Long-term results of corneal wedge excision for pellucid marginal degeneration.

PURPOSE: A retrospective study reporting long-term visual and astigmatic results of patients with pellucid marginal degeneration (PMD) treated by corneal wedge excision. METHODS: The notes of 9 patients (10 eyes) treated by corneal wedge excision were reviewed. All patients had typical PMD and were treated by excision of an inferior crescent of diseased corneal tissue. The excised area measured approximately 2 mm in width and extended from the 4 o'clock to the 8 o'clock meridian. All thinned corneal tissue was removed. Normal-thickness corneal stroma was then reapposed with 10/0 nylon or 10/0 polypropylene sutures. Post-operative selective suture removal was performed until a satisfactory visual and astigmatic result was achieved. This was guided by refraction, keratometry and photo-keratoscopy results. Pre-operative best corrected visual acuity ranged from 6/12 to 6/60 with an associated mean keratometric astigmatism of +13.8 dioptres (range 8-25 dioptres, axis range 30 degrees-175 degrees). Mean follow-up was 59 months (range 14-138 months). RESULTS: Post-operatively a stable corrected visual acuity of 6/9 or better was achieved in all cases in a mean time of 5.4 months (range 3-12 months). Mean post-operative keratometric astigmatism was 1.4 dioptres (range 0.5-4 dioptres). Over the course of follow-up long-term astigmatic drift (LTAD) was noted, mean 2.1 dioptres (range 0.5-5.5 dioptres). Three patients developed mild inferior pannus related to peripherally sited sutures. One case developed apparent corneal hydrops within the corneal wound after 9 years of follow-up. CONCLUSIONS: We believe that corneal wedge excision offers an excellent surgical result for patients with PMD, although modification of the technique may be required to improve long-term astigmatic drift.

Excimer phototherapeutic keratectomy for recurrent granular dystrophy.

PURPOSE: Corneal grafting is a standard treatment for visually disabling granular dystrophy. The visual results of this procedure are generally good, but can be marred by recurrences of granular deposits some years later. We report the results of phototherapeutic keratectomy (PTK) for recurrent granular dystrophy in three eyes from two patients and discuss the possibilities of treating de novo granular dystrophy with excimer laser. METHODS: The records of two patients (three eyes) treated with excimer PTK for recurrent granular deposits on the donor cornea were reviewed. RESULTS: In all cases visual acuity was improved and repeat corneal grafting avoided. No significant complications occurred although one eye had further recurrence of granular deposits which was also successfully retreated with excimer PTK. Follow-up on these cases varies between five months and three years. CONCLUSIONS: We believe that excimer PTK offers a simple, safe and repeatable way of restoring visual acuity to most cases of recurrent granular dystrophy. Visual recovery is fast and all the incumbent problems of repeat grafting are avoided.

Familial Pallister-Hall syndrome: case report and hormonal evaluation.

Pallister-Hall syndrome is a usually lethal dysplasia/malformation syndrome characterized by hypothalamic hamartoblastoma, hypopituitarism, postaxial polydactyly, craniofacial malformations, imperforate anus, and other malformations. We report a familial case in a male infant and his female sib fetus, suggesting autosomal recessive inheritance, or germinal mosaicism for an autosomal dominant mutation, or a segregating submicroscopic chromosome abnormality. Detailed endocrine evaluation on the surviving infant revealed documented pituitary function, pituitary deficit, and hypothalamic deficiency. We suggest that hypothalamic dysfunction contributes to the hypopituitarism seen in Pallister-Hall syndrome.

Long-term intraocular pressure control by trabeculectomy: a ten-year life table.

A retrospective study of 179 eyes in 127 patients who underwent trabeculectomy at Sydney Eye Hospital under the supervision of two surgeons between 1977 and 1982 was carried out. Survival analysis by life table method shows cumulative two, five, and 10 year success rates to be 78%, 70%, and 67% respectively, with mean duration of intraocular pressure control (IOP < 21 mmHg) being 88 months. Antiglaucoma medication improved the long-term survival significantly (Hazard Ratio of 0.49 and P = 0.01) so that when the definition for failure is taken as IOP > 20 mmHg while using medication, the two, five and 10 year success rates were 89%, 87% and 86% respectively. A rise in average intraocular pressure is seen between two weeks and three months after trabeculectomy. The improvement in long-term success rate with use of topical steroids was suggestive (Hazard Ratio of 0.69) but not conclusive (P = 0.21). No difference was found in survival comparing fornix versus limbal based flap technique.

Infectious crystalline keratopathy.

A 76-year-old patient was found to have a crystalline keratopathy with no evidence of acute inflammation in her right corneal graft. The patient had been on long-term topical steroids. Subsequent microscopy of the graft demonstrated pockets of Gram-positive cocci with a paucity of inflammatory cells. This is the tenth reported case of infectious crystalline keratopathy, first reported by Gorovoy in 1983. It does not always occur in corneal grafts, but the use of long-term topical steroids, together with an epithelial defect, allows the microorganism, usually Streptococcus viridans, to invade the corneal stroma and replicate along the lamellar planes, unhindered by the usual inflammatory response. Retrospective analysis of this patient's history suggests that she has suffered the same keratopathy in previous corneal grafts. Treatment involves cessation of steroids, antibiotic cover and often a repeat penetrating keratoplasty. If this is required, the lowest possible dose of steroid cover should be used with prolonged use of topical antibiotics. To the best of our knowledge this is the first reported case of infectious crystalline keratopathy in Australia.

