Children (Mar 2022)

Surgical Management of Pre-Chiasmatic Intraorbital Optic Nerve Gliomas in Children after Loss of Visual Function—Resection from Bulbus to Chiasm

  • Julian Zipfel,
  • Jonas Tellermann,
  • Dorothea Besch,
  • Eckart Bertelmann,
  • Martin Ebinger,
  • Pablo Hernáiz Driever,
  • Jens Schittenhelm,
  • Rudi Beschorner,
  • Arend Koch,
  • Ulrich-Wilhelm Thomale,
  • Martin Ulrich Schuhmann

DOI
https://doi.org/10.3390/children9040459
Journal volume & issue
Vol. 9, no. 4
p. 459

Abstract

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Optic pathway gliomas in children carry significant morbidity and therapeutic challenges. For the subgroup of pre-chiasmatic gliomas, intraorbital and intradural resection is a curative option after blindness. We present a two-center cohort using different surgical approaches. A retrospective analysis was performed, including 10 children. Mean age at surgery was 6.8 years. Interval between diagnosis and surgery was 1–74 (mean 24 ± 5.5, median 10) months. Indications for surgery were exophthalmos, pain, tumor progression, or a combination. Eight patients underwent an extradural trans-orbital-roof approach to resect the intra-orbital tumor, including the optic canal part plus intradural pre-chiasmatic resection. Gross total resection was achieved in 7/8, and none had a recurrence. One residual behind the bulbus showed progression, treated by chemotherapy. In two patients, a combined supra-orbital mini-craniotomy plus orbital frame osteotomy was used for intraorbital tumor resection + intradural pre-chiasmatic dissection. In these two patients, remnants of the optic nerve within the optic canal remained stable. No patient had a chiasmatic functional affection nor permanent oculomotor deficits. In selected patients, a surgical resection from bulb to chiasm ± removal of optic canal tumor was safe without long-term sequela and with an excellent cosmetic result. Surgery normalizes exophthalmos and provides an effective tumor control.

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