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The presence of comorbidities (CM) in congenital lobar emphysema (CLE) has been previously described with varying rates. However, the clinical implication of CM on the clinical presentation and patient outcome in CLE is unclear. This was a retrospective cohort study between 2000 and 2022 in a single institution. The study included pediatric patients diagnosed with CLE. During the study period, 36 patients were identified with CLE. The presence of respiratory symptoms at diagnosis was documented in 69% (24/35) of the patients with 48% admitted to intensive care units. The presence of CM was documented in 14/36 (39%) of the patients, with cardiovascular anomalies as the most common (22%), followed by genitourinary anomalies (11%) and large airway anomalies (8%). When comparing the CM group (n = 14) vs the non-CM group (N = 22), there was no significant difference regarding gender, term birth, age of diagnosis, and the location of the lesion. The CM group had significantly higher rates of intensive care unit (ICU) admissions (75% vs 33%, p = 0.02), longer duration of admission (median 46 days vs 9, p = 0.02), and need for pre-operative non-invasive respiratory support (50% vs 5%, p = 0.002). Conclusion: The presence of CM in CLE was associated with a more severe presentation as reflected with significantly higher rates of ICU admissions, prolonged admissions, and higher need for non-invasive respiratory support. Screening for CM, mostly for cardiac anomalies, is recommended at time of CLE diagnosis. What is Known: • Congenital lobar emphysema (CLE) is a developmental lung anomaly generally associated with other comorbidities; however, their clinical implication on presentation is unclear • The prevalence of comorbidities in CLE was previously reported around 15–20%, with cardiovascular anomalies as the most common What is New: • Comorbidities are very common (39%) in CLE, and their presence is related to a more severe clinical presentation and need for respiratory support • Screening for comorbidities is recommended in CLE, specifically for cardiovascular and genitourinary anomalies [ABSTRACT FROM AUTHOR]

Background: Computed tomography (CT) has a larger role in the noninvasive examination of congenital lung abnormalities, thanks to the introduction of multidetector CT (MDCT). If a surgical lesion is suspected, it is often necessary to use cross-sectional imaging, such as CT, for diagnosis confirmation, further lesion definition, and preoperative evaluation. Aims and Objectives: The aim of this study was to enhance comprehension of the occurrence and varieties of congenital lung abnormalities in children, as well as to assess and compare the diagnostic precision of MDCT with that of surgical intervention. Methodology: MDCT with contrast CT was performed in children with respiratory complaints and prenatal history of suspicion in targeted imaging for fetal anomalies (TIFFA) or congenital lung malformations (CLMs) using TOSHIBA 8 slice CT; the study population consisted of 40 pediatric patients with a history of chest anomalies during pregnancy in TIFFA or children with abnormal chest X-ray suspicion of lung anomalies for 18 months. Results: In a study of 40 pediatric patients who underwent MDCT with contrast to evaluate CLMs, 6 were excluded from further evaluation with contrast due to the absence of pulmonary anomalies on plain CT, leaving a possible diagnosis of s CLM in the remaining 34 cases. Of these, cystic pulmonary airway malformations (CPAM) (41.1%) were the most common, followed by congenital lobar emphysema (17.6%), bronchogenic cyst (14.7%), lung agenesis (11.6%), and phylogenetic lung. Conclusion: To notice the characteristics and related anomalies of CLMs and aid in the presurgical evaluation of CLMs, the study concludes that MDCT with contrast is the imaging of choice.

Congenital pulmonary airway malformations (CPAMs) represent a well-known cluster of rare lung malformations affecting 1 in 2500 live births. The natural history of many CPAMs is to increase their size in the second trimester, reach a plateau, and, in about 50% of cases, regress and to become barely detectable during the third trimester. Little is known about cases of affected neonates born prematurely: only six cases are described in the literature, recording different conduct and outcomes. Herein, we report the case of a very low birth weight infant born at GW 28 without antenatal findings and presenting at birth with severe respiratory distress, requiring ventilation. Chest X-rays and a CT scan showed the presence of a solid mass in the left lung. An initial conservative approach was adopted as the baby gained respiratory stability within the first days of life. Routine ultrasound (US) showed a progressive reduction of the lesion, mimicking the process of involution that CPAM can exhibit during late gestation. The rarity of the condition does not allow the formulation of any suggestions regarding one type of management over the other. An initial conservative approach seems to be appropriate with regards to the outcome and possible intra- and post-operative complications. [ABSTRACT FROM AUTHOR]

There has been an increased recognition of a subset of congenital lobar emphysema (CLE), termed congenital sublobar hyperinflation (CSLH), which may affect only a segment of lung as opposed to an entire lobe. This is an uncommon variant for which there is a paucity of information in published literature. The majority of CLE are managed surgically. Current literature suggests non-operative management for CSLH. However, there has been slow adoption of non-operative management and there is not a well-established observation pathway. A retrospective review of all pediatric patients diagnosed with CSLH at a single institution was performed from 2017 to 2023 to determine if this variant may be safely managed with observation. A total of 10 patients were identified. Of these, three patients had consolidation on cross-sectional imaging; therefore, operative intervention was undertaken given diagnostic uncertainty. All patients managed observationally remained asymptomatic. This case series validates non-operative management for patients with asymptomatic CSLH. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to compare respiratory functions of patients after thoracoscopic lobectomy (TS) with those after thoracotomy (TR). Methods: This retrospective study was conducted in two centers, one of which adapted TS as a standard procedure in 2009 and the other performs it via TR. Data on patients who underwent lobectomy for congenital lung disease between 2009 and 2021 and underwent pulmonary function test (spirometry) were collected. Results: Ten patients underwent TS and 36 underwent TR. Distribution based on sex, prenatal diagnosis, pathological diagnosis, and resected lobe were similar between the two groups. The median [interquartile range] age at procedure in the TR group was significantly smaller than that in the TS group (13 [11–18] months versus 38 [13–79] months, P = .03). The procedure duration in the TR group was significantly shorter than that in the TS group (230 [171–264] minutes versus 264 [226–420] minutes, P = .02). Pulmonary function test was conducted at the age of eight in both groups, but the interval between the procedure, and the test was significantly shorter in the TS group (TR: 7 [5–8] years versus TS: 5 [2–7] years, P = .03). The ratio of forced vital capacity compared to predicted one (TR: 86.6 [76.6–95.3] versus TS: 88.7 [86.8–89.1], P = .58) and the ratio of forced expiratory volume in 1 second against that predicted (TR: 84.0 [80.5–88.7] versus TS: 88.7 [86.8–89.1], P = .08) were not significantly different between the two groups. Conclusions: Although TR was performed earlier than TS, respiratory function was similar between the two groups. [ABSTRACT FROM AUTHOR]

Congenital lung malformations are a constellation of pathologies that can be diagnosed antenatally by ultrasound and fetal MRI. Ultrasound is considered the modality of choice for a routine assessment of second-trimester scans worldwide. Bronchopulmonary sequestration (BPS) and congenital pulmonary airway malformation (CPAM) are the 2 most common echogenic chest masses discovered incidentally during routine ultrasound scans in the second trimester. This paper describes BPS and differentiates it from CPAM sonographically in utero. An extensive literature search involving antenatal ultrasound is undertaken to review the most up-to-date understanding of the BPS. Furthermore, a case study at our institution and the literature review will help better describe the salient features of BPS. A 41-year-old female G3P1 visits our department for a routine second-trimester ultrasound. An echogenic lesion with a cystic component is visualized in this scan. Based on the grayscale and color imaging, this complex echogenic lesion was reported as CPAM and was referred to fetal assessment for confirmation. The fetal assessment diagnosed the lesion as BPS because of the pathognomonic feeding vessel from the thoracic aorta. Regardless of the congenital lung mass, any large mass compromising fetal well-being is an indication for intervention. The prognosis of BPS in the absence of fetal hydrops is excellent. A robust collaboration among radiologists, obstetricians, and pediatricians is required for the best outcome for the pregnancy and the neonate.

