Congenital Lung Malformations Research Articles

Management and Outcomes of Patients With High-Risk (Congenital Lung Malformation Volume Ratio≥ 1.6) Congenital Lung Malformations

IntroductionCongenital lung malformations (CLMs) have a variable natural history. Larger lesions with CLM volume ratio (CVR) ≥ 1.6 are associated with hydrops and fetal mortality. The purpose of this study is to describe the management and outcomes of high-risk (CVR ≥ 1.6) CLM patients. MethodsA retrospective cohort study was performed for all fetuses evaluated between May 2015 and May 2022. Demographics, prenatal imaging factors, prenatal and postnatal treatment, and outcomes were collected. Descriptive statistics were used to compare the cohorts. ResultsOf 149 fetal CLM patients referred to our fetal center, 21/149 (14%) had CVR ≥ 1.6. One CLM patient had intrauterine fetal demise, and 2 patients were lost to follow-up. Of the remaining 18 patients, 11/18 (67%) received maternal steroids. Seven out of 18 patients (39%) underwent resection at the time of delivery with 1/7 (14%) undergoing exutero intrapartum treatment (EXIT)-to-resection, 5/7 (71%) undergoing EXIT-to-exteriorization-to-resection, and 1/7 (14%) undergoing a coordinated delivery to resection; among those undergoing resection, there were 2 fatalities (28.5%). Seven out of 18 (39%) patients required urgent neonatal open lobectomies, and the remaining 4/18 (22%) patients underwent elective thoracoscopic lobectomies with no mortality. ConclusionsThe natural history and outcomes of severe CLM patients remain highly variable. The EXIT-to-exteriorization-to-resection procedure may be a safe and effective approach for a subset of CLM patients with persistent symptoms of mass effect and severe mediastinal shift due to the observed decreased operative time requiring placental support observed in our study.

Experience of video-assisted thoracic surgery treatment of congenital pulmonary airway malformation in infants less than 3 months of age.

Congenital pulmonary airway malformation (CPAM) is the most common type of congenital lung malformation, some children were born with serious respiratory and circulatory disorders, these children tend to have larger cystic lesions and emergency surgery should be taken after birth. This article aimed to explore the feasibility of video-assisted thoracic surgery (VATS) for CPAM in infants less than 3 months of age and analyze the early and mid-term postoperative outcomes. A retrospective analysis was performed on 12 children with CPAM who were admitted to Shanghai Children's Hospital from January 2019 to December 2022. Among them, there were ten boys and two girls. The age ranged from 13 to 89 days, with an average of 60.09±30.13 days. The body weight ranged from 2.8 to 7.5 kg, averaging 5.12±1.56 kg. All patients had severe respiratory symptoms such as persistently tachypneic, recurrent pneumonia or respiratory failure. All of the included children underwent VATS with no cases converted to thoracotomy. The operation time ranged from 40 to 190 minutes, with an average of 124.17 minutes. Among the children in this study, ten cases underwent lobectomy; one underwent segmental resection; one underwent irregular resection. Postoperative mechanical ventilation time ranged from 3 to 49 hours, with an average of 12.75 hours. The indwelling time of the thoracic drainage tube ranged from 2 to 9 days, with an average of 3.92 days. The postoperative hospital length of stay ranged from 5 to 12 days, averaging 8.42 days. None of the children had postoperative complications such as bleeding, bronchopleural fistula or atelectasis. The follow-up time ranged from 3 months to 4 years, and all the children were discharged within 12 days of postoperative hospitalization without need for readmission. Furthermore, no residual lesions were found in the reexamined chest computerized tomography (CT), the lung inflammation dissipated, and no residual cavity was in the affected chest. Infants with symptomatic CPAM less than 3 months of age should be operated on as soon as possible. With the improvement of minimally invasive technology and anesthesia management, VATS is relatively safe and effective in treating children with such conditions.

