Secuestro pulmonar: caracterización y tratamiento en pacientes adultos y pediátricos / Pulmonary sequestration: characteristics and treatment in adult and pediatric patients

Resumen Introducción: El secuestro pulmonar (SP) es una malformación congénita caracterizada por tejido pulmonar con vascularización de una arteria sistémica anómala. Objetivo: Analizar las características y tratamiento de pacientes adultos y pediátricos con secuestro pulmonar. Materiales y Método: Estudio descriptivo transversal. Periodo: enero de 1988 a diciembre de 2018. La información se obtuvo de fichas clínicas y registros de anatomía patológica. Se describen edad, sexo, características clínicas, diagnóstico, tratamiento quirúrgico y hallazgos anatomopatológicos. Se realizó análisis estadístico mediante SPSS25® y se usó la prueba Mann-Whitney y X2, considerándose significativo p < 0,05. Resultados: Total 33 pacientes, 25 (75,8%) mujeres. Edad promedio 30,2 años, rango: 0-68. Adultos 23 (69,7%) pacientes y pediátricos (< 15 años) 10 (30,3%) pacientes. La presentación clínica fue sintomatología pulmonar en 23 (69,7%) casos y 9 (27,3%) eran asintomáticos. Tres (9,1%) presentaron malformación congénita asociada. Diagnóstico preoperatorio en 15 (45,5%) pacientes. La ubicación más frecuente fue lóbulo inferior izquierdo. El tipo intralobar fue el más frecuente en 23 (69,7%) casos. La cirugía más frecuente fue la lobectomía con identificación y ligadura del vaso sistémico. El vaso aberrante se originó en aorta torácica en 27 (81,8%) casos e infradiafragmático (no precisado) en 3 (9,1%) casos. Vaso único en 26 (78,8%) y doble en 5 (15,2%) casos. No hubo mortalidad. Existen diferencias en las características entre los secuestros en pacientes adultos y pediátricos. Discusión y Conclusión: Los SP son infrecuentes, se presentan principalmente en adultos jóvenes como neumopatías a repetición, se distinguen diferencias en las características entre los pacientes adultos y pediátricos, y tienen excelente pronóstico posoperatorio.

Background: Pulmonary sequestration (PS) is a congenital malformation characterized by lung tissue with vascularization from anomalous systemic arteries. Aim: To analyze characteristics and treatment of adult and pediatric patients with pulmonary sequestration. Materials and Method: Transversal descriptive study. Period: January-1988 to December-2018. Information was obtained from clinical files and pathological anatomy records. Age, sex, clinical characteristics, diagnosis, surgical treatment and pathological findings are described. Statistical analysis was performed using SPSS25® and the Mann-Whitney and Chi square test were used, considering p < 0.05 to be significant. Results: Total 33 patients, 25 (75.8%) women. Average age 30.2 years, range: 0-68. Adults 23 (69.7%) patients and pediatric (< 15 years) 10 (30.3%) patients. The clinical presentation was pulmonary symptoms in 23 (69.7%) cases and 9 (27.3%) were asymptomatic. Three (9.1%) presented another congenital malformation. Preoperative diagnosis in 15 (48.4%) patients. The most frequent location was the left lower lobe. The intralobar type was the most frequent: 23 (69.7%) cases. The most frequent surgery was lobectomy with identification and ligation of the systemic vessel. The systemic vessel originated in the thoracic aorta in 27 (81.8%) cases and infradiaphragmatic (not specified) in 3 (9.1%) cases. Single vessel in 26 (78.8%) and double in 5 (15.2%) cases. There was no mortality. Differences were found in characteristics between adult and pediatric patients. Conclusion: SP are infrequent, they mostly appear in young adults as recurrent lung diseases, differences in characteristics are distinguished between adult and pediatric patients and they have an excellent postoperative prognosis.

Successful Repair of Infantile Scimitar Syndrome with Contralateral Pulmonary Vein Stenosis in an Infant.

Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Successful repair of infantile scimitar syndrome with contralateral pulmonary vein stenosis in an infant

Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

Agenesia pulmonar como hallazgo infrecuente en adultos: reporte de caso / Pulmonary agenesis as a rare finding in adults: case report

La agenesia pulmonar es una malformación infrecuente que se caracteriza por la ausencia de uno o ambos parénquimas pulmonares, estructuras bronquiales y vasculares. Posee una incidencia de un caso por cada 10 000 a 15 000 nacidos vivos. Generalmente, el diagnóstico es realizado durante la niñez, aunque puede llegar a presentarse asintomático hasta la edad adulta, lo cual su diagnóstico resulta un hallazgo incidental durante exámenes de rutina. Habiendo pocos casos descritos. Reportamos el caso de una paciente femenina 40 años de edad, quien fue referida al servicio de emergencia de nuestro Hospital del Tórax por presentar dolor torácico, disnea de medianos esfuerzos y fiebre de un mes de evolución.

