Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico / Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente.Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5-4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalida

Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood.A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization.Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.

[Unilateral congenital pulmonary lymphangiectasia in a preterm infant]. / Linfangiectasia pulmonar congénita unilateral en un neonato prematuro.

Unilateral congenital pulmonary lymphangiectasia (CPL) is an extremely rare disease of the pulmo nary lymphatic vessels. OBJECTIVE: to present a case of CPL in a premature newborn. CLINICAL CASE: premature male newborn with severe respiratory failure at 2 hours of extrauterine life was treated with exogenous surfactant, catecholamines and high frequency oscillatory ventilation (HFOV). Chest computed tomography (CT) scan showed bullae and air trapping of the left lung; the histopathological study showed cystic dilation of the bronchoalveolar lymphatic channels. The diagnosis of secondary unilateral CPL was made. The clinical course up to 19 months of age was normal and the chest CT scan showed few emphysematous bullae. CONCLUSIONS: CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress. The prognosis will depend on the type of CPL and lung involvement.

Linfangiectasia pulmonar congénita unilateral en un neonato prematuro / Unilateral congenital pulmonary lymphangiectasia in a preterm infant

La linfangiectasia pulmonar congénita (LPC) unilateral es una enfermedad extremadamente rara de los vasos linfáticos pulmonares. OBJETIVO: presentar un caso de LPC en un recién nacido prematuro. CASO CLÍNICO: recién nacido masculino, prematuro, con insuficiencia respiratoria severa a las 2 horas de vida extrauterina, recibió tratamiento con surfactante exógeno, catecolaminas y ventilación de alta frecuencia oscilatoria (VAFO). La tomografía axial computarizada (TAC) de tórax reveló bulas y atrapamiento de aire de pulmón izquierdo, el estudio histopatológico describió dilatación quística de los canales linfáticos broncoalveolares. Se diagnosticó LPC unilateral secundaria. La evolución clínica hasta los 19 meses de edad fue normal y la TAC de tórax mostró escasas bulas enfisematosas. CONCLUSIONES: La LPC debe ser uno de los diagnósticos diferenciales en neonatos con dificultad respiratoria inexplicable. El pronóstico dependerá del tipo de LPC y de la afectación pulmonar.

Unilateral congenital pulmonary lymphangiectasia (CPL) is an extremely rare disease of the pulmo nary lymphatic vessels. OBJECTIVE: to present a case of CPL in a premature newborn. CLINICAL CASE: premature male newborn with severe respiratory failure at 2 hours of extrauterine life was treated with exogenous surfactant, catecholamines and high frequency oscillatory ventilation (HFOV). Chest computed tomography (CT) scan showed bullae and air trapping of the left lung; the histopathological study showed cystic dilation of the bronchoalveolar lymphatic channels. The diagnosis of secondary unilateral CPL was made. The clinical course up to 19 months of age was normal and the chest CT scan showed few emphysematous bullae. CONCLUSIONS: CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress. The prognosis will depend on the type of CPL and lung involvement.

Congenital pulmonary lymphangiectasia: CT and pathologic findings.

Congenital pulmonary lymphangiectasia is a rare disease characterized by dilation of lymphatic channels without lymphatic proliferation. The disease is seen almost exclusively in infancy and early childhood. The authors report 2 cases of pulmonary lymphangiectasia. The patients were a 12- and a 25-year-old male who presented with progressive dyspnea and hemoptysis. The diagnosis was confirmed by open lung biopsy. The radiographic findings consisted of bilateral reticular changes, peribronchial cuffing, and bilateral pleural effusions. High-resolution CT demonstrated extensive bilateral septal and peribronchovascular interstitial thickening, areas of ground-glass attenuation, and bilateral pleural effusions. The histologic findings consisted of ectatic and tortuous lymphatic channels in the interlobular septa, bronchovascular sheaths, and pleura.

