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 Table of Contents  
Year : 2022  |  Volume : 19  |  Issue : 2  |  Page : 57-61

Ten years experience in surgical management of congenital lung malformations: A prospective, cross sectional study

1 Department of Surgery, Cardiothoracic Unit, College of Medicine, University of Lagos and Lagos University Teaching Hospital, Lagos, Nigeria
2 Department of Paediatrics (Neonatal Unit), College of Medicine, University of Lagos and Lagos University Teaching Hospital, Lagos, Nigeria

Date of Submission25-Nov-2021
Date of Acceptance10-Mar-2022
Date of Web Publication13-Jun-2022

Correspondence Address:
Dr. Ezekiel Ogunleye
Department of Surgery (Cardiothoracic Unit), College of Medicine, University of Lagos and Lagos University, Teaching Hospital, Idi-Araba, Surulere, Lagos
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcls.jcls_53_21

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Background: Congenital lung malformations are very rare lung lesions caused by abnormal lung development occurring at different stages of intrauterine life. They are a spectrum of congenital malformations involving the trachea-bronchial tree, pulmonary parenchyma, and the blood vessels. They are a family of pulmonary lesions that include congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations, bronchogenic cysts (BC), and congenital lobar emphysema (CLE). The aim of the study is to determine the pattern of congenital malformation of the lungs and the outcomes of treatment in Lagos. Methods: This is a prospective study of all patients with congenital lung malformations referred to our center in the period between January 2011 and December 2020. All pediatric cases were diagnosed by the pediatricians in our center and confirmed with chest computed tomography (CT) scan. The adult cases were equally confirmed with a chest CT scan. The lesions encountered in this study include BC, CPAM, bronchopulmonary sequestrations, lung hypoplasia (LH), CLE, and pulmonary arterio-venous malformations. Data collected include biodata, type of malformation, mode of management (surgery), and postoperative outcome and were analyzed as percentages and mean. Results: A total of 18 patients who were diagnosed with congenital lung malformations were recruited into this study. The congenital malformations included CLE, CPAM, BC, lung sequestration, LH, and arterio-venous malformation of the lung. Children accounted for 78% (13) of the population, while the adult population was 28%.(5) The neonates constituted 22% (4) with a mean age of 7.5 ± 5.1, whilst the older children made up 50%, with a mean age of 18.8 ± 10.7 months. The mean age of the adult population was 39 ± 15 years. Seventeen (94.41) had surgery, whilst one opted for continued medical surveillance. Two patients died from postoperative respiratory failure. Conclusion: Congenital lung malformations, though rare, are a heterogeneous group of diseases with presentation, ranging from neonatal respiratory distress to asymptomatic presentation in the adult. The mainstay of management remains surgery with a reasonably good outcome.

Keywords: Lung malformations, outcome, surgical management

How to cite this article:
Ogunleye E, Olusoji O, Fajolu I, Iwuchukwu P. Ten years experience in surgical management of congenital lung malformations: A prospective, cross sectional study. J Clin Sci 2022;19:57-61

How to cite this URL:
Ogunleye E, Olusoji O, Fajolu I, Iwuchukwu P. Ten years experience in surgical management of congenital lung malformations: A prospective, cross sectional study. J Clin Sci [serial online] 2022 [cited 2023 Mar 20];19:57-61. Available from: https://www.jcsjournal.org/text.asp?2022/19/2/57/347246

  Introduction Top

Congenital lung malformations are not very common and they occur due to abnormal development of the lung during intrauterine growth. The malformations are a heterogeneous group of lesions that are pathologically different but have many features in common.

