Case conference Limbal cell deficiency Let me introduce a case of limbal cell deficiency. The presentationer is 4th yr resident J Kim. This case is supervised by associate Professor So Hyang Jung. Case conference Limbal cell deficiency 서울 성모병원 Ap. 정소향/R4 김재련

55/M 김 O 종 C/C Decreased vision, Foreign body sense (OD) for 7 month DM/HBP(-/-) Ocular op/trauma (-/+) Explosion of Natrate induced burn to both eyes 9 month ago AMT(OD)x2 Glasses(-) A 55-yr old man visited our clinic. His chief complaint was recurrent corneal erosion started from 7months ago. He had no underlying disease. On the review of his past history, he had trauma history of both eye due to nitrate explosion 9 months ago. He received amniotic memebrane transplantation two times on his right eye because of the epidefect of the right cornea

Ocular Examination VA OD HM(N-C) EOM straight by ACT OS 0.32(N-C) EOM straight by ACT Lid upper cilia touch to cornea (OD) Conj. mod. Injected (OD) mild Injected (OS) Cornea mod. edematous c diffuse stormal opacity c 4x3 mm sized epidefect c 360 NV involving pph. (OD) mild edematous (OS) His visual acuity of right eye was hand mothion with or without correction & visual acuity of the left eye was 0.32 with or without correction. It was noticed that upper cilia touch to cornea. The conjunctiva of the right eye was injected moderately & that of the left eye was injected mildly. His right cornea was moderately edematous and it showed diffuse stromal opacity. The 4 x 3 mm sized epidefect was located in the center of the cornea. NVS was growing in to the cornea from the limbus about 360 degree. The left cornea was mildly edematous (OD) (OD)

Ocular Examination AC deep & cell(-) (OU) Pupil round & nl. Sized (OU) Lens ant. polar opacity (OD) mild cortical opacity (OS) IOP 12mm Hg (OD) 14mmHg (OS) His right lens showed anterior polar opacity, otherwise unremarkable.

Imp.) Px.) Total limbal cell deficiency after alkali burn (OD) Entropion (OD) Px.) Doxycycline 100mg #1 O-GAF x4, O-FM x4, O-TMO xhs O-3HUM (OD) Blephasol for lid hygiene consider OP 1. Entropion repair (OD) 2. Keratolimbal allograft with AMT (OD) On the basis of his history and ocular examination, our impression is recurrent epitheliopathy and entropion of the right eye due to chronic inflammation and limbal cell deficiency caused by alkali burn. We prescribed Doxycycline 100mg per day ,O-GAFx4, O-FMx4, O-TMOxhs, preservative free artificail eaydreop and Blepharasol for lid hygiene. We decided to do entropion repair surgery of the right eye before the keratolimbal allograft and amniotic membrane transplantation.

POD # 7 after entropion repair VA : OD HM(N-C) Lid: Cornea: no cilia touch to cornea Cornea: decreased epidefect mod. edematous c 360 NV multiple punctate epitheliopathy Px.) Keratolimbal allograft with temporary AMT (OD) prednisolone 60 mg #1 cyclosporine 200mg #2 mycophenolic acid 500mg O-GAF x4, O-FM x4, O-TMO xhs O-3HUM xprn (OD) 7 days after the entropion repair of the right eye, upper cilia no longer touch to the cornea. The size of the epidefect of the right cornea has been decreased. For the treatment of the inflammation, we added prednisolone and cyclosporin. After 1 week, keratolimbal allograft and amniotic membrane transplatation of the right eye was done.

Keratolimbal allograft (OD) Recipient eye 360 degree conjuctivoperiotomy was done Conjunctivalization was removed Donor limbus corneoscleral rim was prepared, leaving approximately 1 mm of sclera peripheral to the limbus lamellar dissection was done by sharp blade The donor limbus was approximated to recipient eye by 10-0 nylon During the keratolimbal allograft operation. for recipient eye, 360 degree conjuctivoperiotomy was done. And the Conunctivalization was removed For Donor limbus, the corneoscleral rim was prepared leaving approximately 1 mm of sclera peripheral to the limbus lamellar dissection was done by sharp blade Then, The donor limbus was approximated to recipient eye by 10-0 nylon

POD # 7 after Keratolimbal allograft + AMT (OD) VA: OD HM(N-C) Cornea: Well attached AM (OD) No corneal neovascualrization (OD) Px.) prednisolone 30mg #1 cyclosporine 200mg #2 mycophenolic acid 500mg O-CS x2 ,O-GAF x4 ,O-FM x4 O-3HUM POD # 14 Removal of AM (OD) (OD) 7 days after the keratolimbal allograft and amniotic membrane transplantation of the right eye, the hazziness and nvs of the right cornea has been decreased. The repopulation of the cornea epithelium was noticed. We decided to continue immune suppressive therapy.

