Diagnosis Of Congenital Lung Malformations Research Articles

Diagnostic accuracy of imaging compared to histology in congenital lung malformations

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

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

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

Congenital lung malformations: can we avoid computed tomography? A five-year study.

Congenital lung malformations (CLMs) consist of a variety of pulmonary development disorders. In the CLM approach, computed tomography (CT) is considered the gold standard imaging technique due to the high-resolution for the lung parenchyma evaluation, the study of the vascular system after contrast injection, and the multiplanar reconstructions. In the paediatric population CT is considered too invasive due to ionizing radiation and the use of contrast agent. Therefore, the indications for the use of magnetic resonance imaging (MRI) are increasing. The aim of our study is to compare retrospectively MRI and CT in the evaluation of CLMs, to reduce or avoid the use of contrast-enhanced CT in the paediatric population. We retrospectively evaluated 22 paediatric patients with prenatal diagnosis of CLMs. All the patients underwent postnatal MRI in the first 2 weeks of life (except for a patient) and pre-surgery contrast-enhanced CT. A total of 7 blinded radiologists divided into 3 different groups independently reviewed each MRI and CT examination. Sensitivity and specificity of radiologists with different years of experience on the field, as well as of MRI findings regarding every pathology, were evaluated using a ROC curve. The interobserver agreement regarding the MRI findings was also measured. Analysing the ROC curves, we observed that MRI provided a satisfactory accuracy for diagnosing most congenital pulmonary diseases. Our study showed that MRI without contrast agent allows us to reach a CLM diagnosis in good agreement with contrast-enhanced CT, which is considered the gold standard imaging technique.

Developing Virtual Reality Head Mounted Display (HMD) Set-Up for Thoracoscopic Surgery of Complex Congenital Lung MalFormations in Children.

Video assisted thoracoscopic surgery (VATS) has been adopted in pediatric age for the treatment of congenital lung malformations (CLM). The success of VATS in pediatrics largely depends on the surgeon’s skill ability to understand the airways, vascular system and lung parenchyma anatomy in CLM. In the last years, virtual reality (VR) and 3-dimensional (3D) printing of organ models and VR head mounted display (HMD) technologies have been introduced for completion of preoperative planning in adult patients. To date no reports about the use of VR HMD technologies in a pediatric setting are available. The aim of this report is to introduce a VR HMD model in VATS procedure to improve the quality of care in children with CLM. VR HMD set-up for planning thoracoscopic surgery was performed in a series of pediatric patients with diagnosis of CLM. The preoperative VR HMD evaluation allowed a navigation into the malformation with the aim to explore, interact, and make the surgeon more confident and skilled to answer to the traps. A development of surgical simulations models and teaching program dedicated to education and training in pediatric VATS is suitable among the pediatric surgery community. Further studies should demonstrate all the benefits of such technology in pediatric patients submitted to VATS procedure.

Prediction of postnatal outcome in fetuses with congenital lung malformation: 2-year follow-up study.

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. 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. 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. 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.

Characterization of the Hyperintense Bronchus Sign as a Fetal MRI Marker of Airway Obstruction.

