Asymptomatic Congenital Pulmonary Airway Malformation Research Articles

Selection of potential targets for stratifying congenital pulmonary airway malformation patients with molecular imaging: is MUC1 the one?

Currently there is a global lack of consensus about the best treatment for asymptomatic congenital pulmonary airway malformation (CPAM) patients. The somatic KRAS mutations commonly found in adult lung cancer combined with mucinous proliferations are sometimes found in CPAM. For this risk of developing malignancy, 70% of paediatric surgeons perform a resection for asymptomatic CPAM. In order to stratify these patients into high- and low-risk groups for developing malignancy, a minimally invasive diagnostic method is needed, for example targeted molecular imaging. A prerequisite for this technique is a cell membrane bound target. The aim of this study was to review the literature to identify potential targets for molecular imaging in CPAM patients and perform a first step to validate these findings.A systematic search was conducted to identify possible targets in CPAM and adenocarcinoma in situ (AIS) patients. The most interesting targets were evaluated with immunofluorescent staining in adjacent lung tissue, KRAS+ CPAM tissue and KRAS- CPAM tissue.In 185 included studies, 143 possible targets were described, of which 20 targets were upregulated and membrane-bound. Six of them were also upregulated in lung AIS tissue (CEACAM5, E-cadherin, EGFR, ERBB2, ITGA2 and MUC1) and as such of possible interest. Validating studies showed that MUC1 is a potential interesting target.This study provides an extensive overview of all known potential targets in CPAM that might identify those patients at risk for malignancy and conducted the first step towards validation, identifying MUC1 as the most promising target.

COllaborative Neonatal Network for the first European CPAM Trial (CONNECT): a study protocol for a randomised controlled trial

IntroductionConsensus is lacking on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM). For future studies, the CONNECT consortium (the COllaborative Neonatal Network for the first European CPAM Trial)—an...

Clinical Symptoms Affect Treatment and Prognosis in Pediatric Patients with Congenital Pulmonary Airway Malformation: A Propensity Score Matching Retrospective Cohort Study

ObjectivesSurgery for asymptomatic congenital pulmonary airway malformation patients is still debatable at this time. This study aims to investigate the safety and efficacy of surgery for asymptomatic patients, as well as the factors influencing the symptoms of this group of patients. MethodsAn institutional database was sampled for congenital pulmonary airway malformation patients. Patients were divided into the symptomatic group and the asymptomatic group. Propensity score matching (PSM) analysis selected patients in each group to compare perioperative outcomes. A multivariable logistic regression analysis was performed to investigate the potential influences on symptomatic lesions. ResultsThe asymptomatic group had better perioperative results than the symptomatic group, including shorter operating times (119.39 ± 49.42 min vs 100.73 ± 23.09 min, P = 0.031), shorter postoperative mechanical ventilation (2 h [0.5–46] vs 1 h [0.5–5], P = 0.002), shorter chest tube durations (4d [2-29] vs 3d [2-10], P = 0.007), and shorter postoperative hospital stays (10d [6–36] vs 8d [6-16], P < 0.001). With the conversion to thoracotomy and postoperative complications, there was no statistically significant difference between the two PSM-matched groups (P > 0.05). Age (p = 0.037), postnatal diagnosis (p = 0.018), and maximum cyst diameter (p = 0.032) were found to be independent variables associated with symptomatic lesions by multivariable logistic regression. ConclusionsPatients with congenital pulmonary airway malformation appear to have better perioperative outcomes before the beginning of symptoms. Symptomatic pulmonary lesions were associated with age, postnatal diagnosis, and maximum cyst diameter. Level of evidenceLevel III.

The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey.

Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers. Proposed outcome parameters were scored according to level of importance, and the final COS was established through consensus. A total of 55 participants (33 surgeons, 22 non-surgeons) from 28 centers in 13 European countries completed the three rounds and rated 43 outcome parameters. The final COS comprises seven outcome parameters: respiratory insufficiency, surgical complications, mass effect/mediastinal shift (at three time-points) and multifocal disease (at two time-points). The seven outcome parameters included in the final COS reflect the diversity in priorities among this large group of European participants. However, we recommend the incorporation of these outcome parameters in the design of future studies, as they describe measurable and validated outcomes as well as the accepted age at measurement.

