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 – Cataracts, Glaucomas, Corneal Opacities
Dec 09, 2022
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Aniridie--Grauer-Grüner-Star-Limbusstammzellinsuffienz-03-06-2013Cataracts, Glaucomas, Corneal Opacities
UniversitUniversitäätsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKS Klinik 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
UniversitUniversitäätsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKS Klinik fKlinik füür 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
2/3 autosomal dominant inherited
Wilms tumor predisposition (sporadic)
Cornea Iris Lens Chamber Angle (Glaucoma)
Fovea Papilla
>> Low Vision >> Nystagmus
Reversible Blindness vs. Irreversible Blindness (Cornea, Lens , Vitreous) (Optic Nerve , Retina, Brain)
Cataract Corneal Opacities
There are no straight-forward generally accepted
treatment modalities !!
Typical Patient‘s Records
Typical Patient‘s Course of Disease - PF
Surgical Treatment Options in Aniridia
15.11.2001: Date of birth 04.04.2003: 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 CPK 07.01.2009: OD/OS CPK 03.03.2010: OD/OS CPK 30.03.2010: OD Phaco + Morcher black iris diaphragm IOL 27.07.2010: OD ppVy with removal of fibrous membrane, hyopotony 25.04.2013: last visit - VA OD LP, OS0.1
Typical Patient‘s Course of Disease - SW
Surgical Treatment Options in Aniridia
03.11.1986: Date of birth 10.03.1987: Date of first presentation
- congenital partial aniridia - macula hypoplasia - nystagmus
14.06.1993: Incipient subcapsular cataract 21.11.1996: OD Phaco + Morcher black iris diaphragm IOL 28.11.1996: OS Phaco + Morcher black iris diaphragm IOL 20.02.1997: OD ppVy with removal secondary cataract 08.05.2001: OS Ahmed valve glaucoma surgery 12.09.2001: OD Ahmed valve galucoma surgery 22.08.2011: OS CPK 25/26.10.2012: OD/OS CPK 12.03.13: 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 Schlemm‘s canal
Pathophysiology of Aniridic Glaucomas
Chamber Angle
congenitalcongenital
BuphthalmusBuphthalmus
Marfan-Syndrom
Marfan-Syndrom
Surgical Treatment Options in Aniridia
Surgical Treatment Options in Aniridia
Aniridic Glaucoma
Glaucoma Diagnostics
Morphology of Optic Nerve Head
Visual FieldsVisual Fields
Gonioscopy Surgical Treatment Options in Aniridia
IOP Measurement Applanation tonometry
Icare tonometer
IOP Measurement
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
Optic Nerve Head
Optic Nerve Head
Visual Fields
Glaucoma Early Stage
Visual Fields
Visual Fields
Topical Antiglaucomatous Therapy
Cyclosdestructive procedures (CPK) – not CCK!!!