Abstract Purpose This study aimed to evaluate the feasibility and limitations of thoracoscopic segmentectomy of the basal segment (S10). Methods Clinical 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. Results There 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. Conclusions Thoracoscopic 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.

Congenital pulmonary airway malformations (CPAMs) represent a well-known cluster of rare lung malformations affecting 1 in 2500 live births. The natural history of many CPAMs is to increase their size in the second trimester, reach a plateau, and, in about 50% of cases, regress and to become barely detectable during the third trimester. Little is known about cases of affected neonates born prematurely: only six cases are described in the literature, recording different conduct and outcomes. Herein, we report the case of a very low birth weight infant born at GW 28 without antenatal findings and presenting at birth with severe respiratory distress, requiring ventilation. Chest X-rays and a CT scan showed the presence of a solid mass in the left lung. An initial conservative approach was adopted as the baby gained respiratory stability within the first days of life. Routine ultrasound (US) showed a progressive reduction of the lesion, mimicking the process of involution that CPAM can exhibit during late gestation. The rarity of the condition does not allow the formulation of any suggestions regarding one type of management over the other. An initial conservative approach seems to be appropriate with regards to the outcome and possible intra- and post-operative complications.

Lobectomy is currently the advised resection for symptomatic congenital lung abnormalities (CLA). Sublobar surgery is suggested as an alternative that enables to conserve healthy lung parenchyma. This systematic review aims to explore the outcomes of sublobar surgery in CLA patients as well as the corresponding surgical terminology and techniques. A systematic literature search was performed in adherence to PRISMA-P guidelines. The target population consists of children undergoing sublobar pulmonary resection for CLA. All studies were independently assessed by two reviewers, and evaluated by a third reviewer in case of disagreement. The literature search yielded 901 studies of which 18 studies were included, comprising 1167 cases. The median chest tube insertion duration was 3.6 days (range 2.0–6.9 days), the median hospital admission was 4.9 days (range 2.0–14.5 days), and residual disease was diagnosed in 2% – leading to re-operation in 70%. The median incidence of postoperative complications was 15% (range 0–67%). Follow-up imaging was standard-of-care in 2/3 of studies. Due to the absence of standardised terminology, operative details and specification of resection type did not typically relate between studies. Sublobar resection of CLA lesions could be a viable alternative to lobectomy in certain cases, with the advantage of conserving healthy lung parenchyma. Peri- and postoperative complications are comparable with those reported for conventional lobectomy. The incidence of residual disease following sublobar surgery appears to be lower than commonly stated. To improve comparability between studies, we recommend reporting perioperative characteristics in a structured format. Level IV. • What is currently known about this topic? Children with congenital lung abnormalities generally undergo lobectomy when surgery is indicated. Sublobar surgery potentially preserves healthy lung parenchyma. • What new information is contained in this article? This systematic review shows similar outcome of sublobar surgery compared to conventional lobectomy and finds great variety in reported terminology and operative details amongst studies, indicating the need for structured reporting. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to evaluate the feasibility and limitations of thoracoscopic segmentectomy of the basal segment (S10). Methods: Clinical 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. Results: There 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. Conclusions: Thoracoscopic 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. [ABSTRACT FROM AUTHOR]

Abstract Background Congenital pulmonary airway malformations (CPAMs) are a heterogenous collection of congenital lung malformations, often diagnosed prenatally. The Stocker Type III CPAM is a rare CPAM sub-type, and, when large, may be associated with hydrops. Furthermore, reports of CPAM management which may include surgical resection in extreme preterm infants are limited. Case presentation We report a case of a female neonate born at 28 weeks of gestation with severe respiratory distress and diffuse pulmonary opacification on the right concerning for a large congenital lung lesion. This lesion was not detected on routine antenatal imaging, and she did not have clinical findings of associated hydrops. Her respiratory status improved dramatically after surgical resection of a mass at 12 day of age. The mass was consistent pathologically with a Stocker Type III CPAM. Lung expansion showed subsequent improvement at 16 months of age. Conclusions Our case describes a preterm neonate with severe respiratory distress that was found postnatally to have a large, unilateral congenital lung lesion despite a normal prenatal ultrasound. Additionally, this lesion required excision early in life due to severity of respiratory compromise. This case highlights that rare congenital lung lesions, like this rare sub-type of CPAM, should remain a diagnostic consideration in neonates with severe respiratory distress. Early lung resection for CPAM in preterm infants is not well described and the favorable outcomes of this case help expand perspectives on potential management strategies.

Abstract Background Thoracoscopic lobectomy is a common treatment for congenital lung malformation. Single-direction thoracoscopic lobectomy may be an effective and safe approach without the need to flip the lung over repeatedly, thus minimizing tissue trauma, but its use has not been reported in children. The purpose of this study was to evaluate the safety and efficacy of single-direction thoracoscopic lobectomy in children. Methods A total of 91 patients who underwent thoracoscopic lobectomy in our hospital from January 2020 to December 2020 were retrospectively analysed. According to the inclusion criteria, 21 children were identified as the single-direction group. The details of the single-direction thoracoscopic lobectomy technique are described. Another 21 patients who underwent conventional thoracoscopic lobectomy in the same period were matched using the propensity score matching and set as the control group, the clinical outcomes between the two groups were compared. Results The median age of the patients was 4.72 months (4.72 ± 0.90) with a mean body weight of 7.43 kg (7.43 ± 1.14). There were no significant differences in intraoperative blood loss (P = 0.549), operation time (P = 0.859), length of chest tube drainage (P = 0.102) and length of hospital stay (P = 0.636) between the 2 groups. No patients experienced bronchopleural fistula and conversion to thoracotomy in either group. All patients recovered well without respiratory symptoms or other complications after follow-up of more than 1 year. Conclusions Our preliminary experience presented a series of single-direction video-assisted thoracoscopic lobectomy for children with satisfactory perioperative results.

