Bronchopulmonary Foregut Malformation Research Articles

Unclassified bronchopulmonary foregut malformation in a 2-year-old girl

BackgroundBronchopulmonary foregut malformation (BPFM) is a rare congenital anomaly characterized by a fistula between an isolated portion of respiratory tissue and the esophagus or stomach. Srikanth et al. reported that BPFMs can be categorized into four groups, and that an unclassified BPFM is extremely rare. Herein, we present an unclassified BPFM group III and IV subtype in a 2-year-old girl.Case presentationAt a gestational age of 36 weeks, a 1535-g female neonate was born as one of the dichorionic diamniotic twins. She had vertebral abnormality, anovestibular fistula, pulmonary artery sling, small right lung, mediastinal shift with dextrocardia, tracheal stenosis, and radial hemimelia. After birth, she was diagnosed with VACTERL association. She exhibited consolidation of the right upper lobe (RUL) in the neonatal period; however, she had no respiratory symptoms and was kept under observation in an outpatient visit. At 24 months, she was urgently admitted with acute pneumonia, and the upper gastrointestinal series revealed the right upper bronchus arising from the lower esophagus. Therefore, she underwent RUL resection. Intraoperatively, the right lung had no lobulations. RUL was ventilated by the esophageal bronchus (BPFM group III); however, the S6 lesion was ventilated by both the normal bronchial system and esophageal bronchus (close to BPFM group IV). The S6 lesion did not satisfy the definition of group IV as it did not have systemic blood supply. Hence, we decided to preserve the S6 lesion to save lung capacity as much as possible. The esophageal bronchus was transected using a 5-mm stapler. Due to lobulation failure, RUL was resected using an electric scalpel and 5-mm staplers along with the demarcation line by ventilation from the normal bronchus. The postoperative course was uneventful, and the patient could regain oral function without pneumonia or respiratory distress.ConclusionsFor neonates with repeated consolidation and pneumonia, BPFM must be considered as one of the differential diagnoses. Regarding BPFM treatment, early recognition and imaging are necessary. To determine the resection area of the lung, it is crucial to consider the segment of ventilation from the normal bronchi.

Esophageal lung complicated by recurrent pneumonia.

A 6-month-old infant with recurrent respiratory infections, rapid breathing, and reduced air entry on the right side was diagnosed with congenital bronchopulmonary foregut malformation (CBPFM). Imaging revealed a collapsed and underdeveloped right lung, with the right bronchus appearing to originate from the lower part of the esophagus. An esophagogram confirmed the diagnosis by showing contrast flowing freely from the lower esophagus to the right bronchus.

Esophageal duplication cysts: a clinical practice review.

Esophageal duplication represents one of the most common types of bronchopulmonary foregut malformations. These rare congenital anomalies occur secondary to embryological aberrations between the 4th and 8th weeks of gestation. In order to be classified as an esophageal cyst a mediastinal cyst must have a close proximity with the esophagus, be lined by alimentary (squamous epithelium) or tracheobronchial mucosa and covered by two smooth muscle layers. These rare anomalies are often asymptomatic during adulthood. However, they can cause symptoms in early childhood, generally during the first 2 years of life. Variations in location, size, presence or absence of heterotopic mucosa, will dictate the clinical presentation. Dysphagia, food impaction, persistent cough and chest pain are common clinical presentations. Imaging studies including esophagram, computed tomography (CT) and magnetic resonance imaging (MRI) can provide key findings to reach the diagnosis. Nonetheless, endoscopic evaluation, particularly endoscopic ultrasound (EUS) is the most valuable tool to determine whether this lesion is cystic versus solid and or if there are abnormal mucosal findings. Needle biopsies are controversial but can help with drainage and to rule out malignant transformation. Therapeutic options include endoluminal drainage. However, more definitive therapies include surgical excision. Open and minimally invasive (laparoscopic and thoracoscopic) techniques have been demonstrated to be safe and effective at completely removing these lesions. Recently, robotic-assisted resections have gained more attention with case reports and series reporting excellent outcomes.

Computed tomography findings of communicating bronchopulmonary foregut malformation: a retrospective study of 18 patients.

