Bronchopulmonary Malformations Research Articles

A Case Report and a Short Literature Review of Pulmonary Sequestration Showing Elevated Serum Levels of Carbohydrate Antigen 19-9

Pulmonary sequestration is a type of bronchopulmonary malformation defined as an isolated portion of lung tissue with systemic arterial supply and no bronchial communication. Carbohydrate antigen 19-9 (CA19-9) has been used for diagnosis and follow-up of gastrointestinal tumors. The current study presents a rare case of intralobar pulmonary sequestration associated with the marked elevation of CA19-9. A 39-year-old female patient was admitted to our hospital due to acute liver injury with marked elevation of serum CA19-9 (3,051.1 μmol/mL), and was then diagnosed with intralobar pulmonary sequestration after examination and surgery. After the pulmonary resection, the serum CA19-9 levels decreased to normal ranges. We briefly reviewed the literature on elevated serum CA19-9 levels and pulmonary sequestration since 1988. We found that serum CA19-9 levels are increased not only in patients with digestive tract cancers but also in those with nonmalignant diseases such as pulmonary sequestration.

Fetal bronchopulmonary malformations

Introduction: Fetal body tumors are rare, but the ability to diagnose them has improved over recent years. Most masses discovered in the chest results from fetal bronchopulmonary malformations, such as congenital cystic adenomatoid malformation and bronchopulmonary sequestration. Congenital cystic adenomatoid malformation and bronchopulmonary sequestration have a reported incidence of 50% and 33% of all prenatally diagnosed lung lesions, respectively.Material and methods: Retrospective analysis of the congenital cystic adenomatoid malformation and bronchopulmonary sequestration cases diagnosed or surveilled at our department, between January 2003 and March 2013. Prenatal examination, evolution, management and patient outcome were analyzed.Results: A total of 918 fetal malformations were diagnosed at our hospital, 17 of them representing fetal bronchopulmonary malformations. The majority were diagnosed during the second trimester and stabilized or regressed during the third trimester of pregnancy. The pregnancies and deliveries had no other relevant findings or complications, except in three cases. Nine children required surgery. All of the children are healthy and have a normal development, with regular surveillance by the pediatricians.Discussion: The majority of these fetal lung masses are isolated findings that partially regress during intrauterine life. With adequate postnatal surveillance and eventual surgery the prognosis is good.

Intralobar pulmonary sequestration in an adult female patient mimicking asthma: A case report

Pulmonary sequestration (PS) is a rare congenital broncho-pulmonary malformation. The main feature of this disease is that partial lung tissues separate from the main lung during the embryonic period, receiving blood supply from systemic circulation arteries. Pathogenesis of PS is not clear, and categorized into congenital and acquired PS. We report a case of a 38 year old woman smoker with medical history characterized by difficult to treat asthma with frequent exacerbations and infections since childhood. CT scan showed a partial PS of left lower lobe, supplied by an abnormal artery arising from supradiaphragmatic aortic diverticulum. Surgical treatment through a lung sequestrectomy and laterobasal segment resection was performed.

Cystic Lung Lesions in Newborns and Young Children: Differential Considerations and Imaging

Numerous diverse entities produce cystic lung changes in neonates and young children. This review provides an evidence-based, age-appropriate, differential diagnostic framework to use when confronted with pulmonary cystic changes. The categories of diseases that have been discussed include congenital cystic bronchopulmonary malformations, neoplastic conditions, infections, collagen or soft tissue abnormalities, and mimics of cystic lung disease. An understanding of the pathophysiology, imaging appearance, and demographics of these entities is essential in guiding optimal care. Important educational points include differentiating bronchopulmonary malformations from neoplasms and the management and surveillance of lung cysts in young children.

Hilar closure using staplers or Hem-o-lok clips in a rabbit model

BackgroundLung lobectomy is necessary in neonatal patients for the treatment of a variety of congenital bronchopulmonary malformations. The working space required for traditional stapling devices limits their use in pediatric video-assisted thoracoscopic (VATS) lung lobectomy. The use of Hem-o-lok clips (Teleflex Medical Inc. Research Triangle Park, NC) has been described for a number of applications in minimally invasive surgery and may provide an alternative to traditional stapling devices for bronchial closure. Materials and methodsTwenty-four New Zealand White rabbits were used in the study. The hilus of each cranial lung lobe was sealed with the Endo GIA (Covidien Inc, Mansfield, MA) stapler or 2 Hem-o-lok clips and the lung lobe was removed. Lung inflation to leakage point was induced by incrementally increasing tidal volume. Side of leakage, volume at leakage, and airway pressure before leakage were recorded. ResultsFourteen rabbits were included in the data analysis. Leakage of air was first observed at the Hem-o-lok closure site in five rabbits and from the Endo GIA site in three rabbits. In the remaining six, both resection sites leaked simultaneously. The mean pressure before failure was 16.39 ± 7.35 mm Hg for the Hem-o-lok clips, 17.98 ± 11.12 mm Hg for the Endo GIA stapler, and 16.95 ± 3.48 mm Hg for the simultaneous failures. No statistical differences were detected in airways pressures before leakage between hilar closure devices. ConclusionsDespite advances in VATS, options for bronchial closure in pediatric patients undergoing VATS lung lobectomy remain limited. Hem-o-lok clips require less working space than traditional stapling devices and may represent an equally efficacious alternative to traditional stapling devices.

