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Is thoracoscopic lung-sparing surgery in treatment of congenital pulmonary airway malformation feasible?

  • M. Lima
  • S. D’Antonio
  • N. Di Salvo
  • M. Maffi
  • M. Libri
  • T. Gargano
  • G. Ruggeri
  • V. D. CataniaEmail author
Original Research
  • 36 Downloads

Abstract

Purpose

Lung-sparing strategies such as segmentectomy or atypical resection have been advocated for small congenital pulmonary airway malformation (CPAM), even by thoracoscopy. The aims of our study were to evaluate surgical and clinical outcome of patients undergoing lung-sparing surgery and to determine whether thoracoscopy is superior to thoracotomy.

Methods

We conducted a retrospective review of patients who underwent lung-sparing resection for CPAM from 2004 to 2018. Demographic data, presenting symptoms, size and location of the CPAM, operative and post-operative data were collected and analyzed according to surgical technique (thoracoscopy—TS, vs thoracotomy—TO). Data were compared using Fisher’s exact test for qualitative values and Mann–Whitney test for quantitative values. P values less than 0.05 were considered as statistically significant.

Results

167 lung-sparing surgery procedures were performed (segmentectomy n = 21 or atypical resection n = 146). 67 procedures were completed in TS. All histological examination revealed negative margins for residual CPAM. Patients in the TS group were older and presented a higher weight compared to the TO group. TS was associated with shorter duration of pleural drainage and shorter hospital stay. Rate of conversion was 35% (n = 29). Location of CPAM in the lower lobe and CPAM size greater than 5 cm were predictor factors of conversion from TS to TO.

Conclusion

Lung-sparing surgery for CPAM is a safe and feasible technique in pediatric patients. TS results in reduced post-operative morbidity compared to TO and should be proposed as first surgical approach for selected patients.

Keywords

Congenital pulmonary malformation Congenital cystic adenomatoid malformation Thoracoscopy Atypical resection Segmentectomy 

Introduction

Congenital pulmonary airway malformation (CPAM) is a developmental malformation of the lower respiratory tract. CPAMs include congenital adenomatoid cystic malformation (CCAM), bronchopulmonary sequestration, bronchogenic and foregut cysts, bronchial atresia with subsequent distal emphysematous progression/development, and congenital lobar emphysema [1, 2].

Although rare, it is the most common congenital lung lesion. Data from large population registries suggest an incidence of congenital lung cysts in the range of 1 per 8300–35,000 live births [3, 4]. Large-cyst subtypes account for about 70% of CPAMs, or 2–8 per 100,000 live births. The diagnosis of congenital pulmonary malformations has increased significantly with the widespread use of prenatal ultrasound screening and, more recently, improvement of malformative characterization with the use of fetal magnetic resonance imaging [5, 6]. Prenatal management is usually conservative because very few of these lesions affect pregnancy or early postnatal course requiring prenatal or urgent postnatal surgery [7, 8, 9, 10, 11, 12]. The optimal postnatal management of CPAM is still under debate. On one hand, there is a wide acceptance that postnatal symptomatic lesions (i.e., symptoms related to infection, hyperinflation, pneumothorax, left to right shunting, and pulmonary hypoplasia due to large mass) require surgical resection [13, 14, 15, 16, 17]. On the other hand, the management of asymptomatic lung lesions is controversial because of concerns related to operative morbidity and mortality along with uncertain outcome due to the lack of long-term follow-up [18, 19, 20, 21]. In the attempt to preserve lung parenchyma, but removing a lung that is at risk of complications (recurrent respiratory tract infections and malignant degeneration), different surgical options have been proposed [22, 23, 24].

In 2013, we have already demonstrated that lung-sparing (LS) surgery (segmentectomy and atypical resection) can be considered as a safe and feasible approach for small CPAMs [12].

The aims of our study were to evaluate the efficacy and the morbidity associated with LS surgery as a treatment of CPAMs and to determine whether thoracoscopy (TS) is superior to thoracotomy (TO).

