Complete thoracoscopic surgery in the treatment of congenital pulmonary airway malformation in children / 中华实用儿科临床杂志

Objective:To summarize the experience and technical difficulties of complete thoracoscopic lobectomy(segmental) in the treatment of congenital pulmonary airway malformation in children.Methods:The clinical data, surgical videos and follow-up results of 38 children suffering from congenital pulmonary airway malformation and underwent complete thoracoscopic lobectomy in Children′s Hospital of Fudan University Xiamen Branch from January 2017 to December 2019 were retrospectively analyzed.A statistical comparisons of operative time, intraoperative blood loss, postoperative pain (the first time of getting out of bed), incision length and postoperative pulmonary complications were made between 28 children who underwent the total thoracoscopic lobectomy and 21 children who accepted open surgical lobectomy before January 2017 in Children′s Hospital of Fudan University Xiamen Branch.Results:Thirty-eight children with congenital pulmonary airway malformation were successfully operated by complete thoracoscopy.There were 28 lobectomies, 6 segmental lobectomies and 4 wedge lobectomies.Postoperative follow-up was conducted for more than 3 months, and no serious surgical complications occurred.Chest radiograph of 2 children with the right lower lobectomy at 48 hours after surgery had the elevation of the right diaphragm (2-3 intercostal), and it returned to normal 3 months after surgery.The CT review of 1 child with pulmonary wedge resection 1 month after surgery displayed a little residual lesion.Thoracoscopic lobectomy was significantly different from open surgery in terms of incision length[(2.0±0.5) cm vs. (10.0±0.5) cm], postoperative pain (the first time of getting out of bed)[(24.0±2.0) h vs. (48.0±1.5) h] and pulmonary complications (0 vs. 5 cases). The operative time of single lung ventilation was remarkably different from that of double lung ventilation in thoracoscopic lobectomy[(96.0±6.0) min vs. (118.0±7.0) min, t=106.501, P<0.001]. Compared with thoracoscopic lobectomy, thoracoscopic pulmonary segmentectomy has a long time and a large amount of blood loss. Conclusions:Complete thoracoscopic lobectomy (segmental) is the first choice for the treatment of congenital pulmonary airway malformation in children, with clear operative field, little trauma, less postoperative pain, quick recovery and fewer respiratory complications.Single-lung ventilation is beneficial for surgical completion.

Type I pleuropulmonary blastoma presenting as congenital pulmonary airway malformation: A report of two cases

Pleuropulmonary blastoma (PPB) is a rare aggressive intrathoracic tumor which is believed to originate from embryonic uncommitted lung mesenchymal cells, which are important for developing the lung. Type I PPB is cystic, type II is cystic and solid, while type III is predominantly solid. Diagnosing type 1 PPB is a challenge for both radiologists as well as pathologists. Owing to its purely cystic nature, type I PPB it is often mistaken for unrelated entities such as congenital pulmonary airway malformation and congenital lobar emphysema which delays surgical intervention. Here, we report two such cases presenting clinically and radiologically as congenital pulmonary airway malformation. On histology, a final diagnosis of type I pleuropulmonay blastoma was made. Thereafter, chemotherapy was administered following complete surgical excision.

Outcomes of cases of prenatally-diagnosed congenital pulmonary airway malformation / Desfechos de casos de malformação das vias respiratórias diagnosticados no pré-natal

Abstract Objective To evaluate the outcomes of cases of prenatally-diagnosed congenital pulmonary airway malformation (CPAM). Methods We retrospectively evaluated cases of prenatally-diagnosed CPAM between 2004 and 2018. Ultrasonographic features such as visualization of a fetal lung mass and heterogeneous pulmonary parenchyma were used for CPAM diagnosis. Prenatal and postnatal findings were compared in terms of accuracy regarding the CPAM diagnosis. Results The sample consisted of 27 cases. There were four cases in which the patients opted for the termination of pregnancy due to the severity of the lesion. A total of 23 neonates were delivered, and CPAM was confirmed in 15 cases. Themedian gestational age at delivery was 37 weeks (28-40 weeks) and the mean birth weight was 2,776 g. There were two neonatal deaths, one due to pneumothorax, and the other due to hypoplastic left heart syndrome (HLHS). A total of five patients with respiratory problems were operated in the postpartum period. There were eight misdiagnosis: bronchopulmonary sequestration (five cases), congenital lobar emphysema (two cases), and congenital diaphragm hernia (one case). Conclusion A precise postnatal diagnosis is very important to organize the proper management of the pregnancies with fetuses with CPAM. The positive predictive value of the prenatal diagnosis of CPAM via ultrasonography is of 70.3%. The differential diagnosis of CPAM may be prolonged to the postpartum period in some cases.

