Cystic Adenomatoid Malformation Research Articles

Percutaneous Drainage of Pediatric Pulmonary Abscesses: An Effective Therapy

IntroductionPulmonary abscess is a complication of lung infection with localized necrosis and purulent cavity formation. Pulmonary abscesses are typically managed using antibiotic therapy with anatomic surgical resection reserved as a rescue. Percutaneous drainage is considered relatively contraindicated in some centers due to perceived risk of bronchopleural fistula. However, drain placement has been frequently employed at our institution. The purpose of this study was to review and describe our longitudinal experience. MethodsMedical records of children diagnosed with lung abscess and treated with percutaneous drainage from 2005 through 2023 were reviewed. Patient clinical parameters, follow-up imaging, and clinical outcomes were evaluated. ResultsPercutaneous drainage (n=24) or aspiration alone (n=4) under imaging guidance was performed by interventional radiologists for 28 children with lung abscesses. A single catheter (8–12 Fr) was deployed in the pulmonary abscess cavity and remained for a median of 6 days (IQR: 6-8 days). The median hospital stay was 10 days (IQR: 8.8-14.8 days). The technical success rate for percutaneous drainage or aspiration of primary pulmonary abscesses was 100% (26/26). Two children were later diagnosed with secondarily infected congenital pulmonary airway malformations that were both successfully drained and ultimately surgically resected. The abscess cavities resolved in all patients and catheters were removed upon clinical, radiographic, and laboratory improvement. Complications included the presence of two bronchopleural fistula, both of which were treated with immediate pleural drain placement. ConclusionPercutaneous drainage of pulmonary abscesses is an effective therapeutic option in children and can be considered alongside antibiotics as part of the initial treatment for pulmonary abscesses. Bronchopleural fistula can occur, but at a lower frequency than previously reported. Level of EvidenceLevel V

Surgical Approach to Pulmonary Cystic Adenomatoid Malformations: About an Observation

Congenital cystic adenomatoid malformation (MAKC) is a congenital anomaly of lung development, and is defined as an adenomatoid proliferation of terminal respiratory structures, bronchial and distal pulmonary cystic dilatation, communicating with the bronchial tree and lacking cartilaginous rings, representing approximately 25% of congenital lung lesions, and in 80 to 85% of cases, the diagnosis is made before the age of 2 and is rarely discovered in adulthood. We report 01 case of MAKC diagnosed at the age of 06 months. The diagnosis was based on clinical elements (episode of bronchiolitis with serious signs at the age of 5 months) and radiological (TLT and CT). The lesion was located in the lower lobe of the right lung. The patient underwent a surgical resection such as a lower right lobectomy removing the lesion in one piece. The discovery of a MAKC must be recognized by clinicians. The clinical diagnosis, strongly oriented by the radiological approach, is confirmed by the anatomical pathology, the therapeutic sanction is surgical in the majority of cases. Most authors indicate surgery, especially in early childhood in order to avoid complications, mainly recurrent infections and pneumothorax, and to take advantage of the potential for lung growth.

Comparison of ICG-Guided Near-Infrared Fluorescence Imaging and Modified Inflation-Deflation Method in Identifying the Intersegmental Plane During Lung Segmentectomy of Infants

BackgroundThe identification of the intersegmental plane (ISP) is a crucial step in segmentectomy for children with congenital pulmonary airway malformation (CPAM) due to complex anatomical variations. However, there is very limited literature available on this aspect specifically for infant. In this study, we compared the intravenous indocyanine green (ICG)-guided near-infrared fluorescence (NIRF) imaging method with the modified inflation-deflation method in terms of their perioperative characteristics and summarized our experience. MethodsFrom June 2021 to November 2022, the data of 83 patients with CPAM who underwent segmentectomy by video-assisted thoracoscopic surgery were retrospectively reviewed. Twenty-eight patients underwent ICG-guided NIRF method, and 56 patients underwent the modified inflation-deflation method, characteristics and clinical outcomes were compared. ResultsThe median age of the patients was 4.99 months (4.99 ± 1.51) with a mean body weight of 7.54 kg (7.54 ± 1.99). Both methods could accurately identify the ISP. The time taken to clearly display the ISP was shorter in ICG group than in the modified inflation-deflation group (0.18 ± 0.08 vs. 6.49 ± 1.67 min; P < 0.001), and the surgical duration (61.32 ± 14.28 vs. 88.18 ± 8.03 min; P < 0.001) were significantly shorter in the ICG group too. The two groups exhibited differences in the length of chest tube drainage (1.75 ± 1.24 vs. 2.36 ± 1.54 days; P = 0.072) and the length of hospital stay (4.61 ± 1.75 vs. 5.20 ± 3.07 days; P = 0.078), however, the differences were not statistically significant. There were no significant differences between the two groups in the blood lost and postoperative complications. At a follow-up of more than 1 year after operation, all patients had recovered well without recurrence. ConclusionsAccording to our experience, the ICG-guided NIRF method was safe and feasible for infants during thoracoscopic segmentectomy, it can quickly display the ISP and shorten the surgical duration compared with the modified inflation-deflation method.

