Bilateral congenital lobar emphysema: how should we proceed?

Maiya, Shreesha; Clarke, JR; More, Bharat; Desai, Maya; Parikh, Dakshesh

doi:10.1007/s00383-005-1425-6

Cited by 19 publications

(30 citation statements)

References 5 publications

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“…(46) According to one group of authors, (46) although the vast majority of patients present with symptoms before age 6 months, some can remain asymptomatic for years. (46,47) Congenital lobar emphysema is twice as common in males as it is in females (47,49) and affects, in order of frequency, the left upper lobe (in 40-50% of cases), (50,51) the middle lobe (in 30-40%), and the right upper lobe (in 20%), (49,52) although bilateral involvement has also been described. (49) One third of all patients are symptomatic at birth, and 50% are diagnosed in the first month of life.…”

Section: Ccammentioning

confidence: 99%

“…(46)(47)(48) The principal cause of congenital lobar emphysema is developmental deficiency of bronchial cartilage, (5,46,49) which occurs in approximately 25% of cases. Obstruction of a lobar bronchus occurs in another 25% of cases and is generally caused by idiopathic bronchial stenosis that leads to a check-valve mechanism, which can also be triggered by mucus plugging or vascular changes.…”

Section: Congenital Lobar Emphysemamentioning

confidence: 99%

“…Obstruction of a lobar bronchus occurs in another 25% of cases and is generally caused by idiopathic bronchial stenosis that leads to a check-valve mechanism, which can also be triggered by mucus plugging or vascular changes. (5,46,49) However, in approximately 50% of all cases, the cause is unknown. (4,46) Congenital lobar emphysema is characterized by hyperinflation of a lung lobe caused by air trapping, resulting in distension of the lobe and causing a mass effect that compresses the remaining lobes and causes mediastinal shift (Figure 4), leading to hemodynamic changes.…”

Section: Congenital Lobar Emphysemamentioning

confidence: 99%

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Malformações pulmonares congênitas

2011

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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.Keywords: Cystic adenomatoid malformation of lung, congenital; Bronchopulmonary sequestration; Pulmonary surgical procedures; Diagnosis. ResumoAs 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. (5) is covered by ...

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Section: Ccammentioning

confidence: 99%

Section: Congenital Lobar Emphysemamentioning

confidence: 99%

Section: Congenital Lobar Emphysemamentioning

confidence: 99%

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Malformações pulmonares congênitas

2011

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“…The exact cause of congenital lobar emphysema is difficult to determine and seems even nonexistent in 50% of cases, as in our 3 patients. The most commonly identified causes are congenital cartilage defect and bronchial obstruction [1].…”

Section: Discussionmentioning

confidence: 99%

Congenital bilobar emphysema

Ghribi

Mekki

Krichène

et al. 2008

Journal of Pediatric Surgery

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“…It usually presents as respiratory distress in the first few days of life, and a majority of reported cases of congenital lobar emphysema have documented its presentation in neonates [1][2][3][4][5][6][7][8][9][10]. Approximately 50% of cases identified in the first month of life, and only 5% of cases are detected after the age of six months [11].…”

Section: Summary and Discussionmentioning

confidence: 99%

Congenital Lobar Emphysema in Adult Treated With Video Assisted Thoracoscopic Lobectomy

Perry¹,

Espiritu²,

Schroeder³

2013

J Pulm Respir Med

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Congenital Lobar Emphysema (CLE) is an uncommon pulmonary disorder in which one or more pulmonary lobes is over-inflated and distended. Fewer than 5% of cases are diagnosed beyond the first 6 months of life. We describe CLE that presented in a 25 yr old female who was successfully treated with minimally invasive video assisted thoracoscopic surgery (VATS). Case ReportA 25-year old Puerto Rican female presented with increased shortness of breath, cough and chest pain. Her past medical history included diagnosis of asthma In Puerto-Rico at the age of five, treated with advair. During her third pregnancy, at the age of 23, the patient experienced shortness of breath and reported "fainting". A chest CT at that time diagnosed her with lobar emphysema.In the United States, she presented to an outside hospital with exacerbation of shortness of breath, where she was thought to have a pneumothorax, and a right chest tube was placed. She was then transferred to the Medical Center ICU. At presentation, She was afebrile, exhibiting tachycardia (117 bpm), with 28 respirations per minute, oxygen saturation of 91%, and decreased breath sounds on the right chest field.Pulmonary function test suggested of Restrictive Lung Disease (RLD) ( Table 1). The patient's initial X-rays, as seen in Figure 1, indicate hyperlucency on the right side, flattening of the right diaphragm, and a slight shift of mediastinum to the left. The right lung is collapsed inferiorly. There are a severe number of bullae within the right hemithorax, with some consolidation or collapse in the region of the right perihilar region.CT taken before surgery documented large bullae, mediastinal shift, and compressed right lung. (Figure 2 and 3). Treatment and Surgical FindingsSince the patient's shortness of breath was not successfully managed with Oxygen support and chest tube drainage, she was scheduled for a bronchoscopy and a Video Assisted Thoracoscopic Surgery (VATS) -right upper lobectomy. On BronchoscopyThe Right Upper Lobe (RUL) takeoff was a slit-like lumen, slightly distorted -confirms the diagnosis of CLE.VATS right upper lobectomy was performed with general endotracheal anesthesia with a double lumen tube and one lung ventilation. We used 3 ports; 10mm port in the seventh intercostal space in anterior axillary line, 10mm trocar in posterior axillary Using a 10 mm 30 degree Olympus scopeThe right upper lobe composed entirely of large bullae. The bullae did not deflate and were densely adherent to the chest wall. There were also aberrant blood vessels from the chest wall to the bullae, with no apparent superior pulmonary vein, pulmonary artery, or bronchi going to the bullous portion. However, the segmental bronchi and major pulmonary vessels were found attached to the area of the hilum of the atritic lobe. An endoscopic EndoGIA (Covidian) used to divide the vessels and the rudimentary bronchus. The lobe removed with an endobag. A 28 french chest tube was left in the chest. PathologyMicroscopic examination of the resected lobe showed dense fi...

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Bilateral congenital lobar emphysema: how should we proceed?

Cited by 19 publications

References 5 publications

Malformações pulmonares congênitas

Malformações pulmonares congênitas

Congenital bilobar emphysema

Congenital Lobar Emphysema in Adult Treated With Video Assisted Thoracoscopic Lobectomy

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