Modified goniosurgery (removal of abnormal tissue
spanning between iris rudimentary stump and trabecular
meshwork)
Options of Glaucoma Surgery
Trabeculectomy with MMC
Surgical Treatment Options in Aniridia
Corneal decompensation in up to 30% of eyes Topouzis et al. Am J Ophthalmol 1999
Glaucoma Drainage Devices
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 des operierten Auges
Gesundes Auge mit intakter Iris
(Foto als Vorlage)
Dr. Schmidt Intraocularlinsen GmbH
Instead of Artificial Iris ……
Major Treatment Problems
Surgical Treatment Options in Aniridia
Lee H, Khan R, O‘Keefe M: Aniridia: current pathology and management. Acta Ophthalmol 2008; 86:708-715
Fragile anterior lens capsule
The Lens in Aniridia
HH 26011999
The Lens in Aniridia
The Lens in Aniridia
The Lens in Aniridia
The Lens in Aniridia
The Lens in Aniridia
The Lens in Aniridia
IOL Calculation
Eye length
IOL Calculation
- 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
> 2000 Cataract Surgeries per Year in HOM
Standard Cataract Surgery + Implantation of Artificial Lens
Microsurgeon: B. Seitz
Black iris diaphragm IOL (Morcher 67F and 67G)
(sulcus fixated or sutured transsclerally)
(via 10 mm incision )
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
Black Iris Diaphragm IOL
Black Iris Diaphragm IOL
Black Iris Diaphragm IOL
Postoperative uveitis
Seconday glaucoma
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
http://www.sciencedirect.com/science/article/pii/S0181551209000722#fig4
Coloured Iris Diaphragm IOL
Morcher Aniridia Ring Type 50C + Yellow IOL
Surgical Treatment Options in Aniridia
http://www.sciencedirect.com/science/article/pii/S0181551209000722#fig4
Major Treatment Problems
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
Anirida -Associated Keratopathy (AAK)
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 involves excessive manipulation of the limbus – or
after application of topical antimetabolites to treat aniridia-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
Dark glasses against photophobia
Conservative Treatment of AAK
Jastaneiah S & Al-Rajhi AA: Association of aniridia and dry eyes. Ophthalmology 2005; 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
Surgical Treatment Options in Aniridia
Anirida -Associated Keratopathy (AAK)
Anirida -Associated Keratopathy (AAK)
Anirida -Associated Keratopathy (AAK)
Limbal stem cell transplantation
Non-matched organ-cultured donor Keratolimbal allograft
Ex vivo expansion of stem cells on amniotic membrane
Options of Corneal Surgery
Systemic immunosuppression necessary !!!!
14 mm peripheral lamellar/central penetrating corneoscleral graft
Boston keratoprosthesis
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
Deutsches Keratoplastikregister Gemeldete Hornhauttransplantationen pro Jahr
1980 - 2011
1980-2011
19 80
19 81
19 82
19 83
19 84
19 85
19 86
19 87
19 88
19 89
19 90
19 91
19 92
19 93
19 94
19 95
19 96
19 97
19 98
19 99
20 00
20 01
20 02
20 03
20 04
20 05
20 06
20 07
20 08
20 09
20 10
20 11
~ 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
Processed Corneas since Foundation of Processed Corneas since Foundation of LIONS Eye Bank in Homburg/SaarLIONS Eye Bank in Homburg/Saar
Hornhautentnahmen Universitäts-Augenklinik Homburg/Saar
Heute: Tag der Organspende !!!
Keratoplastiken Universitäts-Augenklinik Homburg/Saar
Methode der Excimerlaser -Trepanation
Empfänger- maske
Photoablation
Spendermaske mit acht Erlanger/Homburger
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
„Orientation Teeth “
Practical Advantage
Surgical Treatment Options in Aniridia
Excimer Laser Trephination - VIDEO
Typical corneal grafts 6 weeks after keratoplasty
Markus Klingenstein 16121985
Aufn v 050122012
Excimer Laser Keratoplasty with Single Sutures
SF 21031972
Foto 29092010
SF 21031972
Foto 22112010
Excimer Laser Keratoplasty with Single Sutures
SF 1031972
Foto 11052011
Excimer Laser Keratoplasty with Single Sutures
SF 21031972
Foto 26102011
Excimer Laser Keratoplasty with Single Sutures
Indispensable: Save the scleral spur !!
Spender
Central 14 mm Corneoscleroplasty
Central 14 mm Corneoscleroplasty
Boston Keratoprosthesis in Children
Boston Keratoprosthesis Visual results
patno diagnosis BCVA Preop
0.15LP
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
Boston Keratoprosthesis in Children
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
Despite our increasing understanding of the genetics and pathophysiology of congenital aniridia,
effective treatment remains elusive
Strongly individualized approach - stay conservative
Conclusions
Erfahrung ist nicht vererbbar –
1890 - 1935
UniversitUniversitäätsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKS Klinik 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
[email protected]
Augen ärzte sind Organspender !Augen ärzte sind Organspender !
