Congenital Pulmonary Malformation Research Articles

Anesthetic management of pulmonary surgery in newborns and infants.

The main congenital pulmonary airways malformations in newborns and infants requiring surgery are cystic adenoid malformation, congenital lobar emphysema and bronchogenic cyst. The surgical treatment preferably via thoracoscopy is recommended within the first year of life to avoid the risk of pneumopathy. A monopulmonary ventilation is then required by the surgeon to operate the diseased lung. The anesthetic management of intraoperative mono-pulmonary ventilation in newborns and infants is always challenging for the anesthesiologist. The main objective of this study was to describe anesthetic protocol for thoracoscopy and variations of monitored parameters during a mono-pulmonary ventilation procedure in newborns and infants.

What Do You Know About the Imaging Findings in Congenital Pulmonary Malformations in Children?

What Do You Know About the Imaging Findings in Congenital Pulmonary Malformations in Children?

Late diagnosis of complex congenital pulmonary malformation: From symptoms and radiology to histopathology

Late diagnosis of complex congenital pulmonary malformation: From symptoms and radiology to histopathology

Intralobar Pulmonary Sequestration with 3 Aberrant Arteries in a 2-Year-Old Boy

Pulmonary sequestration is a rare congenital pulmonary malformation. This malformation consists of dysplastic lung tissue with no connection to the tracheobronchial tree and with an anomalous systemic arterial supply. The clinical hallmarks of intralobar sequestration are recurrent pulmonary infections. Chest CT scan with contrast is actually gold standard for identifying pulmonary sequestration. CT volume-rendering technique image reconstructions can be particularly helpful in detecting anomalous arterial vessels and show the venous drainage. Surgical resection is the treatment of choice and intralobar sequestration often requires lobectomy. We present an interesting case of intralobar pulmonary sequestration with 3 aberrant arteries arising from the descending thoracic aorta in a 2-year-old boy.

Malformación congénita de la vía aérea pulmonar

La malformación congénita de la vía aérea pulmonar (MCVAP), antiguamente conocida como malformación adenomatoide quística congénita del pulmon (MAQP), pertenece al grupo de las malformaciones congénitas pulmonares, representando al 95% de estas. Se caracteriza por distintos grados de distress respiratorio según el nivel de la vía aérea que comprometa la lesión pudiendo ser desde incompatible con la vida hasta pasar inadvertida hasta adultez temprana. Los adelantos de la biología molecular han descubierto que existe un disbalance entre los genes proliferadores y apoptoticos dentro de estas lesiones interrumpiendo la segmentación del árbol bronquial desencadenando la formación de estas lesiones. Por su lado los avances en la ecografía fetal han permitido que se detecten el 50% de los casos desde la vida fetal, logrando que a estos niños se les extirpe la lesión durante las primeras semanas de vida y su pronóstico de vida mejore. La presente revisión literaria, busca actualizar conceptos sobre los últimos descubrimientos en la fisiopatología, detección y manejo de la MCVAP.

Preoperative delineation of pulmonary fissural anatomy at multi-detector computed tomography in children with congenital pulmonary malformations and impact on surgical complications and postoperative course.

Delineation of the anatomy and integrity of the pulmonary fissures at CT is important because anomalous or incomplete fissures might increase the risk of surgery and of postoperative complications. To preoperatively evaluate the integrity of the pleural fissures in children with congenital lung malformations and determine whether anomalous fissural anatomy is a risk factor for a more complicated surgery and postoperative course. We reviewed preoperative multi-detector CT scans of consecutive children who underwent open or thoracoscopic resection of a congenital pulmonary malformation from 2008 to 2018, to determine the integrity of the fissural anatomy, and compared these findings with the surgical report. We correlated postoperative factors including operating room time, days in hospital and chest tube with the operating room documented fissural integrity. We saw a significant association between the radiologically determined fissural integrity at CT and the operative findings independently for the right, left and both lungs combined (P<0.001). The sensitivity of CT to determine fissural integrity was 76.9%, specificity 95.2%, positive predictive value 95.2%, negative predictive value 76.9%, and accuracy 85.1%. There was a statistically significant association between size of the pulmonary malformation and the integrity of the fissure(s) (P=0.024). Larger lesions also resulted in a significantly longer hospitalization (P=0.024). Chest CT showed high accuracy for delineating fissural anatomy in children with congenital pulmonary malformations, with a good interobserver correlation. Incomplete lung fissures were found more often in children with larger congenital pulmonary malformations. In addition, larger lesions were associated with longer hospital stays. Therefore, children with incomplete fissures may have a longer postoperative course. Analysis of the fissural anatomy should be included in the CT report.