POD # 5 months after Keratolimbal allograft + AMT (OD) VA OD HM(N-C) Cornea: graft rejection sign : ingrowing NV at limbal arcade epithelial defects Px.) subconjunctival dexa injection cyclosporine 200mg #2 mycophenolic acid 500mg #2 Pred-f x4, O-GAF x4 O-3HUM autologous serum x8 blephasol POD # 5 months after Keratolimbal allograft + AMT (OD) (OD) 5 months after the surgery, Increased multiple epidefect of the cornea and nvs of the limbal arcade was noticed. We prescribed mycophenolic acid instead of prednisolone. (OD)

POD # 7 months after Keratolimbal allograft + AMT (OD) VA OD HM(N-C) Cornea: persistent epithelial defects and calcification stop progressive vascularization Px.) cyclosporine 200mg #2 O-FM x4, O-GAF x4 O-3HUM autologous serum x8 blephasol Considering PPKP and cataract OP (OD) 7 months after the surgery, Generalized increasement of the corneal opacity and epidefect associated with calcification was observed. We continued mycophenolic acid and cyclosporin. We are considering cataract operation and PPKP for the improvement of the visual acuity (OD)

69/M 유 O 욱 C/C Visual disturbance (OD) for 20 years DM/HBP(-/-) Ocular op./trauma (-/+) Splashed vinegar(식초) to both eyes 20yrs ago Glasses(-) Let me introduce the second case. A 69-yr old man visited our clinic. His chief complaint was visual disturbance for 20 yrs. She had no underlying disease. On the reviewd his past history, she splashed vinegar on both eyes 20 yrs ago.

Ocular Examination VA OD 0.4(N-C) EOM straight by ACT OS LP(+) EOM straight by ACT Lid no swelling(OU) Conj. mod. Injected (OU) Cornea partial conjunctivalization c NV at upper portion c multiple punctate epitheliopathy (OD) diffuse & thick conjunctivalization (OS) His visual acuity of the right eye was 0.4 with or without correction & that of the left eye was light perception positive. Conjunctiva of the both eye was injected. Right cornea shows partial conjuctivalization and nVS from superior to inferior. Multiple punctate epitheliopathy was noticed on entire cornea. (OD) (OD)

Ocular Examination AC deep & cell(-) (OD) invisible (OS) Pupil round & nl. sized (OD) Lens mild cortical opacity (OD) IOP 14 mm Hg (OD) not checked (OS) Otherwise unremarkable.

Impression cytology of cornea (OD) This is the impression cyctology of the right cornea. It is stage 4, moderate to severe keratinization, greater NC ratio more than 1 over 6. (OD) Stage 4. mod to severe keratinization, greater NC ratio >1:6

Imp.) Px.) Epithelial keratopathy (OD) d/t partial limbal cell deficiency after acid burn Px.) Conservative care O-3HUM (OD) On the review of his ocular examination, it is considered that the epithelial keratopathy was developed due to the partial limbal cell deficiency after the acid burn. As a conservative treatment, we started preservative free artificail eyerops.

F/U 9 months later VA OD 0.25(N-C) Cornea: Px.) advanced conjunctivalization above cornea center increased epithelial irregularity Px.) O-GAF x4, O-FM x4 Autologous serum x8 Temporary AMT (OD) (OD) 9 months later, Advanced conjuntivalization above cornea center and increased epithelial defect was observed. We added O-GAF x3, O-FM x4 , autologous serum x8 and decided to do amniotic memebrane transplantation on the right eye. (OD)

POD #7 days after AMT(OD) VA OD 0.32(N-C) Cornea: Well attached AMT (OD) Px.) O-GAF x3, O-FM x4 autologous serum x8 POD # 14 Removal of AMT (OD) 7 days after the amniotic transplantation of the right eye, Corneal erosion was improved. We decided to keep using the eyedrops. (OD)

POD #6 months after AMT(OD) VA OD 0.5(N-C) Cornea: no SPE(OD) Ingrowing NV- stationary Px.) O-3HUM O-GAF x2 ,O-FM x2 (OD) 6 months after the surgery, The corneal ingrowing neovascularization is stationary. The right cornea is clear without SPE. (OD)