Background: Total thoracoscopic segmentectomy (TTS) is a technically challenging procedure in children but results in more parenchyma preservation, better pain control, better cosmetic results, and a shorter hospital stay. However, definitive data describing the learning curve of TTS has yet to be obtained. Here, we review the safety and efficiency of our initial experiences with pediatric TTS and evaluate our learning curve. Methods: This was a retrospective study of all pediatric patients undergoing TTS between December 2016 and January 2020. Pediatric patients who underwent TTS were included, while those undergoing lobectomy or wedge resection were excluded. Results: One hundred and twelve patients were retrospectively analyzed to evaluate the learning curve and were divided chronologically into three phases, the ascending phase (A), plateau phase (B) and descending phase (C), through cumulative summation (CUSUM) of the operative time (OT). Phases A, B, and C comprised 28, 51, and 33 cases, respectively. OT decreased significantly from phases A to B (p < 0.001) and from phase B to C (p = 0.076). No significant differences were observed in the demographic factors among the three phases. The conversion rate was zero, and the complication rate was 0.9%. Differences in technical parameters, such as length of stay and chest tube duration, were statistically insignificant between phases A and B or B and C. There were no mortalities. Conclusion: CUSUMOT indicates that the learning curve of at least 79 cases is required for TTS in our institute. We emphasize that the learning curve should be cautiously interpreted because many factors in different institutions may influence the exact parabola and actual learning curve. [ABSTRACT FROM AUTHOR]

Congenital pulmonary airway malformation (CPAM) is congenital pulmonary anomaly characterized by multicystic areas, over-distension, and proliferation of terminal bronchioles with lack of normal alveoli. Clinical presentation may vary from mild respiratory symptoms to severe respiratory distress and frequent pneumothoraxes. We report a rare case of neonatal CPAM type I manifested with neonatal respiratory distress and pneumothorax, which was managed successfully with left lower lobectomy. [ABSTRACT FROM AUTHOR]

Background: Congenital pulmonary airway malformations (CPAMs) are a heterogenous collection of congenital lung malformations, often diagnosed prenatally. The Stocker Type III CPAM is a rare CPAM sub-type, and, when large, may be associated with hydrops. Furthermore, reports of CPAM management which may include surgical resection in extreme preterm infants are limited. Case presentation: We report a case of a female neonate born at 28 weeks of gestation with severe respiratory distress and diffuse pulmonary opacification on the right concerning for a large congenital lung lesion. This lesion was not detected on routine antenatal imaging, and she did not have clinical findings of associated hydrops. Her respiratory status improved dramatically after surgical resection of a mass at 12 day of age. The mass was consistent pathologically with a Stocker Type III CPAM. Lung expansion showed subsequent improvement at 16 months of age. Conclusions: Our case describes a preterm neonate with severe respiratory distress that was found postnatally to have a large, unilateral congenital lung lesion despite a normal prenatal ultrasound. Additionally, this lesion required excision early in life due to severity of respiratory compromise. This case highlights that rare congenital lung lesions, like this rare sub-type of CPAM, should remain a diagnostic consideration in neonates with severe respiratory distress. Early lung resection for CPAM in preterm infants is not well described and the favorable outcomes of this case help expand perspectives on potential management strategies. [ABSTRACT FROM AUTHOR]

Background: Thoracoscopic lobectomy is a common treatment for congenital lung malformation. Single-direction thoracoscopic lobectomy may be an effective and safe approach without the need to flip the lung over repeatedly, thus minimizing tissue trauma, but its use has not been reported in children. The purpose of this study was to evaluate the safety and efficacy of single-direction thoracoscopic lobectomy in children. Methods: A total of 91 patients who underwent thoracoscopic lobectomy in our hospital from January 2020 to December 2020 were retrospectively analysed. According to the inclusion criteria, 21 children were identified as the single-direction group. The details of the single-direction thoracoscopic lobectomy technique are described. Another 21 patients who underwent conventional thoracoscopic lobectomy in the same period were matched using the propensity score matching and set as the control group, the clinical outcomes between the two groups were compared. Results: The median age of the patients was 4.72 months (4.72 ± 0.90) with a mean body weight of 7.43 kg (7.43 ± 1.14). There were no significant differences in intraoperative blood loss (P = 0.549), operation time (P = 0.859), length of chest tube drainage (P = 0.102) and length of hospital stay (P = 0.636) between the 2 groups. No patients experienced bronchopleural fistula and conversion to thoracotomy in either group. All patients recovered well without respiratory symptoms or other complications after follow-up of more than 1 year. Conclusions: Our preliminary experience presented a series of single-direction video-assisted thoracoscopic lobectomy for children with satisfactory perioperative results. [ABSTRACT FROM AUTHOR]

Summary: Background: Pediatric thoracoscopic lobectomy is a technically challenging procedure that may result in better pain control, better cosmetic results, and shorter hospital stay. However, data describing the learning curve of total thoracoscopic lobectomy (TTL) have yet to be obtained. To evaluate our learning curve for TTL in children, we reviewed the safety and efficiency of our initial experiences with TTL in pediatric patients with congenital lung malformation. Methods: This was a retrospective study of all pediatric patients undergoing TTL between March 2011 and January 2017. Cumulative summation (CUSUM) analysis of operative time (OT) was used. Results: One hundred patients were retrospectively analyzed and chronologically divided into three phases: the ascending (A), plateau (B), and descending (C) phases of CUSUM of OT. Phases A, B, and C comprised 35, 22, and 43 cases, respectively. OT decreased significantly from phases A to B (P = 0.035) and B to C (P = 0.019). Age and weight of patients both reduced significantly from phase A to B (p = 0.017 and p = 0.012, respectively), while the two measures did not vary from phase B to C (p = 0.987 and p = 0.874, respectively). Chest tube duration and length of hospital stay had similar trend. All complications occurred in five cases in phase A (5/35). Six cases were converted to open surgery (6%). Four conversions occurred within phase A and two in phase C (4/35 vs 2/43, p = 0.490). There were no mortalities. Conclusions: Repeated standardized training plays a role in overcoming the learning curve for thoracoscopic lobectomy in children, and CUSUMOT indicates that a learning curve of approximately 57 cases is required in our institute.

Abstract Objective Meta-analysis was used to evaluate the diagnostic value of a CVR cut-off value of 1.6 for fetal hydrops due to congenital lung malformation (CLM). Methods A systematic search of PubMed, Embase, Web of Science, CNKI, VIP, and Wanfang published before 7/30/2021 for the value of a congenital pulmonary airway malformation volume ratio (CVR) cut-off value of 1.6 for the diagnosis of fetal hydrops. According to the inclusion and exclusion criteria, the literature that met the requirements were obtained. A total of 75 articles were retrieved, and 12 articles were included for further analysis. The quality of these studies was evaluated according to the Quality Assessment for Studies of Diagnostic Accuracy tool (QUADAS-2) criteria. The Q test and heterogeneity I2 were used to evaluate the heterogeneity due to non-threshold effects, and Stata 15.0 was used for statistical analysis to evaluate the diagnostic value of the CVR cutoff value of 1.6 for fetal hydrops due to CLM. Results A total of 12 studies were included. The QUADAS-2 indicated that the risk of bias was relatively low, and the clinical applicability was relatively high. Statistical analysis was performed on included studies using a random effect model. Meta-analysis showed that the pooled sensitivity, specificity, diagnostic ratio and summary receiver operating characteristic (SROC) for the diagnosis of fetal hydrops by CVR were 0.86 (95% CI, 0.72–0.93; I 2 = 59.84), 0.90 (95% CI, 0.88–0.93; I 2 = 31.94), 58 (95% CI, 22–149; I 2 = 100%), 0.93 (95% CI, 0.91–0.95). Conclusions The sensitivity and specificity of CVR cut-off value 1.6 for the diagnosis of CLM-induced fetal hydrops were high, no publication bias was observed, and the CVR cut-off value 1.6 is meaningful for the early diagnosis prediction of CLM-induced fetal hydrops.

