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Surgical Treatment Options in Congenital Aniridia Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit Universit ä ä tsklinikum des Saarlandes tsklinikum des Saarlandes UKS UKS Klinik f Klinik f ü ü r Augenheilkunde, Homburg/Saar r Augenheilkunde, Homburg/Saar Direktor: Prof. Dr. med. Berthold Seitz, ML, FEBO Direktor: Prof. Dr. med. Berthold Seitz, ML, FEBO

Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

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Page 1: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Congenital Aniridia –

Cataracts, Glaucomas, Corneal Opacities

Berthold Seitz

UniversitUniversitäätsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKSKlinik fKlinik füür Augenheilkunde, Homburg/Saarr Augenheilkunde, Homburg/Saar

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

Page 2: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Unsere Augen – das Tor zur Welt

UniversitUniversitäätsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKSKlinik fKlinik füür Augenheilkunde, Homburg/Saarr Augenheilkunde, Homburg/Saar

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

Page 3: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Page 4: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Page 5: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

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

Page 6: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

� Cornea� Iris� Lens � Chamber Angle (Glaucoma)

� Fovea� Papilla

Defective Ocular Tissues in Aniridia

>> Low Vision>> Nystagmus

Page 7: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Anatomy of the Eye

Surgical Treatment Options in Aniridia

Page 8: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

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

CataractCorneal Opacities

Glaucoma

Surgical Treatment Options in Aniridia

Page 9: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

There are no straight-forwardgenerally accepted

treatment modalities !!

Aniridia is an Orphan Disease

Page 10: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Typical Patient‘s Records

Surgical Treatment Options in Aniridia

Page 11: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Typical Patient‘s 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

Page 12: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Typical Patient‘s 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

Page 13: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum
Page 14: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

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

Page 15: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum
Page 16: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� 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 Schlemm‘s canal

Pathophysiology of Aniridic Glaucomas

Surgical Treatment Options in Aniridia

Page 17: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Chamber Angle

Surgical Treatment Options in Aniridia

Page 18: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

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 primären Irisveränderungen - Axenfeld-Rieger-Syndrom, ICE-Syndrome (essenzielle Irisatrophie, Chandler-Syndrom, Cogan-Reese-Syndrom), Aniridie. Ophthalmologe 2011; 108:585-593

Surgical Treatment Options in Aniridia

Page 19: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Aniridic Glaucoma

Page 20: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Glaucoma Diagnostics

3 Approaches3 Approaches

IOPIOPMorphology of Optic Nerve Head

Morphology of Optic Nerve Head

Visual FieldsVisual Fields

Surgical Treatment Options in Aniridia

Page 21: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

GonioscopySurgical Treatment Options in Aniridia

Page 22: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

IOP MeasurementApplanation tonometry

GoldmannImpression tonometry

Schiötz

Surgical Treatment Options in Aniridia

Icare tonometer

Page 23: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

IOP Measurement

Surgical Treatment Options in Aniridia

Always measure corneal thickness !Mean: 631 ± 51 µm 1

691 ± 75 µm 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

Page 24: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Optic Nerve Head

Surgical Treatment Options in Aniridia

Page 25: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Optic Nerve Head

Page 26: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Aniridic Glaucoma

Surgical Treatment Options in Aniridia

Page 27: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Documentation of Optic Nerve Head

Surgical Treatment Options in Aniridia

Page 28: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Visual Fields

Surgical Treatment Options in Aniridia

Page 29: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Glaucoma Early Stage

Surgical Treatment Options in Aniridia

Visual Fields

Page 30: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Glaucoma Late Stage

Surgical Treatment Options in Aniridia

Visual Fields

Page 31: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Topical Antiglaucomatous Therapy

Surgical Treatment Options in Aniridia

Page 32: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Topical Antiglaucomatous Therapy

Page 33: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� 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

Page 34: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Trabeculectomy with MMC

Surgical Treatment Options in Aniridia

Page 35: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

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

Glaucoma Drainage Devices

Surgical Treatment Options in Aniridia

Page 36: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

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

Page 37: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Artificial Iris ?????

