Surgical Treatment Options in Congenital Aniridia .Surgical Treatment Options in Congenital Aniridia

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  • Surgical Treatment Options in Congenital Aniridia

    Cataracts, Glaucomas, Corneal Opacities

    Berthold Seitz

    UniversitUniversittsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKSKlinik fKlinik fr Augenheilkunde, Homburg/Saarr Augenheilkunde, Homburg/Saar

    Direktor: Prof. Dr. med. Berthold Seitz, ML, FEBODirektor: Prof. Dr. med. Berthold Seitz, ML, FEBO

  • Unsere Augen das Tor zur Welt

    UniversitUniversittsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKSKlinik fKlinik fr Augenheilkunde, Homburg/Saarr Augenheilkunde, Homburg/Saar

    Direktor: Prof. Dr. med. Berthold Seitz, ML, FEBODirektor: Prof. Dr. med. Berthold Seitz, ML, FEBO

  • Surgical Treatment Options in Aniridia

  • Surgical Treatment Options in Aniridia

  • Surgical Treatment Options in Aniridia

    Incidence: 1:64.000 1:96.000

    Decreased acitivity of PAX6 geneon chromosome 11p13

    2/3 autosomal dominant inherited

    Wilms tumor predisposition (sporadic)

    Basics in Aniridia

  • Surgical Treatment Options in Aniridia

    Cornea Iris Lens Chamber Angle (Glaucoma)

    Fovea Papilla

    Defective Ocular Tissues in Aniridia

    >> Low Vision>> Nystagmus

  • Anatomy of the Eye

    Surgical Treatment Options in Aniridia

  • Reversible Blindness vs. Irreversible Blindness(Cornea, Lens , Vitreous) (Optic Nerve , Retina, Brain)

    CataractCorneal Opacities

    Glaucoma

    Surgical Treatment Options in Aniridia

  • Surgical Treatment Options in Aniridia

    There are no straight-forwardgenerally accepted

    treatment modalities !!

    Aniridia is an Orphan Disease

  • Typical Patients Records

    Surgical Treatment Options in Aniridia

  • Typical Patients Course of Disease - PF

    Surgical Treatment Options in Aniridia

    15.11.2001: Date of birth04.04.2003: Date of first presentation - VA OD/OS 0.05-0.1

    - congenital total aniridia- macula hypoplasia- nystagmus- hyperopia astigmatism- aniridia associated glaucoma

    07.11.2007: OD/OS CPK07.01.2009: OD/OS CPK03.03.2010: OD/OS CPK30.03.2010: OD Phaco + Morcher black iris diaphragm IOL27.07.2010: OD ppVy with removal of fibrous membrane, hyopotony25.04.2013: last visit - VA OD LP, OS0.1

  • Typical Patients Course of Disease - SW

    Surgical Treatment Options in Aniridia

    03.11.1986: Date of birth10.03.1987: Date of first presentation

    - congenital partial aniridia- macula hypoplasia- nystagmus

    14.06.1993: Incipient subcapsular cataract21.11.1996: OD Phaco + Morcher black iris diaphragm IOL28.11.1996: OS Phaco + Morcher black iris diaphragm IOL20.02.1997: OD ppVy with removal secondary cataract08.05.2001: OS Ahmed valve glaucoma surgery12.09.2001: OD Ahmed valve galucoma surgery22.08.2011: OS CPK25/26.10.2012: OD/OS CPK12.03.13: last visit: VA OD/OS 1/50 LT in 1 m

  • Major Treatment Problems

    Glaucomas ~ 50%Late childhood

    Brunner SC, Walton DS, Chen TC: Aniridia. Int Ophthalmol Clinics 2008; 48 (2): 79-85

    Surgical Treatment Options in Aniridia

  • Obscuration of trabecular meshwork and limbal spur

    by tissue strands containing blood vessels

    Rudimentary iris plane prallel to the axis of the eye

    Absence of Schlemms canal

    Pathophysiology of Aniridic Glaucomas

    Surgical Treatment Options in Aniridia

  • Chamber Angle

    Surgical Treatment Options in Aniridia

  • congenitalcongenital

    BuphthalmusBuphthalmus

    with congenital anomalies

    AniridieAxenfeld-Rieger SyndromSturge-Weber Syndrom

    Marfan-Syndrom

    with congenital anomalies

    AniridieAxenfeld-Rieger SyndromSturge-Weber Syndrom

    Marfan-Syndrom

    Glaucomas in Children

    Ninios K, Jonescu-Cuypers CP, Seitz B: Glaukome bei primren Irisvernderungen - Axenfeld-Rieger-Syndrom, ICE-Syndrome (essenzielle Irisatrophie, Chandler-Syndrom, Cogan-Reese-Syndrom), Aniridie. Ophthalmologe 2011; 108:585-593

    Surgical Treatment Options in Aniridia

  • Surgical Treatment Options in Aniridia

    Aniridic Glaucoma

  • Glaucoma Diagnostics

    3 Approaches3 Approaches

    IOPIOPMorphology of Optic Nerve Head

    Morphology of Optic Nerve Head

    Visual FieldsVisual Fields

    Surgical Treatment Options in Aniridia

  • GonioscopySurgical Treatment Options in Aniridia

  • IOP MeasurementApplanation tonometry

    GoldmannImpression tonometry

    Schitz

    Surgical Treatment Options in Aniridia

    Icare tonometer

  • IOP Measurement

    Surgical Treatment Options in Aniridia

    Always measure corneal thickness !Mean: 631 51 m 1

    691 75 m 2

    1 Brandt JD, Casuso LA, Budenz DL: Markedly increased central corneal thickness: an unrecognized finding in congenital aniridia. Am J Ophthalmol 2004; 137:348-350