Bronchopulmonary sequestrations (BPSs) are rare congenital anomalies characterized by non-functioning embryonic lung tissue receiving anomalous blood supply. They are most commonly located within the thorax (supradiaphragmatic) or into the abdominal cavity (infradiaphragmatic). Intradiaphragmatic extralobar BPs (IDEPS) are an exceptionally rare finding, representing a diagnostic and operative challenge. We report three cases of IDEPS and their surgical management, describing our experience and approach to such rare clinical entities. From 2016 to 2022, we treated 3 cases of IDEPS. Surgical techniques, histopathological findings and clinical outcomes were retrospectively evaluated for each case and compared. Three different surgical techniques were used to approach each lesion, from open thoracotomy to a combined laparoscopic and thoracoscopic approach. Histopathological analysis of the specimens revealed hybrid pathological features, proper of both congenital pulmonary airway malformation (CPAM) and extralobar pulmonary sequestration. IDEPS represent a surgical challenge for pediatric surgeons, given their complex surgical planning. In our experience, the thoracoscopic approach is safe and feasible when performed by trained surgeons, even though a combined thoracoscopic-laparoscopic approach allows for optimal vessels control. The presence of CPAM elements within the lesions supports their surgical removal. Additional studies are required to better characterize IDEPS and their management.

Background: : Sufficient knowledge of anatomy including normal patterns and aberrant variations is crucial in thoracoscopic lobectomy for congenital lung malformations (CLMs). This study aimed to investigate the prevalence of anatomical variations and their correlations with surgical outcome. Methods: : Video-recordings of thoracoscopic lobectomy performed for CLMs between 2009 and 2021 were reviewed. The incidences of surgically relevant variations, including abnormal branches of pulmonary arteries and veins, aberrant systemic arteries, and incomplete fissures (as classified by Craig) were assessed in comparison with the previously published reports. Moreover, multiple univariate analyses were performed to seek correlations between surgical finding and prolonged operative times and postoperative drainage. Results: : Fifty-six lobectomies were reviewed. Variations of pulmonary arteries were more commonly seen in the left lung (29.6%) compared to right (10.7%). The prevalence of atypical pulmonary venous branches was similar between the right (13.8%) and left (18.5%). The right upper oblique fissure was frequently associated with Grade 2 incomplete fissure (44.8%). Grade 3 incomplete fissure was rare (3.4%) in the right lower oblique fissure but relatively common (14.3 – 18.5%) in other locations. Grade 4 incomplete fissure was limited to the right horizontal fissure (1.7%). Multiple univariate analyses demonstrated a significant correlation between any degree of incomplete fissure and prolonged operative time (p < 0.05), whereas no factor was identified to affect need for postoperative drainage. Conclusions: : The incidence of anatomical variations in CLM patients was similar to that reported in adults. Review of intra-operative video footage was useful to closely evaluate and understand anatomical variations.

Introduction Opinion remains divided on whether to resect an asymptomatic congenital lung malformation (CLM) and on optimal timing of resection. This study aimed to determine if age at resection of CLM correlates with the presence of histological inflammation and/or incidence of prior antibiotic administration for lower respiratory tract infection (LRTI). Materials and Methods A retrospective review of all CLMs resected between 2009 and 2021 was carried out. Data on antenatal detection, incidence of preoperative antibiotic use for LRTI, operative details, and histological reports were analyzed. Fisher's exact test and logistic regression were used to look for correlation between age at resection and (1) histological inflammation and/or (2) preoperative LRTI. Results A total of 102 patients underwent resection at age 14 months (interquartile range: 6–23). Eighty percent of children were asymptomatic in the neonatal period and 22% of these went on to develop a respiratory symptom. In total, 59% of specimens had histological evidence of inflammation, with a significantly higher rate of inflammation after 10 months of age (71 vs. 35%; p = 0.0012). Logistic regression showed there was a positive correlation between age at resection and treatment for previous LRTI (p = 0.020). Conclusion Detection rates of inflammation in specimens resected after 10 months of age are double the rates of those resected prior to 10 months. Delaying resection of CLMs showed a higher frequency of treatment of LRTI. Earlier resection may therefore be advantageous for centers pursuing a resection strategy for asymptomatic lesions. [ABSTRACT FROM AUTHOR]

Introduction:  Prenatal ultrasound-guided laser coagulation (USLC) for complicated bronchopulmonary sequestrations has been described but a consensus on the procedure and on the following management is still lacking. We present our experience and provide a literature review.Methods:  Retrospective review of patients treated in our center. Literature review and combined analysis of perinatal data were performed.Results:  Five cases were treated at our center, all presenting with severe hydrothorax. Four met the criteria for fetal hydrops. Four cases underwent postnatal computed tomography (CT) scan: in one case, there was no evidence of persistent bronchopulmonary sequestration. The other three underwent thoracoscopic resection, in two, a viable sequestration was found. Including our series, 57 cases have been reported, with no mortality and a success rate of 94.7%. Mean gestational age (GA) at the procedure was 28 ± 3.4 weeks and mean GA at birth and birth weight (BW) were 38.6 ± 2.3 weeks and 3,276 ± 519.8 g, respectively. In 80.6% of the cases investigated postnatally, a residual mass was found, 50% of cases who showed prenatal arterial flow cessation had a persistent sequestration postnatally, and 26.3% of cases underwent postnatal sequestrectomy. Both patients in our series had pathology examination confirming a viable bronchopulmonary sequestration.Conclusion:  Prenatal USLC seems to be a valid option for bronchopulmonary sequestration complicated by severe hydrothorax and/or fetal hydrops. Authors believe that this procedure should aim to reverse fetal distress and allow pregnancy continuation, and it should not be considered a definitive treatment. The currently available data do not support changes of the common postnatal management. [ABSTRACT FROM AUTHOR]