Utilidad de la quimioterapia con corticoides en la reducción de volumen de las lesiones en fetos con malformación congénita de la vía aérea pulmonar (MCVAP): serie de casos / Usefulness of chemotherapy with corticosteroids in reducing the volume of the lesions in fetuses with congenital pulmonary airway malformation (MCVAP): case series

RESUMEN OBJETIVO: el propósito es describir el efecto de los corticoides prenatales en la malformación congénita de la vía aérea pulmonar (MCVAP) mediante la evaluación secuencial del CVR y mostrar la experiencia en nuestro centro de terapia fetal. MATERIALES Y MÉTODOS: serie de casos en la cual se incluyeron ocho pacientes del Hospital de San José; con diagnóstico de MCVAP; se evaluó edad gestacional, el CVR (longitud x largo x ancho, en cms de la masa) x 0.523/circunferencia cefálica) como factor pronóstico, la desviación mediastínica, la presencia de hidrops, el tipo de MCVAP y la localización. Se aplicó betametasona a dosis de inducción de madurez pulmonar fetal. Se realizó la medición del CVR pre y pos tratamiento. Los datos obtenidos fueron analizados mediante el software estadístico SPSS (Versión 11.0 SPSS Inc. Chicago, IL). Se utilizaron la prueba Wilcoxon y el índice de correlación de Spearman según el caso. Un valor de p<0.05 se consideró significativo. RESULTADOS: ocho pacientes fueron incluidas. La mediana de la edad gestacional fue 30.5 semanas. Cuatro casos fueron tipo I, dos tipo II y dos tipo tres. Cuatro casos presentaron desviación mediastinal. Cinco casos fueron derechos y uno bilateral. Ningún caso presentó hidrops. El CVR pre tratamiento fue 0,93(RIQ:0,17-2,1) y pos tratamiento 0,55(RIQ:0,07-1,39). Se encontró una disminución del CVR pos tratamiento en todos los casos (p 0.0117) e índice de correlación de Spearman 0.9524 CONCLUSIÓN: Después de la aplicación de corticoides encontramos una disminución del CVR en todos los casos descritos. El uso de corticoides podría ser parte de la terapia prenatal con miras a mejorar el pronóstico.

ABSTRACT OBJECTIVE: The purpose of this study is to describe the effect of prenatal corticosteroids in the CPAM by sequential evaluation of the CVR, and to show the experience in our fetal therapy center. MATERIALS AND METHODS: Series of cases in which eight patients from the Hospital of San José were included; with diagnosis of CPAM; gestational age was evaluated, CVR (CVR = length x length x width cm, mass) x 0.523 / head circumference) as a prognostic factor, mediastinal shift, hydrops, type of CPAM, location. Betamethasone was applied to fetal lung maturation dose. In all patients underwent CVR measurement pre and post treatment. The data were analyzed using SPSS statistical software (Version 11.0 SPSS Inc. Chicago, IL). The Wilcoxon test and the Spearman correlation index were used according to the case. A value of p <0.05 was considered significant. RESULTS: eight patients were included. The median of gestational age was 30.5 weeks. Four cases were type I, two type II and two type three. Four cases were mediastinal shift. Five cases were rights and one bilateral. No case presented hydrops. The pretreatment CVR was 0,93(RIQ: 0,17-2,1) and post-treatment 0,55(RIQ: 0,07-1,39). We found a decrease in CVR after treatment in all cases (p 0.0117) and Spearman correlation index 0.9524 CONCLUSION: After application of corticosteroids are a reduction in CVR in all the cases described. The use of corticosteroids may be part of prenatal therapy to improve prognosis.

