The optic chiasm plays a central role in neuro-ophthalmologic diagnosis, as lesions involving this structure typically produce characteristic visual field defects. Compression of the decussating nasal retinal fibers within the optic chiasm most commonly results in bitemporal hemianopia, which serves as a clinical hallmark of sellar and suprasellar pathology. The severity and reversibility of visual impairment depend on multiple factors, including the degree and duration of chiasmal compression as well as the timing of surgical decompression. Pathophysiological mechanisms underlying visual loss and recovery include conduction block, demyelination, ischemia, and irreversible axonal degeneration. Furthermore, structural biomarkers such as retinal nerve fiber layer (RNFL) thickness act as reliable predictors of postoperative visual outcomes.
Craniopharyngiomas are rare, benign epithelial tumors arising in the sellar and suprasellar regions, accounting for approximately 2–5% of all intracranial tumors. These tumors demonstrate a bimodal age distribution and are classified into two distinct histopathological subtypes:
- Adamantinomatous craniopharyngioma (ACP): Predominantly affects pediatric patients and frequently exhibits calcification and cystic components.
- Papillary craniopharyngioma (PCP): Typically occurs in adults and usually lacks calcification.
Due to their close anatomical relationship with the optic apparatus, progressive visual impairment is among the most common presenting manifestations of craniopharyngiomas in both children and adults. In pediatric populations, visual impairment at diagnosis and long-term visual sequelae remain major clinical concerns, as postoperative recovery of visual acuity does not always parallel the recovery of visual fields, reflecting permanent damage to specific optic pathways.
A 13-year-old boy presented with a one-month history of progressive bilateral blurred vision, which was significantly more pronounced in the left eye.
- Initial Visual Acuity: Best-corrected visual acuity (BCVA) was 20/25 in the right eye and limited to light perception in the left eye.
- Ophthalmologic Findings: Initial visual field examination demonstrated severe, asymmetric visual field loss. Fundoscopic examination revealed bilateral optic disc atrophy, and optical coherence tomography (OCT) of the retinal nerve fiber layer showed marked thinning bilaterally.
- Neuroimaging: Magnetic resonance imaging (MRI) revealed a large intrasellar mass with suprasellar extension.
- Intervention & Histopathology: The patient underwent transsphenoidal tumor resection followed by hormone replacement therapy. Histopathological analysis confirmed the diagnosis of adamantinomatous craniopharyngioma.
- Postoperative Outcome: At the three-month postoperative follow-up, visual acuity dramatically improved to 20/20 in the right eye and 20/50 in the left eye. However, visual field testing demonstrated persistent residual bitemporal hemianopia.
Craniopharyngiomas in the sellar and suprasellar regions are well-recognized causes of visual impairment resulting from direct mechanical compression of the optic chiasm and adjacent optic pathways. In children, visual dysfunction is frequently present at initial diagnosis, commonly manifesting as reduced visual acuity, visual field defects, and optic disc pallor. These findings reflect damage to the decussating nasal retinal fibers within the optic chiasm and are typically the result of prolonged, insidious tumor growth before clinical recognition.
The adamantinomatous subtype, which predominates in childhood, is characterized by cystic components, calcifications, and strong adherence to surrounding neurovascular structures. These characteristics contribute to delayed diagnosis and increased surgical complexity. Several pediatric case series and systematic reviews have demonstrated that the duration of optic chiasm compression is a key prognostic factor influencing postoperative visual outcomes. Prolonged symptom duration prior to decompression is consistently associated with poorer visual field recovery, even in cases where central visual acuity successfully improves postoperatively.
In the present case, the patient exhibited classical features of chiasmal syndrome—including bitemporal hemianopia and bilateral optic atrophy—indicative of long-standing optic pathway compression. Despite these unfavorable preoperative findings, substantial recovery of central visual acuity was achieved following surgical decompression.
This dissociation between the improvement of visual acuity (mediated by the papillomacular bundle) and the persistence of visual field defects (due to permanent damage in the peripheral visual pathways) represents an important clinical learning point. Previous studies have shown that optic atrophy does not invariably preclude visual improvement, as a subset of retinal ganglion cells may remain functionally viable despite chronic compression.
This case highlights that in pediatric patients with suprasellar adamantinomatous craniopharyngioma, significant improvement in central visual acuity can be achieved after surgical decompression, despite persistent visual field defects. The presence of optic atrophy and bitemporal hemianopia at presentation suggests prolonged optic chiasm compression, which may predict incomplete visual field recovery. Early neuro-ophthalmologic evaluation, prompt neuroimaging, and coordinated multidisciplinary management remain critical to maximizing visual outcomes and mitigating permanent optic pathway damage.
Author: Dr. Lukisiari Agustini, dr.,Sp.M(K)
Link : https://rjp.com.ro/articles/2026.1/RJP_2026_1_Art-06.pdf
