Endovascular treatment of a right pulmonary sequestration supplied by an aneurysmal aberrant artery originating from the abdominal aorta.

Endovascular embolization of arteries feeding pulmonary sequestrations is a growing therapeutic option. A 51-year-old woman with chest pain and hemoptysis was admitted. During hospitalization she presented 150 mL hemoptysis, hypotension, and hematocrit fell to 23.3%. Contrast-enhanced computed tomography confirmed a pulmonary sequestration irrigated by an aneurysmal artery from the abdominal aorta. The patient underwent endovascular coil embolization of the artery feeding the aneurysm and an Amplatzer device was deployed in the proximal third of the sequestration artery. Subsequent contrast-enhanced computed tomography confirmed complete thrombosis of the aberrant artery feeding the aneurysm and absence of irrigation of the pulmonary sequestration. At 56 months follow-up the patient remains asymptomatic, tomography showed involution of the sequestration and complete thrombosis of the aberrant artery. The challenges presented by the different treatment alternatives are discussed.

A embolização endovascular das artérias que alimentam os sequestros pulmonares é uma opção terapêutica em crescimento. Uma mulher de 51 anos com dor torácica e hemoptise foi internada. Durante a internação, ela apresentou hemoptise de 150 mL, hipotensão e queda do hematócrito para 23,3%. A tomografia computadorizada com contraste confirmou um sequestro pulmonar irrigado por uma artéria aneurismática originária da aorta abdominal. A paciente foi submetida a embolização endovascular da artéria que alimentava o aneurisma com uso de coils e dispositivo Amplatzer no terço proximal da artéria sequestrante. A tomografia subsequente confirmou a trombose completa da artéria aberrante que alimentava o aneurisma e a ausência de irrigação dentro do sequestro pulmonar. No seguimento de 56 meses, a paciente permanecia assintomática, e a tomografia mostrou involução do sequestro e trombose completa da artéria aberrante. Os desafios apresentados pelas diferentes alternativas de tratamento são discutidos neste artigo.

Endovascular treatment of a right pulmonary sequestration supplied by an aneurysmal aberrant artery originating from the abdominal aorta / Tratamento endovascular de sequestro pulmonar direito irrigado por artéria aneurismática aberrante originária da aorta abdominal

Abstract Endovascular embolization of arteries feeding pulmonary sequestrations is a growing therapeutic option. A 51-year-old woman with chest pain and hemoptysis was admitted. During hospitalization she presented 150 mL hemoptysis, hypotension, and hematocrit fell to 23.3%. Contrast-enhanced computed tomography confirmed a pulmonary sequestration irrigated by an aneurysmal artery from the abdominal aorta. The patient underwent endovascular coil embolization of the artery feeding the aneurysm and an Amplatzer device was deployed in the proximal third of the sequestration artery. Subsequent contrast-enhanced computed tomography confirmed complete thrombosis of the aberrant artery feeding the aneurysm and absence of irrigation of the pulmonary sequestration. At 56 months follow-up the patient remains asymptomatic, tomography showed involution of the sequestration and complete thrombosis of the aberrant artery. The challenges presented by the different treatment alternatives are discussed.

Resumo A embolização endovascular das artérias que alimentam os sequestros pulmonares é uma opção terapêutica em crescimento. Uma mulher de 51 anos com dor torácica e hemoptise foi internada. Durante a internação, ela apresentou hemoptise de 150 mL, hipotensão e queda do hematócrito para 23,3%. A tomografia computadorizada com contraste confirmou um sequestro pulmonar irrigado por uma artéria aneurismática originária da aorta abdominal. A paciente foi submetida a embolização endovascular da artéria que alimentava o aneurisma com uso de coils e dispositivo Amplatzer no terço proximal da artéria sequestrante. A tomografia subsequente confirmou a trombose completa da artéria aberrante que alimentava o aneurisma e a ausência de irrigação dentro do sequestro pulmonar. No seguimento de 56 meses, a paciente permanecia assintomática, e a tomografia mostrou involução do sequestro e trombose completa da artéria aberrante. Os desafios apresentados pelas diferentes alternativas de tratamento são discutidos neste artigo.

