Iridocorneal endothelial syndrome in Thai patients: clinical variations.

OBJECTIVE: To evaluate the spectrum of iridocorneal endothelial syndrome, to our knowledge, never studied previously in Orientals. METHODS: From 1986 to 1998, we examined 60 consecutive patients (20 men, 40 women) with characteristic signs of iridocorneal endothelial syndrome and compared the clinical manifestations to those reported in white patients. RESULTS: Cogan-Reese syndrome (CRS) was most common (38 patients), while 14 patients had Chandler syndrome (CS), and 8 had progressive iris atrophy. Three patients initially classified as having CS and 1 as having progressive iris atrophy progressed to CRS. Glaucoma occurred in 46 patients (76.7%), most commonly in patients with progressive iris atrophy or CRS. Ten patients had slow progression of disease during the follow-up period of up to 12 years. Three patients (2 with CRS, 1 with CS) had asymptomatic localized islands of "hammered-silver" appearance and 11 (8 with CRS, 2 with CS, and 1 with progressive iris atrophy) had subclinical abnormal endothelium in the contralateral eyes. A translucent membrane was commonly seen on the brown iris surface. Total endothelial involvement was present in 49 patients, while 6 (4 with CRS, 2 with CS) had focal endothelial abnormalities with sharp demarcation from adjacent normal endothelium. CONCLUSIONS: Iridocorneal endothelial syndrome occurs in Orientals. Cogan-Reese syndrome is the most common form and is strongly associated with glaucoma. Although several clinical manifestations were similar between whites and Orientals (mean age of onset, sex predilection, iris changes, peripheral anterior synechiae formation, or corneal edema), CRS was most prevalent; a translucent membrane were more noticeable in Orientals.

Combined phacoemulsification and goniosynechialysis for uncontrolled chronic angle-closure glaucoma after acute angle-closure glaucoma.

OBJECTIVE: To evaluate combined phacoemulsification, posterior chamber intraocular lens (PCIOL) implantation, and goniosynechialysis (phaco-GSL) prospectively in eyes with more than 180 degrees of peripheral anterior synechiae (PAS) and uncontrolled intraocular pressure (IOP) when performed within 6 months of an attack of acute angle-closure glaucoma (ACG). DESIGN: Prospective, noncontrolled clinical trial. PARTICIPANTS: Patients who had presented with acute ACG and had persistently uncontrolled IOP despite successful laser iridotomy for pupillary block and argon laser peripheral iridoplasty for continued appositional closure after iridotomy. INTERVENTION: After the completion of phacoemulsification and posterior chamber lens implantation, goniosynechialysis was performed in 52 eyes of 48 patients. MAIN OUTCOME MEASURES: Postoperative visual acuity, IOP, extent of PAS, and number of medications, if any, required for IOP control. RESULTS: Intraocular pressure was less than 20 mmHg in 47 eyes (90.4%) without medications; 4 were controlled with medications and 1 required filtration. Mean extent of PAS was reduced from 310 degrees to 60 degrees. Peripheral anterior synechiae formation or IOP elevation did not recur after 3 months after surgery up to 6 years. Eight patients achieved 20/20 visual acuity, while 44 patients had less than 20/20 visual acuity. No patient had worse visual acuity after surgery compared to before surgery. CONCLUSION: Phaco-GSL and PCIOL implantation is effective in reducing PAS and IOP and improving visual acuity in eyes with persistent chronic ACG when performed within 6 months after treatment for acute ACG.

Glaucoma in phakomatosis pigmentovascularis.

BACKGROUND: Sturge-Weber syndrome, Klippel-Trenaunay-Weber syndrome, and oculodermal melanocytosis are neural crest disorders in which glaucoma is known to occur. Phakomatosis pigmentovascularis is a neural crest disorder that is found almost exclusively in Asians and has not been described previously in the ophthalmic literature. METHODS: The authors describe nine patients with combined oculodermal vascular malformations (five pigmentovascularis, two Klippel-Trenaunay-Weber, two Sturge-Weber) and oculodermal melanocytosis. RESULTS: Ocular melanocytosis was present bilaterally in seven patients and unilaterally in two. Of the 16 hyperpigmented eyes, 13 also had episcleral vascular malformations (EVM). Congenital glaucoma developed in all 10 eyes that had total melanocytosis and EVM. Ocular hypertension developed in one eye with diffuse melanocytosis but partial EVM in childhood. Glaucoma did not develop in one eye with extensive EVM but partial melanocytosis or in the four eyes with ocular melanocytosis but not EVM. CONCLUSION: When oculodermal melanocytosis and nevus flammeus (phakomatosis pigmentovascularis) occur together, with each extensively involving the globe, there is a strong predisposition for congenital glaucoma. When one or both are present with only partial involvement, elevated intraocular pressure may develop later in life, and patients should be followed-up at regular intervals for the development of glaucoma. The vascular malformations appear to play a more important role in the predisposition to glaucoma than does the oculodermal melanocytosis.

Posterior polymorphous dystrophy and Alport syndrome.

Seventeen Thai patients from nine families with Alport syndrome underwent complete ocular examination and specular microscopy. Fourteen (82.3%) patients had ocular changes. Eleven (64.7%) had endothelial vesicles compatible with posterior polymorphous dystrophy. Four of these also had subepithelial opacities, a previously undescribed phenomenon. Other ocular changes included lenticonus and macular and midperipheral retinal flecks. A second group of 18 consecutive patients from 14 families with posterior polymorphous dystrophy detected during routine ocular examination underwent renal evaluation. Five had hematuria, four of whom had sensorineural hearing loss. Two of the four patients also had characteristic renal biopsy findings. Another had sensorineural hearing loss without hematuria, and renal biopsy showed a thin glomerular basement membrane. Posterior polymorphous dystrophy is a common but frequently overlooked finding in Alport syndrome. The frequent association of these two hereditary conditions suggests a common defect in basement membrane formation. Patients with posterior polymorphous dystrophy should be examined for renal abnormalities and hearing loss.

