RESUMO
Las malformaciones pulmonares congénitas (MPC) conforman un grupo de entidades originadas por alteraciones en la embriogénesis del pulmón y de las vías respiratorias que se producen de acuerdo al nivel del árbol traqueobronquial donde se dé el insulto o el momento de la edad gestacional. Las entidades que en la actualidad forman parte de las MPC son: malformación congénita de la vía aérea, secuestro pulmonar, quiste broncogénico, enfisema lobar congénito, atresia bronquial. Su diagnóstico puede realizarse desde la etapa prenatal, al momento del nacimiento, en la edad pediátrica o adulta por la aparición de síntomas o incidentalmente en pruebas radiológicas. El manejo de estas lesiones depende del tipo de malformación y de la severidad de los síntomas, por lo que se debe individualizar la conducta a seguir en cada caso. Aunque la mayoría de los autores recomienda la resección de la lesión, no existe en la actualidad un consenso sobre la indicación de cirugía, sobre todo en pacientes asintomáticos. Nuestro objetivo es describir los hallazgos clínicos, radiológicos y en algunos casos anatomopatológicos así como el tratamiento empleado de cuatro casos clínicos, atendidos en un hospital de tercer nivel que ejemplifican las presentaciones más frecuentes de las MPC.
Congenital pulmonary malformations (CPM) make up a group of entities caused by alterations in the embryogenesis of the lung and the respiratory tract that occur according to the level of the tracheobronchial tree where the insult occurs or the moment of gestational age. The entities that are currently part of the CPM are: congenital malformation of the airway, pulmonary sequestration, bronchogenic cyst, congenital lobar emphysema, bronchial atresia. Its diagnosis can be made from the prenatal stage, at birth or in pediatric or adult age due to the appearance of symptoms or incidentally in radiological tests. The management of these injuries depends on the type of malformation and the severity of the symptoms, so the conduct to be followed in each case must be individualized. Although most authors recommend resection of the lesion, there is currently no consensus on the indication for surgery, especially in asymptomatic patients. Our objective is to describe the clinical, radiological and in some cases histopathological findings, as well as the treatment used in four clinical cases, treated in a tertiary level hospital that exemplify the most frequent presentations of MPC.
Assuntos
Humanos , Masculino , Feminino , Gravidez , Recém-Nascido , Adulto , Anormalidades do Sistema Respiratório/diagnóstico por imagem , Pulmão/anormalidades , Pulmão/diagnóstico por imagem , Pneumopatias/diagnóstico por imagem , Diagnóstico Pré-Natal , Recém-Nascido Prematuro , Pneumopatias/congênitoRESUMO
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.
Assuntos
Humanos , Feminino , Lactente , Pneumopatias/congênito , Linfangiectasia/congênito , Derrame Pleural , Propranolol/uso terapêutico , Biópsia , Sirolimo/uso terapêutico , Pneumopatias/patologia , Pneumopatias/diagnóstico por imagem , Linfangiectasia/patologia , Linfangiectasia/diagnóstico por imagemRESUMO
Congenital acinar dysplasia is a lethal, developmental lung malformation resulting in neonatal respiratory insufficiency. This entity is characterized by pulmonary hypoplasia and arrest in the pseudoglandular stage of development, resulting in the absence of functional gas exchange. The etiology is unknown, but a relationship with the disruption of the TBX4-FGF10 pathway has been described. There are no definitive antenatal diagnostic tests. It is a diagnosis of exclusion from other diffuse embryologic lung abnormalities with identical clinical presentations that are, however, histopathologically distinct.
Assuntos
Humanos , Feminino , Recém-Nascido , Insuficiência Respiratória/etiologia , Pulmão/anormalidades , Pneumopatias/congênito , Autopsia , Evolução Fatal , Pulmão/patologiaAssuntos
Pneumopatias/congênito , Linfangiectasia/congênito , Adolescente , Biópsia , Humanos , Imuno-Histoquímica , Pneumopatias/diagnóstico por imagem , Pneumopatias/patologia , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/patologia , Masculino , Derrame Pleural/patologia , Tomografia Computadorizada por Raios XRESUMO
Treats of symptomatic infants with congenital cystic lung lesions (CCLLs) need surgical resection. Variable location and size of the air cyst of involved lung mean that different surgical strategies must be selected. The presence of huge CCLLs mimicking tension pneumothorax would hamper accurate judgment and timely treatment in dealing with respiratory emergencies. Rare experience reports are coping with childhood tension cystic lung lesion. We present a case of childhood huge CCLLs which was similar to tension pneumothorax based on clinical manifestations and imaging evaluations. Implementation of instant decompression measure against tension pneumothorax failed to alleviate respiratory distress of child. We should exclude the possibility of huge lung cystic lesion in children when clinical evidence inclines to the diagnosis of tension pneumothorax.