Malformación adenomatoidea quística congénita asociada a secuestro broncopulmonar en un recién nacido / Congenital cystic adenomatoid malformation presenting with bronchopulmonary sequestration in a newborn

Resumen Antecedentes: La malformación adenomatoidea quística y el secuestro broncopulmonar son malformaciones que se pueden diagnosticar prenatalmente. Los adecuados controles prenatales ayudan a un diagnóstico temprano y su manejo. Objetivo: Presentar el caso de un recién nacido con diagnóstico parental de una malformación pulmonar y su desenlace posterior. Reporte de caso: Recién nacido a término con diagnóstico antenatal de malformación pulmonar, el cual se confirmó tras su nacimiento. Discusión: Estas dos malformaciones son entidades cuya incidencia viene aumentando, razón por la cual es importante el conocimiento del curso clínico, ayudas diagnósticas, complicaciones y las posibilidades de manejo con las que se cuenta.

Abstract Background: Cystic adenomatoid malformation and bronchopulmonary sequestration are malformations that can be diagnosed prenatally. Appropriate prenatal controls helps to early diagnosis and management. Objective: To present the case of a newborn with a parental diagnosis of a pulmonary malformation and its subsequent outcome. Case report: Newborn at term with antenatal diagnosis of pulmonary malformation which was confirmed postnatal. Discussion: These two malformations are entities whose incidence is increasing, which is why knowledge of the clinical course, diagnostic aids, complications, and the management possibilities that are available are important.

Secuestro pulmonar intrapulmonar / Intrapulmonary pulmonary sequestration

RESUMEN El secuestro pulmonar es una de las malformaciones pulmonares más comunes en el adulto joven. Este puede ser originado a partir de un brote pulmonar accesorio que migra junto al desarrollo del esófago, o puede ocurrir por infecciones pulmonares repetitivas; sus manifestaciones clínicas son muy variables y su detección es un hallazgo radiológico. Se presentó un paciente joven con ingresos múltiples por neumonía en el Hospital Universitario Dr. Celestino Hernández Robau. En la radiografía de tórax se apreció una imagen no homogénea en la base pulmonar derecha, que no mejoró a pesar de múltiples tratamientos antimicrobianos de amplio espectro. En una tomografía axial computarizada se observó un grueso vaso que partía de la aorta abdominal hasta la base pulmonar derecha. Se realizó reconstrucción de imágenes y con el diagnóstico de secuestro pulmonar se intervino quirúrgicamente. El paciente tuvo una evolución satisfactoria.

ABSTRACT Pulmonary sequestration is one of the most common lung malformations in young adults. This can be originated from an accessory lung bud that migrates along the development of the esophagus, or it can occur by repetitive pulmonary infections; its clinical manifestations are highly variable and its detection is a radiological finding. We present a young patient with multiple admissions for pneumonia at "Dr. Celestino Hernández Robau" University Hospital. Chest X-ray showed a non-homogeneous image in the right lung base, which did not improve despite several broad-spectrum antimicrobial treatments. A thick vessel that started from the abdominal aorta to the right pulmonary base was observed through a computerized axial tomography. Image reconstruction was performed and with the diagnosis of pulmonary sequestration, surgery was performed. The patient had a satisfactory evolution.

Pulmonary Sequestration Associated with Actinomycosis: A Case Report.

Background: Bronchopulmonary sequestration is a rare congenital malformation of the lower respiratory tract; it consists of a nonfunctioning mass of lung tissue that is irrigated by an anomalous systemic artery. The association with Actinomyces superinfection has not been well established. Methods: We present the case of a 35-year-old woman with a history of recurrent episodes of pneumonia. Based on radiological and histopathological examination, she was diagnosed with intralobar bronchopulmonary sequestration associated with Actinomyces infection. Promoting clinical suspicion is essential to diagnose pulmonary actinomycosis in patients with recurrent pneumonia, to improve early recognition and timely management.

