Imaging spectrum of horseshoe lung: A series of four cases.

Horseshoe lung (HL) is a rare congenital anomaly represented by the fusion of both lungs, posterior to the heart, and is typically associated with various bronchopulmonary and cardiovascular malformations. Multi-detector contrast enhanced CT is the imaging modality of choice to demonstrate the pathology and associated malformations. There has been inconsistency in the nomenclature used for such cases in literature. To resolve ambiguity, the authors emphasise that only two terms: HL and pseudo-HL be used on imaging to describe variants of this congenital malformation. Contribution: A description of the imaging features in four cases of HL, with their associated malformations and a review of the nomenclature.

Bilateral thoracotomy for horseshoe lung complicated with severe aspergilloma: a case report.

BACKGROUND: Horseshoe lung is a rare congenital malformation in which the lung protrudes from the mediastinum to the other side. Owing to the high frequency of other fatal cardiovascular complications, it is often diagnosed in childhood and rarely unnoted until adulthood. We report a case of horseshoe lung in an older patient who underwent thoracotomy. CASE PRESENTATION: The patient was a 69-year-old man with chronic obstructive pulmonary disease (COPD) and a history of heavy smoking. The patient was admitted to the hospital because of acute exacerbation of COPD. Computed tomography revealed horseshoe lung and pulmonary sequestration with pneumonia. This was the first time that he was diagnosed with horseshoe lung; however, he had been treated for pneumonia multiple times before. Surgery for the horseshoe lung was recommended; however, the patient declined it because his symptoms of acute COPD exacerbation were relieved by medication. Aspergillus infection of the horseshoe lung led to frequent bloody sputum, and the patient's respiratory condition gradually worsened. Two years after the initial diagnosis, the patient decided to undergo the surgery. Surgery was performed in the order of left and right thoracotomies, with posterolateral thoracotomies performed bilaterally. Surgery was difficult because of strong adhesions around the inflamed lung; however, the lung was removed in one lump. The patient was extubated on postoperative day (POD) 1, and rehabilitation was initiated. His high sputum volume caused postoperative pneumonia, and the patient was again placed on a ventilator on POD 9. He underwent open-window surgery for concomitant pyothorax. The patient was weaned off the ventilator when the inflammation improved and was discharged on POD 133. The patient lived at home, developed severe pneumonia 4 months later, and died of respiratory failure. CONCLUSION: Pulmonary sequestration and horseshoe lungs are congenital malformations that require surgery. The selection of the optimal time for surgery is important.

Holt-Oram Syndrome with Pulmonary Involvement-A Valuable Algorithm to Follow.

Holt-Oram syndrome comprises a rare spectrum of congenital cardiovascular and appendicular skeletal anomalies. However, only a few cases have reported lung involvement in Holt-Oram syndrome. We reported the rare case of a 1-year-old male child patient who presented with upper limb abnormalities and respiratory distress and was diagnosed with pulmonary agenesis and pulmonary arterial hypertension secondary to an atrial septal defect.

Rare case of horseshoe lung associated with scimitar syndrome in Palestine: a case report.

The caudal and basal parts of the lungs are fused together in a rare congenital bronchopulmonary anomaly known as horseshoe lung. The majority of horseshoe lung cases are associated with scimitar syndrome. Most patients present with nonspecific symptoms. Multidetector pneumoangiography can be used to diagnose horseshoe lung, which shows that the isthmus of the pulmonary parenchyma traverses the midline, connecting the two lungs together. Treatment and prognosis are usually determined depending on the presence of other concomitant anomalies and the degree of symptom severity. Case Presentation: A 3-month-old-male patient presented with respiratory symptoms and a history of chest infection. Chest imaging revealed anomalous venous drainage from the right lower lobe of the lung, right lung hypoplasia with mediastinal shift, and a parenchymal isthmus extending between the two lungs. The patient was diagnosed with horseshoe lungs associated with scimitar syndrome. He was also found to have extralobar sequestration to the right lower lobe of the lung. The patient underwent surgical management in the form of tunneling of the anomalous vein into the left atrium using pericardium autograft ligation of the sequestration artery. Clinical Discussion: Because of its common association with other congenital malformations such as scimitar syndrome and cardiovascular defects, clinicians should be meticulous in the investigation and workup process of patients with horseshoe lung in order not to miss any of these associated abnormalities. Conclusion: Although it is very rare, horseshoe lung should be considered in the differential diagnosis of respiratory distress symptoms, especially in children younger than 1 year.

Rarer Components of a Rare Disease: Accessory Hemidiaphragm and Horseshoe Lung with Scimitar Syndrome; Two Case Reports.

INTRODUCTION: Scimitar syndrome is a rare developmental anomaly with an incidence of 2/100.000 births. Major components of this disease are partial anomalous pulmonary venous drainage, pulmonary hypoplasia, systemic arterialization of the right basal lung, and dextroposition of the heart. Horseshoe lung and accessory hemidiaphragm are two rarer components of this disease. CASE PRESENTATION: In this paper, horseshoe lung and accessory diaphragm associated with Scimitar syndrome have been reported in two cases. CONCLUSION: In conclusion, being aware of rare manifestations of rare diseases is important to fully describe the pathologic spectrum of the disease. This will assist in better management and decision-making process.

Adult congenital horseshoe lung with bilateral pulmonary sequestration: A case report.