The annual incidence is reportedly 30–42 cases per 100,000.[1] The advent of prenatal nonradiating imaging methods such as magnetic resonance imaging (MRI) and ultrasound scanning may improve on the outcome.[2]

There are no identified maternal risk factors (race, age, or exposure in their occurrence) and male preponderance has been described in those early presentations in infancy.[3] While others found no sex preditection.[4]

Congenital lung malformation represents 5%–18.7% of all congenital anomalies; however, this range may be an underestimate because a large number of these cases are either undetected or remain asymptomatic.[5]

The advent of prenatal USS screening continues to play a major role in prenatal detection and subsequent management. Management of these lesions ranges from surgery to medical surveillance. Reports about congenital lung malformations in our environment are scarce; hence the aim of this study is to evaluate the presentation and outcome of surgical intervention in patients with congenital lung malformations in our environment.

  Methods Top

This was a prospective study of all patients who presented to the Lagos University Teaching Hospital, from January 2011 to December 2020. It included all patients who presented either as an emergency or in a stable condition, with any type of congenital lung malformation. Clearance was obtained from the Ethics and Research Committee of the Lagos University Teaching Hospital and permission was obtained from the adult patients and parents of the children who were briefed on the objective of the study.

The main endpoints of our study were patient demographics, clinical presentation, type of congenital lung malformation and anatomical location, mode of management (surgery and conservative management), and postoperative outcome, including complications.

Patients, who presented, as emergencies with respiratory distress, had surgical intervention after proper diagnosis by clinical and radiographic methods.

Preoperative workup

Stable patients were adequately investigated and associated comorbidities properly managed before elective surgery was done. This is especially important in adults with lung sequestration, and associated chest infection. The investigations performed included serum chemistry, hematological profile, chest X-ray, chest computed tomography (CT) scan for all patients, and CT pulmonary angiography in those with bronchopulmonary sequestration and arterio-venous malformations.

The mode of anesthetic induction was modified in neonates with congenital lobar emphysema (CLE) who were oxygenated without positive pressure ventilation. Thoracotomy was done under local anesthesia, with the emphysematous lobe out of the chest before general anesthesia was commenced. Single lumen endotracheal tubes were used for endotracheal intubation in all the patients.


Posterolateral thoracotomy on the ipsilateral side of the lesion was the standard surgical access for resection of the malformed lung. Lobectomy and cystectomy were the main procedures done. All resected specimens were sent for histopathology. All the neonates and older children were transferred to the intensive care unit (ICU) for mechanical ventilator support and or monitoring. All the neonates who had lobectomies from CLE had mandatory mechanical ventilation for 48–72 h to expand the remaining collapsed lung.

Postoperative care

All the children were admitted to the ICU postoperatively based on our laid-down protocol for at least 48–72 h. The adults were reviewed jointly immediately after surgery by the surgical and the anesthetic teams, and they were deemed fit enough based on cardiopulmonary parameters and clinical state not to require ICU admission, hence they were transferred to the recovery room for monitoring and intranasal oxygen to achieve total recovery from the effects of anesthesia.

Postoperative follow-up continued in the outpatient clinic for 2 years

Data analysis was performed using the Statistical Package for the Social Sciences, version 26 (IBM Corp. Released 2019. IBM SPSS Statistics for Windows, Armonk, NY, USA: IBM Corp) for frequencies and percentages, while Microsoft excel 2010 was used to plot the graphs.

  Results Top

Eighteen patients had congenital lung malformations which included, 14 children (78%) and 4 adults (22%). The congenital malformations consisted of seven cases of congenital pulmonary airway malformations (CPAM) (39%), three CLE (17%), three bronchogenic cysts (BC) (17%), three lung sequestrations (17%), one lung hypoplasia (5%) and one patient with pulmonary arteriovenous malformation (5%) [Figure 1].
Figure 1: Pattern of congenital lung malformations. BC: Bronchogenic cyst, CLE: Congenital lobar emphysema, CPAM: Congenital pulmonary airway malformation, EBPS: Extra lobar pulmonary sequestration, IBPS: Intralobar pulmonary sequestration, LH: Lung hypoplasia, PAVM: Pulmonary arteriovenous malformation

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Neonates contributed 22% (4) of the total, with a mean age of 7.5 ± 5.2 days, whilst children above a month constitute 56% (9) with a mean age of 18.8 ± 10 months. The adult population made up 28% (5) with a mean age of 39 ± 15 years.