Surgical techniques of video-assisted thoracic surgery for congenital pulmonary airway malformation in a 1-year-3-month-old (8.6 kg) infant

: We herein report a 1-year-3-month-old boy (8.6 kg) with asymptomatic congenital pulmonary airway malformation (CPAM). The patient had been prenatally diagnosed with CPAM. Since his neonatal condition was stable, surgery was electively planned once he reached 1 year of age. Preoperative chest computed tomography (CT) revealed a multiseptated air-filled cystic lesions in the left lower lobe. Complete video-assisted thoracic surgery (C-VATS) with left lower lobectomy was performed (operation time: 182 min, blood loss: 10 mL). The patient was discharged on postoperative day 7 with no complications. The pathological examination revealed a type 2 CPAM (Stocker’s classification). Although we believe VATS lobectomy for CPAM in infants is feasible, there are some specific hints and pitfalls to be aware of when performing pediatric thoracic surgery: (I) study and improve upon the ligation technique of vessels and bronchus, (II) be aware of the anatomical location of vessels and bronchus, and (III) adjust the position of the bronchial blocker for differential ventilation. When the condition of patients is stable without any symptoms or infection, we recommend that they undergo surgery after the age of one to ensure appropriate growth. In addition, we propose that C-VATS lobectomy may be feasible if patients are older than 18 months old or weigh more than 10 kg from the perspective of vessel/bronchial dissection technique and differential ventilation during anesthesia.

Mucinous adenocarcinoma in a neonate with congenital pulmonary malformation

Congenital pulmonary airway malformation (CPAM) is a rare developmental abnormality of the lower respiratory tract, often diagnosed during routine prenatal ultrasonography. We report a full-term newborn female noted to have a large mass in the left hemithorax during prenatal ultrasound, at 35 weeks gestation. A multicystic lesion in the lower left chest was confirmed using a Chest Radiograph and Computed Tomography (CT) angiogram. Her hospital course was next followed by a left lower lobectomy, with resultant pathology of CPAM Type 1 and invasive mucinous adenocarcinoma. Though patients with asymptomatic CPAM are often managed by observation, our case of unexpected malignancy advocates early considerations towards Surgery.

Quantitative CT imaging analysis to predict pathology features in patients with a congenital pulmonary airway malformation

BackgroundRisk for infection and potential malignant degeneration are the most common arguments for resecting asymptomatic Congenital Pulmonary Airway Malformations (CPAM). We aimed to investigate if CT- imaging characteristics can be used to predict histopathological features, by using an objective quantitative CT scoring method. MethodsArchival CPAM tissue samples were histologically re-assessed and patients who had a pre-operative volumetric CT-scan were included. Lung disease was quantified using the newly-developed congenital lung abnormality quantification(CLAQ) scoring method and obtained percentages were used to predict histopathological signs of inflammation and presence of mucinous proliferation (MP). Because MP is presumed a precursor for mucinous adenocarcinoma in situ (AIS) this method was also used to compare CT-scans of patients with AIS to those with only CPAM. ResultsThirty-three CPAM patients were included of which 13(39%) had histological signs of inflammation and 8(24%) had a MP. Patients with inflammation had a significantly smaller lesion (14% vs 38%) while those with MP had more extensive disease (54%vs17%). Patients with AIS had a significantly smaller lesion compared to CPAM patients (5%vs29%). Significant predictors for inflammation were smaller lesion size and percentage hypodensity within lesions while a larger lesion size and percentage parenchymal hyperdensity (solid lung tissue components) were predictors for MP as well as AIS. ConclusionsSmaller CPAM lesions may be more susceptible to inflammation while larger lesions may be associated with the presence of MP. Parenchymal hyperdensity is found as a predictor for MP as well as AIS and should therefore elicit more extensive gross sampling. Level of evidenceLevel III.

