Skull base surgery for tumors in children: long-term clinical and functional outcome

Abstract

Skull base tumors in children are rare but require complex approaches with potential morbidity to the developing craniofacial skeleton, in addition to tumor-related morbidity. Reports of long-term clinical and functional outcome following skull base approaches in children are scarce. The authors report long-term outcome in children with tumors undergoing multidisciplinary skull base surgery. A retrospective analysis was undertaken of children undergoing surgery at a single institution between 1998 and 2008 for benign and malignant lesions of the anterior, middle, or posterior cranial base. Patients with craniopharyngioma, pituitary tumors, and optic glioma were excluded. Histology, surgical morbidity, length of hospital stay, progression-free survival, and adjuvant therapy were recorded. Functional and cognitive outcome was assessed prospectively using the Late Effects Severity Score (LESS). Twenty-three children ranging in age from 13 months to 15 years underwent skull base approaches for resection of tumors during the study period. The median follow-up duration was 60 months. Tumor types included meningioma, schwannoma, rhabdomyosarcoma, neuroblastoma, angiofibroma, and chordoma. Complete resection was achieved in 12 patients (52%). Thirteen patients (57%) had benign histology. The median hospital stay was 7 days. There were 3 deaths, 1 perioperative and 2 from tumor progression. Two patients had CSF leakage (9%) and 2 developed meningitis. Two children (9%) had residual neurological deficit at last follow-up evaluation. Thirteen (59%) of 22 surviving patients received adjuvant therapy. The majority of the patients remain in mainstream education and 19 of the 20 surviving children have an LESS of 3 or lower. Children tolerate complex skull base procedures well, with minimal surgical-related morbidity as well as good long-term tumor control rates and functional outcomes from maximal safe resection combined with adjuvant treatment when required.