The application of neonate lung ultrasound in congenital pulmonary airway malformation / 中华超声影像学杂志

Objective@#To analyze the characteristics of lung ultrasound (LUS) in congenital pulmonary airway malformation (CPAM), and to compare the difference of image characteristics between LUS and chest X-ray(CXR) and evaluate the value of neonate LUS in CPAM.@*Methods@#From June 2017 to October 2018, the characteristics of LUS and CXR of 32 neonates who were diagnosed with CPAM by prenatal ultrasound and postnatal chest CT were analyzed retrospectively, and the difference of their direct signs and indirect signs were compared.@*Results@#Among 32 CPAM cases, 10 cases (31.25%) showed a single large cystic lesions and 3 cases with consolidation, 4 cases (12.5%) characterized by multiple hypoechoic lesions and 3 cases with consolidation, 16 cases (50%) showed consolidation with or without intensiveaerated bronchus sign or dendritic air-filled bronchi, 2 cases (6.25%) only characterized by dense B-line. The non-characteristic sonographic findings including pleural line was not smooth and not clear; A-line decreased or disappeared, dense B-lines were observed. The ability of LUS in measuring the size of CPAM was limited.Among 32 CPAM cases, 10 cases (31.25%) showed large cystic hypoechoic lesions and 22 cases (68.75%) showed small cystic hypoechoic lesions by CT scan. In this group, there were 11 cases(34.4%) with normal CXR. There was no significant differences of the direct signs (including single large cystic lesions and multiple hypoechoic lesions) between LUS and CXR (P=0.80), however the indirect signs of LUS were more obvious than CXR, with significant difference(P=0.001).@*Conclusions@#The neonatal LUS findings of CPAM is multiple, it can be used as a preliminary qualitative screening method.The diagnosis value of indirect sign of LUS is superior to CXR.

The application of neonate lung ultrasound in congenital pulmonary airway malformation / 中华超声影像学杂志

Objective To analyze the characteristics of lung ultrasound (LUS) in congenital pulmonary airway malformation (CPAM),and to compare the difference of image characteristics between LUS and chest X-ray(CXR) and evaluate the value of neonate LUS in CPAM.Methods From June 2017 to October 2018,the characteristics of LUS and CXR of 32 neonates who were diagnosed with CPAM by prenatal ultrasound and postnatal chest CT were analyzed retrospectively,and the difference of their direct signs and indirect signs were compared.Results Among 32 CPAM cases,10 cases (31.25％) showed a single large cystic lesions and 3 cases with consolidation,4 cases (12.5％) characterized by multiple hypoechoic lesions and 3 cases with consolidation,16 cases (50％) showed consolidation with or without intensiveaerated bronchus sign or dendritic air-filled bronchi,2 cases (6.25 ％) only characterized by dense B-line.The non-characteristic sonographic findings including pleural line was not smooth and not clear;A-line decreased or disappeared,dense B-lines were observed.The ability of LUS in measuring the size of CPAM was limited.Among 32 CPAM cases,10 cases (31.25 ％) showed large cystic hypoechoic lesions and 22 cases (68.75％) showed small cystic hypoechoic lesions by CT scan.In this group,there were 11 cases (34.4％) with normal CXR.There was no significant differences of the direct signs (including single large cystic lesions and multiple hypoechoic lesions) between LUS and CXR (P =0.80),however the indirect signs of LUS were more obvious than CXR,with significant difference(P =0.001).Conclusions The neonatal LUS findings of CPAM is multiple,it can be used as a preliminary qualitative screening method.The diagnosis value of indirect sign of LUS is superior to CXR.

Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience

PURPOSE: Congenital pulmonary airway malformation (CPAM)—a rare developmental anomaly—affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide comprehensive clinical data for the proper management of CPAM. METHODS: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. RESULTS: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive predictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent video-assisted thoracoscopy. Twenty-five and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. CONCLUSION: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with prenatally diagnosed CPAM and to guide appropriate timing of surgery.