Background: Thoracoscopic lobectomy is a challenging procedure in children with congenital lung malformations (CLMs). This study aims to evaluate the safety and efficacy of thoracoscopic clockwise lobectomy (TCL) in CLMs in children and its potential to be a stylized procedure. Methods: All patients with CLMs who received TCL from 2015 to 2019 in our hospital were retrospectively reviewed. Clinical information was extracted from medical records, including patient demographics, operative details, and outcomes. Results: A total of 184 patients with a median age of 6.8 months (range, 3–156) and a median weight of 9 kg (range, 6–45) received TCL. Lesions were all located in the lower lobe and included congenital pulmonary airway malformation (n = 133), intralobar sequestration (n = 44), bronchiectasis (n = 4), and congenital lobar emphysema (n = 3). The mean (±standard deviation [SD]) operating time was 46 ± 7.5 minutes (range, 35–113). The mean (±SD) blood loss was 3.5 ± 0.8 mL (range, 1–60). Three patients converted to thoracotomy, and 162 patients did not have a chest tube placed. The postoperative course was uneventful in all patients except 2 patients who developed air leaks and 23 patients who developed a mild fever. The median length of postoperative hospital stay was 2 days. A total of 163 patients were followed up for more than 1 year without any complications. Conclusion: TCL is suitable for lower lobectomy and is safe and effective in standard and complicated thoracoscopic lobectomy. It could be recommended as a stylized procedure in treating children with CLMs. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to determine the incidence of complications after thoracoscopic lobectomy in children according to a verified classification. Secondary aim was to evaluate the modifications made in our management protocol in order to decrease the rate of complications., Methods: Retrospective study of pediatric patients in whom a lobectomy was performed for congenital lung malformation (CLM) in our institution between 2003 and 2021. The following data were collected: demographics, preoperative clinical symptoms, surgical technical details, and postoperative complications following the Clavien-Dindo (CD) classification. Main outcome measure was the presence of complications in the first month after lung lobectomy., Results: 90 lobectomies were performed in 89 patients. There were 46 girls (51.6%) and 43 boys with a median age of 12.4 months. Most patients (86.6%) remained asymptomatic until the surgical procedure. Postsurgical complications were detected in 26 cases (28.8%) and distributed into their respective category according to CD classification. In our experience, closure of the bronchial stump with interrupted suture or placement of an endoloop showed a statistically significant association with complications (p = 0.022 and p = 0.006 respectively). Moreover, patients in which a device combining sealing and section was used showed significantly fewer complications (p = 0.006)., Conclusions: Thoracoscopic lobectomy continues to be a challenging procedure. The CD grading system for postoperative complications has proved to be useful in this setting. Evolving surgical strategies and new miniaturized endosurgical devices have enabled a safer and quicker procedure with a positive impact in the development of complications., Level of Evidence: III., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Pediatric thoracoscopic lobectomy is a technically challenging procedure that may result in better pain control, better cosmetic results, and shorter hospital stay. However, data describing the learning curve of total thoracoscopic lobectomy (TTL) have yet to be obtained. To evaluate our learning curve for TTL in children, we reviewed the safety and efficiency of our initial experiences with TTL in pediatric patients with congenital lung malformation. This was a retrospective study of all pediatric patients undergoing TTL between March 2011 and January 2017. Cumulative summation (CUSUM) analysis of operative time (OT) was used. One hundred patients were retrospectively analyzed and chronologically divided into three phases: the ascending (A), plateau (B), and descending (C) phases of CUSUM of OT. Phases A, B, and C comprised 35, 22, and 43 cases, respectively. OT decreased significantly from phases A to B (P = 0.035) and B to C (P = 0.019). Age and weight of patients both reduced significantly from phase A to B (p = 0.017 and p = 0.012, respectively), while the two measures did not vary from phase B to C (p = 0.987 and p = 0.874, respectively). Chest tube duration and length of hospital stay had similar trend. All complications occurred in five cases in phase A (5/35). Six cases were converted to open surgery (6%). Four conversions occurred within phase A and two in phase C (4/35 vs 2/43, p = 0.490). There were no mortalities. Repeated standardized training plays a role in overcoming the learning curve for thoracoscopic lobectomy in children, and CUSUM OT indicates that a learning curve of approximately 57 cases is required in our institute. [ABSTRACT FROM AUTHOR]

Objective: Meta-analysis was used to evaluate the diagnostic value of a CVR cut-off value of 1.6 for fetal hydrops due to congenital lung malformation (CLM).Methods: A systematic search of PubMed, Embase, Web of Science, CNKI, VIP, and Wanfang published before 7/30/2021 for the value of a congenital pulmonary airway malformation volume ratio (CVR) cut-off value of 1.6 for the diagnosis of fetal hydrops. According to the inclusion and exclusion criteria, the literature that met the requirements were obtained. A total of 75 articles were retrieved, and 12 articles were included for further analysis. The quality of these studies was evaluated according to the Quality Assessment for Studies of Diagnostic Accuracy tool (QUADAS-2) criteria. The Q test and heterogeneity I2 were used to evaluate the heterogeneity due to non-threshold effects, and Stata 15.0 was used for statistical analysis to evaluate the diagnostic value of the CVR cutoff value of 1.6 for fetal hydrops due to CLM.Results: A total of 12 studies were included. The QUADAS-2 indicated that the risk of bias was relatively low, and the clinical applicability was relatively high. Statistical analysis was performed on included studies using a random effect model. Meta-analysis showed that the pooled sensitivity, specificity, diagnostic ratio and summary receiver operating characteristic (SROC) for the diagnosis of fetal hydrops by CVR were 0.86 (95% CI, 0.72-0.93; I2 = 59.84), 0.90 (95% CI, 0.88-0.93; I2 = 31.94), 58 (95% CI, 22-149; I2 = 100%), 0.93 (95% CI, 0.91-0.95).Conclusions: The sensitivity and specificity of CVR cut-off value 1.6 for the diagnosis of CLM-induced fetal hydrops were high, no publication bias was observed, and the CVR cut-off value 1.6 is meaningful for the early diagnosis prediction of CLM-induced fetal hydrops. [ABSTRACT FROM AUTHOR]

Purpose: Peripheral bronchial atresia is a pulmonary abnormality diagnosed on postnatal computed tomography after prenatal imaging reveals a congenital lung lesion. Debate regarding management of this abnormality prompted us to review our institution's practice patterns and outcomes. Methods: All patients diagnosed with bronchial atresia were assessed from 6/2014 to 7/2020. Pediatric radiologists were surveyed to delineate computed tomography criteria used to diagnose peripheral bronchial atresia. Criteria were applied in an independent blinded review of postnatal imaging. Data for patients determined to have peripheral bronchial atresia and at least an initial pediatric surgical evaluation were analyzed. Results: Twenty-eight patients with bronchial atresia received at least an initial pediatric surgical evaluation. Expectant management was planned for 22/28 (79%) patients. Two patients transitioned from an expectant management strategy to an operative strategy for recurrent respiratory infections; final pathology revealed bronchial atresia in both. Six patients were initially managed operatively; final pathology revealed bronchial atresia (n = 3) or congenital lobar overinflation (n = 3). Conclusions: Peripheral bronchial atresia can be safely managed expectantly. A change in symptoms is suspicious for alternate lung pathology, warranting further workup and consideration for resection. Level of evidence: Level IV. [ABSTRACT FROM AUTHOR]

To the best of our knowledge, double or multiple extralobar pulmonary sequestrations (PSs) with anomalous arterial supply in the ipsilateral thoracic cavity have rarely been reported before. PS can be divided into two types: intralobar sequestration (ILS) and extralobar sequestration (ELS). We encountered a 5-month-old infant with double ELS in the left thoracic cavity that was incidentally detected during thoracoscopic surgery. Surgical exploration revealed two separate, well-circumscribed abnormal masses in the left thoracic cavity, and the patient was successfully treated using thoracoscopic surgery. Postoperative pathology confirmed that both masses were PS tissues. Accurate preoperative diagnosis using CT alone may be inadequate in this type of case. Therefore, thoracoscopy may be more suitable for diagnosing and treating unusual ELS.

