Decompressive hemicraniectomy in pediatric malignant arterial ischemic stroke: a case-based review

Abstract

Purpose: Malignant stroke is a life-threatening emergency, with a high mortality rate (1-3). Despite strong evidence showing decreased morbidity and mortality in the adult population, decompressive hemicraniectomy (DCH) has been scarcely reported in the pediatric stroke population, and its indication remains controversial, while it could be a potential lifesaving option.

Methods and results: We performed an extensive literature review on pediatric malignant arterial ischemic stroke (pmAIS) and selected 26 articles reporting 97 cases. Gathering the data together, a 67% mortality rate is observed without decompressive therapy, contrasting with a 95.4% survival rate with it. The median modified Rankin score (mRS) is 2.1 after surgery with a mean follow-up of 31.8 months. For the 33% of children who survived without surgery, the mRS is 3 at a mean follow-up of 19 months. As an illustrative case, we report on a 2-year-old girl who presented a cardioembolic right middle cerebral artery stroke with subsequent malignant edema and ongoing cerebral transtentorial herniation in the course of a severe myocarditis requiring ECMO support. A DCH was done 32 h after symptom onset. At the age of 5 years, she exhibits an mRS of 3.

Conclusion: Pediatric stroke with malignant edema is a severe condition with high mortality rate if left untreated and often long-lasting consequences. DCH might minimize the vicious circle of cerebral swelling, increasing intracranial pressure and brain ischemia. Our literature review underscores DCH as an efficient therapeutic measure management of pmAIS even when performed after a significant delay; however, long-lasting morbidities remain high.

Keywords: Craniectomy; Malignant edema; Pediatric stroke.

Fig. 1

Anisotropy diffusion coefficient (ADC) map. Initial MRI shows a large subacute ischemic lesion on the right MCA and PCA territory. Restricted diffusion of the splenium and contralateral occipital mesial cortex involvement (A). Coronal T2 FLAIR and axial T2. Deviation of the midline with transtentorial herniation of the frontobasal parenchyma, signs of uncal herniation with effacement of the interpeduncular cistern, and severe mass effect on the midbrain (arrows) are also evident (B, C). Follow-up MRI performed 4 weeks later shows an extensive cortico-subcortical volume loss of the affected parenchyma, including the right putamen. Focal lesions are depicted in the right thalamus and splenium. A minimal parenchymal herniation through the flap is also noted (D)