Bronchial Isomerism Research Articles

Left Bronchial Isomerism with Right-Sided Tracheal Bronchus: A Rare Case Report

The tracheal bronchus is a congenital bronchial branching anomaly defined as an aberrant bronchus arising in either the trachea or a main bronchus. Left bronchial isomerism is characterized by two bilobed lungs, bilateral long main bronchi, and both pulmonary arteries passing superiorly to their respective upper lobe bronchi. Left bronchial isomerism with a right-sided tracheal bronchus is a very rare combination of tracheobronchial anomalies. It has not been previously reported. We present multi-detector CT findings of a left bronchial isomerism with a right-sided tracheal bronchus of a 74 year old man.

Classifying Cardiac Anomalies in Right and Left Isomerism: Concordant and Discordant Patterns

Aims: Evidence is emerging that, in the setting of isomerism, the atrial and bronchial arrangement are not always concordant, nor are these patterns always harmonious with the arrangement of the abdominal organs. We aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism, seeking to determine whether it was feasible to assess complexity on this basis, in this regard taking note of the potential value of bronchial as opposed to appendage morphology. Methods and Results: We studied 78 patients known to have isomerism of the bronchuses, 43 with right and 35 with left isomerism. Appendage anatomy could be determined in 49 cases (63%), all but one of these being concordant with bronchial anatomy. When assessing abdominal features, in only 59 cases (76%) was splenic morphology in keeping with the thoracic findings. As expected, right isomerism was associated with greater complexity of cardiac malformations, with an odds ratio of 6.53, with confidence intervals from 2.2–19.3 (p < 0.001). The odds were slightly decreased with thoraco-abdominal disharmony, when lesions shown to carry higher risk were then found in the setting of left isomerism. Conclusion: Harmony is excellent between bronchial and appendage isomerism, but less so with the arrangement of the abdominal organs. Right isomerism in our cohort, was indicative of a six-fold increase in intracardiac complexity. When discordance was found between the systems, however, the cardiac anomalies were less typical of the anticipated findings for right vs. left isomerism of the appendages.

A confounding bronchopulmonary situs abnormality after bilateral lung transplantation

A 36-year-old female with a past medical history of primary ciliary dyskinesia and bilateral lung transplantation was noted to have a rare and confounding postsurgical anatomy acquired as a result of transplantation. Bronchoscopy and computed tomography showed isomeric main bronchi with a tri-lobed right lung, a bi-lobed left lung and dextrocardia. This rare phenomenon can be observed in lung transplant recipients with situs ambiguous morphology of their native lungs, who receive donor lungs with normal situs solitus morphology. To the best of our knowledge, ours is the first reported case of such a composite bronchopulmonary situs abnormality. Careful review of bronchial anatomy should be done with the help of CT imaging prior to undertaking any bronchial interventions in these subset of patients with bronchial isomerism and bilateral lung transplantation.

Fetal Ultrasonic Evaluation of Bronchial Morphology in Fetuses with Isomerism

Objective: The aim of the study was to evaluate whether fetal ultrasound could determine bronchial isomerism and distinguish left isomerism from right isomerism. Methods: We identified 110 healthy fetuses and 28 fetuses with isomerism. The outer angle between the tracheal midline and the inner margin of the bronchus is measured. The bronchial angles and the ratio of left/right bronchial angle were used to differentiate bronchial morphology and confirm the presence of bronchial isomerism in pathological cases. Results: The normal angles of the left and right bronchi were 146.98° (95% CI, 145.15–147.81°) and 167.37° (95% CI, 166.30–168.44°), separately. The cutoff bronchial angle of 156.5° was used to distinguish left bronchus from right bronchus. The bronchial isomerism could be identified in all pathological cases by autopsy and bronchial-atrial concordance occurred in 27 pathological cases (96.4%). In 21 pathological cases, the bilateral bronchial angle was <156.5 versus >156.5 differentiated left from right isomerism, respectively. The ratio of the left/right bronchial angle of >0.935 identified 92.9% (26/28) of all pathological cases, with a sensitivity of 89.7%. Conclusions: Fetal ultrasound can detect the bronchial morphology and the presence of bronchial isomerism in fetuses with isomerism according to bronchial angles and the ratio of left/right bronchial angle.

