Chylothorax after high translumbar aortography.
(9/145)
Two patients who had high translumbar aortograms for suspected arterial disease developed shortness of breath 48 hours after aortography and were found to have chylothoraces. Conservative management was initially adopted for both, but surgery had to be resorted to, although the first patient died before arriving in the operating theatre. The literature and the management of this condition are reviewed. (+info)
Lymphoscintigraphy using (99m)Tc filtered sulfur colloid in chylothorax: a case report.
(10/145)
OBJECTIVE: A 66-y-old man was diagnosed with esophageal carcinoma and underwent a right thoracotomy and esophagectomy. Postoperatively, a recurring right pleural effusion developed. Because an attempt at lymphangiography failed, lymphoscintigraphy was suggested. Because of the inability to obtain radiolabeled albumin, dextran, or nanocolloid, we used filtered sulfur colloid. (0.1 um). The study confirmed the diagnosis of chylothorax. (+info)
Pulmonary lymphangiomyomatosis. A review.
(11/145)
Anatomic and clinical observations of 28 cases, including 23 previously unpublished, of pulmonary lymphangiomyomatosis are recorded and discussed. This brings the total reported to 57. All patients were women in the reproductive age group with the major complaint of breathlessness. This was usually progressive, and death from pulmonary insufficiency resulted within 10 years. Functional changes were obstructive or restrictive, or both. Pneumothorax, chylous effusions and hemoptysis were frequent complications. Radiographically the lesions initially appear as fine, linear and nodular, predominantly basal densities, and progress to a pattern of bullous change, or honeycombing, involving all portions of the lungs not sparing the region of the costophrenic sinuses as is typical of eosinophilic granuloma. There may be associated pleural effusions. A progressively increasing lung volume is characteristic. The lesions consist of an irregular, nodular or laminar "irrational" proliferation of smooth muscle within all portions of the lung, with loss of parenchyma leading to honeycombing. Proliferated muscle can obstruct bronchioles (with air trapping and formation of bullae often complicated by pneumothorax), venules (with pulmonary hemorrhage and hemosiderosis accompanied clinically by hemoptysis) and lymphatics (with chylothorax or chyloperitoneum). Both thoracic and abdominal lymph nodes and the thoracic duct can also be involved in the myoproliferative process with formation of subsidiary minute channels and obstruction. Renal or perirenal angiomyolipomas can also occur, as exemplified by 2 patients in the present series. Identical pulmonary lesions occasionally occur in tuberous sclerosis. Especially since these patients usually have no neurologic disturbances and are almost women, the possibility of a relationship between tuberous sclerosis and lymphangiomyomatosis must be considered. One feature of note in pulmonary lesions of tuberous sclerosis is the presence of adenomatoid proliferations of epithelium. Such changes were also observed in 2 patients of the present series, and it is remarkable that both of these women had "retarded"children. At present the question of whether by lymphangiomyomatosis is a forme fruste of tuberous sclerosis must be considered as unresolved. It may yield to further investigation, possibility including chromosomal studies. (+info)
Net-targeted mutant mice develop a vascular phenotype and up-regulate egr-1.
(12/145)
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. (+info)
Treatment of symptomatic primary chylous disorders.
