PT  - JOURNAL ARTICLE
AU  - Al-Shamrani, Abullah
AU  - AlSadi, Reem S.
AU  - Elhoury, Motea E.
AU  - AlHarbi, Adel S.
TI  - Infantile scimitar syndrome with unusual associations
AID  - 10.15537/smj.2017.7.18365
DP  - 2017 Jul 01
TA  - Saudi Medical Journal
PG  - 764--767
VI  - 38
IP  - 7
4099  - http://smj.org.sa/content/38/7/764.short
4100  - http://smj.org.sa/content/38/7/764.full
SO  - Saudi Med J2017 Jul 01; 38
AB  - 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.