Abstract:
The Scimitar syndrome or pulmonary venolobar syndrome is a rare, complex and variable congenital anomaly of cardiopulmonary development characterised by an abnormal right-sided pulmonary venous drainage in the inferior vena cava, malformation of the right lung, abnormal arterial supply and sometimes cardiac malformations. These serious anomalies notwithstanding, their presentation is varied, ranging from asymptomatic to severe symptoms, particularly in the neonatal and early infantile period. Some symptomatic cases are misdiagnosed as recurrent lower respiratory tract infections or as heart failure, missing the underlying congenital anomaly. Considering associated serious complications such as pulmonary hypertension and a high mortality rate, accurate and timely diagnosis is mandatory. This requires a high index of suspicion, not only by clinicians but also by radiologists who encounter suggestive chest radiographs in asymptomatic children. Still, the classic radiological finding for which the condition is named is seen in only about 50% of all cases and only 10% in affected infants. We highlight this case to heighten clinicians\' and radiologists\' suspicions about Scimitar syndrome.
UNASSIGNED: None declared.
摘要:
弯刀综合征或肺静脉叶综合征是一种罕见的,心肺发育的复杂多变的先天性异常,其特征是下腔静脉右侧肺静脉引流异常,右肺畸形,动脉供应异常,有时是心脏畸形。尽管有这些严重的异常现象,他们的介绍是多种多样的,从无症状到严重症状,特别是在新生儿和婴儿早期。一些有症状的病例被误诊为反复下呼吸道感染或心力衰竭,缺少潜在的先天性异常。考虑到相关的严重并发症,如肺动脉高压和高死亡率,准确和及时的诊断是强制性的。这需要高度怀疑,不仅是临床医生,也是放射科医生在无症状儿童中遇到暗示性胸部X光片。尽管如此,该疾病被命名的经典放射学发现仅在所有病例中的约50%中可见,在受影响的婴儿中只有10%。我们强调这种情况,以提高临床医生和放射科医生对弯刀综合征的怀疑。
■没有声明。