Congenital lobar emphysema: experience with 21 cases

Thakral, C. L.; Maji, Debasish; Sajwani, Mohammed Jaffer

doi:10.1007/s003830000506

Cited by 102 publications

(135 citation statements)

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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%

“…(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. (47,48,50) Congenital lung malformations J Bras Pneumol. 2011;37(2):259-271…”

Section: Congenital Lobar Emphysemamentioning

confidence: 99%

“…The differential diagnosis of congenital lobar emphysema includes pneumatoceles, pneumothorax, pulmonary atelectasis, and pulmonary hypoplasia. (47) Congenital lobar emphysema is generally treated surgically, and lobectomy through thoracotomy or video-assisted lobectomy (52) is the surgical treatment of choice in symptomatic children. (4,46,47) Although it is logical to recommend surgery for symptomatic patients and observation for asymptomatic patients, the approach to oligosymptomatic children remains controversial.…”

Section: Avmmentioning

confidence: 99%

“…(46) A small proportion of patients have been treated conservatively, with good results. (47,50) Prenatal and postnatal spontaneous resolution suggests that congenital lobar emphysema is a partially reversible process or that the growth of the normal lung parenchyma compensates for the nonfunctioning congenital lobar emphysema. (46) Fiberoptic bronchoscopy is recommended in order to rule out mucus plugging, foreign body, and changes in the airway.…”

Section: Avmmentioning

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%

Section: Avmmentioning

confidence: 99%

Section: Avmmentioning

confidence: 99%

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Malformações pulmonares congênitas

2011

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Congenital overinflation: prenatal MRI and US findings and outcomes

et al. 2016

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The long‐term outcomes of symptomatic congenital lobar emphysema patients

Gatt

Lapidus-Krol

Chiu

2023

Pediatric Pulmonology

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Introduction Surgical (OP) management for symptomatic congenital lobar emphysema (CLE) is the standard of care with nonoperative (NOP) approach applied for asymptomatic cases. The aim of this study is to report the outcomes for NOP approach to the care of symptomatic CLE infants. Methods A retrospective study of CLE patients treated 2000−2021 at a single institution. Patients with CLE and respiratory symptoms were included. Results Overall, 23 children had symptomatic CLE, and 12 had NOP management. The median age at diagnosis was 38.5 days (50) in the NOP group versus 25 days (20) in the OP group (p = 0.31). There was no significant difference in the location of the involved lobe, term birth, postnatal diagnosis and gender, and both groups required noninvasive support in 33% of the cases. There was a trend towards higher frequency of oxygen support in the OP group preoperatively (89% vs. 42%, p = 0.07). The median length of stay was 14 days in the NOP group compared to a median postsurgery stay of 7.5 days in the OP group. In follow‐up, there was no significant difference in respiratory readmission in first year of life, growth delay, treatment with asthma medication or body mass index in the NOP versus OP group. None of the children in the NOP group required surgery during follow‐up. Conclusions A NOP approach for symptomatic CLE infants can have favorable long‐term outcomes. Further studies will be required to identify markers to aid in clinical decision‐making.

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Congenital lobar emphysema: experience with 21 cases

Cited by 102 publications

References 0 publications

Malformações pulmonares congênitas

Malformações pulmonares congênitas

Congenital overinflation: prenatal MRI and US findings and outcomes

The long‐term outcomes of symptomatic congenital lobar emphysema patients

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