RESUMEN
INTRODUCTION: The aim of this study is to evaluate long-term neurodevelopmental and respiratory outcome after fetal therapy for fetal pleural effusion, congenital cystic adenomatoid malformation, and bronchopulmonary sequestration. METHODS: Children ≥18 months of age underwent an assessment of neurologic, motor, and cognitive development. Medical records were reviewed to determine respiratory outcome. Behavioral outcome was assessed using the Child Behavioral Checklist. RESULTS: Between 2001 and 2016, 63 fetuses with fetal hydrops secondary to thoracic abnormalities were treated at our center. Overall perinatal survival was 64% (40/63). Twenty-six children were included for follow-up (median age 55 months). Severe neurodevelopmental impairment (NDI) was detected in 15% (4/26). Three out of 4 children with severe NDI had associated causes contributing to the impairment. Overall adverse outcome, including perinatal mortality or NDI, was 55% (27/49). Fifteen percent (4/26) had severe respiratory sequelae. Parents did not report more behavioral problems than Dutch norms. DISCUSSION: Our results suggest that severe NDI in this specific high-risk cohort occurs in 15%, which is above the range of the incidence of NDI reported in case series treated with other fetal therapies (5-10%). Large multicenter studies and an international web-based registry are warranted to prospectively gather outcome data at fixed time points.
Asunto(s)
Secuestro Broncopulmonar/cirugía , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Enfermedades Fetales/cirugía , Terapias Fetales/efectos adversos , Hidropesía Fetal/cirugía , Trastornos del Neurodesarrollo/etiología , Derrame Pleural/cirugía , Adulto , Secuestro Broncopulmonar/diagnóstico por imagen , Secuestro Broncopulmonar/mortalidad , Niño , Conducta Infantil/fisiología , Preescolar , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico por imagen , Malformación Adenomatoide Quística Congénita del Pulmón/mortalidad , Femenino , Enfermedades Fetales/diagnóstico por imagen , Enfermedades Fetales/mortalidad , Terapias Fetales/métodos , Humanos , Hidropesía Fetal/diagnóstico por imagen , Hidropesía Fetal/mortalidad , Lactante , Masculino , Derrame Pleural/diagnóstico por imagen , Derrame Pleural/mortalidad , Embarazo , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento , Ultrasonografía PrenatalRESUMEN
PURPOSE: The prenatal natural history of intralobar and extralobar bronchopulmonary sequestrations (BPSs), including lesion growth patterns and need for prenatal intervention, have not been fully characterized. We review our series of BPSs to determine their natural history and outcomes in the context of the need for prenatal intervention. METHODS: A retrospective review of the pre/postnatal course of 103 fetuses with an intralobar (n=44) or extralobar BPS (n=59) managed at a single institution between 2008 and 2015 was performed. Outcomes included prenatal lesion growth trajectory, presence of hydrops, need for prenatal intervention, survival, and postnatal surgical management. RESULTS: Most extralobar (71%) and intralobar BPSs (94%) decreased in size or became isoechoic from initial to final evaluation. Peak lesion size occurred at 26-28weeks gestation. Eight fetuses developed hydrothorax, four of which (all extralobar BPSs) also developed hydrops. All four hydropic fetuses received maternal betamethasone, and three hydropic fetuses underwent thoracentesis and/or thoracoamniotic shunt placement with subsequent hydrops resolution. All fetuses survived. Forty-one intralobar (93%) and 35 extralobar BPSs (59%) were resected after birth. CONCLUSIONS: BPSs tend to decrease in size after 26-28weeks gestation and rarely require fetal intervention. Lesions resulting in hydrothorax ± hydrops can be effectively managed with maternal steroids and/or drainage of the hydrothorax. LEVEL OF EVIDENCE: IV.