Page 38: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Artificial Iris

Surgical Treatment Options in Aniridia

Page 39: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� 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

Page 40: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

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

Page 41: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Instead of Artificial Iris ……

Surgical Treatment Options in Aniridia

Page 42: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Major Treatment Problems

Lens Abnormalities and Cataract - 50% - 85%

Surgical Treatment Options in Aniridia

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

Page 43: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� Fragile anterior lens capsule

� Zonular weakness

� Morphological changes at the equator

The Lens in Aniridia

Surgical Treatment Options in Aniridia

Page 44: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

HH26011999

Aufn v 08022011

Surgical Treatment Options in Aniridia

The Lens in Aniridia

Page 45: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

The Lens in Aniridia

Page 46: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

The Lens in Aniridia

Page 47: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

The Lens in Aniridia

Page 48: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

The Lens in Aniridia

Page 49: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

The Lens in Aniridia

Page 50: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

IOL Calculation

Page 51: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

IOL CalculationSurgical Treatment Options in Aniridia

Eye length

Page 52: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

IOL Calculation

Corneal CurvatureCorneal Power

Page 53: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

- 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

Page 54: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

PhacoemulsificationPhacoemulsification

Surgical Treatment Options in Aniridia

> 2000 Cataract Surgeries per Year in HOM

Page 55: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Standard Cataract Surgery + Implantation of Artificial Lens

Microsurgeon: B. Seitz

Surgical Treatment Options in Aniridia

Page 56: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� Black iris diaphragm IOL (Morcher 67F and 67G)

(sulcus fixated or sutured transsclerally)

(via 10 mm incision )

� Morcher aniridia ring type 50C:

designed for intracapsular insertion. Two of these rings

are implanted and turned against each other in order

to produce a full iris diaphragm

� Foldable blue-light-filtering IOL (via 2.5 to 3.0 mm incision)

Options of Lens Surgery

Surgical Treatment Options in Aniridia

Page 57: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

HI 13061962Aufn v 21112011

Surgical Treatment Options in Aniridia

Black Iris Diaphragm IOL

Page 58: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Black Iris Diaphragm IOL

Page 59: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

http://www.sciencedirect.com/science/article/pii/S0181551209000722#fig4

Surgical Treatment Options in Aniridia

Black Iris Diaphragm IOL

Page 60: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Iris Diaphragm IOL - Potential Complications

� Postoperative uveitis

� Seconday glaucoma

� Clinically significant macular edema

� Progression of corneal epithelial disorder

� Hyphaema

Reinhard T, Engelhardt S, Sundmacher R: Black diaphragm aniridia intraocular lens for congenital aniridia: Long-term follow-up. J Cataract Refract Surg 2000; 26:375-381

Page 61: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

http://www.sciencedirect.com/science/article/pii/S0181551209000722#fig4

Surgical Treatment Options in Aniridia

Coloured Iris Diaphragm IOL

Page 62: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Morcher Aniridia Ring Type 50C

Page 63: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Morcher Aniridia Ring Type 50C + Yellow IOL

Page 64: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Page 65: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

http://www.sciencedirect.com/science/article/pii/S0181551209000722#fig4

Surgical Treatment Options in Aniridia

Morcher Aniridia Ring Type 50C

Page 66: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Major Treatment Problems

„Corneal Opacities“Anirida-Associated Keratopathy (AAK)

20% – 30%

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

Surgical Treatment Options in Aniridia

Page 67: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Anirida -Associated Keratopathy (AAK)

Surgical Treatment Options in Aniridia

Page 68: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� Often starting in the first decade of life

� Limbal stem cell deficiency

� Conjunctivalization of the cornea

� Recurrent corneal erosions and ulcerations

� Often worse after surgery that involvesexcessive manipulation of the limbus – or

� after application of topical antimetabolites to treataniridia-associated glaucoma

Surgical Treatment Options in Aniridia

Anirida -Associated Keratopathy (AAK)

Edén U, Riise R, Tornqvist K: Corneal invcolvement in congenital anirida, Cornea 2010; 29:1096--1102

Page 69: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� Dark glasses against photophobia

� Preservative-free lubricants

� Hyaluronic acid containing artificial tears

� 100% autologous serum eye drops

Surgical Treatment Options in Aniridia

Conservative Treatment of AAK

Jastaneiah S & Al-Rajhi AA: Association of aniridia and dry eyes. Ophthalmology2005; 112:1535—1540

Lopez-Garcia JS, Rivas L, Garcia-Lozano I, Murube J: Autologous serum eyedrops in the treatment of aniridic keratopathy. Ophthalmology 2008; 115:262-267

Page 70: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Anirida -Associated Keratopathy (AAK)

Page 71: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Anirida -Associated Keratopathy (AAK)

Page 72: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Anirida -Associated Keratopathy (AAK)

Page 73: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� Amniotic membrane transplantation(incl. enhancing survival of limbal stem cells)

� Limbal stem cell transplantation

� Triplet matching of living related donor

� Non-matched organ-cultured donor� Keratolimbal allograft

� Ex vivo expansion of stem cells on amniotic membrane

Options of Corneal Surgery

Surgical Treatment Options in Aniridia

Systemic immunosuppression necessary !!!!