Horseshoe lung (HL) is an infrequent congenital lung anomaly. Its main feature is that the lower lungs on both sides extend behind the pericardium and fuse across the midline, usually accompanied by pulmonary dysplasia. It is reported that 80% of HL is relevant to the abnormal return of some pulmonary veins from the right lung to the inferior vena cava or right atrium (scimitar syndrome). Most patients are within 5 years old, most commonly within 1 year old, but HL may also have no apparent clinical symptoms or mild symptoms. This case is a 36-years-old adult female who developed left chest pain more than a month ago and continued to worsen for 10 days. The patient also had repeated pulmonary infection with cough and expectoration.

Is horseshoe lung a component of VACTERL spectrum? Case report and review of literature.

Horseshoe lung (HL) is a rare congenital anomaly that has been classically associated with Scimitar syndrome. Very few cases have been described in the context of the VACTERL spectrum. We present a case of a newborn girl with mesocardia, tracheoesophageal fistula, and imperforated anus, who required O2 support at birth and during hospitalization. A chest CT angiography revealed a HL as an incidental finding. We suspect that HL and the VACTERL spectrum, are not separated entities but likely a further expansion of VACTERL-associated symptoms. HL might be underdiagnosed in asymptomatic patients as Chest CT angiography is not part of the routine work up for patients with VACTERL association.

Monochorionic Twin Discordance for Horseshoe Lung and Tricuspid Atresia.

Background: The horseshoe lung is a congenital malformation in which the bases of the right and the left lung are fused. Case report: We describe a monochorionic twin gestation with malformation discordance. The abnormal twin had a horseshoe lung with hypoplasia of the right lung, tricuspid atresia, cleft lip, and a pelvic right kidney. Conclusion: The discordance of anomalies in this monochorionic twin suggests that a postzygotic mutation, epigenetic change, or environmental factors may be responsible for these malformations.

Two rare cases of horseshoe lung with scimitar syndrome in Vietnam.

Horseshoe lung is a rare, congenital, pulmonary anomaly in which the caudal and basal segments of the left and right lungs are joined together. Most cases of horseshoe lung are associated with scimitar syndrome. Horseshoe lung can be diagnosed using pulmonary angiography, showing that the isthmus of the pulmonary parenchyma crosses the midline into the contralateral side. The isthmus parenchyma is typically supplied by the hypoplastic pulmonary artery. Clinical symptoms, therapeutic methods, and prognosis depend on the incidence of pulmonary hypertension, heart failure, recurrent pneumonia, and other combinations of congenital malformations. In this article, we describe two cases of horseshoe lung associated with scimitar syndrome and pulmonary malformation.

Horseshoe lung in a young child at Tygerberg Hospital, South Africa.

Horseshoe lung is a very rare congenital malformation in which the bases of the right and left lung are fused to each other by a narrow isthmus. Although rare, a hyperlucent area in the lower left lung, close to the vertebral column, may represent a horseshoe lung. Horseshoe lung is often associated with scimitar syndrome. Here, we present a case of a 2-year-old girl who presented with recurrent chest infections and wheezing associated with horseshoe lung. The right lung appeared to be hypoplastic.

Congenital venolobar syndrome, pulmonary sequestration, and horseshoe lung in a newborn female.

A 6-day-old female presented to a pediatric cardiologist with tachypnea. A computed tomography angiogram of the chest showed partial anomalous pulmonary venous return, a pulmonary sequestration, and a horseshoe lung. Depicted here are radiographic findings which display this rare combination. This case demonstrates the need for the radiologist to consider additional abnormalities in the setting of anomalous pulmonary venous return.

Clinics in diagnostic imaging (173). Scimitar syndrome with horseshoe lung.

A 56-year-old Chinese man presented with giddiness and vertigo. Subsequent chest radiography showed the classic scimitar sign of an abnormal pulmonary venous return. Further evaluation with non-contrast computed tomography substantiated the finding of a partial anomalous venous drainage pattern and identified an associated rare lung anomaly, horseshoe lung. The imaging findings of scimitar syndrome and its association with horseshoe lung are reviewed.

Elevated Carbohydrate Antigen 19-9 Level in a Patient with Horseshoe Type Pulmonary Sequestration.

Elevated carbohydrate antigen (CA) 19-9 can indicate malignancies of the gastrointestinal, pancreatic, and biliary tracts, and be found in a pulmonary sequestration. A 30-year-old man visited Seoul National University Bundang Hospital due to elevated CA 19-9 levels, representing pulmonary sequestration of the bilateral lower lobes, which were connected with each other. We performed left lower lobectomy and division of the systemic arteries. After operation, CA 19-9 levels decreased to normal range, even though a small amount of sequestrated lung remained in the right lower lobe. It is not uncommon that presence of pulmonary sequestration might elevate serum CA 19-9 levels; however, horseshoe type bilateral pulmonary sequestration is very rare.

Hypoplastic left heart syndrome in PAGOD syndrome.

Chromosomal abnormalities as well as non-cardiac anomalies have been identified as independent risk factors for surgical morbidity and mortality in Fontan palliation. The combination of malformations consisting of pulmonary hypoplasia, agonadism (sex reversal), omphalocele, and diaphragmatic defect is compatible with pulmonary artery and lung hypoplasia, agonadism, omphalocele, and diaphragmatic defect (PAGOD). Most cases have been associated with cardiac disease, particularly hypoplastic left heart syndrome (HLHS) that is potentially destined for Fontan palliation. Reported herein is the case of a Japanese female infant diagnosed with PAGOD syndrome along with HLHS (mitral atresia and aortic atresia), in whom intractable respiratory failure manifested as bilateral eventration of the diaphragm and presumed right lung hypoplasia. These characteristic pulmonary lesions associated with the syndrome precluded use of the Fontan pathway.