Materials and methods

This is a retrospective review of patients (younger than 18 years), referred to Pediatric Surgery Department of Bologna University, between January 2004 and October 2018.

Exclusion criteria included thoracoscopic or open lobectomy for CPAM and patients with a definitive diagnosis of extra-lobar sequestration.

Data collected from charts included: patient’s characteristics defined as prenatal diagnosis (and median gestational age at diagnosis), sex, age at operation, weight at time of operation, presence and age at presentation of preoperative symptoms (defined as pneumonia or respiratory distress), associated malformations. CPAM’s radiological characteristics from CT scan or Visible Patient™ 3D reconstruction defined as: laterality of CPAM, location of CPAM in either upper, middle, or lower lobes of the lung, diameter of CPAM.

Operative and post-operative data were compared between patients who underwent TS with those who underwent TO. Operation-related variables included duration of procedure in minutes, type of LS resection performed defined as segmentectomy or wedge resection, intra-operative complications, and the need for conversion to thoracotomy. Postoperative variables included: duration of postoperative thoracic drainage in days, length of hospital stay in days, histological findings of CPAM, postoperative complications (defined as respiratory complications, which included pneumonia, persistent pneumothorax, respiratory distress, pleural effusion, asthma, and non respiratory complications), and re-intervention.

All patients were followed up with regular clinical monitoring (outpatient clinic visit at the 1st, 6th and every 12th month) as well as radiological evaluation (chest radiography 3 and 12 months after the operation). Postoperative computed tomography (CT) scan was performed only in case of symptoms or persistence of abnormalities on the plain X-ray, and at least 12 weeks after surgery.

Patients were divided according to surgical technique in TS and TO group, and were compared for all the above parameters. Statistical analysis was performed using GraphPad Prism version 7.00 for Windows, GraphPad Software, La Jolla California USA, www.graphpad.com. The bivariate analysis involved the Mann–Whitney U (Wilcoxon rank-sum) test for continuous variables with nonparametric distribution and 2 × 2 contingency tables and Fisher exact test for categorical variables. P values less than 0.05 were considered statistically significant.

Results

One hundred and sixty-seven LS resections for CPAM were performed on 156 patients (3 patients had involvement of both upper and lower right lobes; 4 had involvement of both upper and lower left lobes).

Table 1 reports the patients’ characteristics.
Table 1

Patient’s characteristics

Parameters

Total n = 156 (%)

Prenatal diagnosis

119 (76)

Sex (M/F)

96/71 (1.3)

Preoperative symptoms

47 (30)

 Pneumonia

18 (11)

 Respiratory distress

29 (18.5)

Age at symptoms (mean ± SD), months

23.6 ± 54.5

Asymptomatic

109 (70)

Associated malformations

17 (11)

One hundred and nineteen patients (76%) had prenatal diagnosis by fetal ultrasonography or fetal magnetic resonance imaging (MRI) between 20 and 34 weeks’ gestation (median gestational age at diagnosis 27.5 ± 7.1 weeks).

There was a slight male preponderance (M = 96; F = 71); although no gender predilection was found in patients with multiple lesions.

Forty-seven patients (30%) experienced respiratory symptoms before surgery at a median age of 23.6 ± 54.5 mo. Respiratory distress was observed as presenting symptom in 18.5% (n = 29) of patients, and was more common before 6 months of age (20/29).

Associated malformations were observed in 7% of patients, the most common being cardiac malformations (6/17), followed by laryngo-tracheal and gastrointestinal malformations (respectively, 3/17 and 2/17). Only one case of chromosomal abnormality was observed (trisomy 18).