Secuestro pulmonar intralobar y aspergilosis pulmonar: reporte de un caso / Intralobar pulmonary sequestration and pulmonary aspergilosis: a case report

El secuestro pulmonar es un tipo de malformación congénita pulmonar poco frecuente, con presentación clínica variable y de usual diagnóstico durante la infancia. Puede coexistir con otras patologías pulmonares, principalmente de tipo infecciosas y su manejo definitivo es mediante un procedimiento quirúrgico. Se presenta el caso de una paciente adulta con secuestro pulmonar, asociado a una infección fúngica, manejada con cirugía mínimamente invasiva

Pulmonary sequestration is a rare type of congenital pulmonary malformation, with variable clinical presentation and usually diagnosed during childhood. It can coexist with other pulmonary pathologies, mainly infectious, and its definitive management is through a surgical procedure. We present the case of an adult patient with pulmonary sequestration associated with a fungal infection, managed with minimally invasive surgery

Manejo quirúrgico del secuestro pulmonar complicado. Reporte de caso / Surgical management of the complicated pulmonary sequestration. A case report

Introducción: el secuestro pulmonar es una patología poco frecuente en la edad adulta, su mayor incidencia es en etapas tempranas de la vida. Se acompaña de infecciones pulmonares a repetición o crónicas severas, y puede asociarse a malformaciones congénitas. El diagnóstico se fundamenta en la detección de la irrigación anómala a través de un vaso de la circulación sistémica. Este caso nos permite plantear un diagnóstico diferencial en pacientes de edad adulta, aún cuando se trate de patología con mayor incidencia en niños y jóvenes, y poder orientar el manejo de acuerdo a esta posibilidad diagnóstica. Caso clínico: paciente femenino, de 19 años, con antecedente de asma e infecciones respiratorias a repetición de un mes de evolución. Ingresa con dificultad respiratoria, fiebre y tos productiva. Se realiza tomografía y Radiografía de tórax, en la que se evidencia imágenes compatibles con colección en campo pulmonar izquierdo y derrame pleural. Se realiza BAAR en esputo con resultado negativo para tuberculosis. Se realiza toracotomía posterolateral izquierda, resección de lóbulo inferior izquierdo, y se coloca dren pleural. Se identifica absceso pulmonar en lóbulo inferior izquierdo, y hallazgos anatómicos de secuestro pulmonar. Paciente presenta evolución favorable; el control clínico y radiográfico en el postoperatorio inmediato y mediato fue satisfactorio. Conclusión: presentamos el caso de edad adulta, donde un diagnóstico adecuado y un manejo multidisciplinario permiten una evolución satisfactoria de los pacientes.(AU)

Introduction: pulmonary sequestration is a rare disease in adulthood; its highest incidence is in early stages of life. It is accompanied by repeated or severe chronic lung infections, and may be associated with congenital malformations. The diagnosis is based on the detection of anomalous irrigation through a vessel of the systemic circulation.This case allows us to propose a differential diagnosis in patients of adulthood, even when it is pathology with higher incidence in children and young people, and guide the management according to this diagnostic possibility.Clinical case: female patient, 19 years old, with a history of asthma and respiratory infections after a month of evolution. The patient was admitted with respiratory distress, fever and productive cough.A tomography and chest X-ray are performed; that show images compatible with collection in the left pulmonary field and pleural effusion. BAAR is performed in sputum with a negative result for tuberculosis.Posterolateral left thoracotomy is performed, left lower lobe resection, and pleural drain is placed. Pulmonary abscess is identified in the left lower lobe, and findings of anatomical pulmonary sequestration. Patient presents positive evolution; The clinical and radiographic control in the immediate and intermediate postoperative period was satisfactory.Conclusion: we present the case of adulthood, where an adequate diagnosis and multidisciplinary management allows a satisfactory evolution of the patient.(AU)

Secuestro broncopulmonar. Diagnóstico prenatal, factores pronóstico y tratamiento por cirugía fetal / Bronchopulmonary Sequestration. Prenatal diagnosis, prognostic factors and treatment by fetal surgery

