Successful Postnatal Tracheobronchoplasty for Unilateral Congenital High Airway Obstruction Syndrome due to Mainstem Bronchial Atresia.

INTRODUCTION: Unilateral congenital high airway obstruction syndrome (CHAOS) is caused by a complete obstruction of a mainstem bronchus with resulting hyperinflation and accelerated growth of one lung, severe mediastinal shift, and hydrops. Spontaneous perforation of the atresia has been observed in CHAOS which allows hydrops to resolve but hyperinflation, mediastinal shift and a critical airway obstruction persists as the perforation is usually pinhole-sized. CASE PRESENTATION: We present a case of unilateral CHAOS presenting at 26 2/7 weeks with observed-to-expected total lung volume (O/E TLV) of 203% with spontaneous perforation occurring at 28 weeks with resolution of hydrops but persistence of hyperinflation and mediastinal shift with an O/E TLV of 60.5% on 34 5/7 weeks' magnetic resonance imaging (MRI), successfully managed in a 35 5/7 weeks, 1,670 gm, growth restricted baby, by venoarterial extracorporeal membrane oxygenation (VA ECMO) and resection of the tracheobronchial atresia and tracheobronchoplasty on day of life 5. The baby was separated from ECMO on post-op day 12, required tracheostomy for positive end expiratory pressure for tracheomalacia at 4 months. CONCLUSION: At 2 years of age, she has met all developmental milestones, has been weaned to room air tracheostomy collar, and has been anticipating tracheal decannulation. There is persistent bronchiectasis in the hyperinflated right lung but no malacia. This is the first reported survivor of mainstem bronchial atresia suggesting the importance of preservation of the hyperplastic lung and airway reconstruction to normal long-term outcome.

Congenital lung lesions: a radiographic pattern approach.

Congenital lung malformations represent a spectrum of abnormalities that can overlap in imaging appearance and frequently coexist in the same child. Imaging diagnosis in the neonatal period can be challenging; however, the recognition of several archetypal radiographic patterns can aid in narrowing the differential diagnosis. Major radiographic archetypes include (1) hyperlucent lung, (2) pulmonary cysts, (3) focal opacity and (4) normal radiograph. Here we review the multimodality imaging appearances of the most commonly seen congenital lung malformations, categorized by their primary imaging archetypes. Along with the congenital lung malformations, we present several important imaging mimickers.

A single institution's experience with the management of peripheral bronchial atresia.

PURPOSE: Peripheral bronchial atresia is a pulmonary abnormality diagnosed on postnatal computed tomography after prenatal imaging reveals a congenital lung lesion. Debate regarding management of this abnormality prompted us to review our institution's practice patterns and outcomes. METHODS: All patients diagnosed with bronchial atresia were assessed from 6/2014 to 7/2020. Pediatric radiologists were surveyed to delineate computed tomography criteria used to diagnose peripheral bronchial atresia. Criteria were applied in an independent blinded review of postnatal imaging. Data for patients determined to have peripheral bronchial atresia and at least an initial pediatric surgical evaluation were analyzed. RESULTS: Twenty-eight patients with bronchial atresia received at least an initial pediatric surgical evaluation. Expectant management was planned for 22/28 (79%) patients. Two patients transitioned from an expectant management strategy to an operative strategy for recurrent respiratory infections; final pathology revealed bronchial atresia in both. Six patients were initially managed operatively; final pathology revealed bronchial atresia (n = 3) or congenital lobar overinflation (n = 3). CONCLUSIONS: Peripheral bronchial atresia can be safely managed expectantly. A change in symptoms is suspicious for alternate lung pathology, warranting further workup and consideration for resection. LEVEL OF EVIDENCE: Level IV.

Congenital bronchial atresia complicated by a lung abscess due to Aspergillus fumigatus: a case report.