Percutaneous transthoracic catheter drainage prior to surgery in treating neonates with congenital macrocystic lung malformation presenting with respiratory distress

Percutaneous transthoracic catheter drainage prior to surgery in treating neonates with congenital macrocystic lung malformation presenting with respiratory distress

The complexities in diagnosing intradiaphragmatic extrapulmonary sequestration: a case report

BackgroundExtralobar pulmonary sequestration–a congenital lung malformation characterized by nonfunctional lung tissue with its own visceral pleura and without a connection to the normal tracheobronchial tree–is often surgically resected given the potential for infectious complications. We report a case of a child with extralobar pulmonary sequestration in a rare and challenging intradiaphragmatic location, which made preoperative identification and planning difficult.Case presentationA 2-year-old boy presented for follow-up of a large left extralobar pulmonary sequestration initially diagnosed on an antenatal ultrasound. Follow-up imaging, including prenatal MRI and postnatal CT angiography, was inconclusive on the precise location of the extralobar pulmonary sequestration in relation to the diaphragm but did reveal a subdiaphragmatic arterial supply from a branch off the celiac trunk. Planned resection with diagnostic thoracoscopy revealed the mass to seemingly be below the diaphragm; however, subsequent abdominal laparoscopy identified it to be within the diaphragm. Once the supplying vessel off the celiac trunk was controlled and divided, the mass was circumferentially excised from the edges of the diaphragmatic muscle. The remaining diaphragmatic defect was then closed, and the patient did well postoperatively.ConclusionsThis case demonstrates the difficulty of making a definitive diagnosis of intradiaphragmatic extralobar pulmonary sequestration without operative intervention. CT angiography and identification of the sequestration’s arterial supply may not be conclusive in determining the precise location if in close proximity to the diaphragm.

Diagnostic accuracy of imaging compared to histology in congenital lung malformations

IntroductionThe aim of this study was to evaluate the accuracy of imaging tests (prenatal ultrasound [US] and postnatal computed tomography [CT]) in comparison to histology for diagnosis of congenital lung malformations (CLMs). Material and methodsRetrospective study of patients with a prenatal diagnosis of CLM whose postnatal follow-up included thoracic CT scan and histological examination of the lesion. We collected data on demographic variables, gestational age at diagnosis, US findings and the history of multiple gestation. We used the kappa coefficient to determine the level of agreement between the findings of prenatal US and postnatal tests (CT and histology).We analysed paired data on the size of the lesion, its location and the presence or absence of systemic arterial vascularization. ResultsThe sample included 56 patients with 57 lesions. The mean gestational age at diagnosis was 22.42 weeks (SD, 3.94) and 57% were male. Malformations most frequently involved the left lung and the lower lobes. The agreement between CT and histology in the detection of cystic lesions was moderate (κ = 0.55) but stronger compared to the agreement between US and histology (κ = 0.10). The agreement between CT and histology was substantial (κ = 0.66) in the detection of systemic vascularization of the lesion and stronger compared to the agreement between US and histology. Both imaging methods were highly accurate in the identification of the location of the pulmonary lesions. Conclusionspostnatal CT offers a substantial concordance with histological findings, especially in the detection of systemic vascularization, and an accurate prediction of the anatomy of the lesion.

Novel Clinical Algorithm for Prenatal Monitoring of Congenital Lung Malformations

IntroductionCongenital lung malformations (CLMs) are readily identified early in pregnancy with a variable natural history. Monitoring for lesion size and mediastinal shift (MS) is recommended following diagnosis. The purpose of this study is to propose a risk-stratified clinical algorithm for prenatal monitoring of CLM. MethodsAfter ethical approval, all fetuses with CLMs evaluated at our fetal center from January 2015 to June 2022 were retrospectively reviewed. Patient demographics, imaging characteristics, and fetal interventions were collected. Lesions were stratified by congenital lung malformation volume ratio (CVR) and the presence of MS. Descriptive statistics and receiver operating characteristic curves were employed in the analysis. ResultsWe analyzed 111 patients with a mean of 23.4 wk gestational age, a median CVR of 0.5 (interquartile range, 0.3-1.2), and MS in 76 of 111(68%) patients on initial evaluation. Among low-risk patients (CVR ≤1.1), 96% remained low-risk on final evaluation. No patients transitioned from low to high risk during the growth period. Patients with CVR >1.1 often had persistent MS (P < 0.001). Hydrops (5/111, 5%) and fetal intervention (4/111, 4%) only occurred in patients with CVR >1.1 (P < 0.001, P = 0.002) and MS (P = 0.144, P = 0.214). On receiver operating characteristic curve analysis, initial CVR >1.1 had 100% sensitivity and negative predictive value for hydrops and fetal intervention. ConclusionsCLMs with initial CVR ≤1.1 are low risk for hydrops and fetal intervention. We propose a risk-stratified algorithm for the monitoring of CLM during the growth period based on CVR. While our experience suggests that patients with CLM and MS are at higher risk, the current subjective assessment of MS is not adequately predictive. Incorporating an MS grading system may further refine risk stratification in the management of CLM.