Ultra‐low‐dose chest computed tomography without anesthesia in the assessment of pediatric pulmonary diseases

ObjectiveTo evaluate the feasibility of using ultra‐low‐dose computed tomography of the chest with iterative reconstruction without anesthesia for assessment of pulmonary diseases in children. MethodsThis prospective study enrolled 86 consecutive pediatric patients (ranging from 1 month to 18 years) that underwent ultra‐low‐dose computed tomography due to suspicion of pulmonary diseases, without anesthesia and contrast. Parameters used were: 80kVp; 15–30mA; acquisition time, 0.5s; and pitch, 1.375. The adaptive statistical iterative reconstruction technique was used. Subjective visual evaluation and quantitative assessment of image quality were done using a 5‐point scale in 12 different structures of the chest. ResultsMean age was 66 months (interquartile range, 16–147). Final diagnosis was performed in all exams, and 44 (51.2%) were diagnosed with cystic fibrosis, 27 (31.4%) with bronchiolitis obliterans, and 15 (17.4%) with congenital pulmonary airways malformations. Diagnostic quality was achieved in 98.9%, of which 82.6% were considered excellent and 16.3% were slightly blurred but did not interfere with image evaluation. Only one case (1.2%) presented moderate blurring that slightly compromised the image, and previous examinations demonstrated findings compatible with bronchiolitis obliterans. Mean effective radiation dose was 0.39±0.15mSv. Percentages of images with motion artifacts were 0.3% for cystic fibrosis, 1.3% for bronchiolitis obliterans, and 1.1% for congenital pulmonary airways malformations. ConclusionChest ultra‐low‐dose computed tomography without sedation or anesthesia delivering a sub‐millisievert dose can provide image quality to allow identification of common pulmonary anatomy and diseases.

Rare causes of respiratory insufficiency in newborns.

Congenital lung masses (CLM) the rare group of causes of acute respiratory insufficiency (RI) in newborns include congenital airway pulmonary malformation (CAPM), congenital overinflation, bronchopulmonary sequestration, and bronchial atresia. The presenting group consists of 13 newborns who were admitted to the Neonatal Department of Intensive Medicine (NDIM) during January 1st 2015-December 31st 2019 (8 males, 5 females, 2 premature/11 term newborns, spontaneous delivery: 2, caesarean section: 11) with positive prenatal diagnosis of CAPM in all cases. In 2 cases prenatal intervention was performed (drainage of the amniotic fluid, attempt of thoracentesis). Signs of acute RI immediately after delivery were seen in 5 newborns. Postnatal echocardiographic investigation confirmed the presence of increased pulmonary pressure in 8 patients, no patient had congenital heart abnormality. A thorax x-ray was positive also in asymptomatic patients. Computed tomography in patients brought detailed information about the position, size and character of CAPM. Six patients underwent surgery. In 15.4 % right lungs were affected by cystic malformation and in 23 % left lungs were affected. A final diagnosis of CAPM was confirmed in 5 patients using histopathologic examination. Multidisciplinary cooperation during prenatal as well as postnatal period is necessary.

