Scimitar variant

Pearl, William

doi:10.1007/bf02079472

Cited by 23 publications

(23 citation statements)

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“…[78101112] The forms of infantile scimitar found in our population are similar to the previous publications and include the classic scimitar, a scimitar variant with a meandering pulmonary vein that drains in the left atrium, bilateral scimitar in which all pulmonary veins drain into the right atrium with the presence of a bronchogenic cyst in the right middle lobe,[8] scimitar syndrome with scimitar vein stenosis and contralateral left upper pulmonary vein stenosis,[13] and scimitar syndrome with right and left pulmonary arterial stenosis at multiple levels.…”

Section: Discussionmentioning

confidence: 99%

Clinical spectrum of infantile scimitar syndrome: A tertiary center experience

et al. 2014

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Aim:Infantile scimitar syndrome is a rare condition, with most of the literature reports being limited to case reports and a few case series. The aim of this study was to review patients with infantile scimitar syndrome who presented to our hospital from July 2000 to January 2011.Materials and Methods:In this retrospective study, we evaluated the medical records of patients aged 0–14 years who were symptomatic before the age of 1 year and were subsequently diagnosed with the syndrome. A total of 16 patients with the infantile form of scimitar syndrome were identified from the database.Results:The median age at presentation was 14 days, with a median age at diagnosis of 55 days. Fifty-six percent of the patients were females. Tachypnea was the major presenting symptom and 13 out of 16 patients had pulmonary hypertension. Of the 13 patients with pulmonary hypertension, 7 had systemic collaterals, which were treated by coil occlusion together with medications, and 3 had corrective surgery. The mortality rate was 3/16 (18.8%) over the 10.5 years study period.Conclusions:Infantile scimitar syndrome is a rare congenital anomaly that needs a high degree of suspicion for early referral and management. The association of the syndrome with pulmonary hypertension leads to recurrent and prolonged hospitalization.

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Section: Discussionmentioning

confidence: 99%

Clinical spectrum of infantile scimitar syndrome: A tertiary center experience

et al. 2014

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“…Mit Hilfe der jetzt verfügbaren nichtinvasiven Bildgebungsverfahren, deren Einsatz auch bei asymptomatischen Patien ten gerechtfertigt und aufgrund der im Vergleich zu anderen Methoden hohen Ausagekraft sogar vorteilhafter ist [1,4], lässt sich die oft komplexe anatomische Situation detailgenau darstellen -insbesondere können auch seltenere Varianten mit unterschiedlicher Prognose erkannt werden [3,8,9,10]. In Verbindung mit einer exakten und ausgereiften Funktionsdiagnostik kann dadurch inzwischen in vielen Fällen eine Herzkatheterunter suchung mit Kontrastmittelexposition entbehrlich werden.…”

Section: "Scimitar-syndrom-variante" Bei Unserem Patientenunclassified

“…Dadurch sind Einschätzungen hinsichtlich sich eventuell ergebender therapeutische Konsequenzen und zur Prognose möglich. Unser Patient bot den überraschenden Befund einer Scimitar-Syndrom-Variation [3,9] ohne wesentliche kardiopulmonale Funktionsstörung, die keiner Therapie bedurfte und prognostisch als sehr güns-tig einzuschätzen ist. Einer angestrebten Ausbildung als Feuerwehrmann, die erhöhte Ansprüche an die körperliche Leistungsfähigkeit stellt, konnte somit aus ärztlicher Sicht entsprochen werden.…”

Section: Fazit Für Die Praxisunclassified

Auffälliger Röntgenthorax bei jungem Feuerwehranwärter

2007

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An 18-year-old, asymptomatic male underwent a routine chest x-ray examination which showed a pathological result. It revealed a characteristic crescent-like shadow in the right lower lung field, resembling a muslim sword (scimitar), responsible for the name of the underlying disease. Scimitar syndrome is a rare congenital malformation with a wide clinical spectrum ranging from asymptomatic to severe disturbances manifesting in childhood. The typical findings are hypoplasia of the right lung with marked mediastinal shift to the right and dextrocardia, reduced right pulmonary artery perfusion with maintained bronchial connection, anomalous arterial supply to the right lower lobe from the aorta and abnormal draining of the enlarged right pulmonary vein into the pulmonary circulation, producing the characteristic radiographic "scimitar sign". The development of right ventricular failure due to long-standing right ventricular overload and recurrent respiratory infections depend on the severity of anatomical abnormalities and functional disorders, sometimes requiring surgical intervention. Nowadays, sophisticated imaging techniques allow precise and rapid diagnosis of these complex abnormalities and their functional implications. The findings for our patient differed from the classic constellation, with a regular emptying of the enlarged right pulmonary vein into the left atrium, leading to a decreased burden on the central haemodynamics. In our very rare case of a scimitar syndrome variant, the functional disorders were negligible.

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“…Although a scimitar sign in a female patient with normal pulmonary venous connections may be called a pseudo-scimitar syndrome,9 it was an imprecise and unscrupulous nomenclature. In the literature, we found reports of so-called scimitar vein syndrome,13 scimitar variant,14,15 and incomplete scimitar syndrome.16 None of these 4 reports documented any systemic arterial supply or anomaly of the bronchial airway 13-16. Therefore, the patients in these reports were victims with the isolated scimitar vein anomaly.…”

Section: Discussionmentioning

confidence: 99%

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

Lee

2007

Yonsei Med J

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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.

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Scimitar variant

Cited by 23 publications

References 9 publications

Clinical spectrum of infantile scimitar syndrome: A tertiary center experience

Clinical spectrum of infantile scimitar syndrome: A tertiary center experience

Auffälliger Röntgenthorax bei jungem Feuerwehranwärter

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

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