27/M 곽 O 현 C/C Decreased visual acuity (OD) for 7 months DM/HBP(-/-) Stevens-Johnson syndrome dx: at 7 yrs old Ocular op./trauma (+/-) corneal nodule removal (Salzmann)(OD): 10 yrs ago Hotz op(OU): 8 yrs ago Living related keratolimbal allograft c AMT(OD) :6 yrs ago Glasses(-) Let me introduce the third case. A 27-yr old man visited our clinic. His chief complaint was decreased visual acuity of right eye for 7months. He was diagnosed as Steven Johnson syndrome when he was 7 yrs old, and after that the visual acuity has been decreased. On the reviewed his past history, he received removal of the corneal nodule which was identified as Salzmann by pathologist. He had Hotz operation on both eyes 8 yrs ago. Living related keratolimbal allograft c AMT was done on his right eye 6 yrs ago.

Ocular Examination VA OD 0.02 (N-C) EOM straight by ACT OS F/C 20cm (N-C) EOM straight by ACT Lid no swelling(OU) Conj. symblepharon(OD>OS) Cornea diffuse stormal opacity c NV(OS>OD) His visual acuity of the right eye was 0.02 with or without correction & that of the left eye was finger count 20cm. Symblepharon is much severe on his right eye than left eye. Both of the cornea showed moderately edematous with diffuse stromal opacity with NVS (OD) (OS)

Ocular Examination AC blurry (OU) Pupil blurry (OU) Lens blurry (OU) IOP 8 mmHg (OD) 7 mmHg (OS) Ant. chamber and all other findings were unremarkable

Imp.) Px.) Known Stevens-Johnson syndrome Hx Epitheliopathy d/t total limbal cell deficiency (OD) Symblepharon d/t chronic inflammation Px.) doxycycline 100mg #1 O-GAF x4, O-FM x4, O-CET xhs O-3HUM (OD) blephasol for lid hygiene Removal of conjunctivalization tissue (OD) Keratolimbal allograft +Temporary AMT+ Symblepharolysis (OD) The impression was epitheliopathy and symblepharon due to chronic inflammation and known stevens-johnson syndrome. As a treatment, we prescribed doxycycline, eyedrops, including antibiotics, steroid, artificial tears and blepharasoll We decided to do keratolimbal allograft , AMT, symblepharolysis of the right eye.

POD # 14 days after remval of AMT VA OD 0.2 (N-C) [REF] error [KER] D AX K1 39.75 35 k2 43.50 125 Conj. resolved symblepharone (OD) Cornea: improved corneal opacity (OD) no corneal NVs decreased SPEs (OD) Px.) prednisolone 60mg #1 cycposporine 200mg #2 mycophenolic acid 500mg #2 O-GAF x4, O-CS x4 1% methylprednisolone qid O-CET xhs autologous serum x8 (OD) 7 days after the surgery, The right eye showed improvement of the visual acuity. Symblepharon was resolved, corneal opacity has been improved and SPE’s were decreased. As a treatment, prednisolone, cyclosporine, mycophenolic acid was prescribed. (OD)

POD # 3 months VA OD 0.02 (N-C) Cornea: Px.) (OD) (OD) [REF] error [KER] D AX K1 39.50 14 k2 46.25 104 Cornea: increased corneal haziness ingrowing NV to cornea Px.) cycposporine 100mg #2 mycophenolic acid 500mg #2 O-GAFx4, O-FMx4, O-CET qhs autologous serum x8 RGP fitting for improving VA VA OD (0.16) over R sph +0.75 ~ +0.125 cyl -3 ~ -5 POD # 3 months (OD) 3 months after the surgery, The visual acuity was decreased. The hazziness of the cornea was increased. Ingrowing neovascularization of the cornea was noticed. We continued to use the per oral medication and eyedrops, and prescribed RGP fitting for better visual acuity. (OD)

POD # 5 months VA OD 0.125 (N-C) Cornea Px.) corneal haziness- sl. increased NV-stationary Px.) cycposporine 100mg #2 mycophenolic acid 500mg #2 O-GAF x4, O-FM x4, O-CET xhs Autologous serum x8 (OD) 5 months after the surgery, The visual acuity was improved to 0.125, the corneal hazziness was slightly increased. The neovascularization remains the same. We continued to use immunosupressive agents and eye drops. (OD)