Diagnostic Accuracy of Three-dimensional Turbo Field Echo Magnetic Resonance Imaging Sequence in Pediatric Tracheobronchial Anomalies with Congenital Heart Disease

Tracheobronchial anomalies are common in congenital heart disease (CHD), including tracheobronchial stenosis, tracheal bronchus, cardiac bronchus, and bronchial isomerism, which can cause varying degrees of respiratory illness. It is necessary to assess tracheobronchial anomalies and make a preoperative airway evaluation. Multi-slice computed tomography (MSCT) and cardiac magnetic resonance imaging (MRI) are the most effective noninvasive modalities for the diagnosis of CHD and the associated tracheobronchial anomalies. However, MSCT remains an ionizing procedure despite using low dose protocols. The aim of this study was to evaluate diagnostic accuracy of tracheobronchial anomalies in patients with CHD using three-dimensional turbo field echo(3D-TFE) magnetic resonance imaging sequence for preoperative airway evaluation. The results indicated that 3D-TFE provided better image quality as compared to that of 3D-balanced turbo field echo (3D-bTFE), and it can clearly demonstrated the tracheobronchial tree and tracheobronchial anomalies in CHD. This study confirms the clinical value of 3D-TFE in diagnosing tracheobronchial anomalies and supply helpful tracheobronchial information for preoperative strategies and postoperative follow-up.

Tracheobronchial Branching Abnormalities: Lobe-based Classification Scheme-Erratum.

HomeRadioGraphicsVol. 36, No. 4 Previous ErrataFree AccessTracheobronchial Branching Abnormalities: Lobe-based Classification Scheme—ErratumG Chassagnon, B Morel, E Carpentier, H Ducou Le Pointe, D SirinelliG Chassagnon, B Morel, E Carpentier, H Ducou Le Pointe, D SirinelliG ChassagnonB MorelE CarpentierH Ducou Le PointeD SirinelliPublished Online:Jul 11 2016https://doi.org/10.1148/rg.2016164003MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Originally published in:RadioGraphics 2016;36(2):358–373 • DOI: 10.1148/rg.2016150115Tracheobronchial Branching Abnormalities: Lobe-based Classification SchemeErratum in:RadioGraphics 2016;36(4):1258 • DOI: 10.1148/rg.2016164003Page 361, column 1, lines 10–19: The sentences should read as follows: “Right isomerism (formerly known as asplenia syndrome or Ivemark syndrome) is typically associated with right bronchial isomerism, bilateral right morphologic atria, asplenia, midline liver, and variably located stomach. In contrast, left visceroatrial isomerism (formerly known as polysplenia syndrome [not or Ivemark syndrome]) is usually associated with left bronchial isomerism, bilateral left morphologic atria, polysplenia, midline liver, and variably located stomach.”Article HistoryPublished online: July 11 2016Published in print: July 2016 FiguresReferencesRelatedDetailsCited ByAirflow in Tracheobronchial Tree of Subjects with Tracheal Bronchus Simulated Using CT Image Based Models and CFD MethodShouliangQi, BaihuaZhang, YongYue, JingShen, YueyangTeng, WeiQian, JianlinWu2018 | Journal of Medical Systems, Vol. 42, No. 4Tracheal bronchus diagnosed in children undergoing flexible bronchoscopyEstelaPérez Ruiz, PilarCaro Aguilera, Ana IsabelValdivielso, SoniaSanchís Cárdenas, YazminaMartínez García, JavierPérez Frías2018 | Paediatric Respiratory Reviews, Vol. 28Accompanying This ArticleTracheobronchial Branching Abnormalities: Lobe-based Classification SchemeFeb 1 2016RadioGraphicsRecommended Articles Fetal Cardiac US: Techniques and Normal Anatomy Correlated with Adult CT and MR ImagingRadioGraphics2017Volume: 37Issue: 4pp. 1290-1303Septal Atrioventricular Junction Region: Comprehensive Imaging in AdultsRadioGraphics2016Volume: 36Issue: 7pp. 1966-1986Biatrial Drainage of the Right Superior Vena Cava: Imaging FindingsRadiology: Cardiothoracic Imaging2020Volume: 2Issue: 6Cardiac Type of Partial Anomalous Pulmonary Venous ConnectionRadiology: Cardiothoracic Imaging2020Volume: 2Issue: 1Case 274: Cardiac EchinococcusRadiology2020Volume: 294Issue: 2pp. 478-481See More RSNA Education Exhibits Fetal Cardiac MRI for Structural Congenital Heart DiseaseDigital Posters2018The Puzzle of Describing Congenital Heart Disease: Recognizing the Individual Pieces and Connecting Them Together using Anderson’s Sequential Segmental AnalysisDigital Posters2019Cased-based Imaging Review of Fetal Venous System AnomaliesDigital Posters2020 RSNA Case Collection Horseshoe Adrenal GlandRSNA Case Collection2022Partial anomalous pulmonary venous returnRSNA Case Collection2021Malposition of Umbilical Venous CatheterRSNA Case Collection2020 Vol. 36, No. 4 Metrics Altmetric Score PDF download