Anaesthesia Management of Child with Congenital Cystic Adenomatoid Malformation Posted for Lobectomy

Congenital cystic adenomatoid malformation (CCAM) is a benign lung lesion that appears before birth as a cyst or mass in the chest. It is made up of abnormal lung tissue that does not function properly, but continues to grow. These lesions compress the adjacent normal lung tissues. CCAMs occur sporadically, and there is no genetic predisposition. They are usually unilobed and unilateral, with arterial supply and venous drainage from the pulmonary circulation. The postnatal management of symptomatic CCAM varies on whether the patient has respiratory distress or is asymptomatic. Symptomatic patients require a lobectomy or pneumonectomy. Perioperative anaesthetic management is challenging because it involves thoracotomy in a young patient that may lead to hemodynamic compromise and inadequate ventilation.

A Congenital Pulmonary Airway Malformation (CPAM) Stocker’s Type III

Congenital pulmonary airway malformation (CPAM) is a rare congenital abnormality that can manifest from prenatal period to childhood or uncommonly to adulthood. The diagnosis can be suspected from prenatal ultrasound, postnatal CT or MR imaging, however the mainstay of diagnosis is from histopathology. The rarest type of CPAM along with a poorer prognosis is Stocker’s type III, as it usually involves the entire lobe of the affected lung. We present a case of an infant with type III CPAM, which has difficulty in establishing the diagnosis initially, but later was managed appropriately and showed clinical improvement afterward.

Near-Infrared Fluorescence Imaging with Intravenous Indocyanine Green Method in Segmentectomy for Infants with Congenital Pulmonary Airway Malformation.

Video-assisted thoracoscopic surgery is a commonly used procedure for treating congenital pulmonary airway malformation (CPAM) in infants, particularly when performing segmentectomy for segmental lesions. An innovative technique employing near-infrared fluorescence (NIRF) imaging with intravenous indocyanine green (ICG) has been utilized to delineate the intersegmental demarcation during surgery. However, no previous reports have investigated this method's application, specifically in infants. The primary aim of this study was to assess the safety and efficacy of the NIRF imaging with ICG approach in this context. Between January 2021 and April 2022, a total of 19 consecutive segmentectomies were conducted using the NIRF imaging with ICG method to precisely identify the intersegmental plane. The results were concurrently compared with those obtained using the modified inflation-deflation technique. Comprehensive imaging and clinical data were gathered and analyzed to assess the safety and accuracy of the NIRF imaging with ICG approach. The study involved infants with a median age of 5.12 months (mean body weight of 8.08 g). All segmentectomies were performed successfully without any ICG-related complications. The mean operating time for the surgeries was 88.47 ± 7.94 minutes. Notably, no intraoperative conversions or significant complications were observed in any of the patients. The average hospital stay after surgery was 4.0 ± 0.82 days. During the follow-up period, extending beyond 1-year of postoperation, all patients exhibited excellent recovery with no cases of recurrence. Based on our experience, the NIRF imaging with intravenous ICG method proved to be both safe and effective when performing segmentectomy for infants with CPAM. Low doses of ICG did not hinder the accurate identification of the intersegmental plane.

The Diagnostics and Management of Bronchopulmonary Sequestration: An International Survey among Specialized Caregivers.