Asunto(s)
Secuestro Broncopulmonar/embriología , Secuestro Broncopulmonar/terapia , Terapias Fetales/métodos , Atención Perinatal/métodos , Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/mortalidad , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Masculino , Embarazo , Diagnóstico Prenatal , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Purpose To describe the prenatal course and perinatal outcome, and to define prognostic markers for fetuses with congenital pulmonary airway malformation (CPAM) or bronchopulmonary sequestration (BPS). Materials and Methods A retrospective study was performed at the University Hospital Zurich including pregnancies with either fetal CPAM (nâ=â26) or BPS (nâ=â11) between 2000 and 2013. Results Three patients decided for termination of pregnancy. Two intrauterine deaths (CPAM) occurred at 25 weeks. Minimally invasive interventions were performed in 9/37 (24â%) fetuses, post-interventional survival was 8/9 (89â%). Mean gestational age at delivery was 38.1â+/-2.8 and 39.1â+/-2.5 weeks in fetuses with CPAM or BPS, respectively. In fetuses with CPAM the perinatal mortality rate was 4/24 (17â%); the rate of invasive interventions or surgery during the early neonatal period (neonatal morbidity) was 9/22 (41â%). Prenatal diagnosis of hydrothorax and/or increasing cystic volume ratio (CVR) until delivery preceded perinatal death in 3/5 (60â%). Absent mediastinal shift showed a neonatal morbidity rate of 1/8 (13â%) without any perinatal mortality. In fetuses with BPS the perinatal morbidity and mortality were both 1/10 (10â%). Hydrops predicted morbidity and mortality in 100â% of cases. Absent hydrops was followed by uncomplicated perinatal outcome. Conclusion Fetuses with CPAM or BPS have a good outcome under optimal perinatal care including the possibility to perform minimally invasive prenatal interventions. CPAM without mediastinal shift and BPS without hydrops have an excellent prognosis. Hydrothorax, increasing CVR or hydrops indicates a high risk for perinatal morbidity and mortality.
Asunto(s)
Secuestro Broncopulmonar/diagnóstico por imagen , Anomalías del Sistema Respiratorio/diagnóstico por imagen , Ultrasonografía Prenatal , Aborto Eugénico , Secuestro Broncopulmonar/mortalidad , Secuestro Broncopulmonar/cirugía , Parto Obstétrico , Femenino , Humanos , Pulmón/diagnóstico por imagen , Masculino , Valor Predictivo de las Pruebas , Embarazo , Segundo Trimestre del Embarazo , Pronóstico , Anomalías del Sistema Respiratorio/cirugía , Estudios Retrospectivos , Análisis de Supervivencia , Toracotomía , Resultado del TratamientoRESUMEN
PURPOSE: The goal of this study was to compare the safety and efficacy of treatment for pulmonary sequestration (PS) by transcatheter arterial embolization (TAE) versus surgical resection and to consider the role of a thoracoscopic approach. METHODS: A retrospective review involving 73 children (≤ 15 years of age) with PS between 2002 and 2011 was performed. RESULTS: Forty-two patients were managed with TAE, and 31 underwent surgery alone. Their presenting symptoms were pneumonia (n=11), pneumothorax (n=2), pneumomediastinum (n=1) and respiratory distress (n=6).Fifty-three (72.6%) were asymptomatic. The average age at treatment was 17.0 ± 44.4 and 31.3 ± 41.7 months for the TAE and surgery groups, respectively. In the TAE group, complete regression was observed in only 3 patients, 4 showed no regression, and 35 (83.3%) had residual lesions. Four patients developed sepsis or other blood vessel complications after TAE. The results of resection via thoracotomy versus a thoracoscopic approach were evaluated in 34 patients, including 3 who underwent the operation after TAE. Twenty-seven patients underwent thoracotomy, and 7 underwent thoracoscopic resection. There were no significant differences between the groups except time to chest tube removal, which was shorter in the thoracoscopic group (p=0.046). Complications included a wound infection in 1 patient after thoracotomy. CONCLUSIONS: We believe that even in asymptomatic patients, all PSs should be resected because of the risk of infection, the low rate of natural regression, complications after TAE, and to exclude other pathology. Our experience also shows that thoracoscopic resection of PS is feasible, efficacious, and safe in newborns and infants.