[email protected]@uks.eu
UniversitUniversitäätsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKS Klinik 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
UniversitUniversitäätsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKS Klinik fKlinik füür 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
2/3 autosomal dominant inherited
Wilms tumor predisposition (sporadic)
Cornea Iris Lens Chamber Angle (Glaucoma)
Fovea Papilla
>> Low Vision >> Nystagmus
Reversible Blindness vs. Irreversible Blindness (Cornea, Lens , Vitreous) (Optic Nerve , Retina, Brain)
Cataract Corneal Opacities
There are no straight-forward generally accepted
treatment modalities !!
Typical Patient‘s Records
Typical Patient‘s Course of Disease - PF
Surgical Treatment Options in Aniridia
15.11.2001: Date of birth 04.04.2003: 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 CPK 07.01.2009: OD/OS CPK 03.03.2010: OD/OS CPK 30.03.2010: OD Phaco + Morcher black iris diaphragm IOL 27.07.2010: OD ppVy with removal of fibrous membrane, hyopotony 25.04.2013: last visit - VA OD LP, OS0.1
Typical Patient‘s Course of Disease - SW
Surgical Treatment Options in Aniridia
03.11.1986: Date of birth 10.03.1987: Date of first presentation
- congenital partial aniridia - macula hypoplasia - nystagmus
14.06.1993: Incipient subcapsular cataract 21.11.1996: OD Phaco + Morcher black iris diaphragm IOL 28.11.1996: OS Phaco + Morcher black iris diaphragm IOL 20.02.1997: OD ppVy with removal secondary cataract 08.05.2001: OS Ahmed valve glaucoma surgery 12.09.2001: OD Ahmed valve galucoma surgery 22.08.2011: OS CPK 25/26.10.2012: OD/OS CPK 12.03.13: 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 Schlemm‘s canal
Pathophysiology of Aniridic Glaucomas
Chamber Angle
congenitalcongenital
BuphthalmusBuphthalmus
Marfan-Syndrom
Marfan-Syndrom
Surgical Treatment Options in Aniridia
Surgical Treatment Options in Aniridia
Aniridic Glaucoma
Glaucoma Diagnostics
Morphology of Optic Nerve Head
Visual FieldsVisual Fields
Gonioscopy Surgical Treatment Options in Aniridia
IOP Measurement Applanation tonometry
Icare tonometer
IOP Measurement
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
Optic Nerve Head
Optic Nerve Head
Visual Fields
Glaucoma Early Stage
Visual Fields
Visual Fields
Topical Antiglaucomatous Therapy
Cyclosdestructive procedures (CPK) – not CCK!!!
Modified goniosurgery (removal of abnormal tissue
spanning between iris rudimentary stump and trabecular
meshwork)
Options of Glaucoma Surgery
Trabeculectomy with MMC
Surgical Treatment Options in Aniridia
Corneal decompensation in up to 30% of eyes Topouzis et al. Am J Ophthalmol 1999
Glaucoma Drainage Devices
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 des operierten Auges
Gesundes Auge mit intakter Iris
(Foto als Vorlage)
Dr. Schmidt Intraocularlinsen GmbH
Instead of Artificial Iris ……
Major Treatment Problems
Surgical Treatment Options in Aniridia
Lee H, Khan R, O‘Keefe M: Aniridia: current pathology and management. Acta Ophthalmol 2008; 86:708-715
Fragile anterior lens capsule
The Lens in Aniridia
HH 26011999
The Lens in Aniridia
The Lens in Aniridia
The Lens in Aniridia
The Lens in Aniridia
The Lens in Aniridia
The Lens in Aniridia
IOL Calculation
Eye length
IOL Calculation
- 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
> 2000 Cataract Surgeries per Year in HOM
Standard Cataract Surgery + Implantation of Artificial Lens
Microsurgeon: B. Seitz
Black iris diaphragm IOL (Morcher 67F and 67G)
(sulcus fixated or sutured transsclerally)
(via 10 mm incision )
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
Black Iris Diaphragm IOL
