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Case Twenty Six - Lenticulostriate Vasculopathy

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Clinical History: One week-old full term infant with PPHN and pneumonia. On oscillator ventilator. Possible ECMO candidate.

Findings: Branching linear echogenic foci in region of the lenticulostrriate arteries. Color flow obtained in these regions. These findings are consistent with lenticulostriate vasculopathy (LSV).

Diagnosis:: Lenticulostriate vasculopathy.

Discussion: In normal patients, vessels supplying the basal ganglia cannot be visualized. In patients with lenticulostriate vasculopathy, branching linear echogenicities are seen in the region of the basal ganglia and are felt to represent the arterial walls. Arterial blood flow may be demonstrated in these areas by color or pulse Doppler. In a recent large prospective study, Wang reported the incidence of detection of flow is lower in patients with LSV then in normal patients suggesting that the vessel wall changes are partially obstructive. The ultrasound findings were progressive in 85% of patients and constant in the remaining LSV patients. Pathologic changes of thickened, hypercellular arterial walls, with basophilic deposits (mineralization) in some was reported by Teele but not confirmed by Hughes. No calcification has been detected in the vessel walls.

The numerous etiologies that have been reported include congenital infections, especially CMV, chromosome disorders, particularly Trisomy 21 and 13, maternal drug use, fetal alcohol syndrome, neonatal asphyxia, hydrops fetalis, twin-to-twin transfusion, diabetic fetopathy, sialidosis, RDS, apnea, bacterial meningitis, and Klebsiella pneumonia. Most recently, Wang has associated this finding with neonatal lupus, neonatal hypoglycemia, encephalitis, head injury and uncomplicated maturity. LSV appears to be a nonspecific finding indicating a previous insult to the developing brain.

References:
1. Wang HS, Kuo MF, Chang TC. Sonographic lenticulostraite
vasculopathy in infants: some associations and a hypothesis.
AJNR 1995; 16:97-102.

2. Teele RL, Hernanz-Schulman M, Sotrel A. Echogenic vasculature
in the basal ganglia in neonates: a sonographic sign of vasculopathy.
Radiology 1988; 169:423-427.

3. Hughes P, Weinberger E, Shaw DWW. Linear areas of echogenicity
in the thalami and basal ganglia of neonates: an expanded association.
Radiology 1991; 179:103-105.

4. de Vries LS, Beek FJA, Stoutenbeek P. Lenticulostraite vasculopathy in
twin-to-twin transfusion syndrome: Sonographic and CT findings.
Pediatr Radiol 1995; 25:S41-S42.

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Submitted by:
Dayna Weinert, M.D.
Rainbow Babies and Children's Hospital