Ultrasound biomicroscopy study of the Verisyse aphakic intraocular lens combined with penetrating keratoplasty in pseudophakic bullous keratopathy.

PURPOSE: To evaluate anterior segment modifications after penetrating keratoplasty (PKP), previous anterior chamber intraocular lens (IOL) removal, and Verisyse IOL (AMO) implantation over the iris or under the iris for the treatment of pseudophakic bullous keratopathy (PBK) using ultrasound biomicroscopy. SETTING: Department of Ophthalmology, Poitiers University Hospital, Poitiers, France. METHODS: A prospective randomized comparative case series included 27 patients (27 eyes) with PBK who had PKP and implantation of a Verisyse VRSA54 aphakic IOL. The IOL was implanted over the iris in 13 patients (Group A) and under the iris in a reversed position in 14 patients (Group B). Ultrasound biomicroscopy scans 6 months after surgery measured central anterior chamber depth (ACD), iris thickness (IT), distance of the haptics from the corneal endothelium (CED), distance of the haptics from the ciliary body (CBD), angle opening distance (AOD) 500 mum from the scleral spur (AOD500) and the iridocorneal angle theta on the 4 o'clock meridian lines (AOD3; AOD9; AOD12; AOD6/theta12, theta6, theta3, theta9). RESULTS: No significant difference was found in IT, CBD, or AOD12 between Group A and Group B (P >.05). In Group B, the mean ACD was deeper by approximately 55% (P = .008); CED3 was larger by 69% (P = .0162), CED9 by 80% (P = .0128), AOD3 by 57% (P = .0309), AOD9 by 140% (P = .0057), and AOD6 by 44% (P = .0399); and theta3 was wider by 52% (P = .046), theta9 by 123% (P = .0068), theta12 by 50% (P = .0492), and theta6 by 81% (P = .0237). CONCLUSION: Ultrasound biomicroscopy showed that in eyes that had PKP with Verisyse IOL enclavation to the posterior plane of the iris, which involved posterior translation of the iridal plane, the ACD was significantly deeper and the CED and AOD were significantly larger than in eyes with anterior enclavation of the IOL.

Amniotic membrane transplantation in severe bacterial keratitis.

PURPOSE: To determine whether a combination of early amniotic membrane transplantation (AMT) and early topical corticosteroid treatment could be a safe adjuvant therapy during antibacterial treatment in severe bacterial keratitis (BK) for relieving pain, avoiding iatrogenic epithelial toxicity, and allowing earlier use of topical steroids. METHODS: In a prospective noncomparative case series, 12 patients with severe microscopically-proven BK were treated with immediate maximal topical antibiotics followed by AMT at 48 hours (single-layer epithelial side-down or multilayer epithelial side-up), plus topical steroid treatment at 72 hours. Pain relief (NRS-11 numeric rating pain scale) and the corneal epithelium healing were measured. RESULTS: The follow-up rate was 7.5 person-months, with AMT performed once in 2 patients and twice in 10 patients with BK caused by Pseudomonas aeruginosa (5), Klebsiella pneumoniae (1), Moraxella cattharalis (1), Staphylococcus aureus (1), Staphylococcus epidermidis (2), or Streptococcus pneumoniae (1). A significant decrease in the pain score was noted from the admission day (median, 8; range, 7-10) to shortly after AMT (at day 3: median, 2; range, 1-3). Epithelial healing was achieved between 8 and 45 days (mean, 25.5 +/- 9.7 days). Neither perforation nor neovascularization was observed. CONCLUSIONS: Early AMT combined with topical corticosteroid in severe BK provides immediate pain relief and allows epithelial healing.

Penetrating keratoplasty combined with posterior Artisan iris-fixated intraocular lens implantation.

PURPOSE: To present a new surgical technique combining penetrating keratoplasty and open-sky posterior iris fixation of the Artisan iris-claw intraocular lens (IOL) for treatment of pseudophakic bullous keratopathy in a case series of five patients. METHODS: A graft diameter of 8.25 mm was chosen. The formerly implanted angle-supported IOL was removed. The IOL was enclosed, entrapping a fraction of the mid-peripheral iris within the haptics whilst being held firmly with the implantation forceps. The corneal button was sutured to the recipient bed with 10-0 nylon sutures. A specular microscope was used for making an endothelial cell count. Patients underwent an ultrasound biomicroscope (UBM) scan before and 6 months after surgery and postoperative macular oedema was assessed by optical coherence tomography (OCT). The minimum follow-up was 12 months. RESULTS: Visual acuity (VA) improved in all five cases (mean best corrected VA was 0.4 postoperatively versus 1.28 preoperatively). No complications were noted. The mean endothelial cell density obtained after 1 year was 1508 cells/mm(2). The UBM study showed a deep anterior chamber and an open iridocorneal angle of 360 degrees in all cases. CONCLUSION: The implantation of the Artisan device behind the iris better preserves the anatomy of the anterior segment with respect to the iridocorneal angle.

Traumatic globe rupture following penetrating keratoplasty.