Congenital lung malformations: radiological findings and clues for differential diagnosis

Congenital lung malformations encompass a wide spectrum of conditions with a broadly varying clinical presentation. They are often a source of morbidity in infants and children. Their management depends on the type of malformation and its clinical presentation. Usually, the diagnosis requires an imaging evaluation. Classifications of bronchopulmonary malformations have undergone significant revision in recent years and several theories have attempted to explain their confusing pathogenesis. There are considerable degrees of overlapping and hybrid conditions are common, with interrelated malformations showing various radiologic and pathologic features. Attending to the pathophysiological mechanisms and structures involved, lung malformations can be divided into three categories: bronchopulmonary anomalies, combined lung and vascular abnormalities, and vascular anomalies. The purpose of this article is to review the current imaging techniques for evaluating lung malformations in pediatric patients and their characteristic imaging findings. Moreover, this review discusses a useful classification and offers some clues to facilitate the differential diagnosis.

Use of palivizumab in infants and young children with severe respiratory disease: A delphi study

To achieve a consensus of opinion among an expert group of pediatric pulmonologists regarding the appropriateness of the off-label use of palivizumab for some pediatric patients with severe respiratory diseases. A two-round modified Delphi technique was used. A 43-item self-administered questionnaire grouped into seven clinical scenarios was developed. Level of agreement for each statement was ranked on a 0-9 scale with 0 being total disagreement and 9 total agreement. Consensus was sought through the feedback of information and iteration. The final responses were evaluated for median and interquartile range to determine which questions the group had reached consensus about, either affirmatively or negatively. Consensus was obtained for 24/43 statements (55.81%), including use of palivizumab for prevention of respiratory syncytial virus (RSV) infection in children with severe respiratory involvement due to neuromuscular disease, congenital or acquired immunodeficiency, storage disease, cystic fibrosis, diseases involving impaired ciliary clearance, patients operated on esophageal atresia and/or tracheoesophageal fistula, diaphragmatic hernia, bronchopulmonary malformations, severe tracheomalacia, lung transplant recipients and patients in the waiting list for lung transplant, patients oxygen-dependent for severe interstitial pulmonary disease and patients with severe pulmonary hypertension. Consensus against the use of palivizumab as prevention of RSV infection was also achieved in almost all the recurrent wheezing/asthma attacks situations. A set of indication for off-label uses of palivizumab in pediatric pulmonology was developed in accordance with the degree of professional consensus on which they were based. The applicability of the present results to clinical practice should be evaluated individually and reviewed periodically in the light of new emerging evidence. Further studies are needed to add evidence to the most frequent and clinically oriented scenarios that have shown higher levels of uncertainty.

Infantile pulmonary tuberculosis: the great mimic

Infantile tuberculosis is common in developing countries and rarely presents as space occupying thoracic lesions mimicking congenital malformations. This case series reviews four such infants with varied presentations and their outcome. Four cases of infantile pulmonary/mediastinal tuberculosis that presented like congenital thoracic lesions are described. Details of demography, symptomatology, contact history, immunization status, provisional diagnosis, tuberculin testing, imaging, histopathology, final diagnosis, management and outcome were retrospectively collated and analyzed. They were 4-6-month males, term-born and immunized. They presented with pneumonia/hyperactive airway disease since 2-12 weeks. One had a suspect and another a close tuberculous contact. The provisional diagnosis after imaging were infected congenital lung cyst, posterior mediastinal cyst and bronchopulmonary malformation. Two were tuberculin positive; none had gastric acid-fast bacilli. One underwent a pulmonary lobectomy for necrotic lung cyst; the second had a biopsy and drainage of a posterior mediastinal cyst that contained caseating material and was densely adherent to the esophagus. Surgical biopsy showed necrotizing granulomatous inflammation in both; one with acid-fast bacilli. Both succumbed to postoperative complications. The other two with tuberculous contacts who were managed with early antituberculous therapy, responded well and recovered uneventfully. Infantile pulmonary/mediastinal tuberculosis may mimic congenital thoracic malformations. A review of contact history, investigations and imaging help to establish the tuberculous etiology, avoids surgical misadventures and prompts early antituberculous therapy to achieve a favorable outcome.