Prenatal Measurements of Congenital Lung Malformations: Can They Predict Postnatal Size?

IntroductionPrenatally diagnosed congenital lung malformations (CLMs) are monitored via ultrasound and measured by congenital pulmonary airway malformation volume ratios (CVRs) which can predict postnatal respiratory symptoms. This study compared CVR to postnatal lesion size to help guide prenatal counseling. MethodsA retrospective chart review evaluated the prenatal imaging and postnatal outcomes for patients who were prenatally diagnosed with CLMs and had a postnatal computed tomography (CT) scan at one institution. ResultsFifty-seven patients were included. Four had symptoms requiring urgent resection. The remaining were discharged and had clinic follow-up with CT scan to determine next steps: five had no identified lesions, eight had lesions whose diagnosis did not warrant an operation, and 40 had lesions whose diagnosis rendered size a factor in operative decision-making. Of these 40, 26/40 patients (65%) underwent elective resection (median maximum CVR 0.97; median lesion size 4 cm) and 14/40 patients (35%) were observed without resection (median maximum CVR 0.5; median lesion size 3 cm). There was a positive correlation between prenatal CVR and postnatal lesion size, with R-squared = 0.46. Maximum CVRs were better than last CVRs when predicting whether postnatal CT size would fall above or below our institution's level of recommended resection, with an area under the curve of 0.85 and a CVR cut-point of 0.61. ConclusionsFor newborns with asymptomatic CLMs, higher maximum CVRs correlated with larger size on postnatal CT. A maximum CVR ≤0.6 was correlated with a smaller postnatal CT size that may be eligible for nonoperative management. While these results are not intended to recommend surgery based on higher CVRs alone, this information could potentially be used to reassure expectant parents whose babies’ prenatal imaging demonstrate lower maximum CVRs.

Pulmonary function after lobectomy in children: a systematic review and meta-analysis

BackgroundThe influence of lobectomy on pulmonary function in children was still controversial. A systematic review and meta-analysis were essential to explore whether pulmonary function was impaired after lobectomy in children.MethodsPubMed,...

Pulmonary sequestrations

Congenital lung malformations account for 2.2 – 6.6% of all congenital abnormalities of the lungs. This is a pretty rare problem as compared to acquired lung diseases. Pulmonary sequestrations account for 0.15 – 1.8% in the incidence of all lung malformations, occupying the second place after the complex abnormalities called “lung agenesis-hypoplasia”. Pulmonary sequestrations are common in children and adolescents so one could encounter them in pediatric practice. But pulmonary sequestrations may be associated with insignificant symptoms or even be asymptomatic in selected pediatric cases. Thereby, adult general practitioners, pulmonologists, and thoracic surgeons may encounter such patients. The aim. Review had the purpose to inform the general practitioners, pulmonologists, and thoracic surgeons about pulmonary sequestrations and their diagnostic, clinics course, and treatment. Conclusion. There is a wide range of congenital abnormalities of human body. Pulmonary sequestrations are only a part of this huge problem. But modern medicine has a vast selection of methods for revealing and treating these conditions (including minimally invasive surgery).

Prognosis of the risk progression of post-inflammatory pneumofibrosis in children with chronic nonspecific lung diseases

Introduction. Pneumofibrosis (PF) is a morphological outcome of acute and chronic lung diseases, the progression of which leads to organ deficiency.Aim. Development of a prognostic algorithm for assessing the risk of progression of post-inflammatory PF in children with chronic nonspecific lung diseases (CNSLD).Materials and methods. The examination of 52 children with CNSLD with focal post-inflammatory PF was conducted, of which 26 children with progressive PF and 26 children with non-progressive PF. The children were selected according to the “case-control” scheme. The patients had a dynamic clinical and laboratory examination with multispiral computed tomography of the lungs. Determination of the “zero” genotypes of the detoxification genes GSTM1, GSTT1 was carried out by means of polymerase chain reaction. The odds ratio indicator was used to assess the relative risk.Results. Based on the analysis of medico-social, clinical characteristics and genetic polymorphism, an individual prognostic algorithm for the risk of progression of post-inflammatory PF in children with CNSLD was developed. The algorithm includes the calculation of the total score (TS) of 7 medical and social indicators (living in an urban area; the presence of passive smoking; the duration of a pulmonological history of 4-9 years; the presence of congenital lung malformation; the number of acute respiratory infections (ARI) 4 or more times/year; the duration of ARI 11 days or more; taking antibiotics for more than 3 once/year) and the presence of deletions in the detoxification genes GSTM1 and GSTT1. With a value of TS 6.97 or more, a high risk of progression of PF is predicted, with TS 3.47-6.96 points, a moderate risk of progression of PF is diagnosed, with TS 3.46 or less points, a minimal risk of progression of PF.Conclusion. The proposed algorithm allows predicting the risk of progression of post-inflammatory PF in children with CNSLD at the stage of early diagnosis and will prevent the spread of the process in the lungs with the help of preventive measures and preventive therapy, which will increase the duration and improve the quality of life of the patient.

