Langfristiges Outcome nach dringender und notfallmäßiger Operation bei Kindern mit tumor-assoziierten intrakraniellen Hypertension und zerebralen Herniation: eine monozentrische Studie

Brain tumors, whether supratentorial or infratentorial, can induce life-threatening cerebral herniation necessitating urgent or emergent surgery to normalize intracranial pressure. While survival is the primary surgical goal, the quality of life in children is also of paramount importance.

We conducted a retrospective analysis of 29 pediatric patients with tumor-associated brain herniation (subfalcine, transtentorial and tonsillar) who underwent surgery from 2014 to 2023 at our center.

Of the 29 patients (19 males, median age 4.3 years) 79.3% had infratentorial tumors. Emergent surgeries were performed in 31%, while urgent surgeries within 12 hours took place in 6.9% and within 24 hours in 62%. Histopathological analysis identified medulloblastoma in 37.9%, pilocytic astrocytoma in 31%, anaplastic ependymoma in 10.3%, diffuse high-grade glioma in 6.9%, ATRT in 6.9%, and anaplastic astrocytoma, ETMR and sarcoid tumor in 3.4% respectively. Preoperatively, intracranial pressure symptoms had 89.6%, focal cerebellar symptoms 37.9%, papilledema 31%, reduced alertness 20.7%, cranial nerve disorder 10.3% and focal cerebral symptoms 3.4%. Gross total resection was achieved in 75.9%, near-total in 17.3% and subtotal in 6.8%. Preoperative hydrocephalus was diagnosed in 82.8%, more common in infratentorial tumors (91.3%). External ventricular drain was used in 37.9%, with only 16.7% requiring postoperative shunt placement. In long-term follow-up (median 48.5 months) clinical improvement was observed in 72.4% (73.9% of infratentorial and 66.7% of supratentorial tumors). Tumors in cerebellar hemispheres showed the least clinical improvement (55.6%, p=0.03). Long-term symptoms included cerebellar symptoms in 88.5%, cranial nerve disorder in 23.1% and focal cerebral symptoms in 11.5%. Gross total resection correlated with clinical improvement in 77.3%. 10.3% were asymptomatic in long-term follow-up. Four patients died due to tumor progression (median survival 23.1 months). 14.3% of the children were unable to attend school and 32.1% required special support schools. 50% of the patients participated in sports.

Various factors influence the long-term outcome in children undergoing urgent or emergent tumor surgery. This single-center study underscores the potential for a positive outcome postoperatively. These findings could contribute to managing crucial discussions with caregivers of children facing life-threatening brain tumors regarding clinical outcome postoperatively.