PURPOSE: To evaluate the mechanism, clinical characteristics, management and visual outcome of ocular trauma following penetrating keratoplasty (PK). METHODS: Twenty-six patients (13 men, 13 women) who suffered surgical wound dehiscence after PK because of ocular blunt trauma between 1994 and 2001 were included in this retrospective study. Graft dehiscence was managed with primary wound closure in all patients. Visual acuity, intraocular pressure, and funduscopy were evaluated in the follow-up. RESULTS: The mean age at trauma was 50+/-24 years (range 9-88 years). Mean interval between transplantation and trauma was 45.5+/-64 months (range 1 month to 20 years). Globe rupture occurred at the graft-host junction in all patients. Nine of 13 phakic eyes (69.2%) presented lens expulsion. Eight of nine pseudophakic eyes (88.9%) had lost their implants. Retinal detachment occurred in seven eyes (27%) within 3 months following the trauma. Five patients (19.2%) underwent vitreous surgery for posterior segment damage. Two eyes (7.7%) were regrafted. At the last examination, only seven eyes (27%) had visual acuity of 20/200 or better. CONCLUSIONS: Traumatic wound dehiscence may occur, and the prognosis is poor after the injury. Globe rupture at the graft-host junction showed persistence of wound weakness even a long time after PK. Prevention of ocular trauma should be performed following PK.

Intrastromal corneal ring segments and corneal anterior stromal necrosis.

Poly(methyl methacrylate) refractive intracorneal ring segments (ICRS) can be removed for a refractive miscorrection or for early complications after implantation. We report the first case of a woman who experienced anterior stromal necrosis 5 years after an ICRS surgical procedure.

Truncating mutations in the carbohydrate sulfotransferase 6 gene (CHST6) result in macular corneal dystrophy.

PURPOSE: Identification of mutations in the CHST6 gene in 15 patients from 11 unrelated families affected with recessive macular corneal dystrophy (MCD). METHODS: Genomic DNA was extracted from peripheral blood leukocytes of the affected patients and their healthy family members, and the mutational status of the CHST6 gene was determined for each patient by a PCR-sequencing approach. Serum concentrations of antigenic keratan sulfate for each proband were determined by ELISA. RESULTS: ELISA indicated that all affected patients, except one, were of MCD type I or IA. Fourteen distinct mutations were identified within the CHST6 coding region: 2 nonsense, 2 frameshift, and 10 missense. Of these, 12 were novel, and a nonsense mutation in the homozygous state is reported for the first time. CONCLUSIONS: These molecular results in French patients with MCD combined with those reported in previous studies indicated CHST6 mutational heterogeneity. The characterization herein of nonsense mutations is in keeping with the fact that MCD results from loss of function of the CHST6 protein product.

Phototherapeutic keratectomy for BIGH3-linked corneal dystrophy recurring after penetrating keratoplasty.

PURPOSE: To determine visual results and report side effects and complications after phototherapeutic keratectomy (PTK) for BIGH3-linked corneal dystrophy recurring after penetrating keratoplasty. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Forty-two excimer laser PTK procedures were performed in 42 eyes of 29 patients with BIGH3-linked corneal dystrophies. Genetic status of all patients was determined and allowed us to assess an unambiguous diagnosis. Preoperative diagnoses included LCDIIIA/A546T (1 eye), R124 l+DT125-DE126 (4 eyes), GICD/R555W (14 eyes), LCDI/R124C (6 eyes), SGD/R124 l (16 eyes), and CDBII/R555Q (1 eye). INTERVENTION: Two excimer lasers (Summit Excimed UV 200, Summit Technology, Waltham, MA and Nidek EC 5000, Nidek, Inc., Gamagori, Japan) were used to perform all PTKs. Indications for performing PTK after a graft were severe decrease of the best-corrected visual acuity (BCVA) related to recurrent corneal deposits and/or painful recurrent epithelial erosions. MAIN OUTCOME MEASURES: Preoperative and postoperative BCVA were analyzed, significant recurrences after treatment were noted, and postoperative complications were recorded. RESULTS: Mean preoperative BCVA was 0.2 +/- 0.12 in the decimal chart, mean postoperative BCVA was 0.52 +/- 0.16 with a mean follow-up of 3.13 +/- 1.77 years (range, 0.3-6.65 years). Visual acuity was significantly improved after surgery (P < 0.05). The magnitude of the change in visual acuity was dependent on the mutation (P < 0.001). Seven symptomatic recurrences were observed. One regressive graft rejection and 4 cases of severe postoperative haze were observed. No other complications were noted. CONCLUSIONS: PTK is a simple, safe, and efficient technique for the treatment of recurrent corneal dystrophies; in many cases it prevents or delays the major incumbent problems of repeated grafting.

Long-term outcome of keratolimbal allograft with or without penetrating keratoplasty for total limbal stem cell deficiency.