Pulmonary Sequestration and Congenital Pulmonary Airway Malformation (CPAM) 1 Presented as A Left Suprarenal Mass:ACase Report

Introduction Pulmonary sequestration is a rare condition accounting for 0.1% to 6% of all congenital lung malformations. The correlation with Congenital Pulmonary Airway Malformation (CPAM) type 1 has not been adequately elucidated. Case presentation From routine fetal sonographic screening, a 20-day-old female baby's ultrasound revealed hydronephrosis with enlargement above the left kidney. Magnetic resonance imaging during a follow-up appointment uncovered a left suprarenal lesion of approximately 30 mm, which was not suspected to be adrenal neuroblastoma. After resection, histological analysis was consistent with pulmonary sequestration and confirmed the connection with CPAM type 1. Conclusion Surgery remains the only effective therapeutic choice for intra-abdominal pulmonary sequestration. The definitive diagnosis is confirmed through histological examination.

Concordancia entre el diagnóstico por imagen y la histología en las malformaciones pulmonares congénitas

IntroductionThe aim of this study was to evaluate the accuracy of imaging tests (prenatal ultrasound [US] and postnatal computed tomography [CT]) in comparison to histology for diagnosis of congenital lung malformations (CLMs). Material and methodsRetrospective study of patients with a prenatal diagnosis of CLM whose postnatal followup included thoracic CT scan and histological examination of the lesion. We collected data on demographic variables, gestational age at diagnosis, US findings and the history of multiple gestation. We used the kappa coefficient to determine the level of agreement between the findings of prenatal US and postnatal tests (CT and histology). We analysed paired data on the size of the lesion, its location and the presence or absence of systemic arterial vascularization. ResultsThe sample included 56 patients with 57 lesions. The mean gestational age at diagnosis was 22.42 weeks (SD, 3.94) and 57% were male. Malformations most frequently involved the left lung and the lower lobes. The agreement between CT and histology in the detection of cystic lesions was moderate (κ=.55) but stronger compared to the agreement between US and histology (κ=.10). The agreement between CT and histology was substantial (κ=.66) in the detection of systemic vascularization of the lesion and stronger compared to the agreement between US and histology. Both imaging methods were highly accurate in the identification of the location of the pulmonary lesions. ConclusionsPostnatal CT offers a substantial concordance with histological findings, especially in the detection of systemic vascularization, and an accurate prediction of the anatomy of the lesion.

Initial experience of thoracoscopic segmentectomy of basal segment through the inferior pulmonary ligament approach in treating congenital lung malformations in children

PurposeThis study aimed to evaluate the feasibility and limitations of thoracoscopic segmentectomy of the basal segment (S10).MethodsClinical data of 15 children with congenital lung malformations (CLM) who underwent thoracoscopic segmentectomy of S10 via the inferior pulmonary ligament approach from January to October 2022 were retrospectively analyzed. The demographics, clinical presentation, intraoperative time, blood loss, postoperative events, and follow-up duration were assessed.ResultsThere were 15 patients in this group (nine males and six females). Age ranges from 4.3 to 96.0 months (median, 7.7 months). Fourteen patients underwent S10 segmentectomy, with one undergoing right S10 segmentectomy and right S6 partial wedge resection. The surgical time was 57–125 min (median, 80 min), intraoperative bleeding volume (5–20 ml; median, 10 ml), postoperative drainage tube indwelling (2–4 d; median, 3 d), and postoperative hospitalization time (4–7 d; median, 5 d). No intraoperative conversions, surgical mortalities, or major complications were observed among these patients. Subcutaneous emphysema appeared in three patients; however, it disappeared following conservative observation without pneumothorax or bronchopleural fistula occurrence.ConclusionsThoracoscopic segmentectomy of S10 via the inferior pulmonary ligament approach is technically feasible for treating CLM; however, this surgical approach may have certain limitations for CLM with large cysts.