Determining bronchial morphology for the purposes of segregating so-called heterotaxy.

Introduction Heterotaxy is a unique clinical entity in which lateralisation of the thoraco-abdominal organs is abnormal, typically with isomerism of the bronchial tree and atrial appendages. This study was carried out to determine whether routine clinical imaging such as chest radiographs, angiographic images, and CT/MRI can determine bronchial isomerism, and how sidedness of bronchial isomerism correlates with overall features anticipated in hearts with isomeric atrial appendages. Methods and results We identified 73 patients with heterotaxy, in whom imaging clearly demonstrated the bronchial tree, seen at our institution since 1998. We calculated bronchial angles and lengths using all the available imaging modalities to determine the presence and sidedness of bronchial isomerism. This was then compared with the anticipated presence of isomeric atrial appendages based on the overall clinical findings, as the appendages themselves had not specifically been imaged. The ratio of bronchial lengths revealed bronchial isomerism in all patients, with bronchial angles permitting distinction of right as opposed to left isomerism. We noted discordances between the identified bronchial isomerism and the presumed arrangement of the atrial appendages in nearly 20% of the patients in our cohort. Routine clinical imaging with chest radiographs, angiographic imaging, and CT/MRI can determine the presence of bronchial isomerism in patients with so-called heterotaxy. Right as opposed to left isomerism can be distinguished based on bronchial angles. The finding of bronchial isomerism correlates well, but not totally, with the presumed isomerism of the atrial appendages as predicted from the identified intra-cardiac morphology.

The importance of being isomeric.

In the normal individual, the parietal components of the body are mirror-imaged and appropriately described as isomeric. The thoraco-abdominal organs, in contrast, are lateralized. However, in "visceral heterotaxy," the thoraco-abdominal organs also show some degree of isomerism, best seen in the arrangement of the bronchial tree. Whether isomerism can be found within the heart remains controversial. One of two recent publications in this journal emphasized the crucial features of bronchial isomerism; the other, in contrast, confused the situation of isomerism within the heart. In this review, we show how the topic of cardiac isomerism is clarified by concentrating on the anatomical features of the cardiac components and determining how best they can be described. Appropriate manipulation of developing mice produces unequivocal evidence of isomerism of the atrial appendages, but with no evidence of ventricular isomerism. In hearts from patients with so-called "heterotaxy," only the atrial appendages, distinguished on the basis of the pectinate muscles lining their walls, are uniformly isomeric, permitting the syndrome to be differentiated into the subsets of left as opposed to right atrial appendage isomerism. Thus, controversies are defused by simply describing the isomerism of the atrial appendages rather than "atrial isomerism," recognizing the frequency of abnormal venoatrial connections in these settings. Any suggestion of ambiguity is removed by the equally simple expedient of describing all the variable cardiac features, describing the arrangements of the thoracic and abdominal organs separately should there be discordances.

Exercises in anatomy: cardiac isomerism.