Communicating bronchopulmonary foregut malformation is a rare anomalycharacterized by a patent congenital communication between the esophagus or stomach and an isolatedportion of the respiratory system. An esophagogram is taken as the gold standard for diagnosis. Compared with esophagography, computed tomography (CT) is more widely used and easily obtained, but CT findings have been described as nonspecific. To describe CT findings in 18 patients with communicating bronchopulmonary foregut malformation to assist with early diagnosis. A retrospective review of 18 patients who had proven communicating bronchopulmonary foregut malformation between January 2006 and December 2021 was conducted. For each patient, the medical records, including demographics, clinical manifestations, upper gastrointestinal radiography, magnetic resonance imaging and CT findings, were reviewed. Among the 18 patients, there were 8 males. The right to left ratio was 3.5:1. An entire lung was involved in 10 patients, a lobe or a segment was involved in 7 patients and an ectopic lesion was located in the right neck in 1 patient. The isolated lung may arise from the upper esophagus, mid-esophagus, lower esophagus or stomach, which were detected in 1, 3, 13, and 1 patient, respectively. On chest CT, an extra bronchus which did not arise from the trachea was detected in 14 patients. Contrast-enhanced chest CT was performed in 17 patients, the isolated lung receiving itsblood supply from the pulmonary artery in 13 patients, the systemic artery in 11 patients and both pulmonary and systemic arteries in 7 patients. The presence of an extra bronchus, which does not arise from the trachea, highly suggests the diagnosis of communicating bronchopulmonary foregut malformation. Contrast-enhanced chest CT can provide accurate information regardingthe airways, lung parenchyma and vascular structures that isuseful to plan surgery.

A complete spectrum of congenital cystic adenomatoid malformation of the lung deceptive clinical presentations and histological surprises; a single institutional study from a tertiary care hospital in North India.

Congenital Cystic Adenomatoid Malformations (CCAM) are rare congenital anomalies of the lungs characterised by bronchopulmonary foregut malformations due to a sudden arrest in the development of the bronchial tree in the first trimester of the gestational period. : The present study was aimed to describe the clinical and histopathological profiles of the patients and study patient outcomes after 1 year of surgical resection. All patients diagnosed with CCAM by histological examination of tissue obtained on surgical resection during the study period were included in the study. Data, such as patient demographics and clinical, radiological and histopathological findings, were recorded, and follow-up information was taken on OPD follow-up till 1 year after surgery regarding respiratory infections, haemoptysis or mortality. : Out of 21 patients, 11 were female and included in the study between the ages of 1 month and 32 years, with >50% younger than 2 years. Most patients in the study had recurrent pneumonia, with difficulty in breathing being the second most common presenting complaint. All patients had undergone computed tomography (CT) of the lungs, which was able to diagnose cystic lesions accurately in >80% of cases. Histologically, all cases were classified based on recent Strocker's classification, and Type 1 was the most commonly observed with 13 cases, followed by Type 2 in five and Type 3 in three patients. There was no evidence of malignant transformation in any of the cases. There was 100% survival at the end of 1 year, with six patients having respiratory infections and none of the patients getting hospitalised over 1 year after surgery. CCAM is a rare congenital anomaly associated with significant morbidity and may present at any age. It can be histologically classified into three subtypes, with Type 1 being the most common. Early surgical management is mandatory to prevent complications such as recurrent infections, respiratory distress, pneumothorax, lung abscess and malignant transformation. All patients included in the study had undergone surgical resection, and there was 100% survival at 1-year follow-up.

An unusual case of extrapulmonary sequestration presenting as vomiting in a neonate

Pulmonary sequestration is a rare, mostly sporadic disorder, with an incidence of 0.1–6% of all congenital lung malformations. It is also called as bronchopulmonary foregut malformation (BPFM) or accessory lung. A 16-day-old female child presented with vomiting and inability to take feeds. She was investigated further and differentials included foregut duplication cyst and BPFM. Surgical exploration confirmed the diagnosis of a BPFM. Delay in diagnosis may lead to complications such as torsion and sepsis.

Bronchiectasis without lower respiratory symptoms in the presence of multisystem anomalies – a clinical clue to diagnose esophageal lung anomaly

Esophageal lung is a type of Group-II communicating bronchopulmonary foregut malformations (CBPFM) usually diagnosed beyond neonatal period during investigation for recurrent respiratory symptoms and persistent radiographic features suggesting pneumonia or bronchiectasis. In our case, we noticed bronchiectasis and disproportionately severe volume loss in an infant with associated multisystem anomalies in the absence of "significant" lower respiratory tract symptoms. A detailed evaluation with repeat imaging confirmed a Group-II CBPFM, a congenital pathology instead of an infective cause. Pneumonectomy is a more prudent option instead of undertaking major airway reconstruction for the dysplastic "dysfunctional" tissue. Amongst the various associated anomalies reported till now, the associated rib and renal anomalies noted by us have not been described earlier to the best of our knowledge.