Pulmonary Sequestration with Renal Aplasia and Elevated SUV Level in PET/CT

Extralobar sequestration with other bronchopulmonary malformations is commonly seen; however, the association of extralobar sequestration with renal aplasia is very rare. A 75-year-old female patient was admitted with back pain. Ultrasonography revealed aplasia of the left kidney and tomography showed 6 × 4.5 cm sized tumor in the left hemithorax at the posterobasal area. The lesion has focally increased glycolytic activity (SUVmax: 3.2) at the left upper pole on positron emission tomography scan (PET/CT). Sequestrectomy was performed after the confirmation by frozen section that the lesion was benign and of extrapulmonary sequestration. No complication occurred during postoperative and 50-month follow-up period.

Imaging of fetal chest masses

Prenatal imaging with high-resolution US and rapid acquisition MRI plays a key role in the accurate diagnosis of congenital chest masses. Imaging has enhanced our understanding of the natural history of fetal lung masses, allowing for accurate prediction of outcome, parental counseling, and planning of pregnancy and newborn management. This paper will focus on congenital bronchopulmonary malformations, which account for the vast majority of primary lung masses in the fetus. In addition, anomalies that mimic masses and less common causes of lung masses will be discussed.

Late Diagnosis of Extralobar Pulmonary Sequestration: Two Cases with an Uncommon Malformation

Pulmonary sequestration is a congenital bronchopulmonary malformation, characterized by a mass of nonfunctioning lung tissue which receives its blood supply from a systemic artery. This increased blood flow and the inflammation in the sequestrated segments are positive on PET imaging, arousing the suspicion of malignancy. We report two cases of PET-positive extralobar sequestration that presented in patients of middle age and were successfully treated with surgical resection, indicating the role of surgery and the false-positive PET-CT appearance of sequestration cases.

444: Perinatal outcomes in low risk and high risk congenital pulmonary airway malformations (CPAMs) as indicated by CPAM volume ratio (CVR)

CPAMs are bronchopulmonary malformations frequently identified on routine prenatal ultrasound. Complications related to rapid growth and mass effect in the chest include polyhydramnios, pulmonary hypoplasia, and hydrops. Hydrops in CPAM is usually fatal without intervention. Prenatal treatment options range from steroid administration, open fetal surgery, thoracoamniotic shunting, or third trimester ex-utero intrapartum treatment by EXIT-to-resection. We reviewed a single center experience with CPAMs and the impact on perinatal outcome.

Multislice CT in congenital bronchopulmonary malformations in children

Congenital bronchopulmonary malformations encompass a wide spectrum of pathologies involving the lungs, trachea and bronchi, pulmonary vessels, and oesophagus. These developmental lesions are often isolated, but the association of two or more anomalies is not infrequent. Contrast-enhanced multidetector computed tomography (MDCT), thanks to multiplanar and 3D reconstructions, allows for detailed studies of these malformations, achieving better accuracy compared with conventional techniques such as chest X-ray, fluoroscopy, ventilation and perfusion scintigraphy and ultrasonography. MDCT is characterised by fast data acquisition and does not require sedation in the majority of cases. The main drawbacks of MDCT are the use of ionising radiation and - in many cases -contrast media. Recently, improved CT scanners and optimised CT protocols have made available to children all the benefits of MDCT, thanks to a significant reduction in radiation dose and an improved risk-benefit ratio. The aim of our paper was to evaluate MDCT in children with bronchopulmonary malformations by reporting our experience (about 2,400 studies in 30 months with a 64-slice MDCT scanner) and comparing it with the available literature.

A case of horseshoe lung and complex congenital heart disease in a term newborn

We describe and illustrate a rare association of hypoplastic left heart syndrome, absent hilar left pulmonary artery, and an unusual bronchopulmonary malformation. This case highlights the utility of combination imaging of echocardiography and CT angiography in diagnosing a cyanotic newborn with a chest radiograph that is suspicious for lung hypoplasia.

Bronchopulmonary arteriovenous malformation — demonstrated by multidetector computed tomography angiography

Bronchopulmonary arteriovenous malformation — demonstrated by multidetector computed tomography angiography

Perinatal management of common neonatal thoracic lesions

Esophageal atresia, congenital diaphragmatic hernia, bronchopulmonary malformations and cystic lung diseases are the common neonatal thoracic surgical lesions encountered in practice. The availability of antenatal ultrasonography has lead to these lesions being detected before birth. Antenatal diagnosis can be made with a fair degree of accuracy in tertiary fetal medicine centres. Antenatal intervention is limited in a very few centres in the western world and not being done in India at present. The outcome of these babies with antenatal diagnosis of thoracic lesions has changed in the last decade. Earlier intervention is now possible in cystic lung disease before infectious complication has set in. All these lesions are managed exclusively in well developed neonatal surgery units with excellent outcome in the western world. The present study reviews the antenatal detection, clinical presentation, interventional/surgical procedures [antenatally and postnatally] and outcome of these common neonatal thoracic surgical lesions.