Atypical mycobacterial keratitis.

A case of marginal corneal abscess and hypopyon due to Mycobacterium chelonae is presented. Its therapy is described, followed by a brief discussion of atypical mycobacterial keratitis.

Cyclosporin therapy in Mooren's ulcer.

Mooren's ulcer is a rare disease of presumed autoimmune aetiology. Some cases run a chronic severe course and fail to respond to local and systemic therapy. We report here such a case with bilateral Mooren's ulcer that failed to respond to local therapy with topical corticosteroids, silver nitrate, and conjunctival resection, as well as systemic immunosuppression with corticosteroids, cyclophosphamide, and azathioprine. Systemic cyclosporin (10 mg/kg/day) resulted in resolution of the corneal ulceration within two weeks of beginning treatment, and the patient has remained in remission after 15 months of therapy. Cyclosporin side effects included hirsutism, hypertension, increased blood levels of urea and creatinine, and abnormalities in liver function tests. All these resolved on reducing the dosage of cyclosporin. The results in this case suggest that cyclosporin is an effective agent in patients with severe sight threatening Mooren's ulcer.

Prenatal diagnosis of osteogenesis imperfecta type III.

Ultrasonographic and radiographic evaluation of a fetus at risk for osteogenesis imperfecta (O.I) type III was performed. Real-time ultrasound measurements at 15 weeks gestation were interpreted as normal, but at 20 and 22 weeks of gestation revealed marked shortening of the long bones and deformity of the femurs. The findings were confirmed by fetal radiography at 22 weeks gestation. Radiographic and histologic changes characteristic of O.I. were observed in the aborted fetus. Thus the antenatal manifestations of O.I. type III maybe severe enough to make prenatal diagnosis possible in the second trimester for families at risk for recurrence of this disorder.

Corneal graft survival: a retrospective Australian study.

Four hundred and forty-eight consecutive corneal grafts are analysed and their survival calculated using the actuarial life-table method. Overall survival at two years is 81 +/- 4% and at five years is 65 +/- 5%. Within diagnostic subgroups keratoconus has the best prognosis. Previous graft failure and recipient corneal vascularization are shown to have a negative effect on graft survival. Sex of patient, urgency of operation and use of combined procedures do not affect survival and second regrafts fare no worse than first regrafts. The use of the actuarial life-table method of analysing graft survival is discussed and its importance emphasized.

Evaluation of nine hundred and sixty-seven consecutive intraocular implants.

We undertook a survey of all intraocular lenses (IOLs) implanted by two surgeons from 1976 up to the end of 1983. Of the 967 implants, 164 were excluded because of lack of adequate follow-up. Of the remaining 803 cases there were 104 anterior chamber lenses, 185 iris-supported lenses, and 514 posterior chamber lenses. The overall final visual acuity was 6/12 or better in 740 cases (92.2%). Of the remaining 63 cases, 32 had pre-existing disease or associated conditions not directly related to the operation. By analysing the results within each major group of IOL we found that the posterior chamber IOLs had the best final visual acuity (94.4% 6/12 or better) and fewer postoperative complications. The major problems with iris-supported IOLs were corneal decompensation and cystoid macular oedema.

Keratoplasty following anterior segment trauma.

This paper reports and analyses 20 keratoplasties with or without anterior segment reconstruction carried out for penetrating injuries of the anterior segment. The results show that 80% clear grafts were achieved and 65% of eyes had vision restored to 6/18 or better. No eyes were lost. The complications were retinal detachments 2 cases, corneal graft rejection 2 cases, glaucoma 4 cases (2 mild and easily controlled) and one each of amblyopia and retinal folds through macular area. Eyes that have "quietened" following severe penetrating injuries of the anterior segment should be considered for penetrating keratoplasty and anterior segment reconstruction if they retain normal intraocular pressures and have vision of at least accurate projection of light in all quadrants. As well as achieving clear grafts and improvement of vision as above, all eyes had better cosmetic appearance. Two eyes had an ipsilateral rotational autokeratoplasty. This technique has a role to play when central scarring can be rotated to the periphery if sufficient undamaged cornea remains and interference with angle structures can be minimised.

Simultaneous surgical management of cataract and glaucoma.

This paper reports and analyses 70 combined cataract and trabeculectomy operations carried out on 50 patients by the two authors from 1973 to mid 1979. Control of glaucoma was achieved by surgery alone in 86%, the remaining 14% required additional medical therapy to achieve control. This therapy was less than that used preoperatively. Where the preoperative control was good 96% achieved post operative control without therapy. In eyes with poor preoperative control, 80% achieved post operative control without therapy. The complication rate was low and no eyes were lost in the series. The literature for alternatives for managing cataracts and glaucoma has been reviewed and analysis of this shows the following percentage control by surgery without additional medical therapy. (a) Cataract extraction alone 40.5%. (b) Drainage procedure followed by lens extraction 65.5%. (c) Combined cataract and glaucoma surgery other than trabeculectomy 79%. (d) Combined cataract and trabeculectomy 91%. The latter combined procedure is recommended for all eyes with coexistent cataract and glaucoma.