Page 74: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� Penetrating excimer laser keratoplasty ?

� 14 mm deep lamellar corneoscleral graft

� 14 mm peripheral lamellar/central penetratingcorneoscleral graft

� Boston keratoprosthesis

Options of Corneal Surgery

Surgical Treatment Options in Aniridia

Rixen JJ et al: Treatment of aniridia with Boston Type I keratoprosthesis. Cornea 2013; Feb 26 [Epub ahead of print]

Kang JJ et al: Visual outcomes of Boston keratoprosthesis implantation as primary penetrating corneal procedure. Cornea 2012; 31:1436-1440

Bakhtiari P et al: Surgical and visual outcomes of the type I Boston keratoprosthesis for the management of aniridic fiibrosis sndrome in congenital anirida. Am J Ophthalmol 2012; 152:967-971

Page 75: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Deutsches Keratoplastikregister Gemeldete Hornhauttransplantationen pro Jahr

1980 - 2011

Deutsches Keratoplastikregister gemeldete Hornhauttransplantationen pro Jahr

1980-2011

789 10

45

1145

1246

1203

1262

1407

1363

1470 16

92 1920

3367

3245

4148

2430

3375

3355

3025

2391 27

24

4730

4848

4735

4840

3673

3631 40

64 4380

4257

4030 45

33

0

1000

2000

3000

4000

5000

6000

1980

1981

1982

1983

1984

1985

1986

1987

1988

1989

1990

1991

1992

1993

1994

1995

1996

1997

1998

1999

2000

2001

2002

2003

2004

2005

2006

2007

2008

2009

2010

2011

~ 35.000 pro Jahr in den U.S.A. ↓↓↓↓~ 4.500 pro Jahr in Deutschland ↓↓↓↓

~ 250 pro Jahr in Homburg/Saar ↑↑↑↑

Sektion Kornea in der DOG

Page 76: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Processed Corneas since Foundation of Processed Corneas since Foundation of LIONS Eye Bank in Homburg/SaarLIONS Eye Bank in Homburg/Saar

HornhautentnahmenUniversitäts-Augenklinik Homburg/Saar

154

260

185210

158 172

256

354

288

377 369

414

0

100

200

300

400

500

2000

2001

2002

2003

2004

2005

2006

2007

2008

2009

2010

2011

Kalenderjahr

Anz

ahl H

ornh

aute

ntna

hmen

Surgical Treatment Options in Aniridia

Heute:Tag der Organspende !!!

Page 77: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Corneal Transplantations in theCorneal Transplantations in theDepartment of Ophthalmology in Homburg/SaarDepartment of Ophthalmology in Homburg/Saar

KeratoplastikenUniversitäts-Augenklinik Homburg/Saar

13

8971

113

8071 64

52 57 62

9476

10490 85

94

65

105

171162

202208

243

0

40

80

120

160

200

240

1989

1990

1991

1992

1993

1994

1995

1996

1997

1998

1999

2000

2001

2002

2003

2004

2005

2006

2007

2008

2009

2010

2011

Kalenderjahr

Anz

ahl K

erat

opla

stik

en

Surgical Treatment Options in Aniridia

Page 78: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Methode der Excimerlaser -Trepanation

Photoablation

durch die Maske geschütztesHornhautgewebe

Rotierender Laserstrahlgeführt über HeNe-Laser

Empfänger-maske

Rotierender Laserstrahlgeführt über HeNe-Laser

durch die Maske geschütztesHornhautgewebe

Photoablation

Spender-maske

Spender Empfänger

Surgical Treatment Options in Aniridia

Page 79: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Spendermaske mit acht Erlanger/Homburger