Our objective was to explore the treatment preferences for bronchopulmonary sequestration (BPS) among an international group of specialized caregivers. Sixty-three participants from 17 countries completed an online survey concerning the diagnostics, treatment, and follow-up. Recruitment took place among members of the Collaborative Neonatal Network for the first European Congenital Pulmonary Airway Malformation Trial Consortium and through the Association for European Pediatric and Congenital Cardiology working group database. Most of the 63 participants were pediatric surgeons (52%), followed by pediatric pulmonologists (22%), and pediatric cardiologists (19%). The majority (65%) treated more than five cases per year and 52% standardly discussed treatment in a multidisciplinary team. Half of the participants (52%) based the management on the presence of symptoms, versus 32% on the intralobar or extralobar lesion localization. Centers with both surgical and interventional cardiac/radiological facilities (85%) preferred resection to embolization in symptomatic cases (62 vs. 15%). In asymptomatic cases too, resection was preferred over embolization (38 vs. 9%); 32% preferred noninterventional treatment, while 11% varied in preference. These treatment preferences were significantly different between surgeons and nonsurgeons (p < 0.05). Little agreement was observed in the preferred timing of intervention as also for the duration of follow-up. This survey demonstrates a variation in management strategies of BPS, reflecting different specialist expertise. Most centers treat only a handful of cases per year and follow-up is not standardized. Therefore, management discussion within a multidisciplinary team is recommended. Recording patient data in an international registry for the comparison of management strategies and outcomes could support the development of future guidelines. Level IV.

Congenital pulmonary airway malformation with spontaneous regression in an extremely preterm baby from Sri Lanka

No abstract avialable

Lung Function Evaluated By Structured Light Plethysmography in Children After Lung Surgery: A Preliminary Analysis.

Background: Structured light plethysmography (SLP) is a novel light-based method that captures chest wall movements to evaluate tidal breathing. Methods: Thirty-two children who underwent lung surgery were enrolled. Their clinical history was collected along with spirometry and SLP. Results: Median age of surgery was 9 months (interquartile range 4-30). Most frequent diagnosis was congenital pulmonary airway malformation (14/32), then pulmonary sequestration (9/32), tumor (5/32), and bronchogenic cyst (4/32). The most frequent surgical approach was lobectomy (59%), segmentectomy (38%), and complete resection (3%). More than 80% had surgery when younger than 3 years of age. Eight patients had short-term complications (pleural effusion was the most frequent), while long-term effects were reported in 15 patients (19% recurrent cough, 13% thoracic deformities, 13% airway infections, 9% wheezing, 6% reduced exercise tolerance, and 3% columnar deformities). Spirometry was normal in 9/22 patients. Nine patients had a restrictive pattern, while 4 showed a mild bronco-reactivity. Ten patients did not perform spirometry because of young age. SLP revealed the presence of obstructive pattern in 10% of patients (IE50 > 1.88) and showed a significant difference between the two hemithorax in 29% of patients. Discussion: SLP may be a new method to evaluate lung function, without collaboration and radiation exposure, in children who underwent lung resection, also in preschool age.

The underlying molecular mechanism of ciliated epithelium dysfunction and TGF-β signaling in children with congenital pulmonary airway malformations

The aim of this study was to investigate the variation in gene expression in the complete transcripts of Congenitalpulmonary airwaymalformation (CPAM) of the lung using Next Generation Sequencing (NGS) technology. There were 20 cases involving children with CPAM were used for selection of study sample. NGS was used to establish RNA-Seq libraries for the two groups of samples separately, and both groups were conducted to differential expression analysis and Gene Ontology (GO) functional enrichment analysis. The pathways of the differential genes were analyzed to find the enriched target pathways. A total of 592 genes were expressed with significant differences (CPAM vs. normal tissue, P < 0.05). GO functional analysis of DEGs indicated that abnormal ciliary function played a role in the development of CPAM. Subsequently, analysis of these genes pathways showed the TGF-β signaling pathway was significantly enriched. Finally, the results of immunohistochemical analysis of some DEGs showed that a significant reduction in the expression of SMAD6, a gene related to the TGF-β signaling pathway, led to abnormal activation of the pathway. TGF-β signaling pathway involved in the evolution of the disease obtained by DEGs enrichment pathway analysis. SMAD6, a gene involved in this pathway, might be a potential biomarker for the diagnosis and treatment of CPAM.

[Clinicopathological and genetic characteristics of congenital cystic adenomatoid malformation of lung and its associated lung cancer in adults].