Black Iris Diaphragm IOL
Black Iris Diaphragm IOL
Postoperative uveitis
Seconday glaucoma
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
http://www.sciencedirect.com/science/article/pii/S0181551209000722#fig4
Coloured Iris Diaphragm IOL
Morcher Aniridia Ring Type 50C + Yellow IOL
Surgical Treatment Options in Aniridia
http://www.sciencedirect.com/science/article/pii/S0181551209000722#fig4
Major Treatment Problems
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
Anirida -Associated Keratopathy (AAK)
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 involves excessive manipulation of the limbus – or
after application of topical antimetabolites to treat aniridia-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
Dark glasses against photophobia
Conservative Treatment of AAK
Jastaneiah S & Al-Rajhi AA: Association of aniridia and dry eyes. Ophthalmology 2005; 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
Surgical Treatment Options in Aniridia
Anirida -Associated Keratopathy (AAK)
Anirida -Associated Keratopathy (AAK)
Anirida -Associated Keratopathy (AAK)
Limbal stem cell transplantation
Non-matched organ-cultured donor Keratolimbal allograft
Ex vivo expansion of stem cells on amniotic membrane
Options of Corneal Surgery
Systemic immunosuppression necessary !!!!
14 mm peripheral lamellar/central penetrating corneoscleral graft
Boston keratoprosthesis
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
Deutsches Keratoplastikregister Gemeldete Hornhauttransplantationen pro Jahr
1980 - 2011
1980-2011
19 80
19 81
19 82
19 83
19 84
19 85
19 86
19 87
19 88
19 89
19 90
19 91
19 92
19 93
19 94
19 95
19 96
19 97
19 98
19 99
20 00
20 01
20 02
20 03
20 04
20 05
20 06
20 07
20 08
20 09
20 10
20 11
~ 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
Processed Corneas since Foundation of Processed Corneas since Foundation of LIONS Eye Bank in Homburg/SaarLIONS Eye Bank in Homburg/Saar
Hornhautentnahmen Universitäts-Augenklinik Homburg/Saar
Heute: Tag der Organspende !!!
Keratoplastiken Universitäts-Augenklinik Homburg/Saar
Methode der Excimerlaser -Trepanation
Empfänger- maske
Photoablation
Spendermaske mit acht Erlanger/Homburger
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
„Orientation Teeth “
Practical Advantage
Surgical Treatment Options in Aniridia
Excimer Laser Trephination - VIDEO
Typical corneal grafts 6 weeks after keratoplasty
Markus Klingenstein 16121985
Aufn v 050122012
Excimer Laser Keratoplasty with Single Sutures
SF 21031972
Foto 29092010
SF 21031972
Foto 22112010
Excimer Laser Keratoplasty with Single Sutures
SF 1031972
Foto 11052011
Excimer Laser Keratoplasty with Single Sutures
SF 21031972
Foto 26102011
Excimer Laser Keratoplasty with Single Sutures
Indispensable: Save the scleral spur !!
Spender
Central 14 mm Corneoscleroplasty
Central 14 mm Corneoscleroplasty
Boston Keratoprosthesis in Children
Boston Keratoprosthesis Visual results
patno diagnosis BCVA Preop
0.15LP
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
Boston Keratoprosthesis in Children
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
Despite our increasing understanding of the genetics and pathophysiology of congenital aniridia,
effective treatment remains elusive
Strongly individualized approach - stay conservative
Conclusions
Erfahrung ist nicht vererbbar –
1890 - 1935
UniversitUniversitäätsklinikum des Saarlandes tsklinikum des Saarlandes UKSUKS Klinik 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
[email protected]
Augen ärzte sind Organspender !Augen ärzte sind Organspender !
[email protected]@uks.eu
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