Resumen ANTECEDENTES: El secuestro broncopulmonar suele diagnosticarse antes del nacimiento como una masa sólida intratorácica, homogénea e hiperecogénica, casi siempre localizada en el lóbulo inferior pulmonar. Su signo patognomónico es la demostración con Doppler color de la arteria nutricia sistémica dentro de la masa pulmonar. La historia natural de la enfermedad ha demostrado que durante la vida fetal en la mayoría de los casos la masa puede tener regresión espontánea. Una proporción importante de casos tiene rápido crecimiento de la masa con hidrotórax, compresión pulmonar e hidrops. Estos casos son de mal pronóstico y elevada mortalidad perinatal y, en estas circunstancias, la cirugía fetal es la única opción que puede mejorar el pronóstico. Para este propósito se han intentado varias técnicas de cirugía fetal pero la ideal sigue siendo un tema de controversia. OBJETIVO: Describir los métodos diagnósticos, factores pronóstico y discutir las repercusiones perinatales de cada una de las diferentes técnicas de cirugía fetal descritas en la bibliografía para el tratamiento de fetos complicados con secuestro broncopulmonar. METODOLOGÍA: La búsqueda se efectuó en Medline y PubMed de todos los artículos en inglés y español publicados entre 1990 y 2019 que tuvieran las palabras clave "bronchopulmonary sequestration", "secuestro broncopulmonar", "fetal surgery" y "cirugía fetal". Se eligieron los que describían casos de secuestro broncopulmonar tratados con alguna técnica de cirugía fetal. RESULTADOS: Se encontraron 150 artículos pero solo se seleccionaron 29 estudios que describían casos con diagnóstico prenatal de secuestro broncopulmonar tratados con alguna técnica de cirugía fetal. Para tratar el secuestro broncopulmonar grave se describieron varias técnicas de intervención fetal, entre ellas: cirugía fetal abierta, colocación de catéter de derivación toraco-amniótica, oclusión del vaso nutricio sistémico mediante guía ecográfica con láser, radiofrecuencia, embolización o escleroterapia con inyección de alcohol intravascular e, incluso, broncoscopia fetal. CONCLUSIONES: Al parecer la coagulación láser del vaso nutricio es la técnica con mejores resultados perinatales que evita la muerte fetal, la morbilidad pulmonar y la necesidad de cirugía posnatal en prácticamente todos los casos tratados; se propone como el único tratamiento potencialmente curativo.

Abstract BACKGROUND: The bronchopulmonary sequestration (BPS) is usually diagnosed prenatally as an intrathoracic hyperechoic, homogeneous, solid mass usually located in the lower pulmonary lobe. The pathognomonic sign is the demonstration of its systemic feeding artery into the lung mass by using color Doppler ultrasound. Natural history data reports that a significant proportion of BPS cases usually regress in the intrauterine period. However, a proportion of cases showed a rapid and progressive growth with development of massive pleural effusion and hydrops. Such cases present the poorest prognosis and high perinatal mortality and therefore, fetal intervention should be considered to improve prognosis. Different fetal surgery procedures have been attempted for such purposes. However, the optimal fetal therapeutica strategy remains controversial. OBJECTIVE: In the present manuscript, we describe and discuss the perinatal outcome of the different published fetal interventions in fetuses complicated with BPS. METHODOLOGY: We search in the English and Spanish literature (Medline and PubMed) for cases complicated with BPS and treated with any fetal surgical intervention including the key words "broncopulmonary sequestration" and "fetal surgery" from 1990 to 2019. RESULTS: A total of 150 references were reviewed including only 26 studies describing pregnancies with prenatal diagnosis of bronchopulmonary sequestration that were treated with any fetal surgical intervention. Several fetal surgery procedures have been attempted for the management of complicated fetuses with severe BPS. These include open fetal surgery, placement of thoracoamniotic shunts, and occlusion of the feeding blood vessel by ultrasound-guided intrafetal laser coagulation, radiofrequency ablation, coil embolization, sclerotherapy with intravascular alcohol injection and fetal bronchoscopy. CONCLUSIONS: Laser coagulation of the feeding artery appears to be the best intervention, avoiding fetal death, neonatal pulmonary morbidity and the need for postnatal surgery in virtually all cases postulating itself as the only potential curative management.

Cirugía fetal en tumores pulmonares mediante escleroterapia

Microcystic fetal lung tumors or masses such as the congenital cystic adenomatoid malformation (CCAM) and bronchopulmonary sequestration (SBP) are rare congenital pathologies, diagnosed by ultrasound during prenatal assessment, with a high mortality rate of 95% as a consequence of prenatal complications due to hydrops and postnatal death from pulmonary hypoplasia. Minimally invasive therapies using sclerosing agents are positioned among the low cost and highly effective techniques for such pathologies. Methodology: We conducted a prospective, randomized, blind clinical study in 17 pregnant women with diagnosis of fetal microcystic CCAM or hybrid lesion (CCAM + SBP), who were treated with betamethasone or polidocanol. Alternative therapy was employed in case of failure of the initial procedure. Results: In four (44.4%) of the nine cases treated with betamethasone, the alternative therapy of sclerosis with polidocanol was required for treatment due to fetal compromise. Involution of the lung lesion and the hydropic condition was faster and progressive with percutaneous sclerotherapy compared to the maternal steroid effect. Perinatal results were also more satisfactory in the group with polidocanol (91.7%) than in the group with the steroid (60%). Conclusions: Percutaneous sclerotherapy with polidocanol was found efficacious for CCAM and hybrid lesion treatment, with faster and more efficient resolution of the pathology in cases resistant to previously administered steroid therapy.