Unilateral esophageal lung with dextroposition of the heart

Esophageal lung is a rare type of congenital bronchopulmonary foregut malformation (CBPFM) that is difficult to diagnose. The authors report a case of esophageal lung that presented postnatally with respiratory distress and associated heart and skeletal anomalies. After proper investigations and evaluations, the patient was managed surgically with right total pneumonectomy.

Esophageal lung associated to isolated dextrocardia in a six-month-old infant

Congenital bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and the respiratory system. Esophageal lung is an entity belonging to this group. Few cases have been reported. Diagnostic can be a challenge in some case. Much closer cooperation between pediatric surgeons and radiologists is essential to all investigations of these congenital malformations in particular to those associated to other congeniatl abnormality. Herein we describe the diagnostic and therapeutic approach in a six months old girl with esophageal lung.associated to dextrocardia.

Congenital communicating bronchopulmonary foregut malformation including ectopic pancreatic tissue in an infant

BackgroundSeveral reports have documented that the pulmonary sequestration is in communication with the gastrointestinal tract and the concept of bronchopulmonary foregut malformation (BPFM) has become more widespread. However, there are few reports of the sequestration associated with the pancreas derived from the foregut. We describe the history and pathophysiology of BPFM including pancreatic tissue in a male infant with respiratory distress.Case presentationA male patient was born at 38 weeks of gestation and weighed 2752 g at birth. He developed pneumonia and was hospitalized at 3 months of age. Chest radiographs and CT scans led to the diagnosis of a lung abscess in the left lower intralobar pulmonary sequestration with aberrant arteries from the abdominal cavity. At 4 months of age, when the abscess had resolved, left lower lobectomy and the resection of the intralobar sequestration were performed. The pulmonary sequestration was conjoined with the esophagus. A fistula was found between the lower esophageal wall and the pulmonary sequestration. An additional small segment of the esophageal wall was excised. Histologically, the mediastinal surface of the sequestration tissue contained pancreatic tissue. Furthermore, esophageal and gastric tissue, cartilage tissue, and ciliated epithelium were confirmed. A definitive diagnosis of BPFM was made.ConclusionsWe postulated the rare case of a communicating BPFM with intrapulmonary sequestration on one end and the esophagus on the other forming a mass lesion, which included ectopic pancreatic tissue in a male infant.

Concurrent bronchopulmonary foregut malformations: a rare case of right-sided extralobar pulmonary sequestration and bronchogenic cyst

BackgroundBronchopulmonary foregut malformations are rare congenital malformations. It is extremely rare to have malformations that occur simultaneously. There is literature to show that extralobar sequestration is associated with other congenital anomalies, most commonly diaphragmatic hernias, and also with other bronchopulmonary foregut malformations (e.g., extralobar sequestration and congenital pulmonary airway malformations). However, very few case reports were found that reported extralobar sequestration and foregut duplication cysts and only one report of a right-sided complex foregut malformation with pulmonary sequestration.Case presentationWe present a case of a 3-month-old male infant with a prenatal diagnosis of a cystic lung lesion who, after developing symptoms of respiratory distress, was found to have concurrent right-sided extralobar pulmonary sequestration and a mediastinal bronchogenic cyst.ConclusionsThe concurrent occurrence of these malformations in one patient could help support the theory that these malformations result from an early error in development during the time when both the lung buds and foregut are developing simultaneously.

Successful thoracoscopic treatment for tracheoesophageal fistula and esophageal atresia of communicating bronchopulmonary foregut malformation group IB with dextrocardia: a case report of VACTERL association

