Bilateral acute angle closure glaucoma as a presentation of isolated microspherophakia in an adult: case report

Abstract

Background: Bilateral simultaneous angle closure glaucoma is a rare entity. To our knowledge this is the first reported case of bilateral acute angle-closure glaucoma secondary to isolated microspherophakia in an adult.

Case presentation: A 45-year-old woman presented with bilateral acute angle closure glaucoma, with a patent iridotomy in one eye. Prolonged miotic use prior to presentation had worsened the pupillary block. The diagnosis was not initially suspected, and the patient was subjected to pars-plana lensectomy and anterior vitrectomy for a presumed ciliary block glaucoma. The small spherical lens was detected intraoperatively, and spherophakia was diagnosed in retrospect. She had no systemic features of any of the known conditions associated with spherophakia. Pars-plana lensectomy both eyes controlled the intraocular pressure successfully.

Conclusion: This case demonstrates the importance of considering the diagnosis of isolated microspherophakia in any case of bilateral acute angle closure glaucoma. Lensectomy appears to be an effective first-line strategy for managing these patients.

Figure 1

(Top) Slit-lamp photograph of the both eyes at presentation showing circumciliary congestion and corneal haze. Note the iridotomy in the left eye. (Bottom left) Slit section showing flat anterior chamber with iris apposed to posterior corneal surface. (Bottom right). Diffuse pigmentation seen at the posterior corneal surface.

Figure 2

Magnified picture showing signs of acute angle closure: patches of iris atrophy. (Top left), dilated iris vessels (Top right), and glaucomflecken (Top right and Bottom left). (Bottom right) Completely closed angles on gonioscopy

Figure 3

(Top left) Ultrasound Biomicroscopic scan of the right eye showing anteriorly displaced crystalline lens and forward movement of entire iris-lens diaphragm. (Top right) UBM scan of the left eye showing obliteration of the peripheral anterior chamber by extensive synechiae. (Bottom left) Prominent iris vessels at presentation, which regressed (bottom right) after control of IOP.

Figure 4

(Top left) Slit-lamp photograph of the right eye following cycloplegic therapy showing relief of the acute angle closure. Note the decreased ocular congestion and clear cornea. (Top right) Intra-operative photograph of the left eye showing the edge of the crystalline lens within the pupillary border. (Bottom left) First post-operative day of the left eye after undergoing a pars-plana lensectomy and anterior vitrectomy. (Bottom right) Picture of the right eye after dilatation with phenylephrine showing clearly the small and spherical crystalline lens with the lens edge visible within the pupillary border.

Figure 5

(Top) Final picture of both eyes after surgery. (Bottom left) Pale neuroretinal rim (NRR) of the right eye following prolonged optic nerve head ischemia. (Bottom right) Optic nerve head of the left eye showing advanced glaucomatous optic neuropathy, and pale NRR.

Figure 6

Post-operative gonioscopy pictures of the right eye showing partially opened angles (black arrows) especially in inferior and nasal angles, and also areas of synechial closure (white arrows) in the superior and temporal angle.