RT Journal Article
SR Electronic
T1 Infantile scimitar syndrome with unusual associations
JF Saudi Medical Journal
JO Saudi Med J
FD Prince Sultan Military Medical City
SP 764
OP 767
DO 10.15537/smj.2017.7.18365
VO 38
IS 7
A1 Abullah Al-Shamrani
A1 Reem S. AlSadi
A1 Motea E. Elhoury
A1 Adel S. AlHarbi
YR 2017
UL http://smj.org.sa/content/38/7/764.abstract
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.