Microspherophakia

microspherophakia an uncommon congenital abnormality of the lens

1. MICROSPHEROPHAKIA BY S.DHIVYA CRRI 2009 BATCH

2. DEFINITION UNCOMMON BILATERAL CONGENITAL ABNORMALITY OF CRYSTALLINE LENS. TERM TO DESCRIBE SMALL & SPHERICAL LENS isolated / idiopathic and familial anomaly AUTOSOMAL RECESSIVE MARFANS SYNDROME ALPORTS SYNDROME WEILL-MARCHESANI SYN H yperlysinemea and congenital rubella. 

3. DUE TO UNDER DEVOLPMENT OF ZONULE OF ZINN HOMOZYGOUS MUTATION TO LTBP2 GENE ↑ AP DIAMETER & ↓ EQUATORIAL DM WEAKNESS & NON ATTACHMENT OF POSTERIOR ZONULES TO CILIARY PROCESS LENS MOVES WITH CHANGE IN POSTURE

4. Complications Subluxated / dislocated lens Progressive myopia Defective accommodation GLAUCOMA RD

5. ANGLE CLOSURE GLAUCOMA SHALLOW AC MYOPIA Imp cause of permanent visual loss – phakic PUPILLARY BLOCK GLAUCOMA

6. MECHANISM Forward movement of spherical lens – loose zonules / dislocated lens – inferiorly CHRONIC ANGLE CLOSURE GLAUCOMA Narrowing of AC angle Angle anomaly in spherophakia Iris bowing may also give rise to PAS formation

7. S igns BILATERAL Lens DM small - full mydriasis whole lens visible Moves with change in posture Dislocation Lenticular myopia Defective accommodation Glaucoma Posterior staphyloma Myopic crescent Ectopic pupil RD Blue sclera Hypoplasia of dilator muscles

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

9. Signs Magnified picture showing signs of acute angle closure: patches of iris atrophy. dilated iris vessels and glaucomflecken Completely closed angles on gonioscopy

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

11. Systematic association ,general medical evaluation advisable Systematic connective tissue disorder- Weil- marchesani short stubby fingers short stature Broad hands Joint stiffness Decreased mobility Marfan &syndrome – cardiac,skeletal&muscular system Ectopialentis

12. Pupillary block is exacerbated with miotics and relieved by mydriatics INVERSE GLACOMA Miotics - ciliary muscle contraction,loosening zonules -forward movement-lens AC-shallow cycloplegics -relax-tighten zonules -posterior movement AC deep Unrelieved pupil block-PAS formation-irreversible trabecular damage

13. Various causes of increased IOP- pupillary block, abnorm of trabecular meshwork , inverse glaucoma, dislocation HIGH myopia – second decade – LENTICULAR INCREASED lenticular curvature & forward diasplacement Shallow anterior chamber

14. MANAGEMENT Followed up for glaucoma & systemic workup Periodic refraction, IOP measurement and gonioscopy Lens subluxation with closed angles / rise in iop secondary to pupillary block or angle closure – PERIPHERAL IRIDOTOMY Pretreament with pilocarpine should be avoided Lens extraction Chronic glaucoma - lensectomy with goniosynechialysis – synechial angle closure

15. Gross subluxation – PPV with lensectomy Disc damage – combined lensectomy and trabeculectomy In children bilateral surgery within a short period is essential – visual rehabilitation – prevent amblyopia Antiglaucoma medications and Nd;yag PI Chroni ACG –TRABECULECTOMY – standard Rx Precautions – flat Ac, malignant glaucoma Preop iv mannitol , paracentesis – primary AC reformation to prevent flat AC POST OP CYCLOPLEGICS – DEEP AC

16. INDICATIONS FOR LENS EXTRACTION Cataract Coneo -lenticular touch High myopia Intermittent pupillary block High myopia

17. Phaco with PCIOL ACRYLIC foldable lens used Capsulorrhexis – iris hook - capsular tension ring Early recognition & management prevents secondary glaucoma, iris lens corneal touch & corneal decompensation

18. 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. Intra-operative photograph of the left eye showing the edge of the crystalline lens within the pupillary border. First post-operative day of the left eye after undergoing a pars- plana lensectomy and anterior vitrectomy . Picture of the right eye after dilatation showing clearly the small and spherical crystalline lens with the lens edge visible within the pupillary border.

19. 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 first line strategy for management

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