Dynamics in IgM and IgG antibodies against a polysaccharide capsule-containing complex of various &lt;i&gt;S. pneumonia&lt;/i&gt; and &lt;i&gt;H. influenza&lt;/i&gt; type b serotypes in children with chronic lung and bronchial inflammatory diseases after vaccination with “Pneumo-23” and “Act-HIB”

A lead place among pathogens resulting in pediatric chronic bronchopulmonary diseases is held by S. pneumoniae and Haemophilus influenzae. Vaccination against pneumococcal and hemophilic infections is approved and recommended for patients with chronic pathologies, but no clear recommendations for combined use of Pneumo-23 and Act-HIB vaccines in children with congenital pulmonary malformations and bronchial asthma were proposed.Materials and methods. There were enrolled 92 children aged 0–17 years old with chronic bronchopulmonary diseases; 55 healthy children, and 57 unvaccinated children with chronic broncho-pulmonary pathology were included into control group. Mono- and combination vaccination by Pneumo-23 and/or Act-HIB was performed in remission period. IgM and IgG level against H. influenzae antigens, H. influenzae type b, to S. pneumoniae (serotypes 3, 6B, 9N, 23F) vaccine-specific polysaccharide as well as polysaccharide complex antigens were measured by using ELISA developed by us. Statistical processing was carried out by methods of descriptive, parametric and nonparametric statistics by using Statistica 5.0 software.Results. Vaccination with Pneumo 23 was accompanied by IgG production against serotypes 3, 6, 9N, 23F-derived polysaccharide. A markedly increased anti-serotype 3 and 23F antibody level was observed 6 months after vaccination. Moreover, a significant increase in anti-polysaccharide 23F and anti-vaccine-derived polysaccharide IgM levels was found 1 month after the onset. In addition, anti-serotype 6B and 9N IgM antibodies were maintained 18 months after vaccination at high level, whereas it was significantly elevated against serotypes 3, 23F. Assessing an effectiveness of vaccine prophylaxis against Hemophilus type b infection, it was shown that a significantly increased anti-vaccine-derived polysaccharide IgM level was found 1, 6, 18 months after Act-HIB vaccination. In addition, IgG to the anti-H. influenzae type b polysaccharide tended to rise 1 month after the vaccination.Summary. Children with chronic bronchopulmonary diseases vaccinated by Pneumo-23 and Act-HIB demonstrated activated adaptive immunity manifested by increased vaccine-derived antigen-specific IgM and IgG antibodies.

New insights on congenital pulmonary airways malformations revealed by proteomic analyses

BackgroundCongenital Pulmonary Airway Malformation (CPAM) has an estimated prevalence between 0.87 and 1.02/10,000 live births and little is know about their pathogenesis. To improve our knowledge on these rare malformations, we analyzed the cellular origin of the two most frequent CPAM, CPAM types 1 and 2, and compared these malformations with adjacent healthy lung and human fetal lungs.MethodsWe prospectively enrolled 21 infants undergoing surgical resection for CPAM. Human fetal lung samples were collected after termination of pregnancy. Immunohistochemistry and proteomic analysis were performed on laser microdissected samples.ResultsCPAM 1 and 2 express mostly bronchial markers, such as cytokeratin 17 (Krt17) or α-smooth muscle actin (ACTA 2). CPAM 1 also expresses alveolar type II epithelial cell markers (SPC). Proteomic analysis on microlaser dissected epithelium confirmed these results and showed distinct protein profiles, CPAM 1 being more heterogeneous and displaying some similarities with fetal bronchi.ConclusionThis study provides new insights in CPAM etiology, showing clear distinction between CPAM types 1 and 2, by immunohistochemistry and proteomics. This suggests that CPAM 1 and CPAM 2 might occur at different stages of lung branching. Finally, the comparison between fetal lung structures and CPAMs shows clearly different protein profiles, thereby arguing against a developmental arrest in a localized part of the lung.

Prenatal natural history of congenital pulmonary malformations: MALFPULM population-based cohort study.