Asunto(s)
Secuestro Broncopulmonar/cirugía , Embolización Terapéutica/métodos , Toracoscopía/métodos , Toracotomía/métodos , Factores de Edad , Secuestro Broncopulmonar/diagnóstico por imagen , Secuestro Broncopulmonar/mortalidad , Secuestro Broncopulmonar/terapia , Preescolar , Estudios de Cohortes , Embolización Terapéutica/efectos adversos , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Corea (Geográfico) , Tiempo de Internación , Masculino , Procedimientos Quirúrgicos Mínimamente Invasivos/efectos adversos , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Radiografía , Estudios Retrospectivos , Medición de Riesgo , Estadísticas no Paramétricas , Análisis de Supervivencia , Toracoscopía/efectos adversos , Toracotomía/efectos adversos , Resultado del TratamientoRESUMEN
We examined retrospectively the characteristics and the outcomes of intralobar sequestrations (ILS). We reviewed data we obtained from the medical records of patients with ILS who underwent surgery at Laennec, Georges Pompidou European, and Amiens South Hospital. From 1985 to 2010, 26 consecutive adults patients underwent surgery for ILS. There were 14 males and 12 females. The average age was 37.3 years. The ILS was right-sided in 11 patients (42.3%) and left-sided in 15 patients (57.7%). A systemic artery supply was found during the preoperative period in 11. Surgery consisted of lobectomy (n=20), bilobectomy (n=1), segmentectomy (n=4), and pneumonectomy (n=1). There were no postoperative deaths, and the postoperative course was uneventful in 20 patients. All patients were alive and faring well at long-term follow-up (mean follow-up 36.5 ± 7.2 months). Surgery consisted of lobectomy in most cases. The arterial supply came from the descending thoracic and abdominal aorta. Hemoptysis and/or recurrent infections were present in 14/26 (54%) of patients. These are the same symptoms as those leading to the diagnosis of bronchectasis. This suggests, for similar reasons, that ILS in adults should be nosologically very similar to acquired lesions, such as bronchectasis.
Asunto(s)
Secuestro Broncopulmonar/cirugía , Neumonectomía , Adolescente , Adulto , Anciano , Secuestro Broncopulmonar/mortalidad , Femenino , Francia , Humanos , Masculino , Persona de Mediana Edad , Neumonectomía/efectos adversos , Neumonectomía/mortalidad , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: This study aimed to compare the results of thoracoscopic surgery for congenital lung diseases between infants younger than 6 months and those older than 6 months at the time of surgery in terms of operation duration, surgical complications, chest tube duration, and hospital stay. METHODS: The charts of 30 thoracoscopic resections for congenital lung diseases were retrospectively reviewed. This study compared 17 children younger than 6 months (mean, 3.94 months; range, 0.37-5.7 months; group 1) with 13 children older than 6 months (mean, 12.05 months; range, 6.2-24.63 months; group 2) at the time of surgery. The median follow-up period was 9 months (range, 1-41 months). RESULTS: Lobectomy was performed in 27 cases, bilobectomy in 1 case, and nonanatomic excision in 2 cases. The mean operating time for group 1 (176±54 min) was similar to that for group 2 (160±46 min). The difference is not significant. The mean duration of chest tube drainage was similar in the two groups (4.4 days; range, 1-9 days for group 1 vs. 4.1 days; range, 3-8 days for group 2). The complications included 1 major and 10 minor complications, with no statistically significant difference between the two groups. Three surgical procedures in each group were converted. The hospital stay was not statistically different between the two groups (8 days; range, 3-20 days for group 1 vs. 6 days; range, 4-10 days for group 2). CONCLUSIONS: The study findings showed no statistically significant difference between the two groups in terms of operation time, complication rate, conversion rate, or hospital stay. Lobectomy can be safely and successfully performed by thoracoscopy even for children younger than 6 months.