BACKGROUND: Congenital bronchial atresia is a rare pulmonary abnormality characterized by the disrupted communication between the central and the peripheral bronchus and is typically asymptomatic. Although it can be symptomatic especially when infections occur in the involved areas, fungal infections are rare complications in patients with bronchial atresia. We report a case of congenital bronchial atresia complicated by a fungal infection. CASE PRESENTATION: A 30-year-old man with no previous history of immune dysfunction was brought to a nearby hospital and diagnosed with a left lung abscess. Although antimicrobial treatment was administered, it was ineffective, and he was transferred to our hospital. Since diagnostic imaging findings and bronchoscopy suggested congenital bronchial atresia and a fungal infection, he was treated with voriconazole and surgical resection was subsequently performed. A tissue culture detected Aspergillus fumigatus and histopathological findings were compatible with bronchial atresia. After discharge, he remained well and voriconazole was discontinued 5 months after the initiation of therapy. CONCLUSION: Bronchial atresia is a rare disease that is seldom complicated by a fungal infection, which is also a rare complication; however, physicians should consider fungal infections in patients with bronchial atresia who present with infections resistant to antimicrobial treatment.

Locating the Level and Extent of Congenital High Airway Obstruction: Fluid in the Airway Tract as Reference Points.

Described here are a series of four cases of congenital high airway obstruction in the fetus. All of the patients presented in the second trimester and all had hydrops fetalis. Three cases had bilateral hyperinflated lungs, midline shift of heart, flattening or inversion of the diaphragm, and fetal ascites. Autopsy was performed in one of these three and showed laryngeal atresia. In one fetus, there was only a unilateral huge enlargement of the lung with mediastinal shift. On autopsy, this fetus had atresia of right main bronchus. All parents had terminated the pregnancy following the prenatal diagnosis. Laryngeal atresia is an extremely rare fetal anomaly with dismal prognosis. It is important to differentiate the condition from other lesions with a more favorable prognosis, such as congenital adenomatoid malformation of the lung. Much research is needed in the future to explore the therapeutic options, including fetoscopic intervention or transplantation of stem cell-derived airways.

Anesthetic Management Using a Laryngeal Mask Airway in a Child With Congenital Bronchial Atresia.

Congenital bronchial atresia is a relatively rare malformation that causes a segmental obstruction of the bronchus during the fetal period. The peripheral lung distal from the obstructed bronchus becomes hyperinflated because of the unidirectional flow through collateral check-valve entry. Positive pressure ventilation during general anesthesia may cause a rupture of the bulla, resulting in pneumothorax. An 8-year-old girl, who had to undergo oral surgery, was diagnosed as having congenital bronchial atresia and one-fifth of her lung was poorly ventilated. We planned to perform general anesthesia under spontaneous respiration using a laryngeal mask, which was well tolerated.

In Utero Diagnosis of Unilateral Bronchial Atresia: One Pathologically Proven and 2 Presumed Cases With Similar Sonographic Findings at Presentation.

Unilateral bronchial atresia is a rare prenatal diagnosis that can be confused with other congenital lung abnormalities, particularly congenital pulmonary airway malformation. Accurate distinction between these entities is important for appropriate clinical care and prognosis. Familiarity of the key imaging and clinical features of unilateral bronchial atresia in comparison to other fetal lung abnormalities should increase the likelihood of achieving a timely and accurate diagnosis.

Recurrent pneumothorax associated with bronchial atresia: report of a case.

We herein report a case of recurrent pneumothorax associated with congenital bronchial atresia. A 26-year-old male presented with chest pain. Chest roentgenograms showed left pneumothorax, a left apical bulla and an area of hyperlucency in the left upper lung field, and chest computed tomography revealed a discontinuation of the left superior bronchus. Additionally, both ventilation and perfusion scintigraphy showed a defect in the left superior segment. A thoracoscopy-assisted left superior segmentectomy was performed, and a pathological examination indicated left superior segmental bronchial atresia, which might have predisposed the peripheral lung to emphysematous conditions. No relapse was observed 6 months after the operation. Although this entity is rare, congenital bronchial atresia should be considered in the differential diagnosis when a patient has suffered from a recurrent spontaneous pneumothorax.

A case of congenital bronchial atresia with tracheobronchial stenosis caused by emphysema: Successful management with thoracoscopic surgery.