Pulmonary agenesis is a rare malformation characterized by the absence of one or both pulmonary parenchyma, bronchial and vascular structures. Has an incidence of one case per 10 000 to 15 000 live births. Generally, the diagnosis is made during childhood, although it can be asymptomatic until adulthood, which makes its diagnosis an incidental finding during routine examinations. Few cases have been described. We report the case of a 40-yearold female patient, who was referred to the emergency department of our Hospital del Tórax for presenting chest pain, dyspnea of medium efforts and fever of one month of evolution

Surgical Strategies and Results for Repair of Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals: Experience of a Single Tertiary Center.

OBJECTIVE: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). METHODS: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. RESULTS: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent single-stage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. CONCLUSION: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups.

Surgical strategies and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: experience of a single tertiary center

Abstract Objective: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. Results: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent single-stage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. Conclusion: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups.

[Pleuropulmonary blastoma, case report]. / Blastoma pleuropulmonar. Caso clínico.

Pleuropulmonary blastoma is a rare lung tumor of childhood that can occur with cystic or solid lesions, as a radiological finding with or without respiratory symptoms. We report the case of a 2 year old toddler in his first pulmonary obstructive episode with suspected toracic malformation of the left upper lobe in his chest x-ray and tomography. Surgery was performed showing cystic malformation of the left upper lobe. We received the pathology report with diagnosis of type I pleuropulmonary blastoma. He began follow-up with Oncology initiating treatment with cyclophosphamide and vincristine, well tolerated. Currently, there is controversy about the management of congenital lung cysts, tilting the balance towards the surgical procedure because of serious difficulties in differentiating benign pulmonary cysts from pleuropulmonary blastoma without histopathologic review.

El blastoma pleuropulmonar es un tumor pulmonar raro de la infancia, que puede manifestarse con lesiones quísticas o sólidas, como un hallazgo radiológico o con clínica respiratoria. Presentamos el caso de un niño de 2 años de edad que consultó en su primer cuadro obstructivo con imagen sospechosa de malformación pulmonar en el lóbulo superior izquierdo en la radiografía y la tomografía de tórax. Se realizó cirugía, que evidenció una malformación quistica en el segmento ápico posterior del lóbulo superior izquierdo. Recibimos el informe de anatomía patológica con diagnóstico de blastoma pleuropulmonar tipo I. Comenzó el seguimiento por Oncología e inició el tratamiento con ciclofosfamida y vincristina, con buena tolerancia.

Blastoma pleuropulmonar: caso clínico / Pleuropulmonary blastoma: case report

El blastoma pleuropulmonar es un tumor pulmonar raro de la infancia, que puede manifestarse con lesiones quísticas o sólidas, como un hallazgo radiológico o con clínica respiratoria. Presentamos el caso de un niño de 2 años de edad que consultó en su primer cuadro obstructivo con imagen sospechosa de malformación pulmonar en el lóbulo superior izquierdo en la radiografía y la tomografía de tórax. Se realizó cirugía, que evidenció una malformación quistica en el segmento ápico posterior del lóbulo superior izquierdo. Recibimos el informe de anatomía patológica con diagnóstico de blastoma pleuropulmonar tipo I. Comenzó el seguimiento por Oncología e inició el tratamiento con ciclofosfamida y vincristina, con buena tolerancia.

Pleuropulmonary blastoma is a rare lung tumor of childhood that can occur with cystic or solid lesions, as a radiological finding with or without respiratory symptoms. We report the case of a 2 year old toddler in his first pulmonary obstructive episode with suspected toracic malformation of the left upper lobe in his chest x-ray and tomography. Surgery was performed showing cystic malformation of the left upper lobe. We received the pathology report with diagnosis of type I pleuropulmonary blastoma. He began follow-up with Oncology initiating treatment with cyclophosphamide and vincristine, well tolerated.

