Surgical outcomes of optic nerve sheath fenestration in pediatric pseudotumor cerebri syndrome.

PURPOSE: Pediatric pseudotumor cerebri syndrome (PTCS) is a rare but significant condition that can lead to serious vision loss if left untreated. Although most patients respond positively to medical treatment, surgical intervention may be necessary in resistant cases. This study evaluates the effectiveness and safety of optic nerve sheath fenestration (ONSF) in pediatric PTCS cases that are resistant to medical treatment.

METHODS

This retrospective study examined consecutive pediatric patients diagnosed with PTCS who underwent ONSF for medically refractory disease at a tertiary referral hospital between 2018 and 2023. Clinical findings and demographic characteristics of the patient were analyzed. The outcome measures assessed included best-corrected visual acuity (BCVA), color vision test, visual field test, and peripapillary retinal nerve fiber layer (RNFL) thickness.

RESULTS

A total of 7 pediatric patients (10 eyes) were included in the study. Of the patients, 57.1% (4 patients) underwent unilateral, and 42.9% (3 patients) underwent bilateral ONSF surgery. Statistically significant improvement in average BCVA values (logMAR) was observed at the 6-month follow-up (primary endpoint) after surgery (from 0.435 to 0.165; p = 0.026), corresponding to approximately a three-line improvement in visual acuity. During the same period, RNFL thickness decreased from 194 μm to 101.94 μm (p < 0.001), and the mean deviation in the visual field test improved from - 8.77 dB to -6.17 dB (p = 0.001). No intraoperative or postoperative complications were observed during the 6-month follow-up period. One patient was referred for ventriculoperitoneal shunt surgery due to insufficient anticipated improvement.

CONCLUSION

ONSF appears to be a safe and effective surgical method that provides improvement in visual function and reduction of optic nerve edema in pediatric patients diagnosed with medically resistant PTCS.