Introduction: Congenital bronchial atresia (CBA), as a rare developmental abnormality of the lung, is usually asymptomatic and is accidently discovered in most cases. Currently, no standardized guidelines for the treatment or management of CBA have been established. Case presentation: A 22-year-old male soldier was referred to Shanghai Changhai Hospital, The First Affiliated Hospital of Naval Medical University due to chest tightness and shortness of breath after repeated strenuous activities. Contrast-enhanced computed tomography (CT) revealed an 18mm × 11mm solitary, well-circumscribed, and solid nodule with no enhancement in the right upper lobe (RUL), and emphysematous changes distributed throughout the RUL. A flexible bronchoscopic examination showed extrinsic compression stenosis in the bronchial opening of the right middle lobe (RML). After three-dimensional (3D) reconstruction CT and a multidisciplinary consultation, a diagnosis of CBA in the anterior segment (B3) of RUL was established. Subsequently, thoracoscopic right upper lobectomy was performed and resulted in an improved respiratory capacity 6 months after surgery. To date, the patient has good quality of life without any complication. Conclusion: This study underscores the role of bronchoscopy, 3D reconstruction CT, and a multidisciplinary consultation in the diagnosis of CBA, and highlights that a thoracoscopic intervention should be considered in such case.

Congenital Chest Lesions and Interventions.

Anomalies of the fetal chest require advanced imaging with ultrasound and MR imaging as well as expertise on the part of the interpreting pediatric radiologist. Congenital diaphragmatic hernia and congenital lung malformation are the most frequently seen, and in both conditions, the radiologist should provide both detailed anatomic description and measurement data for prognostication. This article provides a detailed approach to imaging the anatomy, in-depth explanation of available measurements and prognostic value, and keys to identifying candidates for fetal intervention. Less common congenital lung tumors and mediastinal and chest wall masses are also reviewed.

The Effect of Steroids on Prenatally Diagnosed Lung Lesions.

BACKGROUND: Open fetal resection for large lung lesions has virtually been replaced by maternal steroid administration. Despite this paradigm shift, little is known about the effects steroids have on lung lesion growth in utero. METHODS: A 10-year retrospective review of all prenatally diagnosed lung lesions cared for at our fetal care center was performed. We evaluated the effects of prenatal steroids on congenital pulmonary airway malformation (CPAM)-volume-ratio (CVR), distinguishing change in CVR among CPAMs, bronchopulmonary sequestrations (BPS), and bronchial atresias. We also correlated fetal ultrasound and MRI findings with pathology to determine the accuracy of prenatal diagnosis. RESULTS: We evaluated 199 fetuses with a prenatal lung lesion. Fifty-four (27 %) were treated with prenatal steroids with a subsequent 21 % mean reduction in the CVR (2.1 ± 1.4 to 1.1 ± 0.4, p = 0.003). Fetuses with hydrops and mediastinal shift who were treated with steroids rarely had resolution of these radiographic findings. Postnatal pathology was available for 91/199 patients (45.7 %). The most common diagnosis was CPAM (42/91, 46 %), followed by BPS (30/91, 33 %), and bronchial atresia (14/91, 15 %). Fetuses who received steroids and had pathology consistent with CPAM were more likely to have a reduction in their CVR (p = 0.02). Fetal ultrasound correctly diagnosed the type of lung lesion in 75 % of cases and fetal MRI in 81 % of cases. CONCLUSIONS: Prenatally diagnosed CPAMs are more likely to respond to maternal steroids than BPS or bronchial atresias. Knowing the diagnosis in utero could aid to steward steroid usage, however, fetal imagining modalities are not perfect in distinguishing subtype. LEVEL OF EVIDENCE: III.

Congenital bronchial atresia: diagnosis and treatment.

This study aimed to retrospectively summarize the clinical signs, diagnosis, and treatment of congenital bronchial atresia (CBA) in 12 patients. Chest radiographs and computed tomographic (CT) images of 12 patients with CBA treated in the Chinese People's Liberation Army General Hospital were reviewed. Analysis of chest radiographs revealed ten patients had hilar mass-like shadows and two had pneumonia-like shadows; most patients (n = 8) showed hyperlucency of the peripheral lung fields. CT revealed a mucocele in all the patients (n = 12); the mucoceles were round in four patients and club-like in eight. In 80% of the cases (n = 10), associated anomalies, including occlusions of the bronchus central to the mucocele, emphysematous changes of the peripheral lung fields, bronchogenic cyst, and anomalous branching of the bronchial tree and vascular structure were observed. CBA was detected in the right lobe in eight patients and the left lobe in the remaining four. No surgical intervention was performed in 5 CBA patients and the remaining 7 patients underwent surgery, including lobectomy in 5 patients and local resection in 2 patients. Among these 7 patients, 3 had a preoperative diagnosis of malignant disease, and the remaining 4 had severe clinical symptoms that could not be effectively treated by medicines. All patients were followed up, and none experienced obvious discomfort. CBA is a relatively rare and benign malformation disease. Chest CT is the procedure of choice for diagnosis. The presence of a bronchocele and surrounding emphysematous changes are typical radiologic findings in CBA. Surgery should be reserved only for patients with serious complications secondary to the atretic bronchus.

