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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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