There was gender equality, however, there was male predominance in the children age group (61.5%) and female predominance in the adult age group (80%). CLE and BC had a female predominance, in a ratio of 2-1, while CPAM had a male predominance in a ratio of 4–3. Congenital lung sequestration cases were limited only to the female adult population.

Seventeen patients (94%) had posterolateral thoracotomy on the ipsilateral side of the lesion. There were thirteen lobectomies (72%) and two cystectomies (11%). The most affected lobe was the right lower lobe (38%), followed by the left lower lobe (28%).

The cystectomies were done for BC. There were three (two mediastinal and one intra-pulmonary) BC but only two (one mediastinal and one intrapulmonary) cysts were excised; the remaining mediastinal cyst was detected incidentally in the adult patient without symptoms, and is being managed conservatively [Figure 2].
Figure 2: Modalities of management

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Most of the children (80%) were electively ventilated for 4–7 days with a mean of 5.2 ± 1.2 days. It was particularly indicated in neonates with CLE to maximally re-expand the remaining lungs postoperatively. The chest tube drainage duration varied from 5 to 14 days with a mean of 8.7 ± 3.6. Postoperative hospital stay varied from 10 to 7 days with a mean of 14.1 ± 2.3 days.

There were four postoperative complications. One patient with tuberculosis infected BC, which was undiagnosed before surgery, developed total wound breakdown whilst another patient had pneumonia. The patient was commenced on anti-tuberculosis therapy while the wound dehiscence was managed conservatively.

The two mortalities (11%) recorded in our study were neonates that had respiratory failure which led to their death [Figure 3].
Figure 3: Outcome of treatment

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  Discussion Top

Congenital lung malformations are very rare disorders with varied presentations from respiratory emergency in the neonate period to an asymptomatic adult, incidentally discovered on routine imaging or presenting with a complication like infection.[6]

They are caused by aberrant embryological lung development which occurs during intrauterine life. This underscores the need for prenatal screening either by ultrasonography or MRI for detection and diagnosis. In our environment as obtains in most developing countries, most patients with congenital lungs malformation are not detected antenatally but postnatally, even sometimes, they are treated wrongly as lung infections.

In our study, there were more children than adults, and children constituted 72% of the total, with a male predominance of 61%. This contrasts that of the adult population, where there was a female predominance of 80%.

A study by Ferreira et al.[7] in Brazil showed a male predominance (60%) of lung malformation in children, which agrees with our study. Similarly, the study by Saleh et al.[8] in Egypt also revealed a predominance of 60% in male children.

Our patients presented at a mean age of 7.5 ± 5.2 days for neonates, and 18.8 ± 10 months for older children, unlike the 10.73 ± 0.73 months obtained by Ferreira et al. The neonates in our study presented in respiratory emergencies which accounted for earlier presentation, unlike other children whose presentations were delayed due probably to diagnostic error or had been wrongly treated for infection, as occurs in most of the patients with CPAM. One of the patients with CLE was wrongly diagnosed as acute respiratory distress in the neonatal period due to surfactant deficiency, and later again as neonatal pneumothorax which necessitated the referral to us before a correct diagnosis of CLE was made. The other 8-month-old infant with BC was wrongly diagnosed and was treated for bronchopneumonia and intractable asthma for over a month before referral to our center.

Two important factors that assisted in the diagnosis of these patients were the availability of computerized tomography scans and a high index of suspicion for congenital pulmonary malformation as a cause of respiratory distress in this age group.

In our study, CPAM was the commonest lung malformation, constituting 39% of the cases with a male predominance. This agrees with the study of Laberge et al.[9] Repeated chest infections and unresolved pneumonic changes in the lungs constitute the main clinical features of CPAM at presentation and were localized to the right and left lower lobes, and the majority was Type I Stocker classification.[10] This type consists of large cysts majority of which are 3–10 cm in diameter. Transplacental corticosteroid has been found useful in the management of intra-uterine CPAM with observable regression of the mass following two doses of dexamethasone or betamethasone given to the mother.[11] This noninvasive therapy has gradually replaced fetal surgery in the past few years though the mechanism of action is not completely understood.

