[Optic gliomas in the first year of life]

Optic gliomas are mainly observed in pediatric patients. Twenty-five percent of cases are diagnosed in the first year of life. Infants with optic gliomas must be considered distinctly from the rest of pediatric patients with this type of lesion, because of the more invasive nature of the tumour and a worse prognosis. Most authors think that surgical treatment of optic gliomas has to be limited to a diagnostic biopsy of the lesion and, in selected cases, to partial decompressive excision. Chemotherapy is useful in the control of the disease, particularly when combined with surgery. Radiotherapy has important secondary effects on the immature brain, and its use is restricted to patients who have reached the 2nd year of life.

We report our observations on 11 patients with optic gliomas diagnosed in the first year of life, and treated at the Section of Pediatric Neurosurgery of the Catholic University of Rome between 1980 and 1994.

In 45.4% of cases the tumour involved the intracranial optic nerve, the optic chiasm, the optic tract and the thalamus. Nystagmus was the most frequent clinical sign, and it was observed in all cases. Signs of intracranial hypertension were observed in 72.6% of cases. We performed a partial decompressive excision of the tumour in 82% of patients. In 2 of these cases we have observed a spontaneous disappearance of the tumour residue and in 5 cases a prolonged time of survival. Mortality has been lower in our group of patients, compared with other authors' experience (36.4% vs 50-80%). Also morbidity has been lower. A severe neurological worsening was observed in two patients and a complete blindness in other two cases (18.1% vs 66.6-100%).

We think that the lower morbidity and mortality observed in our patients can, at least in part, be ascribed to a more aggressive surgical treatment of the lesions.