To assess prenatal changes in the volume of congenital pulmonary malformations (CPM) and examine whether these changes differ in lesions that appear cystic on ultrasound compared with hyperechoic lesions, and to study the relationship between CPM volume and risk of fetal compression. We conducted a nationally representative, multicenter, prospective cohort study, which included 579 ultrasound examinations in 176 pregnant women with a diagnosis of fetal CPM, between March 2015 and November 2016. Several ultrasound examinations were performed between diagnosis and delivery, including measurement of CPM volume. We modeled changes in CPM volume ratio (CVR) as a function of gestational age, overall and for cystic/mixed vs hyperechoic malformations, and examined the association between CVR and signs of compression during pregnancy. When modeling CVR changes over time, there was a statistically significant decrease in CVR with increasing gestational age (P < 0.001), but the pattern of change differed according to CPM phenotype at first ultrasound examination: cystic/mixed CPM were characterized by a monotonic decrease in CVR with increasing gestational age (P = 0.002), whereas hyperechoic CPM showed an initial increase in CVR up to 27 weeks of gestation, followed by a decrease thereafter (P < 0.001). Peak CVR values were predicted as early as 21-22 weeks for cystic/mixed CPMs compared with 25-26 weeks for hyperechoic malformations. Regardless of CPM phenotype, fetuses that showed no sign of compression at any point had substantially lower CVR at first CVR measurement, and the CVR remained relatively constant thereafter. Among the subpopulation of fetuses with no sign of compression at first CVR measurement, the odds of a subsequent compression was 7-fold higher (adjusted odds ratio, 7.0; 95%CI, 1.6-29.9) if initial CVR was > 0.4 vs CVR ≤ 0.4 cm2 . Predicted changes in CVR during pregnancy differ between cystic and hyperechoic malformations. This may be the result of different pathophysiological mechanisms or differences in the timing of occurrence of these different types of CPM. CVR measured at the initial diagnostic ultrasound examination was strongly associated with the odds of subsequent compression. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Congenital lung cysts in a newborn. About a case

Congenital pulmonary malformations, especially congenital pulmonary cysts, are a heterogeneous group of alterations in lung development that can occur at different stages of embryogenesis, affecting the parenchyma, the arterial supply, the venous drainage or being a combination of them. A clinical case is presented where it analyzes the congenital pulmonary malformations in a newborn patient at the term of adequate weight for the gestational age, in the same as when entering the Neonatology service. To the radiological control in which an image of bubbles of air trapped in the left base is visualized, initial suspicion of diaphragmatic hernia, the same that is discarded at 24 hours, changing to a diagnosis of congenital pulmonary malformations. Congenital lung cysts.

Clinical presentation and outcome in congenital pulmonary malformation: 25year retrospective study in Thailand.

Congenital pulmonary malformations (CPM) are a group of rare abnormal lung development lesions that can have various presentations. The aim of this study was to define the differences in the clinical presentations of CPM in neonates, infants, and children, and to review the outcomes. A retrospective study was conducted at a tertiary care hospital in southern Thailand between 1992 and 2016. Fifty-four patients were diagnosed with CPM, and the median age at onset was 1.7months (IQR, 0.03-10months). There were 33 cases (61.1%) of congenital pulmonary airway malformations, two (3.7%) of bronchogenic cyst, eight of (14.8%) congenital lobar emphysema, seven of(13.0%) pulmonary sequestrations, and fourof (7.4%) congenital lung cysts. Twenty patients under 1 month old and 16 patients who were 1-12 months old had symptoms of respiratory distress. In contrast, 13 patients >1 year old had symptoms of pulmonary infection. There were significant differences in the numbers of patients who had cyanosis (P = 0.006), cough (P < 0.001), and fever (P < 0.001) between the three age groups. Thirty-eight patients (70%) required surgical treatment involving lobectomy (78.9%). Median follow-up duration was 28.1months (IQR, 3.7-9.4months). Nine of 10 patients had abnormal lung function tests, and 80.6% of patients had no subsequent limitations in physical activities. Respiratory distress is the important clinical feature in neonates and infants, whereas the signs of pulmonary infection usually occur in children >1 year old. Good outcomes usually occur after surgery but need long-term follow up including lung function assessment.

Effect of different therapeutic strategies on the clinical outcome of asymptomatic intralobar pulmonary sequestration.

