Endoscopic Endonasal Approach in Pediatric Optic Pathway–Hypothalamic Glioma: Case Report and Systematic Review

Background and Objective: Recent advancements in the endoscopic endonasal approach (EEA) have revolutionized the treatment landscape for optic pathway–hypothalamic gliomas (OPHGS). By providing precise visualization of the lesion neuroanatomic and minimizing brain manipulation, the EEA enhances surgical outcomes reducing tumor burden and alleviating mass effect symptoms. Despite the variable clinical trajectories of OPHGS, from spontaneous regression to progressive growth, the optimal management neurosurgical strategy continues to be a subject of debate. Nevertheless, the EEA represents a promising approach for safe tumor resections, marking a significant leap forward in the management of these challenging tumors.

Methods: We presented a case of OPHG that underwent EEA and performed a systematic review search in English and Spanish case reports from January 2007 (the earliest reported case) to June 2024, focusing on OPHG treated with EEA. Databases such as PubMed, Lilacs, and Embase were queried using keywords like “Optic Glioma,” “Optic Chiasm,” “Hypothalamus,” “Optic Pathway–hypothalamic glioma,” “Endoscopic Endonasal,” and “Endoscopic Transsphenoidal.”

Illustrative Case: An 8-year-old female presented with 6 years of progressive right-sided visual loss, confirmed amaurosis in the right eye, and horizontal nystagmus. Imaging revealed a 27 × 27 × 30 mm pilocytic astrocytoma (PA) in the sellar and suprasellar regions. An expanded EEA enabled maximal safe resection, confirming the tumor's low-grade nature. Postoperatively, she developed central diabetes insipidus and hydrocephalus.

Search Results: Our systematic review encompassed 30 cases of OPHGS treated with EEA. Most patients (86.6%) presented with visual disturbance, 43.3% with headache, and 30% with hormonal alterations. Surgical outcomes included GTR in 20%, STR in 40%, and NTR in 20%. Histologically, PA predominated (73.3%), with varied clinical presentations highlighting the need for tailored management approaches.

Conclusion: The EEA offers safe access to the optic pathway and hypothalamic region, minimizing neurovascular manipulation and brain retraction. Despite limitations in achieving gross-total resection (GTR) compared with other approaches, EEA’s benefits in clinical improvement and neuroanatomical preservation make it a valuable option for select cases of OPHGS.

Fig. 1 Pre- and postoperative MRI. (A) Sagittal, (B) coronal, and (C) axial preoperative enhanced images of the sella demonstrating a lobulated tumor extending into the sellar and suprasellar regions with heterogeneous enhancement, measuring 27 × 27 × 30 cm. In the sagittal view, there is a posterior displacement of the pituitary stalk and inferior compression of the pituitary gland. (C) Sagittal, (D) coronal, and (F) axial postoperative enhanced imaging showing a safe near-total resection of the tumor, while preserving structurally the hypothalamus bilaterally, as well as the pituitary gland and the infundibulum.

 

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
07 February 2025