Congenital Pulmonary Malformation Research Articles

A Case of Congenital Bronchial Pulmonary Arterial Malformation Successfully Treated by Embolization

AbstractIdiopathic bronchial pulmonary arterial malformation (BPAM) is a very rare condition. The authors present a case of BPAM in which a right bronchial artery communicates with a main upper lobe branch of the right pulmonary artery. It was successfully treated by embolization in one setting. The patient remained asymptomatic and well during the follow-up period. The authors therefore conclude that if the embolic material/device is carefully chosen, it is a safe and effective means of treating BPAM.

A case of congenital pulmonary vein malformations undergoing interventional treatment

A case of congenital pulmonary vein malformations undergoing interventional treatment

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.

Genetic testing for pulmonary stenosis

Abstract Pulmonary stenosis (PS) is a congenital pulmonary valve malformation. It can be classified as valvular, subvalvular or supravalvular. Isolated forms of PS are rare. PS is associated with the development of massive pulmonary arterial dilatation. Patients with PS have a high consanguinity rate and the disorder is highly familial, which is why knowing the genetic aetiology of this defect is important. Prevalence is estimated at about 4/10,000 live births, and incidence at about 10% of all children with congenital heart defects. PS has prevalently autosomal dominant and rarely autosomal recessive inheritance. This Utility Gene Test was developed on the basis of an analysis of the literature and existing diagnostic protocols. It is useful for confirming diagnosis, as well as for differential diagnosis, couple risk assessment and access to clinical trials.

Pulmonary sequestration in adults: a retrospective review of resected and unresected cases

BackgroundPulmonary sequestration (PS) is a form of congenital pulmonary malformation that is generally diagnosed in childhood or adolescence and usually resected when diagnosed. We aim to identify the clinical presentation and course of patients diagnosed to have PS during adulthood.MethodsUsing a computer-assisted search of Mayo clinic medical records, we identified adult patients with PS diagnosed between 1997 and 2016. Clinical and radiological data were collected including postoperative course for those who underwent surgical resection.ResultsWe identified 32 adult patients with PS; median age at diagnosis was 42 years (IQR 28–53); 17 patients (53%) were men. The median sequestration size was 6.6 cm (IQR 4.4–9.3). The type of sequestration was intralobar in 81% and extralobar in 19%. The most common location was left lower lobe posteromedially (56%). Forty-seven percent of the patients presented with no relevant symptoms. The most common radiographic finding was mass/consolidation in 61% and the most common feeding artery origin was the thoracic aorta (54%). Surgical resection was performed in 18 patients (56%) and postoperative complication was reported in 5 patients (28%). There was no surgical mortality. Median duration of follow-up after diagnosis for unresected cases, most of whom were asymptomatic, was 19 months (IQR 4–26) with no complications related to the PS reported.ConclusionsNearly one-half of adult patients with PS present with no relevant symptoms. The decision regarding surgical resection needs to weigh various factors including clinical manifestations related to PS, risk of surgical complications, comorbidities, and individual patient preferences.

Safety Assessment for Thoracoscopic Day Case Surgery in Children with Congenital Pulmonary Malformation

No data exist evaluating the utilization and safety of outpatient thoracoscopy in children. The aim of this study was to investigate the safety of outpatient thoracoscopy and to assess parental opinions on the advantages and disadvantages of a pediatric thoracoscopy outpatient setting. A retrospective review of data from patients treated by thoracoscopy for congenital pulmonary malformation between 2013 and 2016 was performed. Study focused on patients who underwent outpatient thoracoscopy. All were placed in a flank position and underwent a three-port (5-mm optical trocar and two 5-mm trocars) thoracoscopy. Insufflation pressure required was 5 mmHg with bilateral lung ventilation. Pain control was provided with multimodal postoperative analgesia and the use of paravertebral block for regional analgesia. No drain was inserted. Outcomes of interest included 30-day overall morbidity, readmission, reoperation, and parental opinions through a phone call questionnaire. A total of 37 thoracoscopies were identified; 11 (30%) with a median age of 5.3 months (4.2-12.3) were performed as an outpatient procedure (10 sequestration, 1 bronchogenic cyst). Median operating time was 51 minutes (34-87). No conversion and no transfusion occurred. No complications occurred (no morbidity, no readmission, and no reoperation). According to parents' view the outpatient setting has no disadvantages. This first analysis of a small monocentric dataset demonstrates that pediatric patients can safely undergo thoracoscopy, an outpatient procedure, with a high rate of parental satisfaction.

