s) in all patients staged (pretreatment) with N2 or greater neck disease and in select N1 cases.

Carcinoma, Squamous Cell↗

Chylothorax as a result of chronic lymphocytic leukemia: case report and review of the literature.

Chylothorax occurs when a disruption in the thoracic duct allows chyle to escape into the pleural space. The most commonly reported cause is malignancy, especially lymphoma. However, chylothorax caused by chronic lymphocytic leukemia is rarely reported in the literature. We describe a patient who developed chylothorax secondary to chronic lymphocytic leukemia. In addition, the pathogenesis, diagnosis, and treatment of chylothorax are reviewed.

Aged↗

Successful management of lymphangiomatosis and chylothorax in a 7-month-old infant.

A 7-month old infant presented with an effusion and multiple lesions in his spleen. A diagnosis of lymphangiomatosis was made based on chylous effusion and an MRI demonstrating numerous enhancing lesions within the spleen on T2-weighed images. Conservative measures, including the withdrawal of feeds and octreotide, did not significantly reduce the rate of chyle production and increasing requirement for respiratory support. Resection of the patient's spleen and partial pleurectomy were associated with a dramatic improvement in his condition. At 27 months the patient was well with no evidence of further lymphangiomas or a recurrence of his chylothoraces.

Chylothorax↗

The effect of a non-ionic detergent (Triton WR 1339) on the removal of triglyceride fatty acids from the blood of the rat.

1. The initial rate of removal from the circulation of injected [(14)C] labelled chylomicron triglyceride is much slower in rats that have been injected with the non-ionic detergent, Triton WR 1339, than in normal animals. Pre-incubation of the lipid with Triton before its injection causes a further reduction in removal rate.2. After the injection of [(14)C]labelled chyle triglyceride into animals given Triton, the rate of disappearance of radioactivity falls markedly as the percentage of the labelled material remaining in the blood declines.3. A much smaller decrease in the rate of removal of injected chylomicron triglyceride occurs with increase in the size of the injected dose and with increase in the time interval between Triton and chylomicron triglyceride injections.4. Serum triglycerides labelled with (14)C are removed from the circulation of rats that have been given Triton at a much slower rate than [(14)C] labelled chylomicron triglycerides.

Animals↗

[Evaluation of a PCR assay commercial kit for quantification of human immunodeficiency virus type-1 RNA].

We studied a basic evaluation of the reliability and usefulness of the test results to assess the validity of the kit "AMPLICOR HIV-1 Monitor" as a laboratory tool, by determining the reproducibility, linearity on dilution, possible effects of interference on the results, and correlation with the results obtained at outside facilities. Furthermore, we compared the HIV-1 RNA load between blood samples obtained from HIV-1 subtype B and E. The HIV-1 RNA load measurement was made according to the pre-determined methods of this kit, in blood samples obtained from HIV-positive outpatients. Simultaneous reproducibility was 23.08%-32.95% in C.V.% and linearity was maintained between 110 copies/ml and 2,184,277 copies/ml, demonstrating favorable performance of the kit. The institution correlation between two facilities were also favorable. Fluctuation of measurement by interference was absent for bilirubin, hemoglobin and chyle, but was significant for heparin.

Evaluation Studies as Topic↗

Intestinal lymphangiectasia. Long-term results with MCT diet.

The clinical course of 6 children with primary intestinal lymphangiectuasia who have been treated with low fat medium chain triglyceride-supplemented diets for between 3 and 8 years (4 for longer than 5 years) is described. Though laboratory findings indicate continuing chyle leak, evidence for long-term benefit from dietary treatment is provided by symptomatic relief while on the diet, clinical relapse upon relaxation of t,e regimen, and improvement in growth rates. In most patients the underlying lymphatic defect, and thus the need for dietary treatment, appears to be permanent.

Body Height↗