Pulmonary sequestration is a rare congenital pulmonary malformation. The aim of this study was to explore the effect of different therapeutic strategies on the clinical outcome of asymptomatic intralobar pulmonary sequestration. We retrospectively reviewed the clinical data of 37 patients diagnosed with intralobar sequestration. All the patients were asymptomatic. Seventeen patients underwent video-assisted thoracoscopic surgery (VATS) once diagnosed and 20 patients chose to undergo observation. Of these 20 patients, 16 patients developed symptoms during the observation period and also underwent VATS; 4 patients never showed symptoms and did not have surgery. The 33 patients who had VATS were divided into 2 groups: group 1, patients who underwent VATS once diagnosed; group 2, patients who underwent VATS once symptoms appeared. Postoperative data and respiratory function data were compared between the 2 groups. Twenty of the patients were men and 17 were women (mean age 37.05 ± 7.89 years). Results of a comparative analysis of the 2 groups indicated that patients in group 1 had better values for median estimated blood loss, median duration of chest tube insertion, postoperative hospital stay and postoperative hospital stay than those in group 2. Postoperative complications were reported in 1 patient in group 1 and in 3 patients in group 2. Meanwhile, the loss of lung function between group 1 and group 2 was statistically significant, which also suggested that patients benefited from surgery once diagnosed. For asymptomatic intralobar sequestration, VATS could be effective and safe. The surgical intervention should be performed once the condition is diagnosed to avoid manifestations occurring and to preserve patients' quality of life.

Type I regressed pleuropulmonary blastoma in a 10-year-old boy.

Pleuropulmonary blastoma (PPB) is a rare, malignant tumor of the lung and is the most common primary pulmonary malignancy in children. Here, we report a case of a boy who was diagnosed with type I regressed PPB after being mislabeled with congenital pulmonary malformation. A 10-year-old boy presented to our hospital with a history of worsening dyspnea. Since birth, his clinical status and radiographic images were concerning for congenital lobar emphysema that was managed conservatively. A chest computed tomography (CT) scan confirmed the persistence of a large cystic lesion and a diagnostic and therapeutic cystectomy was performed. Microscopic examination confirmed the presence of PPB type Ir. Patient was managed surgically alone with no added chemotherapy, as there was no overall survival benefit. PPB Ir has an overall favorable clinical outcome. Limited follow-up data are available due to the rarity of the lesion and the overlap with other congenital cystic lung malformations.

A RARE CONGENITAL PULMONARY MALFORMATION – PULMONARY SEQUESTRATION: A CASE REPORT

PURPOSE: To show a rare case of congenital pulmonary malformation - pulmonary sequestration. METHODS: Medical record review. RESULTS: A 36-year-old man presented to outpatient clinic with severe cough for one month. Chest x-ray revealed airspace opacities in the right lower lung, suggestive of pneumonia. Antibiotics with ampicillin/sulbactam one week was used but his cough persisted. Laboratory examination revealed a low titer of anti-mycoplasma pneumonia IgG reactivity (12.3 AU/mL). And antibiotics was changed to levofloxacin. After two more weeks antibiotics treatment, his symptoms improved. But the follow-up chest x-ray only showed partial clearing of the previous opacity. Triangular shape opacity was still be found in right lower lung field. Chest CT scan was arranged for further survey. A blood vessel arising from aorta and to the right lower lung mass lesion were found. The image and the pulmonary structure are compatible with pulmonary sequestration. This patient had abdominal pain over the right upper quadrant about 20 months later. Abdominal CT showed the same picture in right lower lung without interval change. CONCLUSIONS: Pulmonary sequestration is a relatively rare congenital pulmonary malformation. There are various manifestations, from asymptomatic to pulmonary infections or hemoptysis. Usually, chest x-ray with patients’ presentation may lead to the possible diagnosis of infection or neoplasm. However, our point is that chest CT scan is the most helpful and gold standard to make a definite diagnosis. CLINICAL IMPLICATIONS: Chest CT scan is the most helpful tool to identify the vascular anatomy and make the diagnosis of pulmonary sequestration.

Congenital Lung Malformations.