Abstract Background Congenital lung malformation is an umbrella term and consist of various kind of parenchymal and mediastinal pathologies. Surgical resection is often required for diagnosis and curative treatment. We aimed to review our experience in surgical treatment for congenital lung disease and present the role of minimally invasive surgery. Methods Surgical resections performed for benign lesions of the lung and mediastinum between January 2009 and May 2019 were retrospectively analyzed. Patients who were found to have congenital lung malformation as a result of pathological examination were included in our study. Distribution characteristics of the patients according to congenital lung malformation subtypes, differences in surgical approach and postoperative results were investigated. Results A total of 94 patients who underwent surgical resection and were diagnosed with the bronchogenic cyst, sequestration, bronchial atresia, congenital cystic adenomatoid malformation (CCAM), or enteric cyst as a result of pathological examination were included the study. There were no significant differences between pathological subtypes in the postoperative length of hospital stay and drainage duration however, perioperative complication rate was higher in the sequestration group. In addition, in the first three days postoperatively, the mean pain score was found to be lower in the VATS group compared to thoracotomy. Conclusions Congenital lung malformations consist of a heterogeneous group of diseases and the surgical treatment in these patients can range from a simple cyst excision to pneumonectomy. Video-assisted thoracoscopic surgery should be considered as the first choice in the surgical treatment of these patients in experienced centers.

The benefit of elective resection of congenital lung malformations continues to be debated. Proponents of resection endorse a decreased risk of respiratory complications as one indication for surgery. Our study aimed to compare the prevalence of respiratory infections in cases, before and after resection of congenital lung malformations, to controls without a history of congenital lung malformation. We performed a retrospective cohort study of children born from 1991 to 2007 who underwent congenital lung malformation resection. Patients were identified from Winnipeg´s Surgical Database of Outcomes and Management (WiSDOM), and a 10:1 date-of-birth matched control group was generated from a population-based administrative data repository. International Classification of Disease codes were used to assess pulmonary infection outcomes. Relative rates (RR) were calculated to compare the frequency of pneumonia, respiratory infections and influenza between cases and controls. We included 31 congenital lung malformation cases and 310 controls. Cases consisted of 14 (45.16%) congenital pulmonary airway malformations, 9 (29.03%) bronchopulmonary sequestrations and 8 (25.81%) hybrid lesions. Before resection , pneumonia was more common in cases than controls (RR 6.85; 95%CI 3.89, 11.9), while the risk of acute respiratory infections (RR 1.21; 95%CI 0.79, 1.79) and influenza (RR 0.46; 95%CI 0.01, 3.22) were similar to controls. Post-resection , the risk of pneumonia (RR 9.75; 5.06, 18.50) was still higher in cases than controls , and respiratory infections (RR 1.77; 95%CI 1.20, 2.53) and influenza (RR 3.98; 95%CI 1.48, 9.36) were more common in cases than controls. Our study demonstrated that after resection of congenital lung malformations, children experience more frequent respiratory infections compared to the general population. Resection does not eliminate the increased risk of pneumonia. [ABSTRACT FROM AUTHOR]

Context: Congenital lung malformation (CLM) is a rare developmental anomaly of the lower respiratory tract. The purposes are to define if the presence of respiratory symptoms, in CLM may affect surgical outcomes and to define optimal timing for surgery in asymptomatic patients. Settings and Design: Retrospective review of patients with CLM from 2004 to 2018. Asymptomatic and symptomatic patients were compared. Moreover, asymptomatic patients were stratified according to age at surgery (< or >6 months). Subjects and Methods: Demographic data, prenatal diagnosis, symptoms, CLM's characteristics, operative and postoperative data were collected. Patients were divided into two groups based on the presence or none of respiratory symptoms. Statistical Analysis: Data were compared using the Fisher's exact test for qualitative values and Mann-Whitney test for quantitative values P < 0.05 was statistically significant. Results: One hundred and eighty-six patients were treated. Asymptomatic were 137 (74%), while symptomatic were 49 (26%). The most common presenting symptoms were respiratory distress (n = 30, 61%) followed by pneumonia (n = 18, 38%). Prenatal diagnosis of CLM was performed in 98% of asymptomatic patients compared to 30% of symptomatic (P = 0.001). Surgical excision was performed in all cases, and in 50% by thoracoscopy, without difference between the two groups. In 97% of all cases, a lung sparing surgery was performed without difference between the groups. Symptoms are significantly associated with older age, location in the upper lobe, and lobar emphysema. Length of stay in intensive care, postoperative complications, and reintervention rate were higher in the symptomatic group. Eighty-one asymptomatic patients underwent surgery 6 months (7%). Conclusions: The present study describes a comprehensive picture of CLM. In addition, we emphasize the role of early postnatal management and thoracoscopic surgery, also before 6 months of life, to prevent the onset of symptoms that are associated with worse outcomes.

Aim: To evaluate the impact of previous infection on perioperative outcomes in patients undergoing thoracoscopic lobectomy for congenital lung anomalies. Methods: This was a single-institution retrospective observational study for which patients who underwent thoracoscopic lobectomy for congenital lung disease between 2009 and 2021 were enrolled, and patients with extralobar sequestration were excluded. Patient background and data related to the surgery were compared between patients who had an infection before surgery (Group 1) and those who did not (Group 2). Results: This study included 34 patients, 13 in Group 1 and 21 in Group 2. The sex-based distribution and pathological diagnosis were similar between the two groups. Malformations were prenatally diagnosed in 1 patient in Group 1 (7.7%) and 18 patients in Group 2 (86%; P < .001). The median age and weight at the time of the procedure and procedure duration were comparable between the two groups. The amount of blood loss was significantly higher in Group 1 (60 mL) than in Group 2 (20 mL; P = .0042). Four patients in Group 2 required reoperation due to air leakage, pyothorax, and cardiac tamponade, whereas none of the Group 1 patients required reoperation (P = .12). No conversion to thoracotomy was required in either group. The duration of postoperative admission was similar between the two groups (Group 1: 6 days versus Group 2: 6 days; P = .14). Conclusions: Preceding infection increased the amount of bleeding during thoracoscopic lobectomy but had little effect on other outcomes. [ABSTRACT FROM AUTHOR]