Background Fetal MRI-based differential diagnosis of congenital lung malformations is difficult because of the paucity of well-described imaging markers. Purpose To characterize the hyperintense bronchus sign (HBS) in in vivo fetal MRI of congenital lung malformation cases. Materials and Methods In this retrospective two-center study, fetal MRI scans obtained in fetuses with congenital lung malformations at US (January 2002 to September 2018) were reviewed for the HBS, a tubular or branching hyperintense structure within a lung lesion on T2-weighted images. The frequency of the HBS and respective gestational ages in weeks and days were analyzed. Areas under the curve (AUCs), 95% CIs, and P values of the HBS regarding airway obstruction, as found in histopathologic and postnatal CT findings as the reference standards, were calculated for different gestational ages. Results A total of 177 fetuses with congenital lung malformations (95 male fetuses) and 248 fetal MRI scans obtained at a median gestational age of 25.6 weeks (interquartile range, 8.9 weeks) were included. The HBS was found in 79% (53 of 67) of fetuses with bronchial atresia, 71% (39 of 55) with bronchopulmonary sequestration (BPS), 43% (three of seven) with hybrid lesion, 15% (six of 40) with congenital cystic adenomatoid malformation, and 13% (one of eight) with bronchogenic cyst at a median gestational age of 24.9 weeks (interquartile range, 9.7 weeks). HBS on MRI scans at any gestational age had an AUC of 0.76 (95% CI: 0.70, 0.83; P = .04) for the presence of isolated or BPS-associated airway obstruction at histopathologic analysis and postnatal CT. The AUC of HBS on fetal MRI scans obtained until gestational age of 26 weeks (AUC, 0.83; 95% CI: 0.75, 0.91; P < .001) was significantly higher (P = .045) than that for fetal MRI scans obtained after gestational age 26 weeks (AUC, 0.69; 95% CI: 0.57, 0.80; P = .004). Conclusion The hyperintense bronchus sign is a frequently detectable feature at fetal MRI and is associated with airway obstruction particularly before gestational age 26 weeks. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Dubinsky in this issue.

Is It Better to Operate Congenital Lung Malformations when Patients are Still Asymptomatic?

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.

Establishment of a multidisciplinary fetal center streamlines approach for congenital lung malformations

Purpose Fetuses with a diagnosis of congenital lung malformations (CLM) on prenatal imaging are commonly referred to a multi-disciplinary specialty team for prenatal assessment and postnatal management. The net effect of such services is broadly stated to improve the outcomes of affected newborns. However, these claims are relatively unsubstantiated. Methods After IRB approval, a retrospective review of children diagnosed with CLM from 2008 to 2018 and referred to a large urban children’s hospital was performed. A comparison was performed between prenatally diagnosed patients having a multi-disciplinary fetal center evaluation (FC) and prenatally diagnosed patients who did not receive a referral or were seen prior to the establishment of the center (NON-FC). Results Eighty-eight live-born patients with a prenatal diagnosis of CLM were identified, with 49 in the FC group and 39 NON-FC. Thirty-four (63%) and 23 (59%) patients underwent operative resection of CLM, respectively. FC patients presented earlier at first postnatal follow-up (42 vs. 145 days, p = .03), had fewer preoperative office visits (2.1 vs. 3.4, p = .0003), received fewer preoperative chest radiographs (0.5 vs. 1.3; p = .002) and chest computed tomography (0.9 vs. 1.4; p = .001), and had fewer preoperative pneumonias (0 vs. 17.4%; p = .02) compared to their NON-FC counterparts. FC patients were also more likely to undergo resection at an earlier age (217 vs. 481 days, p = .003) and were more likely to undergo a minimally invasive resection (75% vs. 39.1%, p = .015). There were no differences in post-operative outcomes between the two groups. Conclusion Children with a prenatal diagnosis of CLM appear to benefit from an organized multi-specialty team approach in several impactful parameters. Hospital systems and providers that invest in similar strategies are likely to achieve improved outcomes in the care of newborns prenatally diagnosed with a CLM.

Impact of Additional Anomalies on Postnatal Outcomes in Congenital Lung Malformations

