Journal of Minimal Access Surgery

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Year : 2015  |  Volume : 11  |  Issue : 2  |  Page : 129--133

Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition

Lucile Fievet1, Claudia Natale2, Xavier-Benoit D'Journo2, Stéphanie Coze3, Jean-Christophe Dubus4, Jean-Michel Guys1, Pascal Thomas2, Pascal De Lagausie1,  
1 Department of Paediatric Surgery, Timone and North Children's Hospital, Aix-Marseille University and Assistance Publique-Hôpitaux de Marseille, Marseille, France
2 Thoracic Surgery and Diseases of the Esophagus, North Hospital, Aix-Marseille University and Assistance Publique-Hôpitaux de Marseille, Marseille, France
3 Department of Pediatric and Prenatal Imaging, Timone and North Hospital, Aix-Marseille University and Assistance Publique-Hôpitaux de Marseille, Marseille, France
4 Department of Pediatric Pneumology, Timone and North Children's Hospital, Aix-Marseille University and Assistance Publique-Hôpitaux de Marseille, Marseille, France

Correspondence Address:
Lucile Fievet
Department of Paediatric Surgery, Timone and North Children«SQ»s Hospital, Aix-Marseille University and Assistance Publique-Hôpitaux de Marseille, 264 rue Saint Pierre, 13385 Marseille Cedex 05


In adults, congenital pulmonary malformations are candidates for surgery due to symptoms. A pre-natal diagnosis is simple and effective, and allows an early thoracoscopic surgical treatment. A retrospective study was performed to assess management in two different populations of adults and children to define the best strategy. Subjects and Methods: Pulmonary malformations followed at the University Hospital from 2000 to 2012 were reviewed. Clinical history, malformation site, duration of hospitalisation, complications and pathology examinations were collected. Results: A total of 52 cases (33 children, 19 adults) were identified. In children, 28 asymptomatic cases were diagnosed pre-natally and 5 during the neonatal period due to infections. Surgery was performed on the children between the ages of 2 and 6 months. Nineteen adults underwent surgery, 16 because of symptoms and 3 adults for anomalies mimicking tumours. The mean age within the adult group was 42.5 years. In children, there was one thoracotomy and 32 thoracoscopies, with 7 conversions for difficult exposure, dissection of vascular pedicles, bleeding or bronchial injury. In the adults, there were 15 thoracotomies and 4 thoracoscopies, with one conversion. Post-operative complications in the adults were twice as frequent than in children. The mean time of the children«SQ»s hospitalisation was 7.75 days versus 7.16 days for the adults. Pathological examinations showed in the children: 7 sequestrations, 18 congenital cystic pulmonary malformations (CPAM), 8 CPAM associated sequestrations; in adults: 16 sequestrations, 3 intra-pulmonary cysts. Conclusion: Early thoracoscopic surgery allows pulmonary parenchyma conservation with pulmonary development, reduces respiratory and infectious complications, eliminates a false positive cancer diagnosis later in life and decreases risks of thoracic parietal deformation.

How to cite this article:
Fievet L, Natale C, D'Journo XB, Coze S, Dubus JC, Guys JM, Thomas P, De Lagausie P. Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition.J Min Access Surg 2015;11:129-133

How to cite this URL:
Fievet L, Natale C, D'Journo XB, Coze S, Dubus JC, Guys JM, Thomas P, De Lagausie P. Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition. J Min Access Surg [serial online] 2015 [cited 2019 Jun 16 ];11:129-133
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Sequestration and congenital pulmonary airway malformation (CPAM) are congenital pulmonary parenchyma malformations. Although often asymptomatic, they can lead to severe infection or compression of the thoracic structures. In the absence of pre-natal diagnosis, some initially silent lesions may be diagnosed in adulthood because of complications or suspected malignancy. The percentage of adults with this condition without symptoms is unknown. However, in adults, when a small asymptomatic malformation is identified, no intervention is usually performed. For a small sequestration, embolisation can be proposed and for CPAM, a simple follow up. In contrast, the symptomatic malformations are removed at price of a thoracotomy or thoracoscopy. For children, pre-natal diagnosis is now generally effortless and this can have an important impact on early management by thoracoscopic approach. Nevertheless, no guidelines are yet formalised. The problem is that adult and child populations are different for co-morbidity, performance status, capacity to recover and so on; but it is possible to observe some interesting clinical elements. The purpose of our study is to assess management of these congenital pulmonary malformations for children and to define the best therapeutic strategy.