Congenital lung malformations (CLM) comprise a spectrum of anatomical anomalies of the lungs and respiratory tree. The prenatal growth pattern of CLMs is unpredictable with larger lesions causing life-threatening complications, such as hydrops fetalis, and smaller lesions remaining asymptomatic and potentially regressing. The most common CLMs are congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts. All these lesions have clinically similar presentations when symptomatic, but pathophysiologic differences that must be considered when evaluating and treating. This article reviews the most common CLMs, their pathophysiology, clinical presentation, diagnostic considerations, and current literature on the controversies surrounding CLM management. [Pediatr Ann. 2019;48(4):e169-e174.].

Utility of Fetal Cardiac Axis and Cardiac Position Assessment in Predicting Neonatal Respiratory Morbidity in Fetal Congenital Lung Lesions.

To assess the diagnostic performance of the fetal cardiac axis (CA) and/or cardiac position (CP) versus the congenital pulmonary malformation volume ratio (CVR) in predicting any and severe neonatal respiratory morbidity in fetal congenital lung lesions. This work was an 11-year retrospective cohort study. The sensitivity, specificity, positive predictive value, and negative predictive value of CA and/or CP assessment in prediction of respiratory morbidity were calculated before 24 weeks' gestation and between 24 and 32 weeks and compared to CVR cutoffs obtained from the literature. Fifty-three patients were included. CA and/or CP abnormalities were present in 45% and 38% of patients before 24 weeks and between 24 and 32 weeks and were significantly more common in left- versus right-sided lesions (60% versus 17%; P = .003). The sensitivity, specificity, positive predictive value, and negative predictive value of an abnormal CA and/or CP for any and severe respiratory morbidity were 0.67, 0.61, 0.33, and 0.86 and 0.8, 0.58, 0.17, and 0.97 before 24 weeks and 0.75, 0.73, 0.45, and 0.91 and 0.8, 0.67, 0.20, and 0.97 between 24 and 32 weeks, respectively. An abnormal CA and/or CP had higher sensitivity for any respiratory morbidity compared to the CVR at 0.5 and 0.8 cutoffs both before 24 weeks and between 24 and 32 weeks (P < .05). An abnormal CA and/or CP before 24 weeks and between 24 and 32 weeks has higher sensitivity for the detection of any respiratory morbidity at birth compared to the CVR at both 0.5 and 0.8 cutoffs. A normal CA and CP have a high negative predictive value for excluding any respiratory morbidity at birth both before 24 weeks and between 24 and 32 weeks.

Congenital Pulmonary Adenomatoid Malformation: Unusual Revelation

Congenital pulmonary adenomatoid malformation (CPAM) is a congenital lung malformation that is distinguished by abnormal airway patterning during branching morphogenesis. It may lead to significant morbidity and mortality in infants due to complications such as pulmonary infections, lung hypoplasia, respiratory distress, and fetal hydrops. The diagnosis is usually prenatal thanks to morphological ultrasound; in postnatal, the anomaly may remain asymptomatic or show respiratory signs or complications. The postnatal diagnosis is based on computed tomography. In this article, we report a case of congenital pulmonary adenomatoid malformation in a 7-month-old patient who presented respiratory distress that is due to voluminous infected pleural effusion. The diagnosis of CPAM was suspected at the initial CT and confirmed at the control CT that was realized after treatment of the infection. The therapeutic management of CPAM is depending on the severity of complications and its evolution.

Malformación congénita pulmonar de la vía aérea; caso post mortem

Las malformaciones pulmonares congénitas de la vía aérea, son lesiones del desarrollo anormal embrionario pulmonar, producido en la fase pseudoglandular. Son de poca frecuencia con una incidencia de 1:25,000-35,000 y responsables del 25% de todas las malformaciones congénitas pulmonares. El abordaje es multidisciplinario, en el que la detección temprana disminuye el riesgo de mortalidad y aumenta la supervivencia a largo plazo. Presentamos el caso post mortem de un paciente de dos meses de edad, en el cual se diagnosticó malformación congénita pulmonar de la vía aérea tipo 2.