BackgroundA communicating bronchopulmonary foregut malformation (CBPFM) group IB is very rare congenital malformation. Group IB is associated with tracheoesophageal fistula and esophageal atresia (TEF-EA) and a portion of one lung arisen from the esophagus (Gerle et al. in N Engl J Med. 278:1413–1419, 1968). The coexistence of TEF-EA and dextrocardia is also a rare and challenging setting for repair of TEF-EA. Therefore, the thoracoscopic surgery for TEF-EA require the technical devise because of the small operative space. We herein report a rare case of CBPFM group IB with intralobar sequestration of lung and a successful performing of thoracoscopic surgery for EA with dextrocardia in VACTERL association.Case presentationA 2.2-kg term male neonate was born with an anal atresia, coarctation of the aorta, TEF-EA, renal anomalies, radial hemimelia, limb abnormalities (VACTERL association) and hypoplasia of the right lung with dextrocardia. The patient developed respiratory distress after admission. A two-stage operation for the TEF-EA was planned because of multiple anomalies and cardiac condition. In the neonatal period, esophageal banding at the gastroesophageal junction and gastrostomy were performed to establish enteral nutrition. After gaining body weight and achieving a stable cardiac condition, thoracoscopic surgery for TEF-EA was performed. The thoracoscopic findings revealed a small working space due to dextrocardia. To obtain a sufficient working space and to perform secure esophageal anastomosis, an additional 3-mm assistant port was inserted. To close the upper and lower esophagus, anchoring sutures of the esophagus were placed and were pulled to suspend the anastomotic site. Esophageal anastomosis was successfully performed. An esophagogram after TEF-EA surgery showed the connection between the lower esophagus and right lower lung. The definitive diagnosis was CBPFM group IB with intralobar sequestration. The thoracoscopic surgery was performed again for establishing oral intake. After transection of the bronchoesophageal fistula, the patient could perform oral feeding without pneumonia or respiratory distress.ConclusionsCBPFM type IB with intralobar sequestration is a rare condition. CBPFM type IB should be considered for a patients with respiratory symptom after radical operation for TEF-EA. In the present case, suspending the anastomotic site was effective and useful in thoracoscopic surgery for a TEF-EA patient with dextrocardia.

PULMONARY HYPOPLASIA SECONDARY TO PULMONARY ARTERY STENOSIS: SURGICAL FIX FOR RECURRENT PNEUMONIA?

SESSION TITLE: Global Case Report Posters SESSION TYPE: Global Case Reports PRESENTED ON: October 18-21, 2020 INTRODUCTION: Introduction Congenital bronchopulmonary vascular malformations include a wide spectrum of disorders that compromise one or more of the three main systems of the lung (lung parenchyma, arteries and veins). The stage of gestation where disruption of the pulmonary vessels occurs determines the severity of lung affectation and alveolarization, ranging for segmentary affectation or the complete pulmonary hypoplasia. We present a case of primary pulmonary hypoplasia secondary to pulmonary artery stenosis. CASE PRESENTATION: A 11-year-old boy with history of recurrent pneumonia is evaluated in clinic. He was first admitted to the intensive care unit at age 40 months for pneumonia. Since then, he has had multiple admissions for recurrent pulmonary infections and wheezing treated with beta agonists. His chest x-ray revealed a marked decrease in volume in the right lung, with ipsilateral mediastinal shift. Computerized tomography angiography showed generalized bronchiectasis, main bronchus and pulmonary artery hypoplasia in the right side, with a significant reduction in lung volume. The left lung was hyperinflated and had compensatory emphysema. Scintigraphy revealed a total absence of perfusion in the right lung. Right heart catheterism was consistent with pulmonary artery stenosis. Patient underwent right pneumonectomy. He developed pneumonia and hydrothorax in the postoperative period, and was treated conservatively with antibiotics and incentive spirometer use. Repeat imaging showed a decrease in pleural effusion size. He had clinical improvement and he was discharged home with ambulatory follow up. DISCUSSION: Pulmonary hypoplasia is frequently associated with other malformation, including abnormalities of the thoracic cavity, masses and amniotic fluid disorders. When severe, it is generally diagnosed prenatally or shortly after birth. It can also be associated with congenital heart defects and in rare cases, no cause can be found. The severity of symptoms and their time of occurrence is defined by the degree of hypoplasia present in the patient. There have been reports of diagnosis in adulthood, presenting with recurrent respiratory infections. Clinical exam can show a shorter hemitorax of the affected side, intercostal spaces or diminished breath sounds. Imaging studies like CT can aid diagnosis, showing deviation of the mediastinum to the affected side, thinner vasculature and enlarg. CONCLUSIONS: Pulmonary hypoplasia secondary to pulmonary artery stenosis might present as recurrent respiratory infections and hospitalization. In cases with severe hypoplasia where lung function is severely compromised, pneumonectomy can be considered. Reference #1: Katayama Y, Kusagawa H, Komada T, Shomura S, Tenpaku H. Bronchopulmonary foregut malformation. Gen Thorac Cardiovasc Surg. 2011;59(11):767-770. doi:10.1007/s11748-010-0763-z Reference #2: Abrams, M., Ackerman, V. & Engle, W. Primary Unilateral Pulmonary Hypoplasia: Neonate through Early Childhood — Case Report, Radiographic Diagnosis and Review of the Literature. J Perinatol 24, 667–670 (2004). https://doi.org/10.1038/sj.jp.7211156 Reference #3: Papadopoulos D, Misthos P, Chorti M, et al. Unilateral pulmonary hypoplasia in an adult patient. Monaldi Arch Chest Dis. 2018;88(1):829. Published 2018 Jan 29. doi:10.4081/monaldi.2018.829 DISCLOSURES: No relevant relationships by Amarilys Alarcon-Calderon, source=Web Response No relevant relationships by ISMAR LOPEZ MURALLES, source=Web Response No relevant relationships by LUIS PEREZ MANCILLA, source=Web Response No relevant relationships by Shubert Iván Quiñónez Najera, source=Web Response