Prenatal diagnosis of anomalous origin of pulmonary artery.

Anomalous origin of the pulmonary arteries is a rare congenital pulmonary vascular malformation that includes unilateral absence of the pulmonary artery (UAPA), anomalous origin of unilateral pulmonary artery (AOPA), and left pulmonary artery sling (LPAS). We analyze 15 cases of fetal pulmonary artery abnormalities from 2011 to 2017, detected via prenatal ultrasound at our center. The 15 cases include UAPA (5), AOPA (6), and LPAS (4). Of the UAPA cases, 2 had pulmonary atresia and 3 had pulmonary artery stenosis. In 5 cases, the descending aorta issued collateral vessels to the left lung. Of the AOPA cases, 2 had left pulmonary artery abnormalities originating in the ascending aorta and 4 showed right pulmonary artery abnormalities originating in the ascending aorta. Of the LPAS cases, 2 were type IA, 1 was type IIA, and 1 was type IIB. Two of the LPAS were associated with right lung dysplasia. Abnormal origin of the pulmonary artery has a characteristic ultrasonographic appearance. The branch of the pulmonary artery cross section can provide important clues to the diagnosis of abnormal pulmonary artery origin. Pulmonary artery abnormalities are often associated with pulmonary atresia or stenosis.

Nowoczesna tomografia komputerowa w diagnostyce wybranych wad wrodzonych żył płucnych

An excellent spatial and temporal resolution of computed tomography (CT) provides for precise evaluation of congenital cardiac malformation in selected group of patients. CT allows for quick, simultaneous assessment of all chest structures, such as pulmonary vessels, lung parenchyma and bones. Recent advances in CT hardware and software result in fast scanning of large anatomic ranges with a single lamp rotation without the need for patient's sedation and breath hold. The only limitation of CT is nowadays the exposure to ionizing radiation but with the new generation of scanners equipped with iterative reconstruction it is possible to reduce applied radiation dose while maintaining image quality. CT evaluation of pulmonary venous anomalies is well described, accurate for determining the site of anatomic obstruction in anomalous pulmonary venous return and has been shown to be highly specific when compared to operative findings. This article presents short review on current possibilities of CT diagnostic management of selected congenital pulmonary venous malformations.

Congenital Pulmonary Malformations in Children: Diagnosis and Management in a Pediatric Department in a Low Source Country

Congenital pulmonary malformations (CPM) comprise a group of anatomical abnormalities of the respiratory tree including congenital cystic malformations, bronchopulmonary sequestrations, bronchogenic cyst, bronchial atresia and congenital lobar emphysema.Socioeconomic, demographic, and hospital factors were determinants of survival of CPM. Antenatal diagnosis should be developed in order to improve management and prognosis of CPM in country with low resource.

Retrospective review of the diagnosis and treatment of pulmonary sequestration in 28 patients: surgery or endovascular techniques?