Bronchogenic cyst of the right hemidiaphragm mimicking a hydatid cyst of the liver: report of the first pediatric case

Bronchogenic cyst (BC), which develops from abnormal budding of the tracheal diverticulum or ventral foregut, is a congenital bronchopulmonary malformation. Localization of the BC varies depending on the level of the abnormal budding. Thoracic or abdominal-sited diaphragmatic lesions are the rarest presentations of the BC. We present a case of BC that originated from the diaphragm and mimicking hydatid cyst of the liver in a 19-month-old girl. Diagnosis of a diaphragmatic lesion was confirmed during laparotomy and complete resection was successful.

Congenital bronchopulmonary malformations: A single-center experience and a review of literature

PURPOSE:To present a single-center experience with 25 cases of bronchopulmonary malformations and the review the literature.MATERIALS AND METHODS:We conducted a retrospective analysis of the medical records of patients with congenital bronchopulmonary malformations who were operated between July 1997 and July 2007 in our institute; we examined the modes of presentations, management, and outcome. Outcome of all patients was assessed over a short follow-up period (average 1.8 months).RESULTS:Out of 25 patients, 18 (72%) were male and 7 (28%) were female. Age of patients ranged from 1 day to 11 years. The histopathological diagnosis was congenital cystic adenomatoid malformations [CCAM; n = 14 (56%)], congenital lobar emphysema [CLE; n = 5 (20%)], pulmonary sequestrations [PS; n = 3 (12%)], and bronchogenic cysts [BC; n = 3 (12%)]. Antenatal diagnosis was available in only 2 (8%) patients. The common presenting symptoms were respiratory distress and chest infections. Lobectomy was the procedure of choice . Mortality was 16% (n = 4; M: F = 3: 1). Two patients died because of overwhelming sepsis, one from compromised cardiac function, and one from aspiration which might possibly have been prevented.CONCLUSION:Patients with progressive respiratory distress due to these anomalies may require urgent surgical intervention regardless of age. The surgical outcome is favorable, with manageable complications. Plain x-ray chest and CT of thorax are usually sufficient for diagnosis and planning of treatment. Pathological diagnosis may differ from the imaging diagnosis. Mortality is found to be more in neonates. Apart from initial stabilization, resection of lesion and careful postoperative care is necessary to reduce mortality and morbidity.

Pleuropneumoblastome kystique de découverte fortuite chez un nourrisson

Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, associated with a poor outcome. We report the case of a 7 week-old boy with a pleuropulmonary blastoma classified as type I. Disease was discovered at a chest X-ray performed as a work-up for a benign acute viral bronchiolitis. The final diagnosis was brought by pathology: a bronchopulmonary malformation had not been ruled out by clinical, radiological and macroscopic findings. Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, for which the poor outcome and tough diagnosis justify a surgical attitude when an intrathoracic bullous lesion is found in an infant.

Atopia e auto-imunidade – A propósito de um caso clínico

Atopy, immunodeficiency and autoimmunity are manifestations of immune system dysfunction. Classically atopy and autoimmunity are referred as distinct immunological reactions. Recent studies suggest the existence of common pathogenic mechanisms.We report the case of a teenager with familial history of asthma and miasthenia gravis in her mother (HLAB8+) and personal history of recurrent upper respiratory infections from two to four years old, and pneumonia since five years old (3 or 4 episodes/ year, in three consecutive years), with associated dyspnoea and hypoxemia, requiring frequently hospital admission. Investigation was initially negative for atopy markers, and excluded other hypothesis as tuberculosis, cystic fibrosis, α-1 antitrypsin deficiency, congenital heart disease, bronchopulmonary malformations or foreign body aspiration. Latter, further exams finally confirmed atopy with a rised IgE, positive RAST and cutaneous sensitivity tests (for house dust mites and pollen) and revealed circulating immune complexes and IgG 2, 3 e 4 deficit. Most frequent autoantibodies and precipitins study were negative, and histocompatibility antigens study revealed HLA-B8 (as her mother). Ventilation-perfusion scintigraphy and respiratory function tests were normal. Antihistamines, topical corticoids and bronchodilators were done with an excellent clinical response. At 16 years-old she is admitted again with the diagnosis of erythema nodosum and the clinical suspicion of Sweet's syndrome, having a good evolution.The relation between atopy and autoimmunity is enfatized by the authors. This simultaneous occurrence does not correspond merely to a statistical association, but may represent a global immune system impairment, with the involvement of different types of hypersensibility.