We retrospectively reviewed all cases of congenital pulmonary malformations (pulmonary sequestration and congenital cystic adenomatoid malformations) seen at Children's University Hospital and Cardiothoracic Hospital between 2000 and 2012. We included: Asymptomatic children with a pre-natal diagnosis, symptomatic children post-natally diagnosed and adults with complications. The following items were studied: Date of diagnosis, clinical history, malformation localisation, date and indication for surgery, duration of hospitalisation, pre- and post-operative complications and pathological examination. The computed tomography (CT) scan argument for pulmonary sequestration was a mass or cystic lesion located in the lower lobe with an aberrant arterial supply. The CT scan argument for CPAM was a hyper echogenic mass inside the pulmonary parenchyma, with multiple cyst lesions of different sizes. The malformation was always confirmed by a pathologic examination. We excluded sequestration associated with bronchogenic cysts. We compare these two populations: The child group and the adult group. The two populations are heterogeneous (Student test and chi-square of Pearson with P < 0.05). In each group, we compare thoracoscopic and thoracotomic approaches.


Since the year 2000, 52 patients have been followed for pulmonary congenital malformation. There were 33 children (13 girls and 20 boys) and 19 adults (6 women and 13 men). In the group of children, 28 cases were diagnosed pre-natally and 5 during infancy, due to pulmonary infection [Figure 1]. Surgery was generally performed in children aged between 2 and 6 months when a pre-natal diagnosis was available. Mean age for surgery was 9.19 months (7 days to 5 years). We found malformation of the lower lobe in 27 patients and middle lobe and upper lobe in 6 patients. This malformation is located in the right lung in 17 patients and the left lung in 16 patients. In 14 patients, the malformation was found in the lower right lobe.{Figure 1}

In the group of adults, in 16 cases diagnosis was made through symptoms; and 3 adults underwent surgery for asymptomatic pulmonary anomalies mimicking tumours; since it was impossible to eliminate a cancer diagnosis based only on radiology. A diagnosis of sequestration was called for 12 patients before surgery. In one case, a patient underwent surgery for pulmonary abscess that led to the exeresis of the sequestration. In one case, an emergency intervention for hemothorax leads to the diagnosis of a haemorrhagic sequestration [Figure 2]. A total of 500 cc of blood wasdrained before surgery and transfusion was required. The mean age at the time of surgery was 42.5 years. We found anomalies of the lower lobe in 15 patients, of the upper and middle lobes in 4 patients, of the right lung in 13 patients and left lung in 6 patients. In 10 patients, the anomaly was in the lower right lobe. In the group of adults, 16 underwent surgery because of symptoms [Figure 1] (cough, thoracic pain, haemoptysis, dyspnoea, fever) leading to the discovery of an anomalous image [Figure 3]. The rate of symptomatic patients was significantly higher in the sample of adults than in the sample of children (P < 0.001 with Pearson's chi-squared test).{Figure 2}{Figure 3}

In the group of the children, one of them underwent a thoracotomy. There were 32 thoracoscopies, with 7 conversions in thoracotomy. Three of the conversions were for difficult exposition, two for bleeding, in one case requiring transfusion, one for difficult dissection due to an incomplete fissure, one for a bronchial wound. One child required a second look intervention by thoracotomy because of complications following bronchial injury. Pulmonary resection consisted of a lobectomy in 27 cases, sequestrectomy in 5 cases and bi-lobectomy in 1 case. Overall, eight children underwent a thoracotomy. In the group of adults, four underwent thoracoscopic surgery with one conversion. Pulmonary resection was a lobectomy in 16 cases. This lobectomy was associated with a wedge resection in one case, and a lymphadenectomy in two cases. In one case, the lobectomy was associated with a pleurolobectomy, and in two cases, a simple sequestrectomy was performed. Two patients required a second look intervention for excessive bleeding.