Surgical Treatment for Asymptomatic Congenital Pulmonary Airway Malformations in Children: Waiting or Not?

Infection is undoubtedly the most important factor in influencing the timing and surgical strategy of congenital pulmonary airway malformation (CPAM) surgery. However, there have been no studies on the optimal timing of surgery for patients based on the probability of infection. The aim of this study was performed to explore the optimal timing of surgery of CPAM in children from the risk of infection. The correlation of age distribution and pulmonary infection of 237 children diagnosed by pathology from January 2012 to January 2020 in Guangzhou Women and Children's Medical Center were analyzed retrospectively. We defined the cases with preoperative computed tomographic findings of infection, pathological findings of large number of neutrophils, tissue cells, and abscess formation as the infection group. The rate of infection in patients less than 2 years old was significantly lower than in patients over 2 years old (11.4% vs. 45.7%, p < 0.001). And the pulmonary lobectomy rate of patients with infection (29.0%) was significantly higher than in noninfected patients (17.2%) and whole patients (36.3%), p = 0.033. Patients with infection lose more blood during surgery (noninfected patients: 81.76 ± 13.14 mL, infected patients: 145.10 ± 25.39 mL, p = 0.027). The univariate analysis revealed that the infection rate of patients over 2 years old was 3.084 times that of patients ≤2 years old (odds ratio [OR]: 3.084, 95% confidence interval [CI]: 1.196-7.954; p = 0.020). The infection rate of CPAM types III and IV patients is lower than types I and II (OR: 0.531, 95% CI: 0.307-0.920; p = 0.024). In consideration of the high risk of infection, lower minimally invasive surgery rate, an increased rate of pulmonary lobectomy, and more blood loss in patients over 2 years old, our study also supports early surgical treatment. Therefore, we suggest that for asymptomatic patients with CPAM I and CPAM II, surgical treatment should be performed when they are less than 2 years old, providing more options for surgical strategies and monitoring of CPAM patients.

Early KRAS oncogenic driver mutations in nonmucinous tissue of congenital pulmonary airway malformations as an indicator of potential malignant behavior

The potential for malignant degeneration is the most common reason for some practitioners to resect asymptomatic congenital pulmonary airway malformations (CPAMs). We aimed to investigate the potential of various immunohistochemical (IHC) and genomic biomarkers to predict the presence of mucinous proliferations (MPs) in CPAM. Archival CPAM tissue samples were re-assessed and underwent IHC analysis using a panel of differentiating markers (TTF1/CDX2/CC10/MUC2/MUC5AC/p16/p53/DICER1). In each sample, intensity of IHC staining was assessed separately in normal lung tissue, CPAM, and MPtissue, using a semiquantitative approach. Likewise, next-generation targeted sequencing of known adult lung driver mutations, including KRAS/BRAF/EGFR/ERBB2, was performed in all samples with MP and in control samples of CPAM tissue without MP. We analyzed samples of 25 CPAM type 1 and 25 CPAM type 2 and found MPs in 11 samples. They were all characterized by strong MUC5AC expression, and all carried a KRAS mutation in the MP and adjacent nonmucinous CPAM tissue, whereas the surrounding normal lung tissue was negative. By contrast, in less than half (5 out of 12) control samples lacking MP, the CPAM tissue also carried a KRAS mutation. KRAS mutations in nonmucinous CPAM tissue may identify lesions with a potential for malignant degeneration and may guide histopathological assessment and patient follow-up.

Introduction of thoracoscopic surgery for congenital pulmonary airway malformation in infants: review of 13 consecutive surgical cases.