Pulmonary sequestration (PS) is a rare congenital pulmonary malformation. In this study, we aimed to retrospect and evaluate the diagnosis, treatment, and outcomes of PS in 28 patients at our institute. The files of 28 patients with PS who were treated with surgery (21 cases) or endovascular intervention (7 cases) between May 2005 and June 2016 from a single institute were retrospectively reviewed. The following data of all patients were analyzed: age, sex, clinical symptoms, diagnostic methods, operative techniques, and treatment outcomes. Twenty-eight patients, 15 male and 13 female, with a median age of 42.5 underwent operative intervention for PS. Twenty-one patients showed preoperative symptoms including cough, expectoration, hemoptysis, chest and/or back pain, and fever. General chest computed tomography (CT) scanning; percutaneous needle biopsy, bronchoscopy, enhanced CT scanning, and CT angiography (CTA) were used as diagnostic methods. Twenty-one patients were diagnosed preoperatively by enhanced CT scanning and CTA; seven patients were confirmed by surgery. Twenty-one patients underwent surgery (15 cases via thoracotomy and 6 cases via video-assisted thoracic surgery), seven patients underwent interventional therapy (three cases via endovascular embolization and four cases via thoracic aortic endovascular stent-graft exclusion). Three patients had a complication in surgery group (intraoperative hemorrhage in two patients and postoperative hydropneumothorax in one patient) and two patients had post-embolization syndrome in interventional group (fever and pain at embolism site). During the follow-up period ranging from 6 to 84 months, no recurrences or further complications were observed in two groups. Enhanced CT or CTA may be a potential approach for the diagnosis of PS. Surgical resection for PS is the major treatment approach. Endovascular embolization of PS could be considered when pulmonary lesion is small-sized. Endovascular exclusion could be used to treat combined arterial aneurysm and dissection of PS.

Predicting Pathology from Imaging in Children Undergoing Resection of Congenital Pulmonary Malformations

Predicting Pathology from Imaging in Children Undergoing Resection of Congenital Pulmonary Malformations

Pneumatoceles in pediatric blunt trauma: Common and benign

IntroductionTraumatic pneumatoceles are reported to be rare in children and to have an uncertain clinical significance. We report a single institution series of traumatic pneumatoceles to better define their frequency and clinical significance. MethodsAfter obtaining approval from the IRB, data were extracted from the trauma registry of a level 1 pediatric trauma center on children diagnosed with a pulmonary contusion (International Classification of Diseases-9th edition diagnosis codes: 861.21 to 861.31) who presented between June, 2006 and September, 2016. Patient demographics, mechanism of injury, injury severity score, diagnosis and procedure codes, length of hospital stay, outcome, imaging techniques and findings with attention to the identification of a pneumatocele, were examined. ResultsOf the 10,229 trauma admission, 204 children were identified as having a pulmonary contusion, 25 of whom (12.3%) were diagnosed with a pneumatocele. Their mean age was 13years (3–17). Seventy-six percent (19) were male. The most common mechanism of injury was a motor vehicle collision (10), followed by falls (6), and sports (5). Sixteen children (64%) suffered a long bone fracture, 12 (48%) an abdominal solid organ injury and 3 (12%) a traumatic brain injury. The mean Injury Severity Score was 17 (9–34). Twenty-three patients presented as transfers. There were no fatalities. The pneumatocele was identified on chest computerized tomography (CT) alone in 15 (60%), on chest CT and chest radiograph (CXR) in 3 (12%), the upper portions of an abdominal CT in 6 (24%) and on CXR alone in 1 (4%). Seven patients were found to have a solitary pneumatocele and 18 patients had 2 or more. The largest pneumatocele was 3.7cm in diameter. Ten children (40%) were admitted to the intensive care unit, 3 of whom required intubation. One patient (4%) had a respiratory complication: pneumonia. Three patients underwent chest tube placement for: pneumothorax, hemothorax and hemopneumothorax. No child underwent intervention specific to the pneumatocele. Seventeen (68%) patients were seen in follow-up in Trauma Clinic and the remainder by another practitioner ranging from 1week to 6months after injury. One child (4%) underwent a follow-up chest CT to rule out a congenital pulmonary malformation 6months after injury and this demonstrated complete resolution of the pneumatocele. ConclusionThe incidence of traumatic pneumatoceles among children with a pulmonary contusion was 12.3% in this series, but is probably higher given that only 24% were visible on radiographs and a small minority of children with pulmonary contusions underwent chest CT. Pneumatoceles are common in children with pulmonary contusions, but are usually small. The majority do not appear to be clinically significant nor require follow-up imaging. Level of evidenceIV.