Las tumoraciones o masas pulmonares fetales microquísticas, tales como la malformación adenomatoidea quística congénita (CCAM, por sus siglas en inglés) y el secuestro bronco pulmonar (SBP), representan patologías congénitas poco frecuentes, diagnosticadas por ultrasonido durante la evaluación prenatal, con un alto índice de mortalidad de 95% como consecuencia de las complicaciones prenatales por hidropesía y fallecimiento posnatal debido a hipoplasia pulmonar. Las terapias de mínima invasión usando agentes esclerosantes han tomado posicionamiento entre las técnicas de bajo costo y alta efectividad para dichas patologías. Metodología. Se realizó un estudio clínico prospectivo, aleatorio, ciego, en 17 embarazadas con diagnóstico de CCAM microquística o de lesión híbrida (CCAM +SBP) tratadas con betametasona o con polidocanol, optando por la terapia alternativa en caso de fallar la técnica inicial. Resultados. En 4/9 (44,4%) de los 9 casos tratados con betametasona se requirió la terapia alternativa de esclerosis con polidocanol para la resolución del cuadro, que comprometía el estado fetal. La involución de la lesión pulmonar y del cuadro hidrópico fue más rápida y progresiva con la escleroterapia percutánea en comparación con el efecto esteroideo materno. Los resultados perinatales fueron asimismo más satisfactorios en el grupo con polidocanol (91,7%) que en el grupo con el esteroide (60%). Conclusiones. Se comprobó la eficacia de la escleroterapia percutánea con polidocanol para el tratamiento de la CCAM y de lesión híbrida, con resolución más rápida y eficaz de la patología en los casos donde existía resistencia a la terapia esteroidea previamente administrada.

Secuestro pulmonar en adultos: reporte de tres casos y revisión de la literatura / Adult bronchopulmonary sequestration: report of three cases and review of the literature

Resumen El secuestro pulmonar es una enfermedad congénita infrecuente caracterizada por la presencia de una porción de parénquima pulmonar que recibe vascularización independiente de la circulación sistémica. Se presentan tres casos de secuestro pulmonar intralobares, de presentación tardía, uno de ellos asociado a una malformación adenomatosa quística y en los cuales el manejo se completó por videotoracoscopia: dos de ellos con lobectomía total y uno con lobectomía sublobar. Se trata de una enfermedad de presentación clínica variable y cuyo tratamiento es, en la mayoría de los casos, quirúrgico, requiriéndose usualmente resecciones lobares, las cuales pueden llevarse a cabo de manera eficaz y segura por videotoracoscopia.

Abstract Pulmonary sequestration is an infrequent congenital disease characterized by the presence of a portion of pulmonary parenchyma that receives vascularization independent of the systemic circulation. We present three cases of intralobar pulmonary sequestration of late presentation, one of them associated with a cystic adenomatous malformation and in which the management was completed by videothoracoscopy: two of them with total lobectomy and the other with sublobar lobectomy. It is a disease of variable clinical presentation and whose treatment is, in most cases, surgical, usually requiring lobar resections, which can be carried out effectively and safely by video-assisted thoracoscopy.

Thoracic Changes after Full Laser Ablation of the Feeding Artery in Fetuses with Bronchopulmonary Sequestration.

