Background. Unilateral focal cerebral arteriopathy (UFCA) is a common cause of arterial ischemic stroke (AIS) in children. According to the CASCADE classification, three subtypes are distinguished based on the presence of collaterals and the vascular territory. Data on the clinical course and outcomes of these variants in children are limited.
Objective. To characterize the clinical, neuroimaging, and laboratory features of AIS in children with UFCA based on a retrospective analysis of a pediatric stroke center registry.
Materials and Methods. Data from 94 patients with AIS for the period 2024–2025 were analyzed. Among them, according to the CASCADE classification, 13 patients had UFCA in the middle cerebral artery (MCA) territory (type 2B), and 3 patients had UFCA type 2C in the vertebrobasilar territory. The following parameters were assessed: age, sex, neurological deficit (PedNIHSS score), lesion location, affected artery, preceding factors (infections, trauma), laboratory and instrumental data, treatment, and outcome according to the modified Rankin Scale (mRS) at discharge.
Results. A total of 16 patients (17%) with UFCA were analyzed. Median age was 50.5 months (IQR 18.5–89.5); boys accounted for 56.3% (9/16). The majority of patients presented with the typical pattern of UFCA without collaterals in the MCA territory – 13 patients (81.3%). Patients with UFCA presented to the hospital significantly later compared to other causes – 62.5% of UFCA patients presented more than 24 hours after symptom onset, whereas in the control group, 59% presented within the first 6 hours (p<0.05). Neurological deficit: median PedNIHSS score at AIS onset in UFCA patients was 7 (range 2–9). All UFCA patients had stroke severity less than 15 points. Data on preceding infections were collected – present in 75% (12/16). Varicella zoster infection less than 6 months before AIS n=8, 50% (among these, varicella zoster virus was detected in the cerebrospinal fluid of 6 patients), upper respiratory tract infection within one month before AIS (n=2), herpetic encephalitis (n=1), enteric infection (n=1). Among other causes according to the CASCADE classification, only 19.2% (15/78) had preceding infections (p<0.05). A positive bubble test indicating a patent foramen ovale was noted in 2 patients (12.5%). Epileptic seizures in the acute phase were recorded in 2 children (12.5%) and were controlled after adding valproic acid to therapy. Thrombolysis was administered to one patient (6.3%). Four patients (25%) initially received low-molecular-weight heparin (LMWH) (dalteparin sodium 130 units twice daily subcutaneously), 4 patients (25%) received acetylsalicylic acid from the outset, and 8 patients (50%) received a treatment regimen of unfractionated heparin (UFH) at a dose of 20 units/kg/hour for 7–10 days followed by transition to acetylsalicylic acid. LMWH and UFH dosages were adjusted based on anti-Xa activity levels (target range 0.5–0.8). Hormonal therapy was prescribed to 8 patients (50%) – prednisolone at a dose of 1.5–2 mg/kg/day, max 60 mg/day, for 2 months with gradual tapering. No significant difference in the course of neurological deficit was found between patients receiving hormonal therapy and those not receiving it. Outcomes: at discharge, a good functional outcome (mRS 0–2) was achieved in 69% (11/16), moderate/severe outcome (mRS 3–4) in 25% (4/16). There were no deaths.
Conclusion. In children with unilateral focal cerebral arteriopathy, AIS most commonly occurs in the MCA territory, is associated with a preceding infection (especially varicella zoster), and in most cases has a favorable outcome. The subacute course due to an ongoing process over time and possible development of compensatory collaterals leads to a significant delay in diagnosis because of insufficient awareness among parents and medical personnel. Further prospective follow-up is needed to identify predictors of poor outcomes.