(13/145)
PURPOSE: Primary chylous disorders (PCDs) are rare. Rupture of dilated lymph vessels (lymphangiectasia) may result in chylous ascites, chylothorax, or leakage of chyle through chylocutanous fistulas in the lower limbs or genitalia. Chyle may reflux through incompetent lymphatics, causing lymphedema. To assess the efficacy of surgical treatment, we reviewed our experience. METHODS: The clinical data of 35 patients with PCDs treated between January 1, 1976, and August 31, 2000, were reviewed retrospectively. RESULTS: Fifteen men and 20 women (mean age, 29 years; range, 1 day-81 years) presented with PCDs. Sixteen (46%) patients had chylous ascites, and 19 (54%) had chylothorax (20 patients), and of these, 10 (29%) had both. In 16 patients, reflux of chyle into the pelvic or lower limb lymphatics caused lymphedema (14, 88%) or lymphatic leak through cutaneous fistulae (11, 69%). Presenting symptoms included lower-limb edema (19, 54%), dyspnea (17, 49%), scrotal or labial edema (15, 43%), or abdominal distention (13, 37%). Primary lymphangiectasia presented alone in 23 patients (66%), and it was associated with clinical syndromes or additional pathologic findings in 12 (yellow nail syndrome in 4, lymphangiomyomatosis in 3, unknown in 3, Prasad syndrome (hypogammaglobulinemia, lymphadenopathy, and pulmonary insufficiency) in 1, and thoracic duct cyst in 1). Twenty-one (60%) patients underwent 26 surgical procedures. Preoperative imaging included computed tomography scan in 15 patients, magnetic resonance imaging in 3, lymphoscintigraphy in 12, and lymphangiography in 14. Fifteen patients underwent 18 procedures for chylous ascites or pelvic reflux. Ten (56%) procedures were resection of retroperitoneal/mesenteric lymphatics with or without sclerotherapy of lymphatics, 4 (22%) were lymphovenous anastomoses or grafts, 3 (17%) were peritoneovenous shunts, and 1 (6%) patient had a hysterectomy. Six patients underwent eight procedures for chylothorax, including thoracotomy with decortication and pleurodesis (4 procedures), thoracoscopic decortication (1 patient), ligation of thoracic duct (2 procedures), and resection of thoracic duct cyst (1 patient). Postoperative mean follow-up was 54 months (range, 0.3-276). Early complications included wound infections in 3 patients, elevated liver enzymes in 1, and peritoneovenous shunt occlusion with innominate vein occlusion in 1. All patients improved initially, but four (19%) had recurrence of symptoms at a mean of 25 months (range, 1-43). Three patients had postoperative lymphoscintigraphy confirming improved lymphatic transport and diminished reflux. One patient died 12 years postoperatively, from causes unrelated to PCD. CONCLUSIONS: More than half of the patients with PCDs require surgical treatment, and surgery should be considered in patients with significant symptoms of PCD. Lymphangiography is recommended to determine anatomy and the site of the lymphatic leak, especially if lymphovenous grafting is planned. All patients had initial benefit postoperatively and two thirds of patients demonstrated durable clinical improvement after surgical treatment. (+info)
A case of successful fetal therapy for congenital chylothorax by intrapleural injection of OK-432.
(14/145)
A 38-year-old multiparous woman was referred at 19 weeks' gestation because of hydrops fetalis. Ultrasonic examination revealed severe pleural effusion, ascites and skin edema. Detailed examination of the amniotic fluid, fetal blood and intrathoracic fluid led to a diagnosis of congenital fetal chylothorax. Repeated thoracocenteses were not effective in improving the hydrops fetalis. We introduced fetal treatment for the pleural effusion by an intrapleural injection of OK-432 at 23, 24 and 25 weeks' gestation. The pleural effusion was reduced by adhesion of the intrathoracic space and resulted in the delivery of a neonate who was healthy except for right renal dysfunction. Pulmonary hypoplasia was successfully prevented by OK-432. (+info)
A new fetal therapy for chylothorax: pleurodesis with OK-432.
(15/145)
We present a case of pleurodesis by intrapleural injection of OK-432 for the treatment of fetal chylothorax at an early gestational age. OK-432 injection achieved rapid and effective control of pleural effusion with no adverse effects. (+info)
The lungs in lymphangiomyomatosis and in tuberous sclerosis.
(16/145)
Two cases of pulmonary lymphangiomyomatosis (PL) are described and 33 other cases from the literature are reviewed. These are compared with one case of tuberous sclerosis with pulmonary involvement (PTS) and 32 other cases from the literature. There are no differences in lung function between these two conditions, both of which show airways obstruction associated with diffuse radiological lung changes. There are, however, both clinical and radiological differences and also differences in the distribution of the lesions and the histological location of the excessive smooth muscle; these indicate that PL and PTS are probably different entities and not polar forms of one condition. Finally, the strictly female incidence of PL suggests a sex-linked disorder, and it is postulated that this may be related to congenital pulmonary lymphangiectases. (+info)