Síndrome de la cimitarra: serie de casos / Scimitar syndrome: a case series

Introducción: El síndrome de la cimitarra es una compleja anomalía congénita del desarrollo pulmonar, infrecuente y de múltiples variables. Se denomina así por su semejanza radiológica con la clásica espada torcida. Su característica definitoria es el drenaje pulmonar anómalo. Se asocia con diversas malformaciones cardiotorácicas y un amplio espectro de manifestaciones clínicas. Serie de casos: Se reporta una serie proveniente de la base de datos del Hospital Infantil de México Federico Gómez, revisión del periodo 2009-2013. Se encontraron nueve pacientes con diagnóstico de síndrome de la cimitarra. Se recabaron del expediente médico las características demográficas, estado clínico y parámetros hemodinámicos reportados. Conclusiones: En la presente serie llaman la atención ciertas diferencias entre el grupo de estudio y lo reportado en la literatura internacional. Se encontró que la mayoría de los pacientes fueron del sexo femenino, diagnosticados entre el primero y el vigésimo mes de vida. En el momento del estudio estaban asintomáticos; la mitad contaba con antecedentes de enfermedad respiratoria y el total con hipertensión pulmonar. La tercera parte del grupo requirió manejo quirúrgico.

Background: Scimitar syndrome is a rare and complex congenital anomaly of the lung with multiple variables and is named for its resemblance to the classical radiological crooked sword. Its defining feature is the anomalous pulmonary drainage. It is associated with various cardiothoracic malformations and a wide spectrum of clinical manifestations. Case series: Nine patients diagnosed with scimitar syndrome found in the database of Hospital Infantil de México between 2009 and 2013 were reviewed. Demographic records, clinical status and hemodynamic parameters reported were collected. Conclusions: This case series called attention to certain differences between our group of patients and those reported in the international literature. Patients were predominantly female and were diagnosed between 1 and 20 months of life. All were asymptomatic at the time of the study. Half of the patients had a history of respiratory disease and all patients had with pulmonary hypertension. Surgical management was required in on-third of the patient group.

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.

Quistes pulmonares congénitos / Congenital lung cysts

Introducción: las malformaciones pulmonares congénitas constituyen una alteración en el desarrollo embriológico y fetal de las distintas estructuras pulmonares y una rara expresión de estas son los quistes pulmonares congénitos. Caso clínico: se presenta un recién nacido a término, de buen peso, asintomático y estable hasta el tercer día de vida al debutar con un estado de shock séptico secundario, de bronconeumonía complicada con derrame pleural derecho. En los estudios evolutivos hacia el cuarto de día de vida se le diagnostica como hallazgo radiológico la presencia de quistes pulmonares que evolucionan de forma asintomática. Después de una valoración multidisciplinaria es egresado con un seguimiento por su condición de alto riesgo. Actualmente saludable y con buen desarrollo psicomotor. Conclusiones: la importancia del tema que se presenta es que se describe una presentación rara de quistes pulmonares congénitos en un recién nacido que desarrolló un estadio avanzado de sepsis.

Introduction: congenital lung malformations constitute an alteration in the embryonic and fetal development of the different lung structures and a rare expression of these congenital lung cysts. Case report: termed newborn, good weight, evolving asymptomatic and stable until the third day of life to debut a state of septic shock secondary to complicated bronchopneumonia with right pleural effusion. In evolutionary studies by the fourth day of life it is diagnosed, as radiological finding, the presence of lung cysts evolving asymptomatically. After a multidisciplinary assessment there is a graduate tracking of their status, with high risk. Currently healthy and good psychomotor development. Conclusions: the importance of the issue presented is a rare presentation of congenital lung cysts in a newborn who developed an advanced stage of sepsis described.

[Scimitar syndrome: a case series]. / Síndrome de la cimitarra: serie de casos.

BACKGROUND: Scimitar syndrome is a rare and complex congenital anomaly of the lung with multiple variables and is named for its resemblance to the classical radiological crooked sword. Its defining feature is the anomalous pulmonary drainage. It is associated with various cardiothoracic malformations and a wide spectrum of clinical manifestations. CASE SERIES: Nine patients diagnosed with scimitar syndrome found in the database of Hospital Infantil de México between 2009 and 2013 were reviewed. Demographic records, clinical status and hemodynamic parameters reported were collected. CONCLUSIONS: This case series called attention to certain differences between our group of patients and those reported in the international literature. Patients were predominantly female and were diagnosed between 1 and 20 months of life. All were asymptomatic at the time of the study. Half of the patients had a history of respiratory disease and all patients had with pulmonary hypertension. Surgical management was required in on-third of the patient group.

