NEURORADIOLOGY


Case 37 :
History: 26 week premie with feeding intolerance. Mother had history of hypertension and diabetes during pregnancy. Echoencephalogram was performed to rule out intraventricular hemorrhage. What is the diagnosis?
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Imaging Findings

Sagittal Echoencephalogram demonstrates linear echogenic foci in the distribution of the lenticulostriate branches of the middle cerebral artery in the basal ganglia and the thalami, bilaterally(A,B). Color Doppler confirming the presence of flow in these arteries(C).


Imaging Diagnosis

Mineralising Vasculopathy

Discussion

The entity mineralizing vasculopathy has been infrequently described in literature. On echoencephalograms, it is seen as branched linear pattern of echogenicity in the course of the lenticulostriate branches of the middle cerebral arteries in the basal ganglia and the thalami. Pathologically the various hypotheses for these echogenicities include deposition of amorphous basophilic material, perivascular infiltrates of mononuclear cells, hyalinized thickening of the arterial wall or rarely a true vasculitis (1). Deposition of calcium does not occur, explaining the lack of corresponding hyperdensities in the basal gangia on CT scanning.

No specific cause has been attributed to this finding. This finding can be seen in a variety of conditions including congenital infection(especially CMV), trisomy 13, maternal diabetes, traumatic injury, non-immune hydrops and fetal alcohol syndrome(2). Sometimes no underlying disorder can be found.

This finding should encourage further laboratory and chromosomal investigation for congenital infections or chromosomal abnormality.

References

1.Teele RL, Hernanz-Schulman M, and Sotrel A. Echogenic vasculature
in the basal ganglia of neonates: a sonographic sign of vasculopathy. Radiology 169:423-427, 1988.
2. Wang, HS, Kuo MF, and Chang TC. Sonographic lenticulostriate
vasculopathy in infants: some associations and a hypothesis. AJNR 16:97-102, 1995.

Contribution

DR SUDHA LOLAYEKAR & DR GAURAV SAIGAL
GSaigal@med.miami.edu