BACKGROUND: A congenital lung malformation (CLM) that is diagnosed on prenatal ultrasound exam may subsequently become undetectable on later scans, a "vanishing" CLM. OBJECTIVE: The purpose of our study is to characterize the prenatal natural history and postnatal outcomes of "vanishing" lesions treated at our institution. METHODS: We performed a retrospective chart review of 107 patients diagnosed prenatally with CLM at our institution. Comparisons were made using Kruskal-Wallis or t-test for continuous variables and Fisher's exact test or Chi-Square test for categorical variables. Multivariable analysis using logistic regression was performed. RESULTS: Of the 104 patients, 59 (56.7%) had lesions that became sonographically undetectable on serial ultrasound scans. Patients with lesions that vanished prenatally tended to need less Neonatal Intensive Care Unit (NICU) admission at birth (persistent CLM: 54.8%vs vanished CLM: 28.8%), decreased need for supplemental O2 at birth (persistent CLM: 31.0%vs vanished CLM: 11.9%), and decreased delay in feeds (persistent CLM: 26.2%vs vanished CLM: 8.5%) compared to those with persistent CLM. After multivariate analysis controlling for maternal steroid administration and sex, admission to NICU maintained a slight statistical significance, with patients in the vanishing CLM group 2.5 times less likely to be admitted to the NICU. None of our patients whose lesions vanished prenatally required mechanical ventilation. Eighty-six patients underwent postnatal computed tomography (CT) chest. Only 2 patients had lesions that regressed on postnatal CT. CONCLUSION: Lesions that vanish on prenatal imaging may be associated with improved clinical outcomes. The rate of true regression at our institution was as low as 2.3%. [ABSTRACT FROM AUTHOR]

Anomalies of the fetal chest require advanced imaging with ultrasound and MR imaging as well as expertise on the part of the interpreting pediatric radiologist. Congenital diaphragmatic hernia and congenital lung malformation are the most frequently seen, and in both conditions, the radiologist should provide both detailed anatomic description and measurement data for prognostication. This article provides a detailed approach to imaging the anatomy, in-depth explanation of available measurements and prognostic value, and keys to identifying candidates for fetal intervention. Less common congenital lung tumors and mediastinal and chest wall masses are also reviewed., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: To identify, in fetuses with a congenital lung malformation (CLM), prenatal predictors of the need for postnatal respiratory support and the need for surgery by calculating the CLM volume ratio (CVR), and to evaluate the concordance between the prenatal appearance and the postnatal type of CLM.Methods: This was an analysis of prenatal, perinatal and postnatal data from fetuses diagnosed with a CLM at the Erasmus University Medical Center - Sophia Children's Hospital in Rotterdam, The Netherlands, between January 2007 and December 2016. For all included fetuses, CVR was measured retrospectively on stored ultrasound images obtained at 18 + 1 to 24 + 6 weeks (US1), 25 + 0 to 29 + 6 weeks (US2) and/or 30 + 0 to 35 + 6 weeks' gestation (US3). Postnatal diagnosis of CLM was based on computed tomography or histology. Primary outcomes were the need for respiratory support within 24 h and surgery within 2 years after birth.Results: Of the 80 fetuses with a CLM included in this study, 14 (18%) required respiratory support on the first postnatal day, and 17 (21%) required surgery within 2 years. Only the CVR at US2 was predictive of the need for respiratory support, with a cut-off value of 0.39. Four of 16 (25%) fetuses which showed full regression of the CLM prenatally required respiratory support within 24 h after birth. The CVR at US1, US2 and US3 was predictive of surgery within 2 years. Overall, the prenatal appearance of the CLM showed low concordance with the postnatal type. Prenatally suspected microcystic congenital pulmonary airway malformation (CPAM) was shown on computed tomography after birth to be congenital lobar overinflation in 15/35 (43%) cases. Respiratory support within 24 h after birth and surgical resection within 28 days after birth were needed in all cases of macrocystic CPAM.Conclusions: CVR can predict the need for respiratory support within 24 h after birth and for surgery within 2 years. Regression of a CLM prenatally does not rule out respiratory problems after birth. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. - Legal Statement: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. [ABSTRACT FROM AUTHOR]

Congenital lung malformations (CLMs) include a group of different disorders. With widespread use of antenatal ultrasonography (aUS) and increased use of pre-natal magnetic resonance imaging (MRI), CLMs are increasingly detected, nevertheless the best postnatal imaging approach is not yet well defined: newborns usually undergo several chest X-rays and eventually computed tomography to confirm the diagnosis. In this case series, we show lung ultrasound features of three different cases of congenital lung malformations, describing prenatal and postnatal images comparing different imaging techniques. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to compare the outcomes and pulmonary function test (PFT) of thoracoscopic segmentectomy and lobectomy in infants with congenital lung malformation and study the result of PFT on a medium-term basis.Methods: The clinical data of 19 infants with congenital lung malformation who underwent thoracoscopic surgery in our hospital from January 2018 to March 2019 were retrospectively studied; these infants were paired with another 19 infants who underwent thoracoscopic lobectomy during the same period using propensity score matching. Age-matched healthy individuals with similar body sizes were recruited for PFT as the control group. Patient characteristics, postoperative PFT, and outcomes were extracted for statistical analysis.Results: The average length of hospital stay did not significantly differ between segmentectomy and lobectomy groups. The segmentectomy group had more chest tube drainage than the lobectomy group. PFT 1 month after the operation showed that the tidal volume of the lobectomy group was lower than that of the segmentectomy group. Time to peak expiratory flow/time of expiration and peak flow/terminal airway velocity (V25%) indicated small airway dysfunction in the lobectomy group, and no obvious abnormalities were found in “time of inspiratory/time of expiration” in either group. Reexamination of pulmonary function 2 years after the operation showed that the small airway function of the segmentectomy group returned to normal, and no significant difference in pulmonary function was noted among the three groups.Conclusion: The short-term pulmonary function recovery was better after segmentectomy than after lobectomy. Patients who underwent thoracoscopic lobectomy and segmentectomy have normal lung function 2 years after the operation.

Background and ObjectivesThe treatment of asymptomatic patients with congenital pulmonary malformations (CPMs) remains controversial, partially because the relationship between congenital lung malformations and malignancy is still undefined. Change in methylation pattern is a crucial event in human cancer, including lung cancer. We therefore studied all differentially methylated regions (DMRs) in a series of CPMs in an attempt to find methylation anomalies in genes already described in association with malignancy.MethodsThe DNA extracted from resected congenital lung malformations and control lung tissue was screened using Illumina MethylationEPIC arrays. Comparisons between the group of malformed samples or the malformed samples of same histology or each malformed sample and the controls and between a pleuropulmonary blastoma (PPB) and controls were performed. Moreover, each malformed sample was pairwise compared with its respective control. All differentially methylated regions (DMRs) with an adjusted p-value