OBJECTIVE: To assess longitudinal intrathoracic changes after fetal laser surgery in fetuses with bronchopulmonary sequestration (BPS) with hydrops and/or hydrothorax. STUDY DESIGN: The presence of intrafetal fluid effusions, the lung mass volume ratio (congenital pulmonary airway malformation volume ratio [CVR]), and the observed/expected lung-to-head circumference ratio (O/E-LHR) of both lungs were evaluated in a cohort of BPS fetuses with hydrops and/or hydrothorax treated with full laser ablation of the feeding artery (FLAFA). The longitudinal changes in intrafetal fluid effusions, lung mass volume, and pulmonary growth were analyzed by survival and multilevel analysis against days after FLAFA. RESULTS: FLAFA was successfully performed in 15 cases at a median gestational age of 26.9 weeks. A complete disappearance of the hydrops and hydrothorax was observed a median interval of 7.5 and 21 days after the fetal intervention, respectively. A progressive decrease in the CVR and an increment in the size of both lungs were observed after FLAFA. The O/E-LHR of the lung contralateral and ipsilateral to the side of the BPS became normal on average 8 and 10 weeks after FLAFA, respectively. CONCLUSION: Fetal laser surgery with FLAFA promotes disappearance of all fetal fluid effusions, a lung mass regression sequence, and a normalization of pulmonary growth.

Dolor torácico y hemotórax como presentación atípica de secuestro pulmonar en adulto / Chest pain and hemothorax: an atypical presentation of a pulmonary sequestration

Resumen El secuestro pulmonar es una malformación pulmonar rara, presentándose generalmente en edades tempranas. Se presenta mayoritariamente con neumonías e infecciones repetidas, distrés respiratorio y falla cardíaca; raramente en pacientes de mayor edad se presenta con hemoptisis y dolor torácico. En este artículo se describe el caso clínico de un paciente de 60 años de edad que se presenta con un infarto de un secuestro pulmonar y hemotórax.

Bronchopulmonary sequestration is a rare pulmonary malformation, usually occurring at an early age. It presents mainly with pneumonia and repetitive infections, respiratory distress and heart failure; rarely in aged patients presents with hemoptysis and chest pain. This article describes the clinical case of a 60-year-old male patient who presented an ischemic pulmonary sequestration and hemothorax.

Fetal laser ablation of feeding artery of cystic lung lesions with systemic arterial blood supply.

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.

Atypical presentation of intra-abdominal extralobar pulmonary sequestration detected in prenatal care: a case report / Apresentação atípica de sequestro pulmonar extralobar intra-abdominal detectado no pré-natal: relato de caso

Objective: To describe an unusual clinical presentation of intra-abdominal extralobar pulmonary sequestration in a 2-year, 9 month-old patient and assess diagnostic and treatment aspects of this pathology. Case description: An undefined intra-abdominal mass was identified in the right adrenal region in a male fetus. Postnatal evaluation with ultrasound images, computed tomography, magnetic resonance imaging and laboratory testing was insufficient to determine the nature of the lesion. After two years, laparoscopic resection of the mass and histopathological examination of the surgical specimen allowed to establish the diagnosis of intra-abdominal extralobar pulmonary sequestration. Comments: This malformation can be monitored clinically; however, surgical excision is often performed, probably due to the impossibility of attaining diagnosis with non-invasive methods, such as in the present case, in which the lesion appeared in an unusual position for intra-abdominal extralobar pulmonary sequestration. Therefore, the surgical approach seems to be the key to attain the diagnosis and establish the conduct for this type of congenital malformation.

Objetivo: Descrever apresentação clínica incomum de sequestro pulmonar extralobar intra-abdominal em um paciente de dois anos e nove meses e avaliar aspectos diagnósticos e de tratamento dessa patologia. Descrição do caso: Uma massa intra-abdominal indefinida em topografia suprarrenal direita de feto masculino. A avaliação pós-natal com imagens de ultrassom, tomografia computadorizada, ressonância magnética e testes laboratoriais não foi suficiente para determinar a natureza da lesão. Após dois anos, a resseção laparoscópica da massa e o exame histopatológico do espécime cirúrgico permitiram estabelecer o diagnóstico de sequestro pulmonar extralobar intra-abdominal. Comentários: Essa malformação pode ser monitorada clinicamente; entretanto, a excisão cirúrgica frequentemente é feita, provavelmente devido à impossibilidade de diagnóstico com métodos não invasivos, como ocorreu no presente caso, na qual a lesão apresentou-se em posição não habitual para sequestro pulmonar extralobar intra-abdominal. Desse modo, a abordagem cirúrgica parece ser a chave para o diagnóstico e a condução desde tipo de malformação congênita.

