A 36-year-old woman at 36 weeks gestation presented with 5 months of pain on upgaze and 2 months of progressive vision loss in the right eye. Examination revealed 20/200 acuity, a relative afferent pupillary defect, proptosis, motility restriction, disc edema, and visual field loss. Noncontrast MRI showed a lobulated superolateral orbital apex mass, with differential diagnosis including an orbital vascular malformation enlarged by gestational hypervolemia versus a hormonally sensitive meningioma. Given severe vision loss and advanced gestational age, induced delivery was performed. Postpartum imaging and rapid partial visual recovery favored a vascular etiology. Because spontaneous resolution could not be reliably timed to prevent permanent damage, endoscopic transethmoidal posterior medial wall decompression was performed, restoring vision to 20/20−1 with subsequent lesion regression and normalization of examination findings by 4 months postpartum. Coordinated obstetric–ophthalmic management achieved excellent maternal and fetal outcomes.
We report a case of Erdheim–Chester disease in a patient who presented to the ophthalmology clinic with unilateral vision loss, optic disc swelling, and cluster-type headaches. Erdheim–Chester disease is a rare form of non-Langerhans cell histiocytosis characterized by a xanthogranulomatous process with multisystem organ involvement. The condition can be fatal due to renal or cardiovascular complications. Diagnosis is challenging due to the highly variable presentations and rarity of the disease, as demonstrated in this case. Our patient did not have systemic involvement and tested negative for the BRAF V600 mutation. As the findings are atypical and uncommon, clinicians should be aware of the ophthalmic manifestations and imaging features of this rare disease to establish an early diagnosis.
Linear scleroderma “en coup de sabre” (ECDS) is an uncommon form of localized craniofacial scleroderma, with orbital involvement being exceptionally rare. We present a 29-year-old female with right-sided ECDS who presented with progressive vision loss. Examination revealed an enlarged blind spot with global depression, 6 mm of enophthalmos, ptosis, circumferential optic disc edema, an Adie’s pupil, and a characteristic cutaneous ECDS lesion. MRI revealed severe orbital fat atrophy, enlargement of extraocular muscles, and optic nerve contortion with postcontrast enhancement, with no bony abnormalities or inflammation. The patient was managed conservatively with corticosteroids. A literature review identified 26 cases of orbital ECDS, most commonly presenting with enophthalmos, extraocular muscle atrophy, and occasionally bone remodeling. This case highlights several uncommon manifestations of ECDS in the orbit, particularly paradoxical enlargement of the extraocular muscles. These findings expand the known clinical spectrum of orbital disease in ECDS and highlight the need for regular ophthalmic monitoring.
Purpose: To synthesize evidence on bimatoprost-associated periorbital fat atrophy, compare outcomes across prostaglandin analogues, and evaluate reversibility after discontinuation. Methods: PubMed, Embase, Web of Science, and the Cochrane Library were systematically searched from inception to August 2025 according to an a priori protocol (International Prospective Register of Systematic Reviews CRD420251107027). Studies evaluating periorbital structural changes in human subjects using topical bimatoprost for glaucoma, ocular hypertension, or cosmetic indications were included. Risk of bias was assessed using Joanna Briggs Institute tools. Random-effects meta-analyses estimated risk ratios comparing bimatoprost with latanoprost and travoprost for deepening of the upper eyelid sulcus, periorbital fat loss, enophthalmos, and dermatochalasis involution. Results: Forty-five studies comprising 7,106 participants (3,176 bimatoprost-exposed) were included. Meta-analysis of five comparative studies (742 eyes) demonstrated a significantly higher risk of deepening of the upper eyelid sulcus (risk ratio, 2.92 [95% CI, 1.98–4.30]; Grading of Recommendations Assessment, Development, and Evaluation = low) and periorbital fat loss (risk ratio, 3.10 [95% CI, 1.07–8.99]; Grading of Recommendations Assessment, Development, and Evaluation = very low) with bimatoprost versus latanoprost. No significant differences were observed between bimatoprost and travoprost for any outcome (Grading of Recommendations Assessment, Development, and Evaluation = very low). Reversibility was variable; deepening of the upper eyelid sulcus showed the greatest resistance to recovery. Conclusions: Bimatoprost carries a significantly elevated risk of periorbital fat atrophy compared with latanoprost, with nearly three-fold increased risk; however, the certainty of evidence is low to very low. Bimatoprost and travoprost demonstrate equivalent periorbital adverse effect profiles, suggesting that switching between them is unlikely to reduce periorbital effects. These findings support the preferential use of latanoprost when periorbital appearance is a priority. Limitations include heterogeneity in outcome assessment and predominance of observational designs.
Ocular melanoma is a rare and quite deadly mutation in the eye located in the uvea or the conjunctiva, and its overall 5-year survival prognosis is around a dismal 24 months from the time of diagnosis. This report concerns a 12-year-old male presenting with secondary orbital melanoma of a previously excised conjunctival nevus. The progression of the tumor and treatment approaches are discussed, particularly in regard to the use of immunotherapy with checkpoint inhibitors such as Nivolumab. His first workup indicated a primary malignant melanoma and cervical lymph node metastasis that was suspicious and needed to be addressed via excisional biopsy and follow-up scans. While Nivolumab treated his initial symptoms of care quite effectively, subsequent treatment breaks led to complications, which necessitated a change to a mixed chemotherapy approach. Once Nivolumab was reinstituted, the patient improved significantly, which reinforces the idea that proactive management through surveillance and immunotherapy in combination can control ocular melanoma effectively.