BackgroundApproximately 20% of fetuses diagnosed with congenital lung malformations (CLMs) are found to have additional anomalies. We aim to determine if additional anomalies have an impact on postnatal outcomes for patients with CLMs. MethodsAfter institutional review board approval, we performed a retrospective review of live-born patients with CLMs from 2008 to 2018. All patients were prenatally diagnosed with CLMs. Clinical information pertaining to additional congenital anomalies and outcomes was collected from the electronic health record and analyzed. ResultsOf the 88 patients who had a prenatal diagnosis of CLMs, 20.5% had additional anomalies. Ten of the 18 patients (56%) were considered to have a major anomaly in addition to CLMs. Outcomes for patients electing nonoperative management of CLMs were similar between those with and without an additional anomaly. Although patients with an additional anomaly were more likely to have perinatal respiratory complications (44% versus 17%, P = 0.03), the number of preoperative clinic and emergency department visits, age at surgery, minimally invasive approach to surgical resection of CLM, estimated blood loss, length of hospital stay, intubation, duration of intubation, 30-day postoperative complications, and long term sequelae were not statistically different. This held true when stratified for major versus minor anomalies. ConclusionsTwenty percent of fetuses diagnosed with CLM in our population have additional anomalies. Newborns with additional anomalies have a higher risk of pre-excision pulmonary complications. However, the overall outcomes of all patients with CLMs are similar.

Lung Malformations: Prenatal Ultrasound Diagnosis and Obstetrical Management

ABSTRACT Purpose We present our experience in prenatal diagnosis and obstetrical management of congenital lung malformations (CLM). Materials and methods The diagnosis of CLM was performed during routine second and third trimester fetal morphology assessment. The extent of the disease was established according to the type, localization and size of the pulmonary lesion and the presence of fetal complications (mediastinal shift, fetal hydrops, hydramnios). Termination of pregnancy (TOP) was indicated in cases associated with extrapulmonary anomalies, untreatable cases and fetal hydrops. After delivery a pulmonary X-ray and surgical examination was performed in all newborns. Results We diagnosed 15 cases with congenital lung malformations. Of these, 9 had congenital cystic adenomatoid malformation (CCAM). TOP was performed in 6 cases with CCAM. Three cases had a favorable pre/postnatal evolution. Bronchopulmonary sequestration (BPS) was diagnosed in 3 cases, all with favorable perinatal evolution. Right pulmonary agenesis was diagnosed in one case and the outcome was neonatal death. One case of congenital high airway obstruction syndrome was followed by TOP. One case of severe bilateral pulmonary hypoplasia (secondary to a severe bilateral hydrothorax) resulted in neonatal death. Conclusion Obstetrical management is established individually depending on the severity of the cases. How to cite this article Iliev G, Dumitrascu I, Grigore M, Mihalceanu E, Scripcaru D, Zoican G, Aprodu G, Bivoleanu A, Petrariu A, Stamatin M. Lung Malformations: Prenatal Ultrasound Diagnosis and Obstetrical Management. Donald School J Ultrasound Obstet Gynecol 2014;8(2):134-143.

Prenatal imaging and postnatal presentation, diagnosis and management of congenital lung malformations.

Congenital lung malformations (CLMs) vary in their clinical presentation and severity. Increases in prenatal diagnosis, observed regression of certain lesions, and prognostic uncertainty are driving an evolution in management. There has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. Although prenatal diagnosis of CLMs using ultrasound and MRI has increased, chest radiography and computed tomography still play important roles in diagnosis. The management of these lesions depends on the type of malformation and symptoms. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these lesions is largely unknown. Proponents of early intervention argue that the complications of CLM, which may include infection, pneumothorax, bleeding and malignant transformation, justify surgery. Advocates of conservative management note that some CLMs disappear postnatally, and that the long-term complication rate following surgery is unknown. There is a need to obtain natural history data regardless of the therapeutic recommendations. This article reviews the prenatal radiographic features and postnatal clinical findings of various CLMs and the dilemmas regarding treatment.