A preoperative CT scan was performed in all patients at the average age of 3.7 months; multiplanar reconstruction was used constantly and became essential in those CT cases with the deeper lesions (Fig. 1). In case of anatomical variation or unclear preoperative imaging, a 3D modular anatomical reconstruction was performed by Visible Patient™ 3D technology (Fig. 2, 3).
Fig. 1

CCAM involving both the middle and the basal segments of the lower right lobe. a CT sagittal scan; b anterior view of volume rendering reconstruction

Fig. 2

Bronchial atresia of the lower right lobe (latero-basal segment) with internal emphysema. a: CT sagittal scan that show the lesion of 50 × 40 × 20 mm. b and c 3D modular anatomical reconstruction from standard CT scan performed by Visible Patient™

Fig. 3

Intralobar sequestration of the lower right lobe. A: CT sagittal scan; B: 3D modular anatomical reconstruction from standard CT scan performed by Visible Patient™ that shows a vascular supply from the abdominal aorta

CPAM’s characteristics are reported in Table 2. Concerning CPAM’s location, there was a slight right-sided preponderance (right = 91; left = 76); and 53% (n = 89) of them were in the lower lobe. At histological analysis, the most common findings were suggestive for: congenital cystic adenomatoid malformation (CCAM) (45%).
Table 2

CPAM’s characteristics

CPAM characteristics

N = 167 (%)

Laterality

 Right

91 (54.5)

 Left

76 (45.6)

Location

 Upper

45 (27)

 Middle

12 (7)

 Lower

89 (53)

Histology

 

 CCAM

76 (45.5)

 Hybrid lesions

6 (3.5)

 Intralobar sequestration

41 (24.5)

 Broncogenic cyst

3 (1.7)

 Others

41 (24.5)

CPAM congenital pulmonary airway malformation, CCAM congenital cystic adenomatoid malformation

Operative and post-operative are shown in Table 3 according to surgical approach. Ninety-six patients were approached by TS, but 29 (16%) required conversion to TO. Median age at conversion was (40.7 ± 63 mo). These patients were included in the TO group for the statistical analysis. Age at time of surgery ranged from 13 days to 17 years (mean 8.6 ± 43.5 mo). Patients who had TO (15.45 ± 32.9 mo) were younger compared to those who underwent TS (33.98 ± 58.3 mo, p = 0.01). The mean weight at surgery was greater in the TS group (14.9 ± 15.7 kg), than in the TO group (8.79 ± 8.5 kg, p = 0.001). No differences were observed between the two groups concerning laterality of CPAM, lobar involvement, and type of lung resection. Wedge resection was the most common performed procedure in both groups (respectively, TO n = 89, 89%; TS n = 57, 90%). Twenty-one patients (12.5%) underwent anatomical segmentectomies because these lesions were completely contained in a segment with safe margins. In all cases, histopathological studies demonstrated safe margins. Mean operative time was 122.2 min (ranged from 65 to 255 min). It was longer in TO patients (125.87 ± 40.3 min) compared to those who underwent TS (92.68 ± 32.2 min; p < 0.001). A postoperative chest tube was positioned in all patients for a mean time of 4.5 days (1–10 days). Duration of chest tube in place was statistically longer in patients undergoing TO (6.2 ± 4 days) compared to those who underwent TS (5 ± 3 days; p < 0.05). The length of hospital stay ranged from 7 to 45 days, with a mean of 9 days. It was longer in the TO group (17.4 ± 17.3 days) than in the TS group (12.8 ± 15 days; p = 0.03). Early post-operative complications were observed in 15 (9%) patients, with no differences between the two groups. One case of death was observed due to multiple malformations. About 80% of complications occurred in patients who were symptomatic before lung-sparing surgery.
Table 3

Operative and post-operative data about 167 procedures of lung-sparing surgery for CPAMs, divided according to surgical approach

 

Total n = 167 (%)

Thoracotomy n = 100 (%)

Thoracoscopy n = 67 (%)

p

Operative data

 Age at surgery (mean ± SD), months

27.57 ± 50.9

15.45 ± 32.9

33.98 ± 58.3

0.01

 Weight at surgery (mean ± SD) (kg)

12.35 ± 14.04

8.79 ± 8.5

14.9 ± 15.7

0.001

 Preoperative symptoms

47 (28)

29 (29)

18 (27)

0.86

 Diameter (mean ± SD) (cm)

4.95 ± 2.0

5.2 ± 2

4.3 ± 1.8

0.004

 Duration of procedure (mean ± SD) (min)