Unilateral hyper-translucent lung: a rare case report of bronchial atresia.

A young female patient with chronic left side chest pain warranted a work-up. Chest X-ray suggested left hyper-translucency. High resolution computed tomography scan revealed left atretic apicoposterior segmental bronchus with an area of hyper-translucency suggestive of bronchial atresia. Fiberoptic bronchoscopy findings were normal. Spirometry showed moderate restriction with mild obstruction. It is a rare developmental anomaly characterised by interruption of normal bronchial continuity. Most commonly, it involves the apicoposterior segmental bronchus of left upper lobe as seen in our case. Since it is a benign condition, surgical treatment or any aggressive method of treatment is not required. However, intervention may be required in case of life threatening complications.

Conservative management of congenital bronchial atresia: The Bambino Gesù children's hospital experience.

INTRODUCTION: Congenital bronchial atresia (CBA) is a rare airway malformation. No management guidelines exist because of limited evidence: treatment, surgical or conservative, is based on consensus and opinion. OBJECTIVE: To review the experience of a pediatric tertiary center and provide additional data about nonsurgical management of CBA and its outcomes after a structured follow-up, and to help formulate appropriate evidence-based guidelines. METHODS: A retrospective analysis of clinical, radiological, surgical, and pathological data of all pediatric patients with suspicion of CBA referred to the surgical department of the Bambino Gesù children's hospital of Rome between December 2013 and 2019, along with a review of the literature. RESULTS: Among the 18 children initially included in the study, 2 were lost to follow-up after radiological diagnosis, 4 underwent surgery for radiological suspicion of other pulmonary malformations. The final population is composed of 12 conservatively managed patients. At the end of the follow-up (median: 29 months, range 3-61), 1 patient (8%) was symptomatic. CONCLUSION: Conservative management for CBA appears to be safe. Surgery should be reserved for patients with symptomatic or complicated cases.

Thoracic MDCT findings of a combined congenital lung lesion: Bronchial atresia associated with congenital pulmonary airway malformation.

PURPOSE: To investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven combined congenital lung lesion consisting of bronchial atresia (BA) and congenital pulmonary airway malformation (CPAM) in children. MATERIALS AND METHODS: All pediatric patients (age ≤ 18 years) with a known pathological diagnosis of a combined BA-CPAM congenital lung lesion, who underwent thoracic MDCT studies from January 2011 to January 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung, including nodule, mass, cyst, ground-glass opacity, and consolidation. When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), borders (well-circumscribed vs. ill-defined), contrast enhancement pattern (nonenhancement vs. enhancement), and location (laterality, and lobar distribution) were also evaluated. Interobserver agreement between two independent reviewers was evaluated with κ statistics. RESULTS: Eighteen contrast-enhanced thoracic MDCT studies from 18 individual pediatric patients (8 males (44%) and 10 females (56%); mean age: 4.9 months; SD: 2.6; range: 1-10 months) with a pathological diagnosis of combined BA-CPAM congenital lung lesion comprised the final study population. The most frequent MDCT finding of combined BA-CPAM congenital lung lesion in children was a solitary (18/18; 100%), well-circumscribed (18/18; 100%), both solid and cystic (17/18; 94%) lesion with nonenhancing (17/17; 100%) nodule, reflecting the underlying BA component, adjacent to a well-circumscribed multicystic mass (18/18; 100%), representing the underlying CPAM component. This combined congenital lung lesion occurred in all lobes with similar frequency. There was almost perfect interobserver κ agreement between the two independent reviewers for detecting abnormalities on thoracic MDCT studies (k = 0.98). CONCLUSION: The characteristic thoracic MDCT findings of a combined BA-CPAM congenital lung lesion are a solitary, well-circumscribed solid and multicystic mass, with a nonenhancing nodule, reflecting the BA component, adjacent to a cystic mass, representing the CPAM component. Accurate recognition of these characteristic MDCT findings of combined BA-CPAM congenital lung lesion has great potential to help differentiate this combined congenital lung lesion from other thoracic pathology in children.