Secuestro pulmonar asociado a una malformación congénita de la vía aérea pulmonar / Pulmonary sequestration associated with congenital pulmonary airway malformation

Resumen Introducción: Las malformaciones pulmonares congénitas son una causa poco frecuente de morbilidad neonatal. Algunas de ellas tienen un origen común, lo que permite identificar lesiones combinadas. Su diagnóstico puede realizarse de forma prenatal mediante ultrasonido, con las limitaciones de que solo se realiza en centros especializados y que depende de la pericia del operador. La asociación entre el secuestro pulmonar y la malformación congénita de la vía aérea se ha descrito aproximadamente en 40-60 casos desde 1949, cuando se observó por primera vez. Muchas lesiones no son perceptibles en la vida intrauterina. Sin embargo, en el periodo neonatal se presentan síntomas respiratorios recurrentes que en algunos casos están asociados con una malformación pulmonar. Caso clínico: Se presenta el caso de una lactante diagnosticada con secuestro pulmonar a las 24 semanas de edad gestacional. Recibió tratamiento quirúrgico intrauterino con reporte de resolución completa de la malformación en ultrasonidos posteriores. Fue valorada por neumología pediátrica a los 4 meses de edad. Se realizó una angiotomografía y se confirmó la presencia de secuestro pulmonar, por lo que se realizó una lobectomía. El estudio histopatológico reportó secuestro pulmonar extralobar con malformación congénita de la vía aérea pulmonar tipo 2. Estas lesiones combinadas se identificaron mediante un estudio histopatológico. El tratamiento de elección fue quirúrgico. Conclusiones: Ante la confirmación de una malformación, destaca la importancia de realizar la búsqueda de otras malformaciones que pudieran estar asociadas.

Abstract Introduction: Congenital pulmonary malformations are a rare cause of neonatal morbidity. Some of them have a common origin, which allows the identification of combined lesions. Its diagnosis can be made prenatally by ultrasound, with the limitation that this study is performed in specialized centers and depends on the expertise of the operator. The association of pulmonary sequestration and congenital malformation of the airway has been described in approximately 40-60 cases since its first description in 1949. Many lesions are not perceptible in intrauterine life and in the neonatal period there are recurrent respiratory symptoms that in some cases are associated with a congenital pulmonary malformation. Case report: We report the case of a young infant, who was diagnosed with pulmonary sequestration at 24 weeks of gestational age, undergoing intrauterine surgical treatment with a report of complete resolution of the malformation in posterior ultrasounds. She was valued by pediatric pneumology at 4 months of age, where angiotomography was performed and the presence of pulmonary sequestration was confirmed by lobectomy. The histopathological study reported extralobar pulmonary sequestration with congenital malformation of the pulmonary airway type 2. These combined lesions were identified by histopathological study. The treatment of choice was surgical. Conclusions: Upon the confirmation of a malformation, we emphasize the importance of performing a screening in order to search for other that could be associated.

Diagnosis and treatment of congenital pulmonary airway malformation / 中华胸心血管外科杂志

Objective To summarize the diagnosis and treatment of congenital pulmonary airway malformation in children.Methods The clinical data of 68 children with congenital pulmonary airway malformations from June 2014 to June 2017 were analyzed retrospectively,including 39 males and 29 females with mean age(1.1 ±:0.5) years old(from 2 months 12 days to 15 years),7 cases of pulmonary cyst resection in 68 cases,3 cases with lung segment resection,1 cases with lingulectomy,57 cases with lobectomy.Results 68 cases were cured and discharged without death.Postoperative short-term complications included subcutaneous emphysema in 5 cases,pleural effusion in 2 cases,pneumothorax in 2 cases.All cases were followed up well between 3 months and 1 year.Conclusion The diagnosis of congenital pulmonary airway malformation in children mainly depends on imaging examination.Because it is easy to repeat infection,and can not rule out malignant transformation,early diagnosis and early treatment should be done.Surgical resection is the only way to treat the disease.