A Retrospective Evaluation of Congenital Lung Malformation

Background: Congenital lung malformation (CLM) is a rare condition with high incidence of mortality and morbidity. CLM is often discovered incidentally on routine prenatal ultrasound or other postnatal imaging tests. Depending on the size of lesion and degree of functional impairment, prognosis is different. Today, fetal lung abnormalities are increasingly detected prenatally by ultrasound imaging. Objectives: This study aimed to evaluate the CLM cases in pediatric pulmonary ward of Masih Daneshvari Hospital during an 8-year period from 2002 to 2010. Materials and Methods: In a retrospective study, 21 patients (12 females, nine males), diagnosed with CLM, were enrolled. The mean age of participants was 7.3 years (ranging from 1 month to 16 years). All patients were histopathologically diagnosed with CLM and their demographic data including age, sex, and clinical signs and symptoms were recorded. To confirm the diagnoses and rule out the differentials, patients underwent bronchoscopy, angiography and other proper diagnostic procedures. Afterwards, they were divided into several subgroups based on the malformation and type of involvement, including agenesia, hypoplasia, aplasia, bronchogenic cyst, and sequestration. Results: We reported two agenesis, five hypoplasia, six aplasia, two bronchogenic cyst, and six sequestration cases. All patients were symptomatic at the time of diagnostic workup. The most common clinical signs and symptoms were respiratory distress and recurrent pulmonary infections. Conclusions: Considering the wide range of clinical manifestations from no clinical symptom to severe respiratory distress at birth, imaging including chest X-ray, CT scan with contrast and MRI are of great values for early diagnosis of CLM. Early diagnosis could provide a more appropriate timing of evaluation and treatment options, lower mortality and morbidity, and better prognosis.

Congenital lung abnormalities: a pictorial review of imaging findings

Congenital lung malformations (CLM) comprise a broad spectrum of maldevelopment that spans a continuum of maldevelopment involving parenchyma, bronchi, and vasculature. They may present as respiratory symptoms at birth and can be the source of morbidity and mortality. Early diagnosis is important for adequate therapy to prevent complications, such as recurrent infections, or to help normal lung development. Prenatal diagnosis of CLM using ultrasound (US) and magnetic resonance imaging (MRI) has increased, but chest radiography and computed tomography (CT) still play important roles in making diagnosis. This article reviews the clinical and radiographic features of various CLM.

Congenital lung malformations?antenatal and postnatal evaluation and management

We reviewed our institutional experience with pulmonary resection for congenital bronchopulmonary malformations and analysed the management and outcome of pregnancies with a prenatal diagnosis of congenital lung malformations. Between January 1993 and December 2003, 31 patients underwent evaluation and pulmonary resection for bronchopulmonary malformations. Common clinical presentations were respiratory distress (9), respiratory infections/pneumonias (22), and dyspnoea (9). Diagnostic modalities included chest radiography, CT scan (22), MRI scan (7), arteriography (1), and bronchoscopy (5). There were 13 congenital cystic adenomatoid malformations (CCAM), six pulmonary sequestrations, three bronchogenic cysts, and nine congenital lobar emphysemas (CLE). Fifteen patients who underwent resection were diagnosed by antenatal ultrasound. No foetus had hydrops or associated malformations. No pregnancy was terminated. There was no foetal demise. Regression of the sonographic appearance was observed in six cases. Amniotic puncture was required for hydramnios in three cases. Eight emergency resections were performed (CCAM 4; CLE 3; Bronchogenic cyst 1). Surgical procedures included 24 lobectomies, one right middle lobectomy with a wedge resection of the right lower lobe, one completion right lower lobectomy, four sequestrectomies, one mediastinal mass excision and one wedge resection for a bronchogenic cyst. There were no deaths. Postoperative complications included: persistent air leak (n=2; one requiring completion lobectomy) and pneumothorax (1). Persistent mild symptoms were present in five patients, at long-term follow-up. Congenital cystic adenomatoid malformation and congenital lobar emphysema were the commonest congenital anomalies. Congenital lung malformations are increasingly diagnosed antenatally, sometimes necessitating emergent surgical resection. The natural history is variable. All infants with a prenatal diagnosis require postnatal evaluation. Patients should be evaluated for associated disorders. The presence of mass effects is an indication for therapeutic decompression. The risk of pulmonary compression, infection and malignant degeneration makes resection imperative, even in asymptomatic patients. Lobectomy is the procedure of choice, is well tolerated, and leads to excellent outcomes.