BRONCHOGENIC CYST: A RARE CAUSE OF RESPIRATORY DISTRESS IN NEONATE - A CASE REPORT

Bronchogenic cyst is an unusual congenital pulmonary malformation which may occur at various sites of tracheobronchial tree as well as unusual ectopic site like cervical, scapular region, pericardium and abdomen. Newborn with large cyst can develop respiratory distress, cyanosis and feeding difficulty. A 37 day old male infant presented with failure to thrive, feeding difficulty, respiratory distress and cyanosis. His chest radiograph was suggestive of shift of mediastinum and heart with hyperinflatted left lung. CT thorax showed minimal enhancing cystic lesion posterior to trachea and its bifurcation on right side, it was compressing the right main bronchus. The cyst was successfully surgically excised which was adhering to carina and intrabronchial extension and its histopathological finding confirmed the bronchogenic cyst. Though, bronchogenic cyst is rare cause of respiratory distress, cyanosis, and failure to thrive and feeding difficulty, it should be considered one of the differential diagnoses.

Respiratory Morbidity in Infants Born With a Congenital Lung Malformation

The actual frequency of respiratory symptoms related to congenital pulmonary malformations (CPMs) remains undetermined. The goal of this study was to prospectively evaluate the respiratory symptoms occurring in infants with prenatally diagnosed CPMs, identify factors associated with the occurrence of these symptoms, and evaluate their resolution after surgery. Infectious and noninfectious respiratory symptoms were prospectively collected in a French multicenter cohort of children with CPMs. Eighty-five children were followed up to the mean age of 2.1 ± 0.4 years. Six children (7%) underwent surgery during the first 28 days of life. Of the 79 remaining children, 33 (42%) had respiratory symptoms during infancy before any surgery. Wheezing was the dominant symptom (24 of 79 [30%]), and only 1 infant had documented infection of the cystic lobe. Symptoms were more frequent in children with noncystic CPMs, prenatally (P = .01) or postnatally (P < .03), and with postnatally hyperlucent CPMs (P < .01). Sixty-six children underwent surgery during the follow-up period, and 40% of them displayed symptoms after the intervention. Six children had documented pneumonia during the postoperative period. At the end of the follow-up, pectus excavatum was observed in 10 children, significantly associated with thoracotomy (P < .02) or with surgery before the age of 6 months (P < .002). CPMs are frequently associated with wheezing episodes. Surgery had no significant impact on these symptoms but was associated with a paradoxical increase in pulmonary infections, as well as an increased risk of pectus excavatum after thoracotomy.

Perpetual dilemma: Pleural or parenchymal/congenital or acquired solitary cystic lesion with fluid level.

Congenital cystic adenomatoid malformations (CCAMs) are rare congenital, nonhereditary developmental anomalies of the lung with unknown etiology. CCAM is predominantly a disorder of infancy with the majority of the cases being diagnosed within the first 2 years of life. When CCAM presents in adults, it represents a diagnostic dilemma and requires careful evaluation. We here report a case of large solitary congenital pulmonary cystic adenomatoid malformation with infection and hemorrhage, which was diagnosed as encysted hydropneumothorax on computerized tomography scans but turned out to be infected pulmonary cystic adenomatoid malformation after surgical excision.

Antenatally Detected Congenital Pulmonary Airway Malformations: The Oxford Experience.