Linfangiectasia pulmonar congénita / Congenital pulmonary lymphangiectasis

Se presenta el caso de un recién nacido de término, sexo femenino, de 3.380 g de peso al nacer, producto de un embarazo fisiológico de madre sana, que evolucionó desde el primer minuto de vida con insuficiencia respiratoria y respuesta parcial a ventilación con presión positiva y oxígeno. Una radiografía de tórax mostró sombras pulmonares difusas sin cardiomegalia, sospechándose un drenaje venoso pulmonar anómalo total obstructivo, siendo descartado por ecocardiografía. Los gases arteriales demostraron hipoxemia y acidosis metabólica; las radiografías posteriores mostraron hiperinsuflación pulmonar con sombras reticulonodulares difusas. Se planteó el diagnóstico de linfangiectasia pulmonar congénita y se mantuvo el tratamiento para hipertensión pulmonar con ventilación mecánica, drogas vasoactivas y bicarbonato endovenoso, pese a lo cual presentó deterioro hemodinámico progresivo, falleciendo a las 13 horas de vida. La autopsia confirmó el diagnóstico de linfangiectasia pulmonar congénita. Se presenta el caso clínico con sus hallazgos radiológicos, anatomopatológicos y una breve revisión del tema

Linfangiectasia pulmonar congénita / Congenital pulmonary lymphangiectasis

Presentamos el caso de un recién nacido con linfangiectasia pulmonar congénita no asociada a otras malformaciones congénitas, que falleció dentro de las primeras 24 horas de vida, destacando los aspectos clínicos, radiológicos y anatomopatológicos

[Congenital pulmonary lymphangiectasis]. / Linfangiectasia pulmonar congénita.

Congenital pulmonary lymphangiectasis is a rare cause of severe respiratory distress in the newborn period and most of these patients die, usually within the first 24 hours of life. It may present as an isolated anomaly or it may be associated with other congenital abnormalities, most commonly cardiac in origin, as is the case of total obstruction of the pulmonary venous drainage. Congenital pulmonary lymphangiectasis may be also associated with lymphatic anomalies of other portions of the body or with other non-lymphatic, non-cardiac congenital malformations. Chest radiographs show more commonly that the lungs are hyperexpanded and present a diffuse interstitial reticulonodular pattern. The differential diagnosis from the radiological standpoint should include total anomalous pulmonary venous drainage and pneumonia. We present the case of a newborn infant with congenital pulmonary lymphangiectasis who died within the first 24 hours of life, emphasizing clinical, radiological and pathological findings.

Derrame pericárdio devido à linfangectasia pulmonar / Pericardial effusion due to pulmonary lymphangectasia

Descreve-se um caso de linfangectasia pulmonar congênita, cuja primeira manifestaçäo clínica foi um derrame pericárdico em um menino de quatro anos e meio de idade, näo associada à cardiopatia congênita. Edema intersticial pulmonar difuso associado a quadros repetitivos de volumosos derrames levando a tamponamento cardíaco e insuficiência respiratória aguda, constituíram a clínica básica desta anomalia rara do sistema linfático pulmonar. Os procedimentos clínico-cirúrgicos realizados como o emprego de diuréticos, dieta com triglicérides de cadeia média, pericardiocenteses, pericardectomia, pleurodese com talco bilateral e ligadura do duto toráxico, foram apenas paliativos. Entretanto, houve resposta ao tratamento hormonal com testosterona, tendo sido interrompido pelo aparecimento de sinais de virilizaçäo. Executou-se posteriormente cerclagem de artéria pulmonar direita, porém sem sucesso, devido provavelmente a pequena magnitude da mesma a ao severo comprometimento da funçäo pulmonar do paciente. Os autores propöem uma nova classificaçäo da doença e salientam a raridade do caso, após revisäo extensa da literatura mundial

[Congenital pulmonary lymphangiectasis and hyaline membrane. Report of a case]. / Linfangiectasia pulmonar congénita y membrana hialina. Presentación de un caso.

The case of a newborn with pathological confirmation of congenital pulmonary lymphangiectasis and hyaline membrane is reported. The lymphagiectasis corresponded to type III, with histological characteristics that make it different from other types of lymphatic dilatation. This association is quite uncommon and should be considered in the differential diagnosis of acute respiratory failure in the newborn period.