[OPTIC DISC EDEMA IN McCUNE-ALBRIGHT SYNDROME].
Tsessler. Maria M; Rappoport. Daniel D
Key Findings
- Higher triptorelin dose coincided with worsening optic disc swelling and vision loss
- Stopping triptorelin led to clinical and imaging improvement
- Patients with craniofacial fibrous dysplasia should have regular eye exams and OCT scans when on GnRH analogues
Practical Outcomes
- If you or someone you know has skull bone issues like McCune‑Albright, be cautious with triptorelin or similar hormone drugs. Keep an eye on vision changes and get regular OCT imaging. Consider lowering or stopping the drug if optic nerve swelling appears.
Summary
In a 6‑year‑old girl with McCune‑Albright syndrome, raising the dose of the hormone drug triptorelin (used for early puberty) seemed to worsen swelling of the optic nerve, causing vision problems. When the drug was stopped, her eye health got better. This suggests that triptorelin can worsen optic nerve pressure in people who already have skull bone abnormalities, so they need careful eye monitoring.
Abstract
Craniofacial polyostotic fibrous dysplasia, as part of McCune-Albright syndrome, can have severe complications including vision loss. Also, patients with this syndrome are at greater risk of secondary intra-cranial pressure elevation due to medication side effects. : A 6-year-old girl with McCune-Albright syndrome and polyostotic craniofacial fibrous dysplasia and optic canal narrowing, developed signs of slowly progressive optic nerve compression on clinical examination including deteriorating visual acuity, positive relative afferent pupillary defect )RAPD) and bilateral optic disc swelling. Imaging using optical coherence tomography (OCT) revealed progressive retinal nerve fiber layer thickening. Prior to deterioration, the dose of triptorelin, a gonadotrophin-releasing hormone analogue, she was treated with for precocious puberty, was increased. Medication cessation was followed by improvement in clinical and imaging findings. : McCune-Albright syndrome patients with craniofacial involvement and/or gonadotrophin-releasing hormone analogue treatment should be monitored regularly for clinical signs of optic neuropathy together with routine OCT imaging.
Study Information
pubmed
2022