Surgical resection, postnatally, is indicated to excise the mass, to avoid the complications of CPAM, which includes pneumonia, compression on adjacent structures, and malignant transformation. Pleuro-pulmonary blastoma and bronchoalveolar carcinoma are two notable tumors associated with malignant transformation of CPAM.[12],[13]

Lobar resection is mostly indicated. Occasionally segmental or more extensive resection may become necessary. Early surgery especially during infancy is advised to avoid complications, and allow parenchyma regeneration since alveologenesis continues till 8 years of age.[14] All our seven cases had lobectomy below the age of 2 years.

Concerning CLE, we had 3 cases with two females. The presentation was similar in all three patients; neonatal respiratory distress. Two were misdiagnosed as neonatal pneumothorax despite the absence of risk factors like traumatic forceps delivery. The chest X-ray finding of the emphysematous left upper lobe with contralateral mediastinal shift is a typical finding. All three cases of CLE were located in the left upper lobe which is the commonest location (40%–50%), followed by the right middle lobe (30%–40%).[9]

Two of the three cases of BC in our study were mediastinal, whilst one was intra-pulmonary. There were two pediatric cases that were resected, while the third case was detected asymptomatically in an adult. The latter was a relatively small cyst located in the mediastinum. The patient requested conservative management since it is asymptomatic. However, most prefer excision of the BC due to fear of expansion, infection, hemorrhage, and malignancy. Sarper et al. reported a complication rate of 45% in their study, and most of the complications resulted from compression of mediastinal structures, infection, and rupture into the trachea, pericardial cavity, or pleural cavity.[15]

There were three cases of lung sequestrations in our study with two located in the right lower lobe and the one in the left lower lobe. This was the most common congenital lung malformation in the adult in our study and they all presented with repeated chest infection, and unresolved pneumonia. The symptoms of pulmonary sequestration are consistent in many data series and case reports and similar to our findings.[16],[17],[18] The diagnosis of these cases also required a high index of suspicion and CT angiography because of the anomalies of associated vasculature. This is in agreement with several authors who stated the importance of noninvasive diagnostic techniques like CT or MR angiography to demonstrate aberrant vessels and parenchyma disorders.[19],[20],[21]

The complications (12%) were respiratory failure, wound infection, atelectasis, and fevers. Mortality of 11% included two neonates who died of respiratory failure. Saleh et al. also had a similar complication rate of 17%, but mortality of 3%.[8] The higher mortality in our study may be due to a smaller volume of the study and infrastructural deficit characteristic of low-resource economies.

A patient whose BC was located in the mediastinum and has remained asymptomatic is been managed conservatively with medical surveillance. The management of the asymptomatic patient is controversial, while some authors agree to nonsurgical intervention,[22] others advocate for surgery,[23] because this effectively prevents the risk of infection and malignant transformation, occasionally noted with congenital lung malformations.

Early surgical intervention for asymptomatic cases is advocated on the premise that it allows for compensatory lung growth than late resection. This is because lung growth and development continue into childhood until about 8 years or beyond with an increase in the number and size of the alveoli.[14] Nakajima et al. reported in their study better pulmonary function when resection was carried out before 4 years of age.[24]

  Conclusion Top

Congenital lung malformation remains a rare disorder with a varied presentation from neonatal respiratory emergency to incidental detection in adulthood. Prenatal screening should be encouraged in our environment to enhance early detection. A high index of suspicion is also needed to pick up these cases in children with repeated chest infections, respiratory emergencies, or radiological abnormalities.

The optimal treatment modality remains surgery with the availability of postoperative intensive care, especially in neonates and other children presenting either as an emergency or electively. This goes a long way in reducing postoperative complications while enhancing surgical outcomes generally.

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Conflicts of interest

There are no conflicts of interest.

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  [Figure 1], [Figure 2], [Figure 3]


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