[Atypical presentation of intra-abdominal extralobar pulmonary sequestration detected in prenatal care: a case report]. / Apresentação atípica de sequestro pulmonar extralobar intra-abdominal detectado no pré-natal: relato de caso.

OBJECTIVE: To describe an unusual clinical presentation of intra-abdominal extralobar pulmonary sequestration in a 2-year, 9 month-old patient and assess diagnostic and treatment aspects of this pathology. CASE DESCRIPTION: An undefined intra-abdominal mass was identified in the right adrenal region in a male fetus. Postnatal evaluation with ultrasound images, computed tomography, magnetic resonance imaging and laboratory testing was insufficient to determine the nature of the lesion. After two years, laparoscopic resection of the mass and histopathological examination of the surgical specimen allowed to establish the diagnosis of intra-abdominal extralobar pulmonary sequestration. COMMENTS: This malformation can be monitored clinically; however, surgical excision is often performed, probably due to the impossibility of attaining diagnosis with non-invasive methods, such as in the present case, in which the lesion appeared in an unusual position for intra-abdominal extralobar pulmonary sequestration. Therefore, the surgical approach seems to be the key to attain the diagnosis and establish the conduct for this type of congenital malformation.

[Intradiaphragmatic bronchopulmonary sequestration: a case report and review]. / Secuestro broncopulmonar intradiafragmático: reporte de caso y revisión de la literatura.

Bronchopulmonary sequestration is a rare congenital malformation, consisting in a mass of nonfunctioning lung tissue with no connection to the tracheobronchial tree. It can be classified into intra- and extra-lobar. Extra-lobar bronchopulmonary sequestration accounts for 25% of them; of these, only 1% are intra-diaphragmatic. There is little international literature about intra-diaphragmatic bronchopulmonary sequestration and its diagnosis and treatment remain a challenge for the surgeon. Our case is a newborn with antenatal diagnosis of an abdominal mass by ultrasound and magnetic resonance imaging (MRI) showing a left adrenal tumor that appeared to be a neuroblastoma. Postnatal computed tomography (CT) revealed the likelihood of an intra-diaphragmatic bronchopulmonary sequestration, although neuroblastoma could not be ruled out. Abdominal laparoscopy was performed in the bulging left hemidiaphragmatic area. A combined thoracoscopic approach was decided which showed that the defect was located in between both cavities. This is the first case of intra-diaphragmatic bronchopulmonary sequestration reported in Chile.

El secuestro broncopulmonar es una malformación congénita rara, consistente en una masa de tejido pulmonar no funcionante sin conexión al árbol traqueobronquial. Esta puede clasificarse en intra y extralobar. El extralobar corresponde al 25% de los secuestros broncopulmonares y sólo el 1% es intradiafragmático. Existe escasa literatura internacional sobre secuestro broncopulmonar intradiafragmático, por lo que su diagnóstico y tratamiento es un desafío para el cirujano. Nuestro caso es un recién nacido con diagnóstico antenatal de una masa abdominal por ecografía y resonancia magnética nuclear, las cuales mostraron un tumor adrenal que impresiona ser un neuroblastoma. Se realizó tomografía axial computarizada postnatal donde el secuestro broncopulmonar intradiafragmático apareció como más probable. No se pudo excluir el neuroblastoma. Se realizó laparoscopía abdominal en la que se observó un bulto en el hemidiafragma izquierdo. Se decidió un abordaje toracoscópico combinado, el cual mostró que el defecto se encontraba entre ambas cavidades. Este es el primer caso reportado de secuestro broncopulmonar intradiafragmático en Chile.

Malformaciones pulmonares congénitas / Congenital lung malformations

Congenital lung malformations (CLM) comprise a heterogeneous group of lung diseases. They vary widely in their clinical presentation and severity, depending on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Patients with CLM can present respiratory symptoms at birth or can remain asymptomatic for long periods. There has been an increase in early diagnosis of CLM attributable to the routine use of prenatal ultrasound. Management of these lesions depends on the type of malformation and symptoms. Treatment of asymptomatic patients is controversial, because the prognosis of these diseases is unpredictable. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. This review describes the principal CLM, their diagnosis, and the controversies regarding treatment.