It is well recognized that the patients with the most complex cardiac malformations are those with so-called visceral heterotaxy. At present, it remains a fact that most investigators segregate these patients on the basis of their splenic anatomy, describing syndromes of so-called asplenia and polysplenia. It has also been known for quite some time, nonetheless, that the morphology of the tracheobronchial tree is usually isomeric in the setting of heterotaxy. And it has been shown that the isomerism found in terms of bronchial arrangement correlates in a better fashion with the cardiac anatomy than does the presence of multiple spleens, or the absence of any splenic tissue. In this exercise in anatomy, we use hearts from the Idriss archive of Lurie Children's Hospital in Chicago to demonstrate the isomeric features found in the hearts obtained from patients known to have had heterotaxy. We first demonstrate the normal arrangements, showing how it is the extent of the pectinate muscles in the atrial appendages relative to the atrioventricular junctions that distinguishes between morphologically right and left atrial chambers. We also show the asymmetry of the normal bronchial tree, and the relationships of the first bronchial branches to the pulmonary arteries supplying the lower lobes of the lungs. We then demonstrate that diagnosis of multiple spleens requires the finding of splenic tissue on either side of the dorsal mesogastrium. Turning to hearts obtained from patients with heterotaxy, we illustrate isomeric right and left atrial appendages. We emphasize that it is only the appendages that are universally isomeric, but point out that other features support the notion of cardiac isomerism. We then show that description also requires a full account of veno-atrial connections, since these can seemingly be mirror-imaged when the arrangement within the heart is one of isomerism of the atrial appendages. We show how failure to recognize the presence of such isomeric appendages can lead to spurious diagnoses of discordant atrioventricular connections. Overall, we show that the finding of isomeric atrial appendages, guided by the finding of bronchial isomerism, is but the prelude to full segmental sequential analysis.

Bronchial isomerism in a Kabuki syndrome patient with a novel mutation in MLL2gene

BackgroundKabuki syndrome (KS) is a rare, multiple congenital anomalies/intellectual disability syndrome caused by mutations of MLL2 gene, which codifies for a histone methyltrasferase that regulates the embryogenesis and the tissue development. Left-bronchial isomerism is a rare congenital abnormality that can be defined as the absence of the normal lateralizing features which distinguish right and left-sides in the lungs. To date, this is the first report of left-bronchial isomerism in association with KS.Case presentationA one-month-old Caucasian male patient underwent our attention for microcephaly, dysmorphic features (long palpebral fissures, eyebrows with sparse lateral third, everted lower eyelids, blue sclerae, large dysplastic ears, lower lip pits), persistent fetal fingertip pads, short stature, heart defects (interventricular defect and aortic coarctation), unilateral cryptorchidism, hypotonia and delay in gross motor skills. These features suggested a diagnosis of KS and a molecular analysis confirmed a novel frame-shift mutation in the exon 11 of MLL2 gene. Subsequently, given recurrent respiratory infections with a normal immunological status, he underwent a chest CT scan that showed a left bronchial isomerism.ConclusionWe report a patient affected by KS, with a novel MLL2 mutation and an atypical phenotype characterized by left-side bronchial isomerism. Interestingly, genes involved in the heterotaxia/isomerism such as ROCK2 and SHROOM3 are known to interact with MLL2 gene. In order to achieve a correct diagnosis and an appropriate therapy, the presence of pulmonary anatomical variations should be investigated in KS patients with respiratory signs not associated to immunological deficiency. Finally, our findings support the hypothesis that the mutations leading to a complete loss of function of MLL2 gene is often associated with complex visceral malformations.

Right Pulmonary Artery Sling in a Single Lung with Bronchial Isomerism

Pulmonary artery slings are rare congenital anomalies seen only on the left side. The presence of right pulmonary artery (RPA) sling is extremely rare. We report a case of a RPA sling with a single lung associated with tracheal stenosis and its management.