108.9 ± 40.1

125.87 ± 40.3

92.68 ± 32.2

< 0.001

Type of procedure

  Segmentectomy

21 (12.5)

11 (11)

10 (15)

0.48

  Wedge resection

146 (87.5)

89 (89)

57 (85)

0.48

  Intra-operative blood transfusion

8 (5)

5 (5)

3 (4)

 

Post-operative data

 Duration of post-operative drainage (mean ± SD), days

5.8 ± 4.51

6.2 ± 4

5 ± 3

0.03

 Length of hospital stay (mean ± SD), days

29.5 ± 30.8

17.4 ± 17.3

12.8 ± 15

0.03

 Postoperative complications

15 (9)

8 (8)

7 (4)

0.32

  Pneumonia

4 (2)

1 (1)

3 (4)

 

  Persistent pneumothorax

6 (3.5)

3 (3)

3 (4)

 

  Respiratory distress

4 (2)

3 (3)

1 (1)

 

 Duration of follow-up (mean ± SD), months

29.53 ± 30.8

35.7 ± 35.9

25 ± 20

 

 Late complications

5 (3)

2 (2)

3 (4)

 

  Pneumonia

3 (1.7)

1 (1)

2 (3)

 

  Persistent pneumothorax

2 (1)

1 (1)

1 (1)

 

Reintervention

3 (1.7)

1 (1)

2 (3)

0.56

P values less than 0.05 were considered statistically significant (in bold)

Mean duration of follow-up was 65.2 months (range 12–115 months). Late postoperative complications were observed in 5 (3%) of patients. In 3 of them a second surgical exploration and LS was performed because CT scan revealed the suspicion of persistent CPAM. However, histological assessment revealed compensatory emphysema.

Twenty-nine (35%) of patients required conversion from TS to TO. The most common reasons were: patients’ anesthetic instability or inability in recognizing the anatomic limits of the lesion due to adhesion after previous pneumonia. Factors associated with conversion are shown in Table 4. Statistical analysis did not show sex, age (58% age < 6 months vs 42% age > 6 months; p > 0.05) or weight-related risk of conversion to TO. There were no differences between patients who required conversion to TO with those who underwent successful TS concerning pre-operative symptoms (respectively, 38% vs 27%; p = 0.60). Otherwise, location of CPAM in lower lobe and diameter greater than 5 cm can be considered as predictors of conversion from TS to TO.
Table 4

Factors associated with conversion from thoracoscopy to thoracotomy

Predictor variables

Conversion n = 29 (%)

No conversion n = 67 (%)

p

Female

10 (34)

26 (39)

0.60

Age at surgery (mean ± SD) months

27.68 ± 52.3

33.98 ± 58.3

0.61

Weight at surgery (mean ± SD) (kg)

12.5 ± 13.9

14.9 ± 15.7

0.47

Preoperative respiratory symptoms

11 (38)

18 (27)

0.33

Laterality: right sided (r/l)

16/13

38/29

 

Location of CPAM

 Upper

3 (10)

23 (34)

0.02

 Lower

22 (76)

33 (49)

0.02

 Middle

2 (7)

5 (7)

1.00

 Upper and lower

1 (4)

3 (4)

1.00

Diameter (mean ± SD) (cm)

5.2 ± 2.3

4.3 ± 1.8

0.04

P values less than 0.05 were considered statistically significant (in bold)

Discussion

Herein, we report the largest experience of CPAM treated with LS surgery defined as segmentectomy and wedge resection. This approach can be considered as safe and effective in the treatment of CPAM, as no residual disease was observed at histological examination. Moreover, we showed that patients who underwent successful TS had better clinical outcomes, faster removal of thoracic drainage and shorter hospital stay. Finally, we acknowledge that the presence of lesions in the lower lobe or those greater than 5 cm is associated with an increased risk of conversion from TS to TO.

CPAM is a rare congenital anomaly of the lung; however, the incidence of its diagnosis is rising as a result of antenatal ultrasound screening and improvements in imaging quality [5, 6, 7].