Background and Objectives: The treatment of asymptomatic patients with congenital pulmonary malformations (CPMs) remains controversial, partially because the relationship between congenital lung malformations and malignancy is still undefined. Change in methylation pattern is a crucial event in human cancer, including lung cancer. We therefore studied all differentially methylated regions (DMRs) in a series of CPMs in an attempt to find methylation anomalies in genes already described in association with malignancy. Methods: The DNA extracted from resected congenital lung malformations and control lung tissue was screened using Illumina MethylationEPIC arrays. Comparisons between the group of malformed samples or the malformed samples of same histology or each malformed sample and the controls and between a pleuropulmonary blastoma (PPB) and controls were performed. Moreover, each malformed sample was pairwise compared with its respective control. All differentially methylated regions (DMRs) with an adjusted p-value <0,05 were studied. Results: Every comparison highlighted a number of DMRs closed to genes involved either in cell proliferation or in embryonic development or included in the Cancer Gene Census. Their abnormal methylation had been already described in lung tumors. Conclusions: Methylation anomalies already described in lung tumors and also shared by the PPB were found in congenital lung malformations, regardless the histology. The presence of methylation abnormalities is suggestive of a correlation between congenital lung malformations and some step of malignant transformation. [ABSTRACT FROM AUTHOR]

Background: Congenital lung malformation is an umbrella term and consist of various kind of parenchymal and mediastinal pathologies. Surgical resection is often required for diagnosis and curative treatment. We aimed to review our experience in surgical treatment for congenital lung disease and present the role of minimally invasive surgery.Methods: Surgical resections performed for benign lesions of the lung and mediastinum between January 2009 and May 2019 were retrospectively analyzed. Patients who were found to have congenital lung malformation as a result of pathological examination were included in our study. Distribution characteristics of the patients according to congenital lung malformation subtypes, differences in surgical approach and postoperative results were investigated.Results: A total of 94 patients who underwent surgical resection and were diagnosed with the bronchogenic cyst, sequestration, bronchial atresia, congenital cystic adenomatoid malformation (CCAM), or enteric cyst as a result of pathological examination were included the study. There were no significant differences between pathological subtypes in the postoperative length of hospital stay and drainage duration however, perioperative complication rate was higher in the sequestration group. In addition, in the first three days postoperatively, the mean pain score was found to be lower in the VATS group compared to thoracotomy.Conclusions: Congenital lung malformations consist of a heterogeneous group of diseases and the surgical treatment in these patients can range from a simple cyst excision to pneumonectomy. Video-assisted thoracoscopic surgery should be considered as the first choice in the surgical treatment of these patients in experienced centers. [ABSTRACT FROM AUTHOR]

Context: Congenital lung malformation (CLM) is a rare developmental anomaly of the lower respiratory tract. The purposes are to define if the presence of respiratory symptoms, in CLM may affect surgical outcomes and to define optimal timing for surgery in asymptomatic patients. Settings and Design: Retrospective review of patients with CLM from 2004 to 2018. Asymptomatic and symptomatic patients were compared. Moreover, asymptomatic patients were stratified according to age at surgery (< or >6 months). Subjects and Methods: Demographic data, prenatal diagnosis, symptoms, CLM's characteristics, operative and postoperative data were collected. Patients were divided into two groups based on the presence or none of respiratory symptoms. Statistical Analysis: Data were compared using the Fisher's exact test for qualitative values and Mann-Whitney test for quantitative values P < 0.05 was statistically significant. Results: One hundred and eighty-six patients were treated. Asymptomatic were 137 (74%), while symptomatic were 49 (26%). The most common presenting symptoms were respiratory distress (n = 30, 61%) followed by pneumonia (n = 18, 38%). Prenatal diagnosis of CLM was performed in 98% of asymptomatic patients compared to 30% of symptomatic (P = 0.001). Surgical excision was performed in all cases, and in 50% by thoracoscopy, without difference between the two groups. In 97% of all cases, a lung sparing surgery was performed without difference between the groups. Symptoms are significantly associated with older age, location in the upper lobe, and lobar emphysema. Length of stay in intensive care, postoperative complications, and reintervention rate were higher in the symptomatic group. Eighty-one asymptomatic patients underwent surgery <6 months of life; they had a lower rate of surgical complications (2%) compared with those >6 months (7%). Conclusions: The present study describes a comprehensive picture of CLM. In addition, we emphasize the role of early postnatal management and thoracoscopic surgery, also before 6 months of life, to prevent the onset of symptoms that are associated with worse outcomes. [ABSTRACT FROM AUTHOR]

Majority of sequestrations fall into two categories: Intra-Lobar (ILS) and Extra-Lobar (ELS). Rarely the abnormal lung could be attached to the gastrointestinal tract, Bronchopulmonary Foregut Malformation (BPFM). We described a case of a girl of 3-years-old with antenatal diagnosis of left intrathoracic mass of the inferior lobe. Postnatal Computed-Tomography (CT) revealed a bilateral ELS with an isthmic bridge crossing the vertebral spine. She follows a MRI follow-up at 18months/30months confirming the lesion. Before surgery, a three-dimensional-CT-angiography was performed to study the mass, its blood supply and to plan surgery. She underwent to thoracoscopic resection. Two aberrant blood vessels were dissected from the thoracic aorta and ligated. The postoperative course was uneventful. She was discharged after 3 days. The rarity of our case is due to the bilateral extension. An appropriate preoperatory imaging study is necessary for the success of surgery while thoracoscopy is particularly appropriate in surgical treatment.

Introduction: Thoracoscopy represents the most challenging area of pediatric minimally invasive surgery due to its technical difficulty. A standardized training program would be advisable. The aim of this study is to evaluate the results of our surgical training.Materials and Methods: A retrospective, single-center, cohort study was performed. The following four-step program was tested: (1) theoretical part; (2) experimental training; (3) training in centers of reference; (4) personal operative experience. Particular attention was focused on the choice of mentor. Times and modality of adherence to the program were evaluated. The effectiveness and safety of the training were evaluated according to the surgical results of esophageal atresia (EA/TEF) repair and resection of congenital lung malformations (CLM). The study was conducted from January 2014 to May 2020. Attending surgeons with previous experience in neonatal and pediatric laparoscopy were selected for the training program after being evaluated by the head of Department.Results: The training program was fully completed in 2 years. Twenty-four lobectomies, 9 sequestrectomies, 2 bronchogenic cyst resections and 20 EA/TEF repair were performed. Thoracoscopy was always feasible and effective, with no conversion. The operative times progressively decreased. Only three minor complications were recorded, all treated conservatively.Conclusions: A standardized training program is highly desirable to learn how to safely perform advanced pediatric thoracoscopy. The 4-steps design seems a valid educational option. The choice of the mentor is crucial. An experience-based profile for pediatric surgeons who may teach thoracoscopy is advisable.

The most common congenital lung malformations are congenital pulmonary airway malformations and pulmonary sequestrations. Many surgeons advocate resection to prevent complications of infection, malignancy, and pneumothorax. The standard of care is lobectomy, but segmentectomy and embolization alone have been reported. These methods avoid the complications of lobectomy but are not widely practiced due to concerns about incomplete resection or involution of the lesion. We present a novel approach to the treatment of a pulmonary sequestration in a 7-month-old male using preoperative embolization followed by a sublobar pulmonary resection. The embolization clearly demarcated the affected lung intraoperatively, thereby facilitating complete removal of the lesion with a segmental lung resection rather than complete lobectomy.