Right-sided bronchial isomerism diagnosed in adulthood

This case report describes a diagnosis of right-sided isomerism and specifically right-sided bronchial isomerism in a patient who was being investigated for deteriorating bronchiectasis. Right-sided bronchial isomerism is a variation of the normal bronchial anatomy (situs solitus) consisting of a left lung that is identically configured at the bronchial and lobar level to the right. It is sometimes referred to as bilateral right lung and is usually associated with congenital asplenia and therefore impaired immunity with susceptibility to pneumococcal sepsis and cardiac abnormalities which may be severe and result in a high mortality in infancy. Ivemark syndrome (also known as right atrial isomerism) combines these associations with malrotation of the gut and a midline liver.2 Interestingly, left-sided isomerism is associated with polysplenia as well as midline liver, malrotation of the gut, partially anomalous pulmonary venous drainage and cardiac septal defects.3 To the best of our knowledge cases of right-sided isomerism are sufficiently rare in adulthood that there are only two other reports in the literature and only one of the patients had bronchial isomerism.4,5.

A new syndrome with noncompaction cardiomyopathy, bradycardia, pulmonary stenosis, atrial septal defect and heterotaxy with suggestive linkage to chromosome 6p

We report a three-generation family with nine patients affected by a combination of cardiac abnormalities and left isomerism which, to our knowledge, has not been described before. The cardiac anomalies include non-compaction of the ventricular myocardium, bradycardia, pulmonary valve stenosis, and secundum atrial septal defect. The laterality sequence anomalies include left bronchial isomerism, azygous continuation of the inferior vena cava, polysplenia and intestinal malrotation, all compatible with left isomerism. This new syndrome is inherited in an autosomal dominant pattern. A genome-wide linkage analysis suggested linkage to chromosome 6p24.3-21.2 with a maximum LOD score of 2.7 at marker D6S276. The linkage interval is located between markers D6S470 (telomeric side) and D6S1610 (centromeric side), and overlaps with the linkage interval in another family with heterotaxy reported previously. Taken together, the genomic region could be reduced to 9.4 cM (12 Mb) containing several functional candidate genes for this complex heterotaxy phenotype.

Isolated and Complex Scimitar Vein Anomalies and Their Differentiation from the Meandering Right Pulmonary Vein

PurposeFour pediatric patients with isolated (the adult form) and complex (the infantile form) scimitar vein anomalies were reviewed and compared with patients with meandering right pulmonary veins.Materials and MethodsFrom January 1990 to December 2006, 4 female patients, aged 2 days to 3.5 years, with isolated and complex scimitar vein anomalies were retrospectively studied. The clinical features, chest radiographs, echocardiography, magnetic resonance imaging, magnetic resonance angiography, computer tomography, bronchography, cardiac catheterization with angiography, surgery, and autopsy were reviewed to substantiate the diagnosis of isolated and complex scimitar vein anomalies.ResultsClinical manifestations were lung infections with radiographic scimitar signs in 4 patients (3 right; 1 left), respiratory distress, congestive heart failure, pulmonary hypertension and/or cyanosis in 3 patients, dextrocardia, heterotaxy, and/or right atrial isomerism in 2 patients, as well as dextroversion, right bronchial isomerism, bronchial stenosis, and/or sepsis in 1 patient. Two patients with right atrial isomerism expired.ConclusionThe clinical discrimination between scimitar vein anomaly with and without cardiovascular and bronchopulmonary malformations is crucial for the outcomes vary.

Left Bronchial Isomerism

Left Bronchial Isomerism

Left bronchial isomerism, normal atrial arrangement and bronchomalacia mimicking asthma: a new syndrome?

Three children who presented with steroid-resistant airflow obstruction are described. They all had bronchomalacia in the setting of a rare visceral arrangement, namely left bronchial isomerism with normal atrial arrangement. Imaging and, in two cases, a normal residual volume in the face of severe airflow obstruction were diagnostic pointers to a nonasthmatic cause of wheeze. Although the association of these abnormalities may be coincidental, together they may constitute a new clinical syndrome.

Left bronchial isomerism associated with bronchomalacia, presenting with intractable wheeze.

The cause of the Williams Campbell syndrome (bronchomalacia with bronchiectasis) is controversial. A boy with bronchomalacia, bifid ribs, and left bronchial isomerism presented with intractable wheeze mimicking asthma. The combination of the abdominal, bronchial, and atrial anatomy seen in this child has been described only once previously. The coexistence of these congenital abnormalities in this boy supports a congenital cause for the Williams Campbell syndrome. The need to assess wheezy children critically is emphasised.