Although complete video-assisted thoracic surgery (C-VATS) has been demonstrated to have several advantages compared with conventional thoracotomy, there are few reports on the clinical feasibility of C-VATS for CPAM in infants. We retrospectively evaluated 13 consecutive patients (neonates 4; infants 9) surgically treated for congenital pulmonary airway malformation (CPAM) from 1 January 2008 to 31 March 2017. In the group of neonates, all 4 cases were prenatally diagnosed and they underwent semi-emergent surgery after birth due to respiratory failure. In the group of 9 infants, 5 cases were prenatally diagnosed and 4 cases were diagnosed at age >2.5 years due to symptoms associated with pulmonary cystic infection. Pulmonary resection consisted of the following: 8 lobectomies, 1 segmentectomy, 2 wedge resection, 1 fractionated lung resection and 1 lobectomy with segmentectomy. Overall, there were 9 thoracotomy and 4 thoracoscopic surgeries. Mean operation time was 162 min (range, 67-290 min) and blood loss was 21 mL (range, 0-74 mL) on average. There were no complications such as thoracic deformity or respiratory failure, however in 2 of those who underwent segmentectomy the cystic remnant remained. No statistically significant differences were observed between the thoracotomy Group and C-VATS group in terms of age and height at intervention, operation time, blood loss, postoperative day of drain removal, and length of hospital stay after surgery. However, only the average body weight was heavier in C-VATS group (P=0.03). Since early surgical resection of asymptomatic CPAM is often recommended for the prevention of infections and the development of lung malignancy, we recommend performing surgery after the age of 1 year if the patient's condition is stable. Furthermore, C-VATS lobectomy may be feasible if they are older than 18 months or weigh more than 10 kg.

Cancer gene mutations in congenital pulmonary airway malformation patients.

BackgroundNewborns affected with congenital pulmonary airway malformations (CPAMs) may present with severe respiratory distress or remain asymptomatic. While surgical resection is the definitive treatment for symptomatic CPAMs, prophylactic elective surgery may be recommended for asymptomatic CPAMs owing to the risk of tumour development. However, the implementation of prophylactic surgery is quite controversial on the grounds that more evidence linking CPAMs and cancer is needed. The large gap in knowledge of CPAM pathogenesis results in uncertainties and controversies in disease management. As developmental genes control postnatal cell growth and contribute to cancer development, we hypothesised that CPAMs may be underlain by germline mutations in genes governing airways development.MethodsSequencing of the exome of 19 patients and their unaffected parents.ResultsA more than expected number of mutations in cancer genes (false discovery rate q-value <5.01×10−5) was observed. The co-occurrence, in the same patient, of damaging variants in genes encoding interacting proteins is intriguing, the most striking being thyroglobulin (TG) and its receptor, megalin (LRP2). Both genes are highly relevant in lung development and cancer.ConclusionsThe overall excess of mutations in cancer genes may account for the reported association of CPAMs with carcinomas and provide some evidence to argue for prophylactic surgery by some surgeons.

Symptom development in originally asymptomatic CPAM diagnosed prenatally: a systematic review.

For the approximately three quarters of patients with a prenatal diagnosis of congenital pulmonary airway malformation (CPAM) who are asymptomatic at birth, the chance of eventually developing symptoms is unknown. We sought to explore the natural history of asymptomatic CPAM. We searched EMBASE, MEDLINE, and the first 50 results from Google Scholar. Studies describing the natural history of prenatally diagnosed, initially asymptomatic CPAM were included. For asymptomatic patients initially managed non-surgically, we tabulated the proportion who went on to develop symptoms as well as the median age at symptom development. We included data from 19 retrospective studies on 353 patients. Of the 128 patients managed expectantly, 31 (24.2%) developed symptoms requiring surgical intervention. The median age at symptom development was 7.5months (range 15days-5years). The risk for developing respiratory symptoms exists with originally asymptomatic CPAM patients, but the exact risk is difficult to predict. Parents may be given the value of approximately 1 in 4 as an estimate of the proportion of asymptomatic CPAM patients who go on to develop symptoms, which will help them make an informed decision regarding the option of elective surgery.

Optimal timing for elective resection of asymptomatic congenital pulmonary airway malformations.