While postnatal management for children with symptomatic CPAM is reasonably straightforward, there is an ongoing debate regarding the need and timing of surgery in children with asymptomatic lesions [8]. The natural history of CPAM is variable and to date there is no conclusive publication that demonstrates the resolution of a typical cystic CPAM confirmed on postnatal CT scan. All infants with a prenatal diagnosis require close postnatal evaluation because of the risk of pulmonary compression, infection and malignant degeneration [2, 9, 13, 25]. Many authors recommend that asymptomatic CPAM should be resected prior to the onset of symptoms. Their position is based on the risk of developing infection or symptoms during the period of observation [2, 8]. In our series, 28% experienced clinical symptoms before surgery, in more than 60% of the cases within the first 2 years of life. Most common symptoms were pneumonia and respiratory distress. However, no cases of malignant tumors were observed or identified at time of histological analysis. Our data are concordant with current literature; in fact, the risk of infection for congenital lung lesions has been estimated to range between 10 and 30% within the first year of life, while one series demonstrated a 43% of infection at 2 years, and another recent series had 15% [26, 27, 28]. Moreover, even if rare, some cases of spontaneous pneumothorax have been reported in patients affected by CPAM that may cause adverse cardio-respiratory events. Finally, up to 5% of all CPAMs have been shown to harbor cancer in a retrospective series [29]; whereas in a retrospective series and review, malignant transformation has been reported in 1% of CPAM [30]. Malignant tumors include pleuropulmonary blastoma, bronchoalveolar carcinoma and rhabdomyosarcomas [31].

If surgical resection is chosen for an asymptomatic low-risk patient, it is usually performed after the neonatal period and before 12 months of age, but the optimal timing has not been established, and practice varies [32, 33]. Many authors operate when the infant is between six and 12 months of age [34]. Other groups have suggested earlier time frames for elective surgery, between 3 and 6 months of age [35, 36], or even between one and two months of age [37]. The viewpoint in our center is to operate on asymptomatic infants with a prenatal diagnosis of CPAM within 6 and 12 months of age [12].

Surgical options in the treatment of CPAM include removal of the diseased bronchopulmonary segment (segmentectomy), wedge resection, lobectomy, or complete pneumonectomy [8, 24]. Pulmonary lobectomy, via TO or TS, has been considered as standard in the resection of parenchymal CPAM. In most recent years, many authors, and our center too, have advocated lung-sparing strategies such as segmentectomy or atypical resection in the resection of parenchymal CPAM. In our institution, since 2001, the sparing-lung technique has been employed for smaller, well-defined segmental lesions and in children with bilateral or multilobar disease. The possibility of TS and avoiding lobectomy in CPAM has yet been described and it is very appealing to preserve normal lung parenchyma and improve clinical outcome [23, 24]. Moreover, extended lung reductive surgery may cause an alteration of the architecture of the tracheo‐bronchial tree, thereby leading to an abnormal air flow in the airways and lungs. Segmentectomy or wedge resection may also be beneficial in patients who are diagnosed at an older age. Although many pediatric patients will undergo compensatory lung growth, the efficacy of that growth diminishes in the first few years of life. Thus, as age increases, the potential benefit of segmentectomy also increases [24]. The main controversy concerning lung-sparing surgery is the risk of residual disease and potential for malignant transformation. The systematic review by Stanton et al. demonstrated a 15% rate of residual disease after segmental resection as opposed to 0% with lobectomy [8]. Thus, the number of cases of residual disease in overall literature is considerable, even though publication bias is likely; therefore, there is adequate evidence to recommend a formal lobectomy for typical cases of intralobar CPAMs, except in multilobar or extremely localized disease. Furthermore, the existence of multifocal lesions, not always evident at imaging, supports the hypothesis of a genetic alteration of the lung tissue [22].