The rate of thoracoscopic surgery was significantly higher in children (25/33) than in adults. (3/19): P < 0,001 (Pearson's chi-squared test).

Rate of complications in children was 15% (5/33), including:

An iatrogenic middle lobar bronchus injury detected pre-operatively during thoracoscopic lobectomy. The patient required a middle lobe bronchus re-implantation. Due to the poor result of the re-implantation, a middle lobectomy by thoracotomy was carried out [1] ;Two cases of recurrent pneumothorax and respiratory distress, with one requiring a stay of 4 days in the intensive care unit (ICU);One pulmonary infection required antibiotics;One paralysis of the diaphragm.

Rate of surgical complication in the adult was a 30% (6/19), including:

A broncho-aspiration endoscopy was required due to Nelson atelectasis;One case of pulmonary infection;One case of pneumothorax;Three cases of hemothorax requiring a second look surgery;

The difference in the rate of complications between the children and the adult population was not significant (P = 0.18 Fisher test).

The mean duration of hospitalisation was 7.75 days for children versus 7.16 days for adults. There was a non-significant difference in the length of hospitalisation between the two groups (P = 0.75) (Ki2 non-significant difference).

The pathological examination concluded that there were 7 sequestrations, 18 CPAM and 8 CPAM associated with sequestrations in the sample of children. In adults, the diagnosis of sequestration was made in 16 cases and CPAM in 3 cases.


The purpose of this study is to suggest the best therapeutic strategy for congenital pulmonary malformations in asymptomatic children. In fact, the two populations are different and each one has their own therapeutic indications for surgery. However, there are several arguments in favour of an early treatment of the pulmonary malformation in children: Low complications in children, high rate of symptoms in adults, necessity of an aggressive surgery in adults with increase of complications. Ethically, we cannot propose a prospective study analysing the impact of early thoracoscopic management versus radiological follow up in neonatal population with pulmonary malformation.

In our sample, the children are asymptomatic before surgery. They underwent a thoracoscopy, aged mostly between 4 and 6 months, with few complications. This strategy maximises compensatory lung growth [2] and decreases thoracic wall aggression. In our adults group, most patients undergo surgery after presenting with more or less severe complications. A thoracotomy is often required and the rate of post-operative complication is doubled. These data are congruous with literature about the fact that the risk of complication due to congenital pulmonary malformations increases with time. [3] In children with a pre-natal diagnosis, a CT scan was performed at 1 month, to confirm and precisely locate the malformation [Figure 4]. Due to rapid surgical intervention, the risk of over-infection is decreased. This can also be explained by the protection of the maternal antibody (the high levels of Immunoglobulin G (IgG) that are transferred to the foetus in utero are still observed at 1.5 months of age, and decrease in the first 6 months of life). [4] Previous studies have shown that the risk of complications secondary to congenital pulmonary malformation is evaluated at 84%. [5] Congenital anomalies of lung parenchyma in adults are a very rare pathology but it can be marred by severe complications. [6] Ninety-two per cent of our patients presented with symptoms linked to their congenital pulmonary malformation. The symptoms were cough, haemoptysis, fever, chest pain or pneumothorax. Antibiotics were often necessary for the 45% of patients who developed an infection. [7] For pulmonary sequestration, only 13.36% of patients remained asymptomatic. [8]{Figure 4}

In adults, the problem of a differential diagnosis is still open: Pulmonary malformation imaging can be inconclusive and a diagnosis of cancer is often called, increasing the level of anxiety of the patient. The removal of the lesion and its pathological examination is the only way to confirm the diagnosis and more, the risk of malignant transformation is well documented. [9] Epithelial metaplasia in congenital cystic disease may lead to carcinoma of the bronchus. [10] CPAM Type 1 is a precursor of mucinous bronchiolo-alveolar carcinoma. CPAM type 2 is a precursor of rhabdomyosarcoma. [11] Isolated cysts could evolve into pleuropulmonary blastoma, often identified during infancy. [12] Radiological examinations are not sufficient to identify the type of CPAM. In some studies, pathological examination showing a pulmonary malformation with the expression of an epidermal growth factor receptor positively correlated with malignant transformation. [13] CPAM type II is sometimes associated with extra lobar sequestration. The pathology report for CPAM type II show that 48% of cases present with displayed rhabdomyomatousdys genesis. This justifies the recommendation of an early surgical management of these lesions.