We sought to determine optimal timing for CPAM resection within the first year of life. We queried the National Surgical Quality Improvement Program pediatric database from 2012 to 2015 for elective CPAM resections on patients less than 1year of age. Patients were divided by age in months: 1-3 (n=57), 4-6 (n=135), and 6-12 (n=214). Patient operative variables and 30-day postoperative outcomes were compared. A total of 406 patients were included with no differences in demographics or comorbidities. Median operative time increased with each older age category (115min, 152min, 163min, respectively; p<0.01). Thoracoscopic approach was less utilized in 1-3months (40.4%) compared to the older two age categories (65.9% and 69.6%, respectively; p<0.01). There were no differences by age in major complications, conversion to open, or readmissions. On multivariate analysis, ASA class≥3 (p<0.01) and prolonged operative time (p<0.01) were associated with a major complication. Furthermore, operations on patients aged 6-12months were associated with increased operative time (p<0.01) regardless of operative approach. Elective CPAM resections are equally safe in patients 1-12months of age. Earlier resection including both open and thoracoscopic resection is associated with decreased operative time. IIc, Outcomes Research.

Decision-Making Criteria for Observational Management of Congenital Pulmonary Airway Malformations (CPAMs)

The purpose of this study was to determine practice patterns of Canadian surgeons managing congenital pulmonary airway malformations (CPAMs) and factors influencing practice. Pediatric surgeons in Canada were surveyed regarding their experience, evaluation, and management CPAMs, and what factors they feel qualify patients for observation vs resection. Data were summarized, and Fisher's-Exact and Kruskal-Wallis Tests applied where appropriate. Sixty eight percent (n=46) of surgeons responded. However, three surveys were incomplete and excluded. The median age of initial assessment by a pediatric surgeon was one month. 98% (42/43) use CXR for initial imaging, and 83% (36/43) recommend CT scan for further evaluation. Observation is offered always, almost always, or sometimes by 2%, 35% and 37%, respectively. Only 16% almost never, and 9% never offer it. Years in practice was not associated with this decision (p=0.41). Of surgeons who offer observation, 78% (28/37) use morphology to guide their decision, and 63% (21/37) use lesion size (<1cm to <5cms). 68%(23/37) consider the number of lesions, and 61%(14/23) of those only offer observation to solitary lesions. Most pediatric surgeons in Canada offer observational management to patients with asymptomatic CPAMs. While practice variations exist, detailed imaging with a CT scan early in life to determine the morphology, size, and number of lesions guides practice. V.

Erratum to: Current Management of Congenital Pulmonary Airway Malformations: A "European Pediatric Surgeons' Association" Survey.

Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%). Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. Conclusion Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.

Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use.

Surgery remains the cornerstone treatment of symptomatic lesions but the postnatal management of asymptomatic CPAM remains controversial. There are pros and cons of surgical resection, as increasing rate of infections over time renders the surgery more difficult after months or years of evolution, as well as risk of malignancy, though exact incidence is still unknown. What is known: • Congenital pulmonary airway malformations (CPAM) are rare developmental lung malformations mainly antenatally diagnosed. • While the neonatal management of symptomatic CPAM is clear and includes prompt surgery, controversies remain for asymptomatic CPAM due to risk of infections and malignancies. What is new: • Increased rate of infection over time renders the surgery more difficult after months or years of evolution and pushes for recommendation of early elective surgery. • New molecular or pathological pathways may help in the distinction of type 4 CPAM from type I pleuropulmonary blastoma.

Current Management of Congenital Pulmonary Airway Malformations: A “European Pediatric Surgeons' Association” Survey

To define current management of congenital pulmonary airway malformation (CPAM). A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%).Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.

Treatment of congenital pulmonary airway malformations: a systematic review from the APSA outcomes and evidence based practice committee.