Structural and perfusion magnetic resonance imaging of congenital lung malformations

BackgroundA radiation-free advanced imaging modality is desirable for investigating congenital thoracic malformations in young children.ObjectiveTo describe magnetic resonance imaging (MRI) findings of congenital bronchopulmonary foregut malformations and investigate the ability of lung MRI for their classification.Materials and methodsThis is a retrospective analysis of consecutive MRI examinations performed for suspected congenital lung anomalies in 39 children (median age: 3.8 months, range: 2 days–15 years). Morphological and perfusion findings were characterised on respiratory-gated fast spin echo and dynamic contrast-enhanced sequences obtained at 1.5 tesla. Abnormalities were classified independently by two readers and compared to an expert diagnosis based on pathology, surgery and/or other imaging.ResultsMain diagnoses included bronchopulmonary lesions in 33 patients, scimitar syndrome in 4 patients, pulmonary arteriovenous malformation and oesophageal duplication cyst in one patient each. Of 46 observed abnormalities, 44 (96%) were classified correctly with very good interobserver agreement (96% concordance rate). The 39 detected lung lesions included isolated overinflation (17/39, 44%), cystic pulmonary airway malformation (8/39, 21%), bronchopulmonary sequestration (7/39, 18%), bronchogenic cyst (4/39, 10%) and hybrid lesion (3/39, 8%). All lung lesions presented as perfusion defect at peak pulmonary enhancement. Non-cystic lesions showed a delayed peak (median delay: 2.8 s, interquartile range: 0.5 to 4.0 s) in relation to normal lung parenchyma.ConclusionA dedicated lung MRI protocol including respiratory compensated sequences, dynamic angiography and perfusion is able to reliably delineate parenchymal and vascular components of congenital bronchopulmonary foregut malformations. Therefore, MRI may be considered for comprehensive postnatal evaluation of congenital thoracic malformations.

Recurrent Peumonia in an Infant With an Esophageal Lung

Esophageal lung is a rare variety of communicating bronchopulmonary foregut malformation with anomaolous communication between an isolated portion of respiratory tissue and esophagus. Children present in early life with recurrent cough and pneumonia. Majority of the reported cases are associated with other anomalies like tracheoesophageal fistula. We report a case of a 7-month-old girl with right sided esophageal lung who was misdiagnosed as dextrocardia with right sided pneumonitis.

Thoracoscopic (3-D) pneumonectomy of congenital bronchopulmonary foregut malformation in an infant

Abstract Congenital Bronchopulmonary Foregut Malformations (CBPFMs) are a wide spectrum of congenital malformations that arise due to a communication between the respiratory and alimentary tracts. Considering the rarity of the disease, there is a wide range of presentations, investigations and management options described in literature. We describe one such case that was managed by 3D thoracoscopic pneumonectomy and also discuss in detail our review of literature of almost 60 years about this condition.

Bronchopulmonary and vascular anomalies are frequent in children with oesophageal atresia.