El tratamiento de los lactantes con quistes pulmonares congénitos conlleva una resección quirúrgica. Las variaciones en la ubicación y el tamaño del quiste de aire en el pulmón afectado implican la selección de diferentes estrategias quirúrgicas. La presencia de quistes pulmonares congénitos gigantes que imitan un neumotórax a tensión no permitiría tomar una decisión precisa y realizar un tratamiento oportuno durante una emergencia respiratoria. Los escasos informes sobre este tipo de experiencia hacen referencia a quistes a tensión durante la niñez. En este artículo, presentamos el caso de un niño con un quiste pulmonar congénito gigante que imita neumotórax a tensión según las manifestaciones clínicas y las evaluaciones del diagnóstico por imágenes. La descompresión instantánea del neumotórax a tensión no logró aliviar la dificultad respiratoria del niño. En los niños que presentan evidencia clínica de neumotórax a tensión, se debe excluir la posibilidad de un quiste pulmonar congénito gigante.
Assuntos
Pneumopatias/congênito , Pneumopatias/diagnóstico , Pneumotórax/diagnóstico , Diagnóstico Diferencial , Humanos , Lactente , Pneumopatias/patologia , MasculinoAssuntos
Humanos , Masculino , Adolescente , Pneumopatias/congênito , Linfangiectasia/congênito , Derrame Pleural/patologia , Biópsia , Imuno-Histoquímica , Tomografia Computadorizada por Raios X , Pneumopatias/patologia , Pneumopatias/diagnóstico por imagem , Linfangiectasia/patologia , Linfangiectasia/diagnóstico por imagemRESUMO
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.
Assuntos
Doenças do Prematuro/diagnóstico , Pneumopatias/congênito , Linfangiectasia/congênito , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Pneumopatias/diagnóstico , Linfangiectasia/diagnóstico , MasculinoRESUMO
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.
Assuntos
Humanos , Masculino , Recém-Nascido , Doenças do Prematuro/diagnóstico , Pneumopatias/congênito , Linfangiectasia/congênito , Recém-Nascido Prematuro , Pneumopatias/diagnóstico , Linfangiectasia/diagnósticoRESUMO
BACKGROUND: Treatment of congenital pulmonary airway malformations (CPAMs) is generally surgical resection; however, there is controversy regarding the optimal timing of surgical intervention, especially in asymptomatic patients. STUDY DESIGN: Using the American College of Surgeons National Surgical Quality Improvement Program-Pediatric Participant Use Files from 2012 to 2015, children who underwent lung resection for CPAMs were identified. Outcomes in children who underwent lung resection during the neonatal period were compared with those who underwent resection beyond the neonatal period, but during the first year of life (non-neonates). RESULTS: A total of 541 patients (20.7% neonates and 79.3% non-neonates) were identified. Neonates had higher rates of preoperative comorbidities and worse postoperative outcomes when compared with non-neonates (morbidity 19.6% vs 5.4%, p < 0.0001). On multivariable regression analysis, the presence of preoperative symptoms (defined as oxygen dependence or ventilatory support) was independently associated with increased morbidity (odds ratio 3.91 [range 1.6 to 9.57], p = 0.003). In a subgroup analysis of asymptomatic neonates compared with asymptomatic non-neonates, there was no difference in overall morbidity (7.4% vs 4.4%, p = 0.33). CONCLUSIONS: These data suggest that lung resection for CPAMs in the neonatal period in asymptomatic children are not associated with increased 30-day morbidity. The presence of preoperative symptoms was independently associated with increased morbidity in a multivariable regression model. More data are needed to better understand the long-term outcomes and better define the optimal timing of surgery in this patient population.
Assuntos
Pneumopatias/congênito , Pulmão/anormalidades , Pneumonectomia/métodos , Melhoria de Qualidade , Canadá/epidemiologia , Feminino , Seguimentos , Humanos , Incidência , Lactente , Recém-Nascido , Pulmão/cirurgia , Pneumopatias/cirurgia , Masculino , Complicações Pós-Operatórias/epidemiologia , Prognóstico , Estudos Retrospectivos , Fatores de Tempo , Estados Unidos/epidemiologiaRESUMO
OBJECTIVE: To assess the effectiveness of laser surgery in fetuses with a cystic lung lesion with systemic arterial blood supply (hybrid lung lesion) at risk of perinatal death. METHODS: A cohort of five consecutive fetuses with a large hybrid lung lesion associated with hydrops and/or pleural effusion with severe lung compression was selected for percutaneous ultrasound-guided fetal laser ablation of the feeding artery (FLAFA) before 32 weeks' gestation in a single tertiary national referral center in Queretaro, Mexico. The primary outcomes were survival and need for postnatal surgery. RESULTS: FLAFA was performed successfully in all cases at a median gestational age of 24.9 (range, 24.4-31.7) weeks. After fetal intervention, dimensions in both lungs increased and fluid effusions resolved in all cases. All cases were delivered liveborn at term at a median gestational age of 39.6 (range, 38.0-39.7) weeks, without respiratory morbidity or need for oxygen support, resulting in perinatal survival of 100%. During follow-up, three (60%) cases showed progressive regression of the entire lung mass and did not require postnatal surgery, whereas in two (40%) cases a progressive decrease in size of the mass was observed but a cystic portion of the lung mass persisted and postnatal lobectomy was required. CONCLUSION: In fetuses with large hybrid lung lesions at risk of perinatal death, FLAFA is feasible and could improve survival and decrease the need for postnatal surgery. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.