Intralobar bronchopulmonary sequestration: an example of the late onset of this rare congenital pathology

Bronchopulmonary sequestration (BPS) refers to a rare congenital lung malformation with a nonventilated dysplastic fragment of parenchyma separated from the main bronchial tree. This segment has a systemic blood supply through aberrant arteries and venous outflow into the systemic venous bed or pulmonary veins and has a common with the rest of the lung (intralobar BPS) or an independent (extralobar BPS) visceral pleura. Purpose of the study is to describe an unusual late onset of right-sided intralobar BPS characterized by a pseudopneumonic course in a patient with concomitant anomalies of the thoracic spine and diaphragm; to discuss the issues of formation, manifestation, diagnosis, differential diagnosis, complications, and treatment of intralobar BPS, and to consider its combination with other developmental anomalies. Conclusion. BLS should be considered in patients with an unusual course of pulmonary anomalies, especially localized in the basal segments of the left (more common) or right (less common) lung and characterized by abnormal blood supply and other malformations.

Intralobular Pulmonary Sequestration of the Right Upper Lobe Supplied by Ascending Aorta.

Pulmonary sequestration (PS) is a rare congenital lung malformation that is more common in the left lower lobe. In 95% of cases, the artery supplying the sequestration usually originates from the thoracic and abdominal aorta. We report a rare intralobular PS case for a feeding artery from the ascending aorta. Angio-computed tomography should be performed for diagnosis once PS is suspected.

Congenital Lung Malformations: Pulmonary Function Assessment Before and After Surgery

BackgroundThe management of asymptomatic congenital lung malformations (CLM) is debated, and pulmonary function of patients with CLM is seldom discussed. Short-term respiratory outcome in patients operated for CLM was assessed in order to better define surgical impact. MethodsA retrospective study on patients operated for CLM between 2012 and 2021 was performed. Respiratory function was assessed with tidal breath analysis (TBA) in spontaneous sleep within 2 years of life. Patients with comorbidities affecting pulmonary function were excluded. Three variables were studied: tPTEF/tE (time to peak tidal expiratory flow/total expiratory time), tV (tidal volume), RR (respiratory rate). Results were assessed as z-score (normal ±1.64) and expressed as median (range). Pre- and post-operative results were compared, as well as post-operative results of disease and surgery type subgroups. ResultsThirty-nine patients were included. Median pre- and post-operative tPTEF/tE were normal (pre: −0.32 (−2.12; +1.56); post: −0.18 (−1.62; +2.43)). Patients with extralobar sequestration had pre-operative high median RR, which improved after sequestrectomy (pre: +1.99 (−2.49; +7.43); post −0.22 (−3.01; +1.18)). All groups had reduced pre-operative median tV, which restored after surgery (pre: −2.15 (−9.75; +0.91); post: −0.35 (−6.65; +3.14)). Patients undergoing lobectomy and patients with intralobar sequestration showed greater improvement. Median post-operative TBA results were normal for all sub-groups except for reduced tV in patients operated with thoracotomy (−2.27 (−6.12; +5.26)). ConclusionsPatients with clinically asymptomatic CLM can have pathologic pulmonary function tests, which improve after surgery. These results add to the elements in favor of an interventional approach. Level of evidenceIII, Treatment study.

Spectrum of Congenital Lung Malformations in Children: Experience from A Tertiary Care Center

Spectrum of Congenital Lung Malformations in Children: Experience from A Tertiary Care Center

