Al-Shamrani Abullah, AlSadi Reem S, Elhoury Motea E, AlHarbi Adel S
Department of Pediatric, Prince Sultan Medical Military City, Riyadh, Kingdom of Saudi Arabia. E-mail.
Saudi Med J. 2017 Jul;38(7):764-767. doi: 10.15537/smj.2017.7.18365.
Scimitar syndrome is a variant of partial anomalous pulmonary venous connection (PAPVC), in which all or part of the right lung is drained by right pulmonary veins that anomalously connect to the inferior vena cava (IVC). The affected lung and its associated airways are often hypoplastic. In addition, aortopulmonary collateral vessels may be involved on the affected side, causing sequestration of that side; such involvement is commonly associated with cardiac defects. We report a case of infantile scimitar syndrome that involved a typical association with the right lung, but with extremely unusual associations with congenital hydrocephalus and heart blockage. The presentation of this case and the role of different diagnostic approaches and management are discussed.
弯刀综合征是部分性肺静脉异位连接（PAPVC）的一种变异型，其中右肺的全部或部分由异常连接至下腔静脉（IVC）的右肺静脉引流。患侧肺及其相关气道常发育不全。此外，患侧可能存在主肺动脉侧支血管，导致该侧肺隔离；这种情况通常与心脏缺陷有关。我们报告一例婴儿弯刀综合征病例，该病例与右肺有典型关联，但与先天性脑积水和心脏传导阻滞存在极其罕见的关联。本文讨论了该病例的表现以及不同诊断方法和治疗措施的作用。