The optimal period to treat patients with pre-natal diagnoses is not defined. Although it is widely accepted that early surgery is necessary for symptomatic patients, the management of asymptomatic patients is somewhat contentious. [14] There are two different approaches: The first approach consists of an early intervention between 6 months and 1 year of life. A more conservative approach consists of repeated CT scan surveillance. [15],[16] We advocate performing a CT scan and MRI at the age of 1 month, followed by early surgery between the ages of 4 and 6 months. In our opinion, this strategy can decrease the risk of pulmonary infection, considering that the child is still protected by maternal antibodies, and lung growth will continue throughout the first 3 years of life. [17] About the conservative approach, we consider that radiological follow-up for small children based on annual CT scans may be a source of concern due to the radiation induced by such examinations. The anxiety level of the parents in relation with the follow-up should also be taken into consideration. Even if it is possible to monitor a lesion over time, there is no general consensus on timing and strategy of the radiological examinations (e.g. CT scans every 2 or 5 years). For adults who are asymptomatic, we can propose a conservative approach of follow up after diagnosis for minor CPAM, and embolization for minor sequestration. Concerning symptomatic malformation and cancer suspicion, surgical exploration by thoracotomy or thoracoscopy is necessary.

In children, lobectomy, segmentectomy or sequestrectomy are easily performed by thoracoscopy. [18],[19],[20],[21] It has been shown to be safe and effective. This technique avoids thoracotomy, which can induce thoracic deformation and scoliosis. Thoracoscopic surgery is also associated with a lower rate of scarring, a reduced length of hospitalisation time, as well as a decrease in the duration of surgical drainage. In children, the rate of conversion in thoracotomy is low, as like the rate of complications. [20] In CPAM, lobectomy is necessary and sufficient, and provides better results than segmentectomy. [22] Early surgery performed because of asymptomatic lesions can limit the risk of extensive resection and decrease the risk of conversion. Although there were seven conversions in our sample of children, we think that this risk will decrease over time as the team gains experience. Over the last few years, thoracoscopic surgery has been adapted to adults. It is probably effective to pain reduction, reduced hospitalisation time and decreased scarring, [3],[23],[24] but this kind of surgery might not be suitable for malformation with complications. More aggressive approach may limit the further exploration by thoracoscopic surgery in adults. For patients with congenital cystic lung disease, extensive pulmonary resection is necessary with total pneumonectomy totalling 36.8% of surgeries in a series of 68 patients. [25] This shows that delayed diagnosis and surgical treatment usually results in significant mortality and morbidity. [25] In our sample of 19 adults, only 3 thoracoscopies were carried out and 1 thoracoscopy required a conversion. We started the practice of thoracoscopy for this malformation 3 years ago. Even though we can increase the number of thoracoscopic interventions in adults, complete resection of super-infected congenital lesions remains difficult by thoracoscopy. There is a bias in our sample: The proportion of adults that have asymptomatic malformations remains unknown; and if an asymptomatic malformation is identified, no intervention is usually conducted. The good or bad evolution of congenital malformations is impossible to predict.


It appears that congenital pulmonary malformations identified pre-natally are often less symptomatic, and an early thoracoscopic surgery is indicated, with few pre - and post-operative complications, limited chest deformation during growth and limited impact on pulmonary growth and low parental anxiety. In contrast, in adults, the practice of thoracoscopy is still developing, and thoracotomy is more often used, mainly after severe complications. Surgery is only for symptomatic malformations or if cancer is suspected. It is not possible to predict the evolution of this malformation, but the risk of cancer cannot be eliminated. Resuming, it seems legitimate to propose an early thoracoscopic resection between 4 and 6 months of life for pulmonary malformation discovered pre-natally.

Thanks to Professor De Lagausie for his encouragement and advice. Thanks to Adjunct Professor James Carson for his assistance.


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