Congenital lobar emphysema: 30-year case series in two university hospitals / Enfisema lobar congenito: serie de casos de 30 anos em dois hospitais universitarios

OBJECTIVE: To review the cases of patients with congenital lobar emphysema (CLE) submitted to surgical treatment at two university hospitals over a 30-year period. METHODS: We reviewed the medical records of children with CLE undergoing surgical treatment between 1979 and 2009 at the Botucatu School of Medicine Hospital das Clínicas or the Mogi das Cruzes University Hospital. We analyzed data regarding symptoms, physical examination, radiographic findings, diagnosis, surgical treatment, and postoperative follow-up. RESULTS: During the period studied, 20 children with CLE underwent surgery. The mean age at the time of surgery was 6.9 months (range, 9 days to 4 years). All of the cases presented with symptoms at birth or during the first months of life. In all cases, chest X-rays were useful in defining the diagnosis. In cases of moderate respiratory distress, chest CT facilitated the diagnosis. One patient with severe respiratory distress was misdiagnosed with hypertensive pneumothorax and underwent chest tube drainage. Only patients with moderate respiratory distress were submitted to bronchoscopy, which revealed no tracheobronchial abnormalities. The surgical approach was lateral muscle-sparing thoracotomy. The left upper and middle lobes were the most often affected, followed by the right upper lobe. Lobectomy was performed in 18 cases, whereas bilobectomy was performed in 2 (together with bronchogenic cyst resection in 1 of those). No postoperative complications were observed. Postoperative ...

OBJETIVO: Revisar os casos de enfisema lobar congênito (ELC) operados nos últimos 30 anos em dois hospitais universitários. MÉTODOS: Foram revistos os prontuários médicos das crianças com ELC operadas no Hospital das Clínicas da Faculdade de Medicina de Botucatu e no Hospital da Universidade de Mogi das Cruzes entre 1979 e 2009. Dados sobre sintomas, exame físico, achados radiológicos, diagnóstico, tratamento cirúrgico e seguimento pós-operatório mediato e tardio foram analisados. RESULTADOS: Durante o período estudado, 20 crianças com ELC foram submetidas a tratamento cirúrgico. A média de idade dos pacientes no momento da cirurgia foi 6,9 meses (variação, 9 dias a 4 anos). Todos os casos apresentaram sintomas ao nascimento ou nos primeiros meses de vida. Em todos os casos, a radiografia simples de tórax foi importante na definição do diagnóstico. Em casos de dificuldade respiratória moderada, a TC de tórax auxiliou no diagnóstico. Um paciente com desconforto respiratório grave foi diagnosticado erroneamente com pneumotórax hipertensivo e submetido a drenagem torácica. A broncoscopia só foi realizada nos pacientes com dificuldade respiratória moderada e não foram encontrados sinais de anomalias traqueobrônquicas. A abordagem cirúrgica foi realizada através de toracotomia lateral poupadora de músculo. O lobo superior esquerdo e o lobo médio ...

Hernia diagfragmática congénita derecha en el Hospital Universitario de Santander / Right congenital diaphragmatic hernia at the Hospital Universitario de Santander

Introducción: La hernia diafragmática del lado derecho es una variable poco frecuente de los defectoscongénitos diafragmáticos que permiten el paso del contenido abdominal a la cavidad torácica, causandograves trastornos en el desarrollo pulmonar fetal. Objetivo: Describir las características patológicasencontradas en la autopsia perinatal de un paciente con Hernia diafragmática congénita derecha en elHospital Universitario de Santander. Caso clínico: Neonato de 35 semanas de gestación con diagnósticoprenatal de Hernia Diafragmática Congénita, quien fallece minutos después de su nacimiento debido ainsuficiencia respiratoria aguda. En los hallazgos de autopsia se encuentra ausencia de la mayor parte delhemidiafragma derecho, herniación del contenido abdominal al tórax y una severa hipoplasia pulmonar.Conclusión: La Hernia diafragmática congénita del lado derecho se asocia con alta mortalidad neonataly los hallazgos encontrados en el presente caso se correlacionan con los graves defectos estructuralespulmonares que se describen en otros casos reportados en la literatura.

Introduction: The congenital diaphragmatic hernia of the right side is the least common type ofthe congenital diaphragmatic defects which allows the passage of abdominal contents to the thoraciccavity, causing serious disorders on lung development. Objective: To describe the pathological featuresfound in perinatal autopsy of a patient with Congenital Diaphragmatic Hernia of the right side at theHospital Universitario de Santander. Case report: 35 weeks gestation neonate with prenatal diagnosisof congenital diaphragmatic hernia, who died due to acute respiratory failure. In the autopsy be founda severe pulmonary hypoplasia and in the microscopic examination, the pulmonary alveoli collapsed.Conclusion: The congenital diaphragmatic hernia of the right side is associated with high neonatalmortality and the findings in this case are correlated with severe pulmonary structural defects that aredescribed in other cases reported in the literature.