Outcome of Bubble CPAP in Neonate with Respiratory Distress

Background: Continuous Positive Airway Pressure (CPAP) is a well-established mode of respiratory support in newborns. Bubble CPAP (bCPAP) is safe, efficacious and easy to use in preterm and term neonates with mild to moderate respiratory distress.&#x0D; Objectives: To find out the outcome of bCPAP in neonate with respiratory distress.&#x0D; Methods: This cross sectional study was conducted over 6 months in Bangladesh Shishu Hospital &amp; Institute. Total 108 term and preterm neonates were enrolled who were presented with respiratory distress. Neonate with type II respiratory failure, congenital heart disease and structural malformations of lung and GI tract causing respiratory distress at birth and neonate needed intubatuin at birth were excluded. Detailed information were obtained in each case. Thorough clinical examinations were done. Relevant investigation reports were collected. All the information were recorded. Statistical analysis was done by using SPSS version 23.&#x0D; Results: In this study the mean age was found 43.3±43.1 hours with range from 2 to 204 hours. Majority 62(57.4%) patients were male and male female ratio was 1.3:1. Two third (66.7%) patients had birth weight ³2500 gm, 14(12.9%) had &lt;1499 gm and 22(20.4%) patients had birth weight 1500-2499 gm. Majority (59.3%) patients belonged to gestational age between 37-41 wks, followed by 27(25%) belonged between 33-36 wks and 17(15.7%) belonged between 28-32 wks. Among the enrolled cases 18(16.7%) were RDS, 18(16.7%) were PNA, 14(13%) were PPHN, 9(4.3%) were MAS, 6(5.6%) were TTN, 13(12%) were congenital Pneumonia, 15(13.9%) were Pneumonia, 10(9.3%) were Sepsis and 5(4.6%) were Laryngomalacia. Among 108 patients who were put on bCPAP, 85(78.7%) patients were weaned and 23 (21.3%) were failed and needed mechanical ventilation. Out of 85 weaned patients hundred percent were survived and got discharge. Out of 23 failed cases 16(69.6%) cases were died and 7 (30.4%) cases were survived and got discharge.&#x0D; Conclusion: It is concluded from this study that bCPAP is an effective way of management of neonates with respiratory distress due to various causes. Patients who were failed in bCPAP, died more in final outcome.&#x0D; DS (Child) H J 2021; 38(1): 27-33

Thoracoscopic segmentectomy in children with congenital lung malformation

Congenital lung malformations (CLM) are most commonly treated with a pulmonary lobectomy. However, due to technological advancement, video-assisted thoracoscopic surgery (VATS) segmentectomy is becoming an attractive alternative to VATS lobectomy. This study aimed to evaluate the safety, feasibility, and efficacy of VATS segmentectomy as a lung parenchyma-saving strategy in children with CLM. A retrospective analysis was performed on 85 children, for whom VATS segmentectomy was tried for CLM between January 2010 and July 2020. We compared the surgical outcomes of VATS segmentectomy with the outcomes of 465 patients who underwent VATS lobectomy. Eighty-four patients received VATS segmentectomy and thoracotomy conversion was necessary for one patient for CLM. The mean age was 3.2 ± 2.5 (range 1.2–11.6) years. The mean operative time was 91.4 ± 35.6 (range 40–200) minutes. The median duration of chest tube drainage was 1 (range 1–21) day, and the median length of postoperative hospital stay was 4 (range 3–23) days. There were no postoperative mortality and postoperative complications developed in 7 patients (8.2%), including persistent air leakage in 6 patients (7.1%) and postoperative pneumonia in 1 patient (1.2%). The median follow-up period was 33.5 (interquartile range 31–57) months and there were no patients requiring re-intervention or reoperation during the follow-up period. In the VATS segmentectomy group, the persistent air leakage rate was higher than in the VATS lobectomy group (7.1 vs. 1.1%, p = 0.003). Otherwise, postoperative outcomes were comparable between the two groups. VATS segmentectomy in children with CLM is a technically feasible alternative to VATS lobectomy with acceptable early and mid-term outcomes. However, the persistent air-leakage rate was higher in VATS segmentectomy.

A Rare Case of Congenital Cystic Adenomatous Malformation of Lung

ABSTRACT Congenital pulmonary airway malformation (CPAM) is because of excessive and disordered proliferation of bronchial tubular structures of the lung. CPAM is slightly infrequent with the unknown etiology and incidence. The diagnosis of these cases is most of the time performed prenatally by routine ultrasonography scanning. Examination of the resection specimen is mandatory for determining occult malignancy. Complication of untreated congenital cystic adenomatous malformation of lung includes pulmonary hypertension; pulmonary hypoplasia; respiratory distress because of hydrops, pneumothorax, or prematurity; recurrent respiratory tract infection; recurrent pneumonia; hemothorax; pleural effusion; and pneumatocoele. A correct prenatal diagnosis and a clinical assessment of the congenital cystic lung diseases authorize an advanced and successful surgical timing. Surgery is the only definitive treatment and should be undertaken as early as possible to prevent infection and other complications.