„Orientierungszähnchen“

Empfängermaske mit acht „Orientierungskerben“

Page 80: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Spender trepanation unmittelbar vor der

Perforation

Orientierungszähnchen

Behrens A, Seitz B, et al:"Orientation teeth" in nonmechanical laser corneal trephination for penetrating keratopasty: 2.94 µm Er:YAG vs. 193 nm ArF excimer laser.Br J Ophthalmol 1999; 83:1008-1012

Page 81: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

„Orientation Teeth “

Practical Advantage

for the Microsurgeon

Exact positioning of the second cardinal suture

Surgical Treatment Options in Aniridia

Page 82: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Excimer Laser Trephination - VIDEO

Surgical Treatment Options in Aniridia

Page 83: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Typical corneal grafts6 weeks after keratoplasty

Page 84: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Markus Klingenstein 16121985

Aufn v 050122012

Surgical Treatment Options in Aniridia

Excimer Laser Keratoplasty with Single Sutures

Page 85: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

SF21031972

Foto 29092010

Surgical Treatment Options in Aniridia

Irreversible Graft Failure After Keratoplasty

Page 86: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

SF21031972

Foto 22112010

Surgical Treatment Options in Aniridia

Excimer Laser Keratoplasty with Single Sutures

Page 87: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

SF1031972

Foto 11052011

Surgical Treatment Options in Aniridia

Excimer Laser Keratoplasty with Single Sutures

Page 88: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

SF21031972

Foto 26102011

Surgical Treatment Options in Aniridia

Excimer Laser Keratoplasty with Single Sutures

Page 89: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� Indispensable: Save the scleral spur !!

Spender

Surgical Treatment Options in Aniridia

Central 14 mm Corneoscleroplasty

Page 90: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� Systemic Immunosuppression (Cyclosporin A oder MMF)

Central 14 mm Corneoscleroplasty

Surgical Treatment Options in Aniridia

Page 91: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Boston Keratoprosthesis in Children

Surgical Treatment Options in Aniridia

Page 92: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Boston Keratoprosthesis Visual results

patno diagnosisBCVAPreop

BCVAPostop

1 chem burn LP0.5�re OK�

0.15�LP

2 chem burn HM 0.9

3 chem burn LP 0.2

4 chem burn LP 1.2--> LP� OOKP

5 Limbal stem def eci FC 0.01

6 Limbal stem def eci LP LP

7 Limbal stem def eci FC FC

8 Aniridia LP 0.15

9 Aniridia LP 0.1

10 Aniridia FC 0.15

Lies Remeijer, SOE 2007

Page 93: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Surgical Treatment Options in Aniridia

Boston Keratoprosthesis in Children

Page 94: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

�Anirida is a profibrotic disorder.�Therefore, many interventions - incl. penetrating keratopalsty and filtering surgery – tend to fail.�The Boston KPro may provide a more effective approach in the management of anirida-associated keratopathy AAK.�Guarded filtration surgery appears to be effctive in glaucoma.�Small incision cataract surgery seems to have advantages over large incisions for black iris diaphragm IOLs.

Summary

Surgical Treatment Options in Aniridia

Page 95: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

� Despite our increasing understandingof the genetics and pathophysiologyof congenital aniridia,

� effective treatment remains elusive

� with many of the conditions recurringafter surgical intervention.

�Strongly individualized approach -stay conservative

Conclusions

Surgical Treatment Options in Aniridia

Page 96: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Erfahrung ist nicht vererbbar –

Jeder muß seine eigenen machen.

Kurt TucholskyJournalist und Schriftsteller

1890 - 1935

Surgical Treatment Options in Aniridia

Page 97: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

UniversitUniversitäätsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKSKlinik fKlinik füür 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

Surgical Treatment Options in Aniridia

Page 98: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

[email protected]

Ophthalmologist are Organ Donors!Ophthalmologist are Organ Donors!

Thank you very much for your attention !

Page 99: Surgical Treatment Options in Congenital Aniridia · Surgical Treatment Options in Congenital Aniridia – Cataracts, Glaucomas, Corneal Opacities Berthold Seitz Universit ätsklinikum

Augen ärzte sind Organspender !Augen ärzte sind Organspender !

[email protected]@uks.eu

Vielen Dank für Ihre Aufmerksamkeit !Vielen Dank fVielen Dank f üür Ihre Aufmerksamkeit !r Ihre Aufmerksamkeit !