Asunto(s)
Pulmón/anomalías , Neumonectomía/métodos , Anomalías del Sistema Respiratorio/cirugía , Toracoscopía/métodos , Factores de Edad , Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/mortalidad , Secuestro Broncopulmonar/cirugía , Estudios de Cohortes , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/mortalidad , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Neumonectomía/mortalidad , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/fisiopatología , Pronóstico , Anomalías del Sistema Respiratorio/diagnóstico , Anomalías del Sistema Respiratorio/mortalidad , Estudios Retrospectivos , Medición de Riesgo , Análisis de Supervivencia , Toracoscopía/mortalidad , Resultado del TratamientoRESUMEN
PURPOSE: This study evaluates the safety and efficacy of thoracoscopic lobectomy in infants and children. METHODS: From January 1995 to March 2007, 97 patients underwent video-assisted thoracoscopic lobe resection. Ages ranged from 2 days to 18 years and weights from 2.8 to 78 kg. Preoperative diagnosis included sequestration/congenital adenomatoid malformation (65), severe bronchiectasis (21), congenital lobar emphysema (9), and malignancy (2). RESULTS: Of 97 procedures, 93 were completed thoracoscopically. Operative times ranged from 35 minutes to 210 minutes (average, 115 minutes). There were 19 upper, 11 middle, and 67 lower lobe resections. There were 3 intraoperative complications (3.1%) requiring conversion to an open thoracotomy. Chest tubes were left in 88 of 97 procedures for 1 to 3 days (average, 2.1 days). Hospital stay ranged from 1 to 12 days (average, 2.4 days). CONCLUSIONS: Thoracoscopic lung resection is a safe and efficacious technique. It avoids the inherent morbidity of a major thoracotomy incision and is associated with the same decrease in postoperative pain, recovery, and hospital stay as seen in minimally invasive procedures.
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Secuestro Broncopulmonar/cirugía , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Neumonectomía/métodos , Enfisema Pulmonar/cirugía , Cirugía Torácica Asistida por Video/métodos , Adolescente , Secuestro Broncopulmonar/mortalidad , Niño , Preescolar , Estudios de Cohortes , Malformación Adenomatoide Quística Congénita del Pulmón/mortalidad , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Tiempo de Internación , Masculino , Procedimientos Quirúrgicos Mínimamente Invasivos/efectos adversos , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Dolor Postoperatorio/fisiopatología , Neumonectomía/efectos adversos , Complicaciones Posoperatorias/epidemiología , Enfisema Pulmonar/congénito , Enfisema Pulmonar/mortalidad , Estudios Retrospectivos , Medición de Riesgo , Análisis de Supervivencia , Cirugía Torácica Asistida por Video/efectos adversos , Factores de Tiempo , Estados UnidosRESUMEN
Pulmonary sequestration refers to the situation whereby a portion of lung tissue receives its blood supply from an anomalous systemic artery. Three main variants exist: intralobar, extralobar and communicating bronchopulmonary foregut malformations. Venous drainage is typically via a systemic vein, although drainage into the pulmonary veins is well documented. Pulmonary sequestrations are the second commonest congenital lung anomaly. Affected individuals often have other anomalies which are responsible for most of the mortality associated with sequestrations. Diagnosis requires a high index of suspicion particularly in any child with a chest x-ray suggesting the presence of a mass, those with recurrent chest infections and those with other anomalies seen with the pulmonary sequestration spectrum. Surgical excision is usually advised, although embolisation of the feeding vessel has a role in selected cases.