Congenital airway pulmonary malformations are increasingly being diagnosed, but their management continues to remain controversial. Our approach has been to offer surgery to mitigate the risk of infection and possible malignancy. All patients routinely undergo a CT scan of the chest postnatally and once the diagnosis is confirmed, minimal access surgery is offered. Our anesthetists provide single-lung ventilation to enhance the operative view. We conducted a retrospective review over a 10-year period, during which 91 patients were prenatally suspected to have a cystic lung lesion. There were 88 live births of which 29 (33%) cases were initially managed conservatively based on CT findings. Five of these patients, however, became symptomatic needing surgery. A total of 64 (73%) patients underwent surgery with the most common lesions being congenital pulmonary airway malformations (CPAMs) (24), hybrid lesions (19), and pulmonary sequestrations (12). The median age at surgery was 5 months (1 day to 17 months). Using a minimal access approach, 41 (64%) cases were completed with 17 performed open from the onset. Open surgery was indicated in neonates who became symptomatic within the first few weeks of life as well as patients in respiratory distress that would not tolerate either single-lung ventilation or gas insufflation. There were six further conversions to open from minimal access surgery due to poor visualization or technical difficulties. One patient needed a perioperative blood transfusion and one patient had a more prolonged stay due to persistent air leak managed conservatively. Among asymptomatic patients, evidence of microscopic disease was seen, which included infection as well as two cases of tumors, one pleuropulmonary blastoma seen as part of a CPAM, and one rhabdomyomatous dysplasia seen in the CPAM component of a hybrid lesion. In our experience, excising asymptomatic lesions is safe with minimal complications. Single-lung ventilation in combination with thoracoscopy provides excellent vision. There is a risk of infection and a definite, albeit low, risk of malignancy, which may outweigh the benefits of conservative management.

Pulmonary Sequestration: A Congenital Abnormality Seen in the Adult Pulmonologist’s Office

SESSION TITLE: Fellow Case Report Poster - Genetic and Developmental Disorders SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Pulmonary sequestration is a rare congenital malformation. Diagnosis is often delayed until the 2nd or 3rd decade. Common symptoms including recurrent infections and hemoptysis. Surgical resection is usually recommended due to the risk of massive hemoptysis. The following is a case of a 42 year old male presenting with his sentinel event of sequestration, which underscores the importance of adult pulmonologists recognizing congenital conditions. CASE PRESENTATION: A 42 year old male, with minimal past medical history, presented with small volume hemoptysis. In the Emergency Department, he was hemodynamically stable without hypoxia. CT of the chest with IV contrast was obtained due to the concern of pulmonary embolus. The CT showed ground glass infiltrates in the medial right lower lobe with an aberrant feeding vessel arising from the thoracic aorta. He was discharged in stable condition with pulmonary follow up. DISCUSSION: Pulmonary sequestration is a rare finding accounting for about only 5% of congenital pulmonary malformations. It is characterized by dysplastic lung tissue fed by an aberrant artery arising directly from the aorta. In extralobar sequestration, there is a distinct visceral pleura surrounding the dysplastic parenchyma. It is usually diagnosed in vitro, but often does not cause symptoms until adulthood. On imaging, it is commonly seen as a mass because it is rarely aerated. Intralobar sequestration, on the other hand, is more common and is not surrounded by an independent visceral pleura. It is more likely to result in recurrent infection, and therefore, earlier presentation in childhood. Intralobar sequestration imaging shows aerated but abnormal lung tissue. Both forms of sequestration usually require surgical resection due to the risk of massive, life-threatening hemoptysis. Standard thoracotomy or video assisted thoracic surgery (VATS) can be performed. There is limited data but increasing interest in endovascular intervention in adults. CONCLUSIONS: Pulmonary sequestration is a congenital malformation, but often presents in the adult pulmonologist’s office. Recognition is imperative because intervention can be life-saving. Reference #1: Qian X, Sun Y, Liu D, Wu X, Wang Z, and Tang Y. 2015. Pulmonary sequestration: a case report and literature review. Int J Clin Exp Med. 8(11):21822-21825. Reference #2: Leonici G, Rossi UG, Ferro C, and Chessa L. 2011. Endovascular treatment of pulmonary sequestration in adults using Amplatzer® vascular plugs. Interact Cardiovasc Thorac Surg. 12:98-100. DISCLOSURE: The following authors have nothing to disclose: Laurie Manka No Product/Research Disclosure Information

Outcome of infants operated on for congenital pulmonary malformations.