PURPOSE: To evaluate the long-term outcome of ocular surface reconstruction, including keratolimbal allograft (KLAL) and amniotic membrane transplantation (AMT) with or without penetrating keratoplasty (PKP), in patients with nonambulatory vision secondary to total limbal stem cell deficiency (LSCD). DESIGN: Retrospective, non-comparative interventional case series. PARTICIPANTS: Thirty-nine eyes in 31 consecutive patients with total LSCD, as defined by impression cytology, who had a preoperative best-corrected visual acuity of less than 20/200 and a minimum follow-up of 12 months. Patients were divided into three groups: group 1 (16 eyes) with chemical burns, group 2 (9 eyes) with Stevens-Johnson syndrome (SJS), and group 3 (14 eyes) with other causes of LSCD, including ocular cicatricial pemphigoid, atopic keratoconjunctivitis, and aniridia. INTERVENTION: All patients underwent KLAL and AMT by one surgeon (SCGT). If needed, PKP was performed at the same surgical setting using tissue from the same donor. MAIN OUTCOME MEASURES: Cumulative rates of survival of ambulatory vision (> or = 20/200), survival of KLAL, survival of PKP, and incidence of complications. RESULTS: Fifty-three KLAL with AMT procedures were performed in 39 eyes, of which 23 eyes received simultaneous PKP at the time of the first KLAL. The mean follow-up was 34.0 +/- 21.5 months (range, 12-117.6). The mean period of ambulatory vision was 23.9 +/- 20.9 months (range, 0-104). The overall survival of ambulatory vision was 53.6% at 3 years and 44.6% at 5 years. The survival of ambulatory vision was significantly worse in SJS compared with other causes (67%, 81%, and 92% for groups 1, 2, and 3, respectively; P = 0.06 for group 1 versus 2, P = 0.0008 for group 1 versus 3). KLAL performed alone resulted in higher survival of ambulatory vision at 2 years (86.1% +/- 9.1%) compared with KLAL with PKP (46.9% +/- 10.6%, P = 0.100). The survival of PKP was significantly worse in SJS compared with the other causes (20.0% +/- 17.9% compared with 55.6% +/- 11.7%, respectively, P = 0.028). After 2 years, the survival of the second KLAL was better than that of the first: 68.2% +/- 15.4% compared with 27.3% +/- 13.4%, respectively (P = 0.041). CONCLUSIONS: Ambulatory vision for a period of more than 2 years can be achieved by KLAL with or without PKP in eyes with severe ocular surface disorders caused by total LSCD. However, a progressive decline of the visual outcome and graft survival is evident with time. Performing PKP simultaneously with KLAL may be associated with a less favorable outcome. The failure of KLAL is associated with the loss of donor cells in the recipient. Augmentation of ocular surface defense is essential in securing the success of KLAL and PKP. Future modifications of the surgical procedure and of the immune suppressive protocols may improve survival of the allogeneic grafts and the final visual outcome.

Clinical outcome of eight BIGH3-linked corneal dystrophies.

OBJECTIVE: To determine whether the mutational pattern of BIGH3-linked corneal dystrophies (CDs) can accurately predict the clinical course of the disease and be helpful in planning adequate surgical treatment. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Chart review of 73 patients (110 eyes) with recently confirmed BIGH3 mutations who underwent a penetrating keratoplasty (PK) from 1978 through 1999. Diagnoses included Thiel-Benhke CD (TBCD/R555Q) (13 eyes), classic granular CD (CGCD/R555W) (28 eyes), superficial variant of granular dystrophy (SVGD/R124 l) (27 eyes), lattice CD type I (LCDI/R124C) (20 eyes), Avellino CD (ACD/R124H) (2 eyes), H626R-lattice dystrophy (LCD/H626R) (6 eyes), and two novel dystrophies: a French variant of granular dystrophy (FVGD/R124 l+DT125-DE126) (9 eyes) and a French lattice CD type IIIA (LCDIIIA/A546T) (5 eyes). METHODS: The mutation of the BIGH3 gene was characterized for all patients. Clinical data were reviewed for each patient, and included age at first PK and elapsed time before significant recurrence (as defined by a severe decrease in best-corrected visual acuity related to recurrent deposits in the graft). MAIN OUTCOME MEASURES: Mean age at first PK and delay before a significant recurrence. RESULTS: Mutational pattern was highly correlated with the clinical course of each dystrophy. According to the genetic mutation, two groups with different prognosis were identified. Group 1 was defined by the presence of the FVGD/R124 l+DT125-DE126 and SVGD/R124 l mutations and was characterized by the early need for treatment and early recurrence of deposits. Group 2 was molecularly defined by the presence of any of the following mutations: LCDI/R124C, CGCD/R555W, LCDIIIA/A546T, TBCD/R555Q, and LCD/H626R. In group 2, mean age at first treatment was older, and delay before a significant recurrence was longer as compared with group 1 (P = 0.0001). CONCLUSIONS: These results demonstrate that there is a direct correlation between the molecular defect and the clinical course of BIGH3-linked CDs. They also indicate that molecular characterization of the genetic defect will help predict and design adequate surgical treatment for patients with ambiguous clinical diagnosis.