Identification and analysis of left atrial isomerism

The veno-atrial connections, atrial morphology, atrioventricular (AV) junction, ventricular mass, ventriculoarterial (VA) connection and great arteries in 22 autopsied hearts, diagnosed as having bilateral left-sidedness because of the morphology of the atrial appendages, were studied. The findings were correlated with the arrangement of the thoracic-abdominal organs. A solitary spleen was found in 3 and double spleens in 2 hearts (the remaining 17 hearts had multiple spleens) but left bronchial isomerism existed in all hearts in which bronchial arrangement could be determined. The heart was in the left chest in 14 cases, in the right chest in 5 and midline in 3. The apex pointed to the left in 18 hearts while in 4 hearts it pointed to the right. Fifteen hearts had a biventricular and ambiguous AV connection, 3 hearts had an absent left AV connection and 4 had double-inlet connection via a common valve (to the left ventricle in 3 and the right ventricle in 1). The VA connection was concordant in 14 hearts, discordant in 1, double outlet from the right ventricle in 4, double outlet from a solitary indeterminate ventricle in 1 and single outlet from the right ventricle through a pulmonary trunk with aortic atresia in 2 hearts. Superior caval veins were present bilaterally in 13 hearts. There was interruption of the infrahepatic inferior caval vein with azygos or hemiazygos continuation in 19 hearts while in 3 hearts the inferior caval vein continued upwards to drain into the right-sided morphologically left atrium. The hepatic veins drained bilaterally in 9 hearts, via a supraheptic confluence in 8 hearts and separately to one or the other atrium in 5 hearts. The pulmonary veins were bilaterally connected to the atria in 11 hearts. Aortic outflow tract atresia or obstruction was present in 10 hearts.

Intralobar pulmonary sequestration in conjunction with bronchial isomerism.

<h3></h3> The traditional hospice setup has been developed with silos across therapeutic services - resulting in families receiving multiple referrals for support with little or no understanding of the wider influences and having to retell their stories. Combining Social work, Counselling, Physio, OT, Creative therapy, Complementary therapy, Family support and Spiritual care into a wellbeing department streamlines the process with one referral, one holistic assessment, one MDT and one family-centred therapeutic plan. Families access the right support at the right time. <h3>Aims</h3> Early intervention; holistic family support; integrated services; robust referral pathways; preventing crisis. <h3>Methods</h3> Internal audits. Review of current counselling demand. Review of best practice guidance. Embedding a social care model for social and wellbeing needs. Client feedback. Evaluations. Referral trends. <h3>Results</h3> Reduction in counselling waiting lists. Improved early intervention to prevent crisis. Streamlined referral processes – one referral to wellbeing services. Improved multidisciplinary working. Strengthen relationships with stakeholders. Embedded holistic family assessments. <h3>Conclusion</h3> Therapeutic services improve wellbeing and should be integrated. Families consistently tell professionals that they do not want to keep repeating their stories and communication is key. To provide person-centred care we must look at all the influencing factors and family dynamics and support play a key role. To improve family wellbeing, we must look at the whole picture. This way of working is innovative in hospices but best practice in all therapy and family-based settings. Further research in hospices could strengthen this practice.

Use of high kilovoltage filtered beam radiographs for detection of bronchial situs in infants and young children.

Determination of atrial situs is of cardinal importance in the analysis of complex congenital heart lesions, and is best predicted from bronchial situs. Previous methods for assessing bronchial morphology, however, are unsuited to the very young patient. To assess bronchial morphology, 100 consecutive patients under 18 months of age (medium 57.5 days) with suspected congenital heart disease were studied by high kilovoltage filtered beam radiographs, before cardiac catheterisation. This low radiation dose technique clearly defined bronchial anatomy in 95 patients. The lengths of the left and right main bronchi were compared and 10 cases (10%) had a ratio less than 1.5 suggesting bronchial isomerism. Discriminant function analysis based on tracheal width and bronchial length enabled clear distinction of right from left bronchi. Four patients had bilateral right and six had bilateral left bronchi. Four of these 10 cases died and had necropsy confirmation of the radiological diagnosis. Practical prediction about cardiac anatomy, particularly the systemic and pulmonary venous return, may be made when bronchial morphology is known.