Nanophthalmos presenting with acute refractory angle-closure glaucoma: acute-phase histopathological evidence and bilateral comparative findings

BACKGROUND: Nanophthalmos is a rare congenital ocular disorder characterized by a microphthalmic eye with abnormally thickened sclera. Although scleral histopathology has been described in elective surgical cases with normal intraocular pressure (IOP), scleral architecture during acute IOP elevation exceeding 60 mmHg remains unclear. This article reports unique histopathological findings obtained during an episode of refractory acute angle-closure glaucoma. CASE PRESENTATION: A 63-year-old man with lifelong high hyperopia (corrected with + 10.25 D spectacles) presented with acute severe ocular pain, headache, nausea, and blurred vision in the right eye for 2 days. Best-corrected visual acuity was 20/2000 in the right eye and 20/70 in the left eye; IOP was > 60 mmHg and 18 mmHg, respectively. Examination revealed extremely shallow anterior chamber (2.15 mm), corneal edema, and nuclear cataract in the right eye; the fellow eye showed similar anterior chamber depth (2.23 mm) with slit-like angle opening, remaining in a preclinical state as an internal control. Axial lengths were 16.3 mm and 16.5 mm, with markedly thickened sclera in both eyes on B-scan ultrasonography. Despite maximal medical therapy, IOP remained > 60 mmHg. Combined lamellar sclerectomy with full-thickness sclerostomy, phacoemulsification with intraocular lens implantation, and posterior capsulotomy was performed, with scleral tissue obtained for histopathological examination. On the first postoperative day, IOP decreased to 17 mmHg, with inflammation resolving within 1 week and scattered superficial retinal hemorrhages completely resolving within 6 weeks. At 8-month follow-up, best-corrected visual acuity improved to 20/80 (with spectacles correcting residual hyperopia of + 10.5 D) with IOP stabilized at 15-18 mmHg without medication. The left eye remained stable (visual acuity 20/70, IOP 16-19 mmHg). Histopathological analysis revealed disorganized collagen architecture in nanophthalmic sclera, showing irregular fiber bundles and loosely arranged connective tissue; in contrast, normal control sclera showed dense, regularly layered collagen with uniform tissue structure. CONCLUSIONS: This case provides the first acute-phase histopathological evidence that characteristic scleral abnormalities in nanophthalmos persist during IOP elevation >60 mmHg, supporting their congenital etiology. Combined sclerostomy and cataract surgery successfully treated refractory nanophthalmos-associated glaucoma in the acute setting, and bilateral findings provide valuable comparative data supporting prophylactic surgical intervention in the fellow eye.