In our institution, since its first description, LS was routinely performed in patients with both asymptomatic and symptomatic CPAM. In our study we collected data of 167 procedures, on 156 patients, of LS surgery with a wide range of age at surgery, from neonatal period to older children. In all cases histological examination performed showed clean resection margins. We believe that this is the result of an accurate pre-operative multi-plane CT reconstruction of the lesion. This is mandatory for a proper resection: this anatomical reconstruction allowed us to assess the lesion along the whole surface of the lung, delineating the anatomy of the lung and of the malformation and supporting its resection. At a median follow-up of 50 months, no cases of late lung tumors were observed and an overall incidence of post-operative complications of 4.7% was assessed. This figure is lower than our previous reported study in which the complication rate raised up to 14%, and is concordant with current literature that reports a complication rate, for LS surgery, ranging from 8 to 30% [23, 38]. The low rate of post-operative complication may be partially justified by the fact that 70% of our procedures were performed on asymptomatic patients, who are at lower risk of adverse outcomes [39]. Furthermore, the risk of post-operative complications, in particular those due to air leak, reported as a limiting factor to the extent of LS surgery, was significantly reduced by low-energy bipolar cauterization, LigaSure, stapler device and running sutures.

Since the first publication by Albanese et al. in 2003, several reports have described the feasibility and safety of thoracoscopic lung resections, including a meta-analysis [39, 40, 41, 42]. TS is a safe and feasible option in selected children with CPAM, particularly those without previous infection. In our series, we observed that patients who underwent successful TS were older and with a greater weight compared to those who underwent TO. Our data confirm the statement reported by Rothenberg et al. [43] who described TS as much more challenging if performed in patients weighing less than 10 kilograms. Moreover, we observed that TS resection of CPAM resulted in shorter operative time, duration of post-operative drainage, and length of hospital stay. These results are concordant with current literature, even if all comparative studies published evaluating both the open and the minimally invasive approaches for CPAM were on patients who underwent lobectomy [42]. Our study shows that there were no differences between patients treated by TO and TS for CPAM in terms of intra-operative bleeding, overall complications, and re-interventions.

In our previous study, we reported a conversion of 70% but this result was influenced by the high prevalence (70%) of patients younger than 6 months who undergo TS. In the current study, we decreased the conversion rate up to 36%, thanks to the more accurate selection of patients for TS and gained experience by our medical staff (anesthesiologists and surgeons) in approaching and managing TS also in small infants. This is still higher than the conversion rate reported in current literature, that rates generally from 0 to 33% [23, 42, 44]. With the prospective to improve our results in the near future, we evaluated which factors could be considered as predictors for conversion by comparing patients who successfully underwent TS with those who required conversion to TO. Moreover, the recent use of specific 3D reconstructions of the CPAM performed by Visible Patient™ has improved the preoperative imaging study, and it has allowed to plan accurately a safe surgical procedure.

We identified the location of CPAM in the lower lobe and lesion diameter greater than 5 cm as a potential predictor for conversion from TS to TO. Both can be considered as limitation factors for visibility under TS and mobilization of the lung. However, in our series, the presence of pre-operative symptoms was no different between the two groups.

Our study has several limitations since our data were retrospectively collected, and follow-up was limited over a short and medium period of time.

This is, to our knowledge, the largest reported series of atypical resection in pediatric patients for CPAM. Atypical resection for CPAM is a safe means of lung parenchymal preservation, suitable for both symptomatic and asymptomatic patients. Thoracoscopic approach is associated with a shorter length of hospital stay. TS resection can be considered as feasible and effective for lesions located in the upper/middle lobe, smaller than 5 cm. A prospective randomized trial would be the gold standard to definitively determine the difference in outcomes between lobectomy and LS surgery for CPAM.

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer Nature Singapore Pte Ltd 2019

Authors and Affiliations

  • M. Lima
    • 1
  • S. D’Antonio
    • 1
  • N. Di Salvo
    • 1
  • M. Maffi
    • 1
  • M. Libri
    • 1
  • T. Gargano
    • 1
  • G. Ruggeri
    • 1
  • V. D. Catania
    • 1
    Email author
  1. 1.Department of Pediatric Surgery, Sant’Orsola HospitalUniversity of BolognaBolognaItaly

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