Microsurgical LVA have a place in the treatment of peripheral lymphedema, and should be the therapy of choice in patients who are not sufficiently responsive to nonsurgical treatment.
Authors report over 30 years of their own clinical experience in the treatment of chronic peripheral lymphedemas by microsurgical techniques performed at the Center of Lymphatic Surgery of the University of Genoa, Italy. Over 1,500 lymphedema patients were treated with microsurgical techniques. Derivative lymphatic-venous techniques were most often used. For those cases where a venous disease was associated to lymphedema, reconstructive lymphatic microsurgery techniques were performed (lymphatic-venous-lymphatic-plasty). Objective assessment was undertaken by water volumetry and lymphoscintigraphy. Volume changes showed a significant improvement in over 83%, with an average follow-up of more than 10 years. There was an 87% reduction in the incidence of cellulitic attacks after microsurgery. Microsurgical lymphatic-venous anastomoses have a place in the treatment of peripheral lymphedema and should be the therapy of choice in patients who are not sufficiently responsive to nonoperative treatment. Improved results can be expected with operations performed at earlier lymphedema stages.
Congenital pulmonary lymphangiectasia (PL) is a rare developmental disorder involving the lung, and characterized by pulmonary subpleural, interlobar, perivascular and peribronchial lymphatic dilatation. The prevalence is unknown. PL presents at birth with severe respiratory distress, tachypnea and cyanosis, with a very high mortality rate at or within a few hours of birth. Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. Secondary PL may be caused by a cardiac lesion. The diagnostic approach includes complete family and obstetric history, conventional radiologic studies, ultrasound and magnetic resonance studies, lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy, and pleural effusion examination. During the prenatal period, all causes leading to hydrops fetalis should be considered in the diagnosis of PL. Fetal ultrasound evaluation plays a key role in the antenatal diagnosis of PL. At birth, mechanical ventilation and pleural drainage are nearly always necessary to obtain a favorable outcome of respiratory distress. Home supplemental oxygen therapy and symptomatic treatment of recurrent cough and wheeze are often necessary during childhood, sometimes associated with prolonged pleural drainage. Recent advances in intensive neonatal care have changed the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive infancy, present medical problems which are characteristic of chronic lung disease.
Disease name and synonyms
Pulmonary lymphangiectasiaPulmonary cystic lymphangiectasis
Pulmonary lymphangiomatosis
Definition and diagnostic criteriaCongenital pulmonary lymphangiectasia (PL) is a rare developmental disorder involving the lung and is characterized by pulmonary subpleural, interlobar, perivascular, and peribronchial lymphatic dilatation. On the basis of improved characterization of the clinical presentation and recent noteworthy advances in intensive neonatal care, the original classification [1] has been modified and PL
Primary chylous ascites is closely correlated to lymphatic-lymphonodal dysplasia that does not involve a single visceral district alone. Medical preoperative treatment played an essential role in the global management of this complex pathology. We demonstrated that the use of laparoscopy is remarkably advantageous for confirming diagnosis, for draining the ascites, and for evaluating the extension of the dysplasia. Our diagnostic work-up provided us with an exact diagnostic assessment and allowed us to plan a precise surgical approach.
Over the past 25 years, 665 patients with obstructive lymphedema have been treated with microsurgical lymphatic-venous anastomoses; of these, 446 patients were available for long-term follow-up study. Objective assessment was undertaken by water volumetry and lymphoscintigraphy. Lymphangioscintigraphy, lymphangiography (in patients with gravitational reflux pathology), and echo-Doppler were used preoperatively. Subjective improvement was noted in 578 patients (87%). Objectively, volume changes showed a significant improvement in 552 patients (83%), with an average reduction of 67% of the excess volume. Of those patients followed up, 379 patients (85%) have been able to discontinue the use of conservative measures, with an average follow-up of more than 7 years and average reduction in excess volume of 69%. There was a 87% reduction in the incidence of cellulitis after microsurgery. In those patients who improved, drainage resulted in increased softness of the limbs. Peripheral edema (hand and foot) diminished considerably in most patients. These long-term results indicate that lymphatic-venous anastomoses have a place in the treatment of obstructive lymphedema and should be the therapy of choice in patients who are not sufficiently responsive to nonsurgical treatment. Improved results can be expected with earlier operations because patients referred earlier usually have fewer lymphatic alterations.
The LYMPHA technique appears to be feasible, safe, and effective for the prevention of lower limb lymphedema, thereby improving the patient's quality of life and decreasing health care costs.
This study shows for the first time the feasibility of LVA in patients with vulvar cancer undergoing ILND. Future studies including larger series of patients should clarify whether this microsurgical technique reduces the incidence of LLL after ILND.
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