The diagnosis and treatment of congenital pulmonary airway malformation in children / 中华胸心血管外科杂志

Objective The purpose of the study is introducing the concept and classification of congenital pulmonary airway malformation(CPAM),and summarizing the clinical features,imaging,pathological features and treatment methods of congenital pulmonary airway malformation.Methods 283 cases were retrospectively analyzed from April 2012 to November 2017,which were treated by thoracic surgery,pathological diagnosis were isolated congenital pulmonary airway abnormalities except combination of other deformity surgery at the same time.we analyze the clinical data,surgical methods,operation and postoperative pathology classification.Results All the 283 cases were successfully performed without severe surgical complications and secondary thoracotomy.There were 167 males and 116 females,male∶ female =1.44∶1;Age:0.77-166 months,median age 20.67 months;The operation time was 25-220 min,median time was 75min,and the bleeding volume was 1-250 ml,and the median blood volume was 5 ml.Among them,80 patients underwent thoracotomy,203 cases of thoracoscopic surgery,19 cases were transferred.Postoperative pathology 57 cases of type 1,194 cases of type 2,5 cases of type 3,9 cases of type 4.Conclusion CCAM has been renamed as congenital pulmonary airway malformation (CPAM) and has been reclassified.CPAM can be detected by imaging examination,and the diagnosis is based on histopathological examination,and the prognosis is better after the complete surgical resection of lung lesions.Thoracoscopic minimally invasive surgery could make less surgical trauma,small and beautiful wound,which is suggested to be promoted widely.

Congenital pulmonary airway malformation with atrial septal defect and pulmonary hypertension for lobectomy‑anesthetic considerations.

The association of congenital pulmonary airway malformation (CPAM) with congenital heart disease is rare. We present the case of a 6‑month‑old child with atrial septal defect and pulmonary hypertension (PH) who presented with severe respiratory distress and hypoxia. The patient underwent right lobectomy for CPAM. With timely management, real‑time monitoring, one lung ventilation, and adequate analgesia, we were able to extubate the child in the immediate postoperative period. We conclude that with meticulous planning and multidisciplinary team approach, such complex cases can be managed successfully.

Congenital pulmonary airway malformation / 中华实用儿科临床杂志

Congenital pulmonary airway malformation (CPAM),which was previously known as congenital cystic adenomatoid malformation,is one of the most common congenital lung abnormalities.In recent years,more and more reports about the disease have been published and its classifications also have new changes.This article introduces the concept of CPAM,classification,clinical manifestations,differential diagnosis,and treatment strategies.

Malformación congénita de la vía aérea pulmonar o malformación adenomatoidea quística congénita: Serie clínica de 16 pacientes / Congenital pulmonary airway malformation or congenital cystic adenomatoid malformation

Objective: To describe 16 patients treated for a congenital pulmonary anomaly named congenital pulmonary airway malformation (CPAM) or congenital cystic adenomatoid malformation (CCAM) in Roberto del Río children S Hospital. Methods: We retrospectively analyzed the medical records of patienes who had had surgery for CPAM/CCAM from January 2000 to July 2014. Results: We found 16 cases, 9 of them were males. Nine had surgery within the first year of life (5 of them within 3 weeks of life). According to Stocker 's classification CPAM/CCAM was type 1 in 9 patients, and type 2 in 7. Diagnosis was based on antenatal ultrasonography s (n = 10), persistent abnormalities in Chest X-Ray (n = 4), recurrent pneumonia (n = 1) and incidental radiological findings (n = 1). Computed Tomography (CT) was performed in 13 patients, CT angiography in 2 and only chest x-ray in one. The lesions were located in right upper lobe (3), right middle lobe (2), right lower lobe (4), left upper lobe (4) and left lower lobe (3). One patient had pectus excavatum and another one had ventricuar septal defect. They all had open thoracotomy with lobectomy of the affected area. Postoperatively was uneventful except for transient lobar atelectasis in 2 patients. There was no mortality. Conclusions: We emphasize the early diagnosis and good outcome of our patients treated which this disease. Our findings are consistent with those that has been reported in the literature.