Las malformaciones pulmonares congénitas (MPC) constituyen un grupo heterogéneo de enfermedades. Varían en su presentación clínica y gravedad en función al grado de afectación pulmonar y a su ubicación en tórax. Se pueden manifestar a cualquier edad y pueden condicionar morbilidad y mortalidad significativa en lactantes y niños. Los pacientes pueden presentar síntomas respiratorios desde el nacimiento o permanecer asintomáticos durante largos períodos. Se ha observado un aumento en el diagnóstico precoz de las MPC atribuible al uso rutinario de la ecografía prenatal. El manejo de estas lesiones depende del tipo de malformación y de los síntomas. El tratamiento de pacientes asintomáticos es controvertido debido a que el pronóstico de estas enfermedades es desconocido. La mayoría de los autores recomiendan la resección de la lesión en el momento del diagnóstico debido al riesgo de complicaciones. Esta revisión describe las principales malformaciones congénitas del pulmón, su diagnóstico y las estrategias de tratamiento.

Tumor carcinoide e sequestro pulmonar / Carcinoid tumor and pulmonary sequestration

O sequestro pulmonar é definido como uma massa de tecido pulmonar separada da árvore traqueobrônquica e irrigada por uma artéria sistêmica anômala. Sua associação com neoplasias pulmonares é rara. Relatamos o caso de uma paciente de 39 anos com o diagnóstico de tumor carcinoide localizado no brônquio intermediário, associado a alterações caracterizadas como bronquiectasias em lobo inferior direito. A paciente foi submetida à toracotomia para ressecção da área acometida e, durante a cirurgia, apresentou hemorragia importante decorrente da transecção da artéria anômala que nutria o sequestro pulmonar intralobar localizado em lobo inferior direito, não identificado nos exames pré-operatórios.

Pulmonary sequestration is defined as a mass of lung tissue separated from the tracheobronchial tree and irrigated by an anomalous systemic artery. It is rarely seen in conjunction with lung neoplasms. We report the case of a 39-year-old female patient diagnosed with a carcinoid tumor, located in the intermediate bronchus and accompanied by bronchiectasis in the right lower lobe. The patient underwent thoracotomy for the resection of the affected area. During surgery, she presented with significant hemorrhage resulting from the transection of the anomalous artery that irrigated an intralobar pulmonary sequestration, which was located in right lower lobe and had not been identified in pre-operative examinations.

Malformações pulmonares congênitas / Congenital lung malformations

As malformações congênitas do pulmão são raras e variam muito na sua forma de apresentação clínica e gravidade, dependendo principalmente do grau de envolvimento pulmonar e de sua localização na cavidade torácica. Elas podem se manifestar em qualquer idade e podem ser fonte de importante morbidade e mortalidade em lactentes e crianças. Os indivíduos com malformações congênitas do pulmão podem apresentar sintomas respiratórios ao nascimento, enquanto outros podem permanecer assintomáticos por longos períodos. Atualmente, com o uso rotineiro da ultrassonografia pré-natal, vem ocorrendo um aumento no diagnóstico mais precoce dessas malformações. A manifestação clínica dessas malformações varia desde uma disfunção respiratória pós-natal imediata a um achado acidental na radiografia de tórax. O diagnóstico precoce e o tratamento imediato oferecem a possibilidade de um desenvolvimento pulmonar absolutamente normal. Quando assintomáticos, a conduta para o tratamento dos pacientes com malformações pulmonares ainda é controversa, uma vez que o prognóstico dessas afecções é imprevisível. O manejo dessas lesões depende do tipo de malformação e de sintomas. Devido ao risco de complicação, a maioria dos autores sugere a ressecção da lesão no momento em que essa é identificada. A lobectomia é o procedimento de escolha, fornecendo excelentes resultados a longo prazo. Este artigo descreve as principais malformações pulmonares congênitas, seu diagnóstico e controvérsias quanto o tratamento.

Congenital lung malformations are rare and vary widely in their clinical presentation and severity, depending mostly on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Individuals with congenital lung malformations can present with respiratory symptoms at birth or can remain asymptomatic for long periods. Recently, there has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. The clinical manifestation of these malformations varies from respiratory distress in the immediate postnatal period to an incidental finding on chest X-rays. Early diagnosis and prompt treatment offer the possibility of absolutely normal lung development. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable. The management of these lesions depends on the type of malformation and symptoms. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. Lobectomy is the procedure of choice and yields excellent long-term results. This article describes the principal congenital lung malformations, their diagnosis, and the controversies regarding treatment.