Objective: To investigate the clinicopathological features and genetic characteristics of congenital cystic adenomatoid malformation (CCAM) of lung and CCAM associated lung cancer in adults. Methods: A total of 13 cases of CCAM of lung in adults, diagnosed from June 2015 to May 2023, were collected from the Department of Pathology, Shanghai Pulmonary Hospital, Tongji University School of Medicine, China. Their histopathological features were correlated with probable development into lung cancer. Next-generation sequencing was performed on the benign and malignant areas of all cases. Results: The pathological classification of all cases were of CCAM of lung type 1. There were 4 male and 9 female cases, age ranged from 18 to 65 years, with a mean age of 41 years. Six cases were accompanied by lung cancer, all of them were mucinous adenocarcinoma. Next-generation sequencing showed no gene mutation in 2 of the 13 cases; KRAS mutations in exon 2 were detected in 7 cases, in which there were 6 cases complicated with lung mucinous adenocarcinoma and no matter in the malignant or benign regions, the same case exhibited the same mutation sites in KRAS gene. Conclusions: CCAM of the lung is a congenital disease, and in adults, type 1 is most commonly found in the pathological classification, and it is often accompanied by cancer. Gene mutations are frequently detected in CCAM of the lung, KRAS being the most recurrent mutation which may play an important role in the carcinogenesis.

Assessment of mediastinal shift angles in congenital pulmonary airway malformation: a new fetal magnetic resonance imaging indicator of congenital lung disease.

The mediastinal shift angle is a new fetal magnetic resonance imaging (MRI) index that is reportedly correlated with postnatal survival in fetuses with congenital diaphragmatic hernia. However, its correlation in patients with congenital pulmonary airway malformation (CPAM) has not been assessed. This study aimed to establish a normal range for the right/left mediastinal shift angles, to evaluate the mediastinal shift angle in fetuses with CPAM, to compare the mediastinal shift angle with the CPAM volume ratio, and to evaluate the predictive value of the mediastinal shift angle measurements. To establish the normal range, we measured the mediastinal shift angle bilaterally in 124 fetuses without any lung abnormality (the control group). Subsequently, the mediastinal shift angle was measured in 32 fetuses pathologically diagnosed with CPAM. Moreover, the mediastinal shift angle and CPAM volume ratio were compared using fetal MRI. The mean values for the right/left mediastinal shift angles were 18.6°/26.3° and 39.2°/35.9° for control fetuses and fetuses with CPAM, respectively. The mediastinal shift angle and the CPAM volume ratio showed a positive statistical correlation. The area under the curve demonstrated high discriminatory accuracy for the mediastinal shift angle (0.76). The mediastinal shift angle has potential to replace the CPAM volume ratio for evaluating the severity of CPAM infetal MRI.

A radiolucent cystic lesion in a chest radiograph of a breathless child − a case report

A radiolucent cystic lesion is a common finding on chest radiographs of children presenting with acute breathlessness, and congenital pulmonary airway malformation (CPAM) is a relatively uncommon cause of it. The majority of children with CPAM present with respiratory distress of varying severity in the neonatal period. Here, we discuss the case of an 18-month-old child who had a recent history of worsening breathlessness, and on thorough evaluation, he was diagnosed as having CPAM (type 1 variant). Inadvertent chest tube insertions should be avoided in these cases, as it can worsen the underlying condition. A computerized tomography (CT) chest scan plays a key role in diagnosing and assessing the therapeutic intervention in these cases. Surgical resection of the lesion is curative and has an excellent prognosis in the majority of cases of type 1 CPAM.

Prognosis of infants with congenital pulmonary airway malformations after surgery: a short and mid-term evaluation

Prognosis of infants with congenital pulmonary airway malformations after surgery: a short and mid-term evaluation

754 Ultrasound and Clinical Characteristics of Congenital Pulmonary Airway Malformations (CPAM) and Neonatal Care Needs

754 Ultrasound and Clinical Characteristics of Congenital Pulmonary Airway Malformations (CPAM) and Neonatal Care Needs

510 Congenital pulmonary airway malformations and bronchopulmonary sequestrations: features of prenatal evaluation that predict postnatal surgery

510 Congenital pulmonary airway malformations and bronchopulmonary sequestrations: features of prenatal evaluation that predict postnatal surgery

A Rare Combination of Multisystem Congenital Anomalies; Interrupted Aortic Arch, Congenital Cystic Adenomatoid Malformation of the Lungs and Horseshoe Kidneys, A Case Report

Combinations of multisystem congenital anomalies have been frequently reported. However, this article presents a rare combination of congenital anomalies affecting the cardiovascular system, lungs, and kidneys in a neonate, which was detected through a computed tomography exam. The authors conducted a comprehensive search of the English literature and found no previous reports of such a combination in online literature.

Spontaneous Resolution of Congenital Pulmonary Airway Malformation (CPAM) Type 1 in an 8-Month-Old Female from Ethiopia: A Rare Case Report

Spontaneous Resolution of Congenital Pulmonary Airway Malformation (CPAM) Type 1 in an 8-Month-Old Female from Ethiopia: A Rare Case Report

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

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

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.