Objetivo: Describir los pacientes tratados por malformación congénita de la vía aérea pulmonar (MCVAP) o malformación adenomatoidea quística congénita (MAQC) en el Hospital de niños Roberto del Río desde el año 2000 hasta el segundo semestre de 2014. Métodos: Revisión retrospectiva de fichas clínicas. Resultados: 16pacientes, 9 hombres. Se operaron antes del año de vida 9 pacientes (5 de ellos antes de las 3 semanas de vida). Tipo de MCVAP (según clasificación de Stocker) tipo 1 fue 9/16 y tipo 2 fueron 7/16. La sospecha diagnóstica fue en base a diagnóstico prenatal 10/16, imagen radiológica persistente 4/16, neumonías repetidas 1/16 y hallazgo radiológico 1/16. Estudio imagenológico: Tomografia computada (TC) 13/16, Angio-TC 2/16y radiografía de tórax 1/16. Ubicación: lóbulo superior derecho 3/16, lóbulo medio 2/16, lóbulo inferior derecho 4/16, lóbulo superior izquierdo 4/16, lóbulo inferior izquierdo 3/16. Comorbilidad: ninguna 14/16, pectus excavatum 1/16, comunicación interventricular 1/16. Tratamiento quirúrgico: en todos los pacientes se realizó lobectomía total del lóbulo afectado por vía abierta. Evolución postoperatoria: Buena 14/16, atelectasia 2/16. No se registró mortalidad. Conclusiones: Se destaca el diagnóstico precoz y buena evolución de nuestros pacientes concordantes con la literatura.

Malformación congénita de la vía aérea pulmonar: reporte de un caso adulto / Congenital pulmonary airway malformation: an adult case report

Congenital pulmonary airway malformation or CPAM (congenital cystic adenomatoid malformation) that involve the lung is an embryonic anomaly. CPAM is a rare condition in chilhood, and even more so in adulthood. In this article we report the case of a young adult man with CPAM and present a brief review about this condition. Case study: 23 years old man with several episodes of pneumonia and obstructive bronchial syndrome during his childhood. The patient complained of pleuritic chest pain, malaise and fever. On physical examination lung sound was diminished in the lower right zone of the chest, abundant coarse crackles were detected and there was dullness on percussion. Chest roentgenogram and computed tomography (CT) revealed a complex image of multiple cysts with air and fluid in the right lung. Because of the lack of response to medical treatment a right lower lobectomy was performed. Histopathology revealed a type 1 CPAM. After being discharged from the hospital the patient developed a broncho-pleural fistula, that was succesfully treated by a second surgical intervention. Three years after surgery the patient is free of symptoms. Discussion: CPAM is a rare congenital hamartomatous condition. This malformation in adults it is exceptional. Chest CT is the choice diagnostic technique. Considering the high neoplastic potential of this malformation, its treatment should be the surgical resection, even in asymptomatic patients.

La malformación congénita de la vía aérea pulmonar (MCVAP), llamada también malformación congénita adenomatoidea quística que compromete al pulmón es una anomalía embrionaria. La MCVAP es de rara presentación en la infancia y es excepcional en los adultos. En este artículo comunicamos el caso de un hombre adulto con MCVAP y presentamos una breve revisión sobre esta condición. Caso clínico: Hombre de 23 años de edad con varios episodios de neumonía y de síndrome bronquial obstructivo en su niñez. El paciente se quejaba de dolor torácico pleurítico, malestar general y fiebre. En el examen físico se encontró disminución del murmullo pulmonar en la parte inferior del hemitórax derecho, abundantes crepitaciones y matidez. La radiografía y la tomografia computada de tórax (TC) revelaron múltiples imágenes hidro-aéreas complejas en el pulmón derecho. Debido a la falta de respuesta al tratamiento médico, se practicó una lobectomía inferior derecha. La histopatología demostró una MCVAP tipo 1. Después de ser dado de alta el paciente presentó una fístula broncopleural, la cual fue tratada exitosamente con una segunda intervención quirúrgica. Tres años después de la cirugía el paciente está asintomático. Discusión: La MCVAP es una malformación congénita hamartomatosa muy poco frecuente. En adultos es excepcional. La técnica diagnóstica de elección es la TC de tórax. Considerando el alto potencial neoplásico de esta malformación, su tratamiento debe ser la resección quirúrgica, incluso si el paciente está asintomático.