MICROSPHEROPHAKIA

BY S.DHIVYA CRRI 2009 BATCH

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 Hyperlysinemea and congenital rubella. 

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

Complications Subluxated / dislocated lens

Progressive myopia

Defective accommodation

GLAUCOMA

RD

ANGLE CLOSURE GLAUCOMA

SHALLOW AC MYOPIA Imp cause of permanent visual loss – phakic PUPILLARY BLOCK

GLAUCOMA

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

Signs 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

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.

Signs

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

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

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

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

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

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 avoidedLens extraction Chronic glaucoma - lensectomy with goniosynechialysis –synechial angle

closure

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

INDICATIONS FOR LENS EXTRACTION Cataract

Coneo-lenticular touch

High myopia

Intermittent pupillary block

High myopia

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

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.

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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