Patients operated on for congenital pulmonary malformations (CPM) have excellent survival rates, but little is known about long-term morbidity. Our aim is to report the sequelae in patients operated on for CPM in infancy and to define factors that may influence their outcome. All patients operated on for major congenital anomalies are followed in a dedicated outpatient program and evaluated at 6, 24, and 48 months of life (corrected for gestational age) and at school age at 4, 6, 8, and 12 years of life. The data are prospectively collected. Patients operated on for CPM and enrolled in the follow-up clinic between January 2004 and December 2010 are compared with a control group of term infants operated on for inguinal hernia, without other major congenital or acquired abnormalities. The two groups were compared for auxological, respiratory, and orthopedic outcome. In the study period, 76 consecutive patients with CPM attended our dedicated follow-up clinic. Eight non-operated patients were excluded from the study. Age at follow-up was 82.0 (56.1-103.7) months in CPM patients and 83.5 (75.2-90.4) months in controls (P = 0.79). Fifty-three patients with CPM (78%) had one or more clinical or radiological abnormality versus six (16%) control patients (OR [95%CI] 16.5 [5.8-47.2]; P < 0.0001). Over 50% of patients with CPM present long-term sequelae, regardless type of malformation. Therefore, long-term follow-up of patients operated on for CPM is recommended. Further studies are needed to define if, in asymptomatic patients, surgery may modify the natural history of CPM. Pediatr Pulmonol. 2016;51:1367-1372. © 2016 Wiley Periodicals, Inc.

Congenital Lung Malformations: Shifting from Open to Thoracoscopic Surgery

Over the years the need for surgical treatment, timing of intervention, and the type of surgical approach have been discussed, but the treatment of congenital lung malformations remains controversial. The aim of this study was to compare the thoracotomy approach with the thoracoscopic technique by evaluating different surgical outcomes (duration of surgery, postoperative hospital stay, and complications). All patients operated from January 2011 to March 2015 for suspected congenital cystic lung were included in the study. Patients treated for congenital lobar emphysema and tracheobronchial neoplasms were excluded from the study. In the analyzed period, 31 asymptomatic patients were treated: 18 lung resections were performed with thoracotomy (Group A) and 13 with the thoracoscopic approach (Group B). No significant differences were observed between the age and weight at surgery, length of the procedures, complications, and the need for postoperative intensive care between the two groups. The postoperative hospital stay in Group A was twice that for Group 2 (p=0.0009). Comparing thoracoscopic surgery with the traditional open approach, we confirmed the superiority of minimally invasive treatment in terms of postoperative hospital stay. Common technical recommendations can help pediatric centers to develop the thoracoscopic approach for the treatment of congenital pulmonary malformations.

Ultrasound assessment of congenital fetal lung masses and neonatal respiratory outcomes.

Fetal congenital lung masses generate concern for compromised postnatal respiratory function. Congenital pulmonary adenomatoid malformation volume ratio (CVR) has been used to predict the risk of hydrops fetalis and need for antenatal intervention. This study investigates whether CVR could be used to predict neonatal respiratory outcomes. The ultrasounds of fetuses diagnosed with a lung mass between 2005 and 2013 were reviewed. CVR was calculated at each ultrasound using the formula for a prolate ellipse divided by head circumference. The pregnancy outcome and information about NICU admission for respiratory insufficiency were collected. Forty-two fetuses were diagnosed with a lung mass during the study period. CVR prior to 24 weeks and between 24 and 32 weeks were associated with NICU admission (p < 0.0001 and <0.008, respectively). CVR increased up to 32 weeks and decreased thereafter for most subjects. The decrease in CVR beyond 32 weeks was larger for cases that required NICU admission (p = 0.002). For a CVR cut-off of <0.5, the sensitivity was 100%, the specificity 85.7%, and negative predictive value was 100% for regular nursery care. In pregnancies diagnosed with fetal lung masses, CVR predicted normal respiratory outcomes and need for NICU admission. This information may be helpful for delivery planning. © 2015 John Wiley & Sons, Ltd.