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Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/cirugía , Diagnóstico por Imagen/métodos , Angiografía/métodos , Secuestro Broncopulmonar/mortalidad , Femenino , Humanos , Recién Nacido , Imagen por Resonancia Magnética/métodos , Masculino , Pronóstico , Medición de Riesgo , Índice de Severidad de la Enfermedad , Análisis de Supervivencia , Toracotomía/métodos , Ultrasonografía DopplerRESUMEN
UNLABELLED: Pulmonary sequestration is rare and serious dysplasia, which has clinical signs of pneumonia and respiratory distress syndrome and radiological signs of polymorphic shadows. Pulmonary sequestration masses have autonomic blood supply. For confirmation of the diagnosis we use ultrasound examination, CT scan, MRI, angiopulmography and aortography. AIM OF THE STUDY: To present pathogenesis, diagnostic features and surgical treatment tactics of rare and serious pulmonary dysplasia. MATERIAL AND METHODS: During the period of 1979-2002, we have examined and treated 9 patients because of pulmonary sequestration. We analyzed clinical signs, results of the examination before operation, indications for operation and results of surgical treatment. RESULTS: Seven patients after the operation recovered, 1 patient died due to infection and cardiovascular complications. One patient is supposed to be operated on in the near future. CONCLUSIONS: 1. Pulmonary sequestration is rare and serious bronchial - pulmonary dysplasia, which has clinical signs of pneumonia and respiratory distress syndrome and radiological signs of polymorphic shadows. 2. Pulmonary sequestrations are classified as intralobar, extralobar and extrapulmonary sequestration. 3. Pulmonary sequestration is often associated with cardiovascular dysplasias and diaphragmatic malformations. 4. Methods of examination: X-ray examination, CT scan, MRI, ultrasound examination, aortography. 5. Complications of pulmonary sequestration: pneumonia, pleuritis, necrosis of the sequestration, chylothorax, respiratory distress syndrome, hemothorax, cystic transformation, and pneumothorax. 6. Treatment - surgery or embolisation of abnormal supply vessels.
Asunto(s)
Secuestro Broncopulmonar , Angiografía , Secuestro Broncopulmonar/clasificación , Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/diagnóstico por imagen , Secuestro Broncopulmonar/mortalidad , Secuestro Broncopulmonar/cirugía , Secuestro Broncopulmonar/terapia , Niño , Preescolar , Embolización Terapéutica , Humanos , Lactante , Recién Nacido , Laparotomía , Imagen por Resonancia Magnética , Neumonectomía , Radiografía Torácica , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Ultrasonografía DopplerAsunto(s)
Secuestro Broncopulmonar/diagnóstico por imagen , Enfermedades Fetales/diagnóstico por imagen , Ultrasonografía Prenatal , Secuestro Broncopulmonar/mortalidad , Secuestro Broncopulmonar/cirugía , Femenino , Enfermedades Fetales/mortalidad , Enfermedades Fetales/cirugía , Estudios de Seguimiento , Humanos , Recién Nacido , Masculino , Monitoreo Fisiológico , Tasa de SupervivenciaRESUMEN
Between November 1986 and April 1993, 22 cases of intrathoracic abnormality were detected prenatally by ultrasound, and examined postnatally. There were 11 cases of diaphragmatic hernia, 5 cases of cystic adenomatoid malformation of lung, one case of chylothorax, two cases of lung sequestration, and three cases of bronchogenic cyst. The total number of deliveries during that period was 48,281 and the total number of major anomalies at that time was 669 (1.38%). Cases of hydrothorax of various etiology, as well as thoracic cage anomalies were excluded. Prenatal diagnosis allows planned delivery and the assembly of neonatologists and pediatric surgeons.