Oesophageal atresia is frequently associated with other malformations, and our aim was to use computed tomography (CT) to explore intrathoracic malformations in patients with this condition. This was retrospective study of children aged 0-16 with oesophageal atresia who were born in 1996-2013 and followed up at the French reference centre for rare oesophageal diseases at the University of Lille. Computed tomography scans were available for 48 of the 234 patients during follow-up visits, and these were reviewed by a thoracic radiologist. More than two-thirds of the scans were performed to explore persistent respiratory symptoms. We found that six patients had a pulmonary malformations: four lobar agenesis, one right pulmonary aplasia and one congenital cystic adenomatoid malformation. Computed tomography enabled us to diagnose unexpected thoracic malformations in 16 patients: four lobar agenesis, six arteria lusoria, five persistent left superior vena cava and one partial anomalous pulmonary venous return. It also confirmed the diagnoses of suspected malformations in five patients: one congenital cystic adenomatoid malformation, one pulmonary hypoplasia, two right-sided aortic arches and one communicating bronchopulmonary foregut malformation. Intrathoracic anomalies were frequently associated with oesophageal atresia, and contrast-enhanced chest CT scans should be performed on patients with persistent respiratory symptoms.

Communicating Bronchopulmonary Foregut Malformation Type III with Pulmonary Sequestration Diagnosed in a Newborn: A Case Report

Communicating Bronchopulmonary Foregut Malformation Type III with Pulmonary Sequestration Diagnosed in a Newborn: A Case Report

Congenital bronchopulmonary foregut malformation: systematic review of the literature

BackgroundCongenital bronchopulmonary foregut malformation (CBPFM) is a rare congenital malformation involving both the digestive and respiratory system. Early diagnosis is difficult, and delayed recognition may result in considerable complications. The aim of the study was to identify and analyze the clinical characteristics and radiological features of CBPFMs.MethodsA systematic review was conducted in accordance with PRISMA guidelines. PubMed, Ovid database, EMBASE were searched for relevant publications to identify all published case-reports of CBPFM since 1992. Data about the demography, clinical presentation, pathology, imaging features, treatment and prognosis were collected.ResultsSixty-one cases were included in our study. Cases were aged from 1 day to 59 years with the majority aged 3 years or younger. The most common type was group III (37.7%), followed by group II (29.5%)group I (27.9%) and group IV (4.9%). The presentations included respiratory distress (32.8%), cough/choking following food intake and other presentations associated respiratory infection. Thirty-eight cases (62.3%) were diagnosed by upper gastrointestinal series (UGI). Misdiagnosis was common. Eight cases (13.1%) of the included cases died.ConclusionsEarly recognition and extensive delineation of the anatomy of CBPFM are important to correct these anomalies successfully. UGI is the first choice to confirm the abnormal bronchus communicating with the esophagus. Resection of abnormal pulmonary tissue, lobe or even unilateral lung is preferred. Reconstruction procedures are feasible in selected patients.

DOZ047.40: Bronchopulmonary and vascular anomalies are frequent in children with esophageal atresia

Abstract Introduction Esophageal atresia (EA) is frequently associated with other malformations although few data are available. Objective Describe tracheobronchial, pulmonary and/or vascular malformations in patients with EA using chest CT scans. Methods Monocentric retrospective study in children with EA, born between 1996 and 2013, who had a CT scan during their follow-up, reviewed by a pediatric radiologist. Results Among 234 children with EA, 48 patients underwent a CT scan available for interpretation, among which 69% were performed to explore persistent respiratory symptoms. Thirty-nine children (81%) were type III EA, 13 (27%) had a VACTERL association. Six patients (13%) had a pulmonary malformation: 4 lobar agenesis, 1 right pulmonary aplasia, and 1 congenital cystic adenomatoid malformation. All these patients presented with at least one associated malformation. Combined with the results of laryngotracheal endoscopy (n = 30), 43 patients (90%) had a tracheobronchial anomaly: tracheomalacia for 40 (83%), tracheal stenosis for 12 (25%), right tracheal bronchus for 2 (4%), communicating bronchopulmonary foregut malformation for 1 (2%). Combined with the results of echocardiography (n = 47), 7 patients (15%) had an isolated vascular anomaly, 8 (17%) had an isolated congenital heart disease and 7 (15%) had both. CT scans permitted the diagnosis of 6 pulmonary malformations (13%), 15 tracheobronchial anomalies (31%), and 2 vascular anomalies (4%). Only one patient (2%) in our study presented with an isolated EA. Conclusion Our study confirms the association of tracheobronchial, pulmonary, and vascular anomalies in patients with EA. Contrast-enhanced CT scan is complementary to echocardiography and laryngotracheal endoscopy in the exploration of persistent respiratory symptoms.