Familial congenital non-immune hydrops, chylothorax, and pulmonary lymphangiectasia.
(41/145)
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 lymphangiectasia (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 vascular endothelial growth factor receptor-3 (VEGFR3) in families with Milroy disease, mutations of FOXC2 in the lymphedema-distichiasis syndrome, and fatal chylothorax in alpha9-deficient mice are potential candidate genes. (+info)
Thoracic duct variations may complicate the anterior spine procedures.
(42/145)
The aim of this study is to localize and document the anatomic features of the thoracic duct and its tributaries with special emphasis on the spinal surgery point of view. The thoracic ducts were dissected from nine formaldehyde-preserved male cadavers. The drainage patterns, diameter of the thoracic duct in upper, middle and lower thoracic segments, localization of main tributaries and morphologic features of cisterna chyli were determined. The thoracic duct was detected in all cadavers. The main tributaries were concentrated at upper thoracic (between third and fifth thoracic vertebrae) and lower thoracic segments (below the level of ninth thoracic vertebra) at the right side. However, the main lymphatic tributaries were drained into the thoracic duct only in the lower thoracic area (below the level of the tenth thoracic vertebra) at the left side. Two major anatomic variations were detected in the thoracic duct. In the first case, there were two different lymphatic drainage systems. In the second case, the thoracic duct was found as bifid at two different levels. In formaldehyde preservation, the dimensions of the soft tissues may change. For that reason, the dimensions were not discussed and they may not be a guide in surgery. Additionally, our study group is quite small. Larger series may be needed to define the anatomic variations. As a conclusion, anatomic variations of the thoracic duct are numerous and must be considered to avoid complications when doing surgery. (+info)
Postoperative chylothorax in patients with a thoracic aortic aneurysm.
(43/145)
OBJECTIVES: Postoperative chylothorax in patients with a thoracic aneurysm is generally infrequent. We report a mode of surgery to reduce the incidence of paraplegia. We review our experience with chylothorax after resection of an aneurysm to find its cause and to evaluate the success of management. PATIENTS AND METHODS: For descending thoracic aneurysms, intercostal arteries in the aneurysm were exposed before incising the aneurysm and, they were only sacrificed no change in motor-evoked potentials (MEPs) occurred after temporary occlusion. Between January 2001 and December 2003, out of a total of 147 aneurysms including thoracic and thoracoabdominal, 4 consecutive patients (2.7%) with chylothorax were reviewed. RESULTS: The chylothorax was diagnosed 1.5 days after operation (range 1 to 2 days). All patients were initially treated by the cessation of oral intake. This treatment was successful for 2 patients and the remaining 2 required surgical intervention to control the fistula. Chylothorax was cured in all patients. CONCLUSION: We postulate that chylothorax is caused by injury of the thoracic duct or its branch during the exposing the intercostal arteries. In the management of chylothorax, early intervention is recommended when the volume of chylous fluid is not decreased by conservative treatment. (+info)
Successful octreotide treatment of chylothorax following coronary artery bypass grafting procedure. A case report and review of the literature.
(44/145)
Chylothorax occurs in 0.25 to 0.50% of cardiac operations performed through thoracotomy incisions and is more unusual after median sternotomy. A case of chylothorax following coronary artery bypass grafting is presented. Combined treatment with pleural drainage, "nothing per os", total parenteral nutrition and subcutaneous injection of somatostatin was effective and led to rapid cessation of chyle production. (+info)
Modified and bilateral retroperitoneal lymph node dissection for testicular cancer: peri- and postoperative complications and therapeutic outcome.
(45/145)
OBJECTIVE: To characterize surgical invasiveness and morbidity and to verify therapeutic efficacy, we reviewed perioperative and postoperative courses and therapeutic outcomes of patients who underwent retroperitoneal lymph node dissection (RPLND) for testicular germ cell cancers. METHODS: The study included 31 patients who underwent retroperitoneal lymph node dissection. A modified template was used if retroperitoneal metastasis was limited to the areas below the level of the renal hilus and above the level of the inferior mesenteric artery (IMA). Perioperative and postoperative courses and complications were reviewed as well as therapeutic outcomes. RESULTS: Overall, 27 perioperative and postoperative complications were observed in 15 patients (48.4%). Superficial surgical site infection and paralytic ileus were seen most frequently. All of them were resolved without special additional treatments; however, patients who underwent retrocrural dissection had a tendency to have severe symptomatic complications such as chylothorax, phrenic nerve palsy and orthostatic hypotension. Antegrade ejaculation was preserved in 94.1% of patients with modified template dissection, whereas no patients with additional dissection below the level of the IMA had the function preserved. One patient (3.2%) developed a postoperative recurrent disease in the retroperitoneum, which was outside the dissection field, as well as in the mediastinum. CONCLUSIONS: Although RLND had high morbidity, most peri- and postoperative complications were manageable conservatively. Modified template dissection enabled patients to preserve antegrade ejaculation without compromising its therapeutic efficacy, if the disease extension allowed us to use the template. (+info)
Bilateral agenesis of the superior vena cava associated with congenital hydrothorax.
(46/145)
Agenesis of the superior vena cava is a rare anomaly that is generally asymptomatic in the neonate. We report a male neonate with bilateral (total) agenesis of the superior vena cava with obstructed thoracic duct and subsequent congenital hydrothorax, anomalies that were detected by prenatal ultrasound at 25 weeks' gestation. The cardiac anomaly was confirmed by postnatal magnetic resonance angiography. The chylothorax disappeared with conservative therapy. (+info)
Massive unilateral chylous pleural effusion: a rare initial presentation of Behcet's disease.
(47/145)
Pulmonary manifestations of Behcet's disease are not very common and usually include pulmonary artery aneurysms, central venous thrombosis, pneumonia and pleurisy. Chylothorax secondary to superior vena caval obstruction is a rare complication and has been reported in only a few cases. We report a case of a 24-year-old man presenting with massive chylothorax as the initial presentation of Behcet's disease that was successfully treated conservatively. (+info)
Platelet-derived growth factor receptor-beta in Gorham's disease.
(48/145)
BACKGROUND: A 17-year-old male presented with pain in his lower-left chest. He had no significant medical history and was previously in good health. He had a fractured ninth left anterior rib and the tenth, eleventh and twelfth ribs were absent, which was thought to be a congenital anomaly. Several months later, he presented again with back pain, an enlarging mass in the lower-left chest wall, erosion of the lateral pedicles of the lower thoracic vertebrae and pleural effusion. INVESTIGATIONS: Physical examination, chest X-ray, MRI of the spine, incisional biopsy, serial CT imaging of the hemithorax, immunohistochemistry, flow cytometry, and enzyme-linked immunosorbent assays. DIAGNOSIS: Gorham's lymphangiomatosis with expression of platelet-derived growth factor receptor-beta and elevated circulating platelet-derived growth factor-BB. MANAGEMENT: Spine stabilization, thalidomide, celecoxib, interferon-alpha2b, pamidronate, zoledronate, thoracotomy, pleurectomy, talc pleurodesis, and imatinib mesylate. (+info)