    2 Whitson JT, Liang C, Godfrey DG, Petroll VM, Cavanagh HD, Patel D, Fellman RL, Starita RJ: Central corneal thickness in patients with congenital aniridia. Eye Contact Lens 2005; 31:221-224

  • Optic Nerve Head

    Surgical Treatment Options in Aniridia

  • Surgical Treatment Options in Aniridia

    Optic Nerve Head

  • Aniridic Glaucoma

    Surgical Treatment Options in Aniridia

  • Documentation of Optic Nerve Head

    Surgical Treatment Options in Aniridia

  • Visual Fields

    Surgical Treatment Options in Aniridia

  • Glaucoma Early Stage

    Surgical Treatment Options in Aniridia

    Visual Fields

  • Glaucoma Late Stage

    Surgical Treatment Options in Aniridia

    Visual Fields

  • Topical Antiglaucomatous Therapy

    Surgical Treatment Options in Aniridia

  • Surgical Treatment Options in Aniridia

    Topical Antiglaucomatous Therapy

  • Goniotomy

    Trabeculotomy

    Trabeculectomy with Mitomycin C

    Glaucoma drainage devices (Molteno, Baerveldt, Ahmed)

    Cyclosdestructive procedures (CPK) not CCK!!!

    Modified goniosurgery (removal of abnormal tissue spanning between iris rudimentary stump and trabecular

    meshwork)

    Trabectome in pseudophakia or simultaneous with phaco ???

    Options of Glaucoma Surgery

    Surgical Treatment Options in Aniridia

  • Trabeculectomy with MMC

    Surgical Treatment Options in Aniridia

  • Corneal decompensation in up to 30% of eyesTopouzis et al. Am J Ophthalmol 1999

    Glaucoma Drainage Devices

    Surgical Treatment Options in Aniridia

  • Major Treatment Problems

    Iris Hypoplasia

    Brunner SC, Walton DS, Chen TC: Aniridia. Int Ophthalmol Clinics 2008; 48 (2): 79-85

    Surgical Treatment Options in Aniridia

  • Surgical Treatment Options in Aniridia

    Artificial Iris ?????

  • Artificial Iris

    Surgical Treatment Options in Aniridia

  • Eye lid surgery Iris imitating contact lens Corneal tattooing (Alio JL, et al. Br J Ophthalmol 2011; 95:1397-1401) Artificial iris into the capsular bag during cataract surgery

    We do NOT advocate implantation of artificial irides into the sulcus often worsening of glaucoma !! risk of progressive anterior fibrosis syndrome

    Options for Compensation of Iris Hypoplasia

    Surgical Treatment Options in Aniridia

    CAVE: PANAMA - NewColorIris

    Tsai JH, Freeman JM, Chan CC et al: A progressive anterior fibrosis syndrome in patients with postsurgical congenital aniridia. Am J Ophthalmol 2005; 140:1075-1079

    George MK, Tsai JC, Loewen NA: Bilateral irreversible severe vision loss from cosmetic iris implants. Am J Ophthalmol 2011; 151:872-875

  • Simulation zur Implantation einer Iris-Teilprothese

    Endergebnis desoperierten Auges

    (Die Naht ist praktisch nicht sichtbar)

    Gesundes Augemit intakter Iris

    (Foto als Vorlage)

    Verletztes Auge -partielle Aniridie

    Fixierung derIris-Teilprothese

    Surgical Treatment Options in Aniridia

    Artificial Iris from Gore -Tex

    Dr. Schmidt Intraocularlinsen GmbH

  • Instead of Artificial Iris

    Surgical Treatment Options in Aniridia

  • Major Treatment Problems

    Lens Abnormalities and Cataract - 50% - 85%

    Surgical Treatment Options in Aniridia

    Lee H, Khan R, OKeefe M: Aniridia: current pathology and management. Acta Ophthalmol 2008; 86:708-715

  • Fragile anterior lens capsule

    Zonular weakness

    Morphological changes at the equator

    The Lens in Aniridia

    Surgical Treatment Options in Aniridia

  • HH26011999

    Aufn v 08022011

    Surgical Treatment Options in Aniridia

    The Lens in Aniridia

  • Surgical Treatment Options in Aniridia

    The Lens in Aniridia

  • Surgical Treatment Options in Aniridia

    The Lens in Aniridia

  • Surgical Treatment Options in Aniridia

    The Lens in Aniridia

  • Surgical Treatment Options in Aniridia

    The Lens in Aniridia

  • Surgical Treatment Options in Aniridia

    The Lens in Aniridia

  • Surgical Treatment Options in Aniridia

    IOL Calculation

  • IOL CalculationSurgical Treatment Options in Aniridia

    Eye length

  • Surgical Treatment Options in Aniridia

    IOL Calculation

    Corneal CurvatureCorneal Power

  • - 90% as out-patient- Local anesthesia (RBA/PBA/Topical)- Clear cornea incision (2.5 mm)-Bimanual phacoemulsification- Foldable IOL implantation via inserter

    Surgical Treatment Options in Aniridia

    > 2000 Cataract Surgeries per Year in HOM

  • PhacoemulsificationPhacoemulsification