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Enfermedades Torácicas/congénito , Tórax/anomalías , Ultrasonografía Prenatal , Adenomatosis Pulmonar/diagnóstico por imagen , Adenomatosis Pulmonar/mortalidad , Adolescente , Adulto , Secuestro Broncopulmonar/diagnóstico por imagen , Secuestro Broncopulmonar/mortalidad , Quilotórax/congénito , Quilotórax/diagnóstico por imagen , Quilotórax/mortalidad , Extracción Obstétrica , Femenino , Hernia Diafragmática/diagnóstico por imagen , Hernia Diafragmática/mortalidad , Hernias Diafragmáticas Congénitas , Humanos , Recién Nacido , Grupo de Atención al Paciente , Embarazo , Tasa de Supervivencia , Enfermedades Torácicas/diagnóstico por imagen , Enfermedades Torácicas/mortalidad , Tórax/diagnóstico por imagenRESUMEN
Fourteen patients with scimitar syndrome, aged from 4 days to 20 years, underwent surgical treatment between September 1980 and August 1988. Patients were separated into two groups. Group A comprised four neonates with heart failure and severe pulmonary hypertension; part of the right lung was supplied by large aberrant systemic subphrenic arteries (ASSAs) in each. Group B included ten patients (nine children and one adult) with mild to moderate symptoms, normal pulmonary artery pressures; only two had ASSAs. In group A, one neonate with multiple ventricular septal defects underwent pulmonary artery banding but later required a lobectomy because of a lung abscess. The other three neonates underwent ligation of ASSAs; two improved rapidly, and one died of sepsis. In group B, all patients survived intracardiac repair and remain asymptomatic during a follow-up of 24 to 108 (mean 54.9) months. In summary, prognosis after intracardiac repair is excellent in patients without pulmonary hypertension. Neonates with heart failure usually improve after ligation of ASSAs, and pulmonary resection is only indicated in patients with intractable pulmonary sequestrations.
Asunto(s)
Síndrome de Cimitarra/cirugía , Adolescente , Adulto , Anastomosis Quirúrgica , Secuestro Broncopulmonar/diagnóstico por imagen , Secuestro Broncopulmonar/mortalidad , Secuestro Broncopulmonar/cirugía , Niño , Preescolar , Femenino , Estudios de Seguimiento , Atrios Cardíacos/cirugía , Insuficiencia Cardíaca/diagnóstico por imagen , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/cirugía , Humanos , Hipertensión Pulmonar/diagnóstico por imagen , Hipertensión Pulmonar/mortalidad , Hipertensión Pulmonar/cirugía , Lactante , Recién Nacido , Masculino , Complicaciones Posoperatorias/diagnóstico por imagen , Complicaciones Posoperatorias/mortalidad , Venas Pulmonares/anomalías , Venas Pulmonares/cirugía , Radiografía , Síndrome de Cimitarra/diagnóstico por imagen , Síndrome de Cimitarra/mortalidad , Técnicas de SuturaRESUMEN
This report describes the antenatal sonographic identification of a hyperechoic intra-abdominal mass in a 30-week fetus. The mass was proved by autopsy to be an extralobar pulmonary sequestration. Although a rare type of congenital malformation, pulmonary sequestration should be included in the differential diagnosis of an echodense intra-abdominal mass seen during antenatal ultrasound examination.
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Abdomen , Secuestro Broncopulmonar/diagnóstico , Enfermedades del Prematuro/diagnóstico , Diagnóstico Prenatal , Ultrasonografía , Adulto , Secuestro Broncopulmonar/mortalidad , Secuestro Broncopulmonar/patología , Diagnóstico Diferencial , Femenino , Humanos , Recién Nacido , Enfermedades del Prematuro/mortalidad , Enfermedades del Prematuro/patología , Neoplasias Renales/diagnóstico , Embarazo , Tumor de Wilms/diagnósticoRESUMEN
Pulmonary sequestration is a congenital anomaly in which an aberrant systemic artery arising from the thoracic or upper abdominal aorta supplies part of the lungs, usually the lower lobe. The sequestered lung may be anatomically distinct from the remainder of the lobe (extralobar), or may be included in the substance of the lobe, in which case it may or may not have bronchial communication with the rest of the bronchial tree. The patients present, often in the first two decades of life, with recurrent and severe bronchopulmonary infections. Associated anomalies are present, especially in the extralobar variety. Nine cases of sequestration are reviewed, stressing significant clinical, radiological, and arteriographic findings. Preoperative demonstration of the anomalous vessel by aortography has contributed significantly to the planning and safety of the surgical procedure, which was generally a lower lobectomy. Eight out of nine patients survived the procedure.