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a six week old male infant is noted to be tacchypneic and distressed. A CXR demonstrates cardiomegaly with right sided predominance, as well as superior mediastinal widening. A possible cause includes
a) Moya Moya disease
b) pseudotumour cerebri
c) encephalitis
d) aneurysmally dilated great vein of Galen
e) thrombosis of a major dural sinus
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what's the right answer ?
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The correct answer is D
Explanation
An aneurysmally dilated great vein of Galen causes high output congestive heart failure in neonates whilst in older children it obstructs the Aqueduct of Sylvius, producing non communicating hydrocephalus. This malformation is a congenital arteriovenous malformation with blood shunting from cerebral arteries into an aneurysmally dilated great vein of Galen and the straight sinus. The major feeding vessels are the posterior cerebral artery and its branches the posterior choroidal and posterior perforating artery Chest film shows cardiomegaly with right sided predominance. The superior mediastinum is widened due to dilation of the brachiocephalic arteries and veins. Pulmonary vascularity is frequently normal because of the pulmonary arterial hypertension of the newborn. On angiography rapid arteriovenous shunting into a dilated saclike vein of Galen and straight sinus is seen. The majority of patients diagnosed in the neonatal period present with life-threatening, intractable cardiac decompensation and high-flow arteriovenous fistulae, CHF due to AV shunting (L -> R) and predominantly RIGHT-sided cardiomegaly, a wide superior mediastinum and retrosternal fullness. In neonates it causes high output congestive heart failure. In older children it obstructs the Aqueduct of Sylvius, producing non communicating hydrocephalus.
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what is moya moya disease ?
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moya moya
realy i didn't know it so i searched in cecil
MOYAMOYA. Moyamoya disease is a chronic, noninflammatory occlusive vasculopathy of unknown etiology. It is a rare condition that is most common among the Japanese. It has a bimodal age distribution, with peaks in the first and fourth decades. Diagnostic criteria include stenosis or occlusion involving the bifurcation of the ICA and proximal portions of the ACA and MCA, presence of unusual netlike (œpuff of smoke) collateral arteries arising from the circle of Willis, and bilateral occurrence. In adults, the clinical manifestation is usually hemorrhages. Moyamoya is diagnosed by cerebral angiography. No treatment has been proved effective.