Pediatric Cystic Lung Lesions: Where Are We Now?

Pediatric cystic lung lesions have long been a source of confusion for clinicians, radiologists, and pathologists. They encompass a wide spectrum of entities with variable prognostic implications, including congenital lung malformations, pulmonary neoplasms, and hereditary conditions. As our understanding of the developmental and genetic origins of these conditions has evolved, revised nomenclature and classifications have emerged in an attempt to bring clarity to the origin of these lesions and guide clinical management. This review discusses cystic lung lesions and the current understanding of their etiopathogenesis.

"Not all bullae are synonymous with COPD"-a rare congenital anomaly described.

Hyperlucent areas with thin walls and absent vascular markings in chest X ray are described as radiological findings of a bullae. We present the case of an adult male referred for coronary revascularisation and bullectomy in the right lung. A non-smoker, without any significant past medical history, made us think of bronchial atresia. He was planned for coronary artery bypass grafting with close follow-up of lung anomaly. Clinicians should be aware of this entity in non-smokers with unilateral bullous lesion and calcification as other close clinical differentials warrant aggressive medical management.

Fetal Congenital Peripheral Bronchial Atresia Diagnosed by Magnetic Resonance Imaging: Two Case Reports.

Two types of congenital bronchial atresia (proximal and peripheral) have been classified. We report two cases of peripheral bronchial atresia diagnosed by prenatal ultrasonography (US) and magnetic resonance imaging (MRI). Evaluating an enlarged lung mass that is homogeneously hyperechoic on US and hyperintense on T2-weighted MRI can help in determining whether bronchial atresia is present. Proximal type is suggested when a dilated main bronchus is observed as a tubule structure of an involved lung hilum. In our cases, T2-weighted MRI revealed homogeneously hyperintense lung lesion with decreased signal intensity of adjacent lobe, flattening diaphragm, and mediastinal shift. Dilatation of the main bronchus was not observed and the opposite lung was normal in appearance. These findings were explained by secondary compression due to enlargement of the involved lung. The preservation of vascular structure and the retained normal shape, though enlarged, in the affected lobe were observed, which demonstrated undisrupted pulmonary architecture of the lobe. Thus, congenital cystic adenomatoid malformation was excluded because pulmonary architecture was relatively preserved. Finally, presumed diagnoses of the peripheral bronchial atresia were made and confirmed by postnatal chest computed tomography.

Predictors of early lobectomy after birth in prenatally diagnosed congenital pulmonary airway malformation.

PURPOSE: The purpose of this study was to clarify the relationship between congenital pulmonary airway malformation volume ratio (CVR) of bronchial atresia (BA), CVR of congenital cystic adenomatoid malformations (CCAM), and time of surgery after birth. METHOD: We retrospectively analyzed data of 36 BA and CCAM cases, prenatally diagnosed as CPAM from 2009 through 2014. RESULTS: Within 2 h after birth, 12 neonatal patients underwent emergent (EMG) lobectomy. Five cases of lobectomy were performed urgently (UG) from 12 to 48 h after birth. Four cases of lobectomy were required within 30 days after birth (early = EAG). We performed lobectomy in 15 other patients at 11 months after birth (late = LG). Of the EMG cases, 11 were macrotype CCAM (maximal CVR >2.0), and 4 of 5 UG cases were microtype CCAM (CVR >2.0). Of the EAG cases, 3 of 4 were macrotype CCAM with CVR of <1.5. Of 15 LG, 13 were BA and showed a CVR of 0.13-3.0 (median, 0.78). The CVR of the cases operated on within 48 h after birth was significantly larger than that of the cases operated on after 2 weeks (p = 0.001). CONCLUSION: EMG or UG lobectomy was usually required after birth in CCAM, indicating maximal CVR >2.0. By contrast, elective surgery was performed in most BA cases. LEVEL OF EVIDENCE: IV.