Variation in management characterizes treatment of infants with a congenital pulmonary airway malformation (CPAM). This review addresses six clinically applicable questions using available evidence to provide recommendations for the treatment of these patients. Questions regarding the management of a pediatric patient with a CPAM were generated. English language articles published between 1960 and 2014 were compiled after searching Medline and OvidSP. The articles were divided by subject area and by the question asked, then reviewed and included if they specifically addressed the proposed question. 1040 articles were identified on initial search. After screening abstracts per eligibility criteria, 130 articles were used to answer the proposed questions. Based on the available literature, resection of an asymptomatic CPAM is controversial, and when performed is usually completed within the first six months of life. Lobectomy remains the standard resection method for CPAM, and can be performed thoracoscopically or via thoracotomy. There is no consensus regarding a monitoring protocol for observing asymptomatic lesions, although at least one chest computerized tomogram (CT) should be performed postnatally for lesion characterization. An antenatally identified CPAM can be evaluated with MRI if fetal intervention is being considered, but is not required for the fetus with a lesion not at risk for hydrops. Prenatal consultation should be offered for infants with CPAM and encouraged for those infants in whom characteristics indicate risk of hydrops. Very few articles provided definitive recommendations for care of the patient with a CPAM and none reported Level I or II evidence. Based on available information, CPAMs are usually resected early in life if at all. A prenatally diagnosed congenital lung lesion should be evaluated postnatally with CT, and prenatal counseling should be undertaken in patients at risk for hydrops.

Optimal age for elective surgery of asymptomatic congenital pulmonary airway malformation: a meta-analysis.

Controversy exists on the optimal age for elective resection of asymptomatic congenital pulmonary airway malformation. Current recommendations vary widely, highlighting the overall lack of consensus. A systematic search of Embase, MEDLINE, CINAL, and CENTRAL was conducted in January 2016. Identified citations were screening independently in duplicate and consensus was required for inclusion. Results were pooled using inverse variance fixed effects meta-analysis. Meta-analysis results indicate no statistically significant differences for complications within the 3-month and 6-month age comparison groups [odds ratio (OR) 4.20, 95% confidence interval (CI) 0.78-22.77, I 2 = 0%; OR 2.39, 95% CI 0.63-9.11, I 2 = 0%, respectively]. Older patients were significantly favoured for 3-month and 6-month age comparison groups for length of hospital stay [mean difference (MD) 4.13, 95% CI 2.31-5.96, I 2 = 0%; MD 3.38, 95% CI 0.44-6.31, I 2 = 0%, respectively]. Borderline statistical significance was observed for chest tube duration in patients ≥6 months of age (MD 1.06, 95% CI 0.02-2.09, I 2 = 0%). No mortalities were recorded. Surgical treatment appears to be safe at all ages, with no mortalities and similar rates of complications between age groups. The included evidence was not sufficient to make a conclusive recommendation on optimal age for elective resection.

Morbidity after elective resection of prenatally diagnosed asymptomatic congenital pulmonary airway malformations.

The optimal management of prenatally diagnosed asymptomatic congenital pulmonary airway malformations (CPAM) is controversial. Since there is a paucity of data relating to surgical risks in this specific population, we reviewed our experience to further inform this controversy. Ethically approved 10 year (2004-2013) retrospective review. Patients were included only if the CPAM was diagnosed prenatally and remained asymptomatic. Indication for surgery was physician recommendation and/or parental choice. Sixty patients were identified. Median age at surgery was 6.5 months (range 65 days to 9.6 years). Resections were performed thoracoscopically (n = 51, one conversion) or by thoracotomy (n = 9). Surgical time was 2.5 hr (43 min to 4.75 hr). A chest drain was used in 58/60 and remained in situ 53 hr (23-108). There were no intra-operative complications or blood transfusions. All patients were extubated at the end of the procedure with no re-intubations. Post-operative hospitalization was 73.4 hr (23.8 hr to 4.2 days). Overall, complications occurred in 14/60 (23%). Eleven were minor but three were major: tension pneumothorax associated with new presentation of a small previously undiagnosed diaphragmatic hernia 5 days following resection; aggressive fibromatosis of the chest wall in the region close to resection 2 years later; and near-fatal hypovolemic cardiac arrest due to massive haemorrhage from a feeding vessel on postoperative day 7. There were no deaths and no cases of pleuropulmonary blastoma. Resection of prenatally diagnosed asymptomatic CPAM is associated with a significant risk of complications, which may be life threatening. These data contribute to a balanced discussion of risks and benefits for these children.