Ocular findings in oculodermal melanocytosis.

We examined 194 patients with oculodermal melanocytosis. Dermal involvement alone was present in 67 (34.5%) patients, while 12 (6.2%) had only ocular involvement. The remaining 115 (59.3%) patients had both ocular and dermal pigmentation. Dermal hyperpigmentation in the combined distribution of the ophthalmic and maxillary divisions of the trigeminal nerve and hyperpigmentation of the nasal or buccal mucosa were closely associated with ocular involvement. Ocular hyperpigmentation most commonly involved the episclera. Associated ocular findings included elevated intraocular pressure with or without glaucoma (10.3%), uveitis (2.6%), cataract (1%), asymmetric cupping of the optic nerve head unassociated with glaucoma (9.8%), and orbital melanoma (0.5%). The most serious complication of oculodermal melanocytosis is malignant transformation, while glaucoma appears to be the more common one. Patients with oculodermal melanocytosis and ocular hyperpigmentation should be followed up at regular intervals for the development of either of these complications.

Combined exfoliation and pigment dispersion syndrome.

Both exfoliation syndrome and pigmentary dispersion syndrome can lead to secondary glaucoma. We treated five patients who had pigmentary dispersion and who subsequently developed exfoliation. In one patient who had bilateral pigment dispersion syndrome, the presence of exfoliation increased the difficulty of controlling intraocular pressure in the affected eye. In a monocular patient, exfoliation and pigmentary dispersion were concurrently present with characteristic defects of both pigmentary dispersion and exfoliation as seen by transillumination defects of the iris. A third patient had bilateral pigmentary dispersion and uncontrollable intraocular pressure in an eye with exfoliation. Despite argon laser trabeculoplasty, the pressure remained uncontrolled. Another patient had a filtering operation in the left eye, with gradually increasing intraocular pressure in the right eye. Exfoliation material complicated the pressure control in the unoperated on eye. Another patient had an eight-year history of glaucoma in the left eye for which filtering surgery was performed. Exfoliation was present in both eyes with controlled pressure in the filtered eye but uncontrolled pressure in the nonfiltered eye.

Glaucoma in oculodermal melanocytosis.

Oculodermal melanocytosis (ODM) is a developmental disorder of the embryonic neural crest involving virtually all of the structures of the eye derived from this tissue. The authors examined 194 previously unreported Thai patients with ODM. Elevated intraocular pressures (IOPs) developed in the ipsilateral eye of 15 patients at initial examination and in 5 others during follow-up, for a total of 10.3% of the patients. Three patients had congenital or late congenital glaucoma, 14 had ocular hypertension or open-angle glaucoma, and 3 had acute angle-closure glaucoma. Five of the patients with open angles had acute pressure rises accompanied by anterior uveitis. Melanocytic hyperpigmentation of the anterior chamber angle was extensive in all patients except those with acute angle-closure glaucoma. There was no consistent correlation between the extent or density of angle pigmentation and the development of elevated IOP. A larger cup-to-disc ratio in the involved eye, presumably congenital, was present in an additional 19 patients. Glaucoma is common in eyes with ODM and may develop at any age. Patients with ODM and initially normal IOP should be examined at regular intervals. Patients with open angles and acutely elevated IOP should be examined carefully for signs of anterior uveitis and treated medically.

Fibronectin and corneal epithelial wound healing in the vitamin A-deficient rat.

The time course of the appearance of fibronectin (Fn) on wounded corneal surfaces was studied in vitamin A-deficient (A-) and pair-fed control rats. At various times following a central epithelial abrasion, the tissue was harvested, and Fn was localized on frozen corneal sections by an indirect immunofluorescence technique. There was no detectable Fn in intact, nonwounded control or intact A-corneas, except for Descemet's membrane. Within a half hour after abrasion, a band of Fn appeared on the denuded corneal surface of pair-fed control rats and became a continuous, prominent layer at four hours. This layer remained until 16 hours but disappeared at 24 hours when the epithelium had resurfaced over the defect. In severely A- rats, reepithelialization following central epithelial abrasion was delayed, and no Fn band was discernible at any time from one to 32 hours after injury. Light microscopy revealed a progressive increase in polymorphonuclear neutrophil infiltration with time in the underlying stroma in severely A- rats. This study indicated that in severe vitamin A deficiency, delayed epithelial migration is associated with an inflammatory cell layer and occurred in the absence of Fn.

Elevated intraocular pressure following penetrating keratoplasty.

IOP was retrospectively studied in 229 consecutive cases of PK, with a mean follow-up period of 84 weeks. Twenty-seven percent of the cases had preoperative glaucoma, three-fourths of which were medically controlled prior to keratoplasty. Following PK, 34% of the total sample developed sustained elevated IOP but only five (2%) patients required surgical treatment for glaucoma. The mean time from PK to first IOP rise was 24 weeks. Variables which were significantly associated with IOP rise included preoperative glaucoma (P less than .001), aphakia (P less than .01), and IOL removal (P less than .01). When eyes with preoperative diagnoses of glaucoma were excluded from the analysis, aphakia was no longer associated with postoperative IOP rise. Keratoconus patients were at significantly less risk than patients with other corneal diagnoses. Factors not associated with IOP rise included previous PKs, pseudophakia, intraoperative vitrectomy, PAS lysis, iridoplasty, secondary IOL placement, and concomitant cataract extraction with IOL (triple procedure).

Glaucoma filtration surgery in rabbits using bioerodible polymers and 5-fluorouracil.

A prospective, randomized, double-masked, and placebo-controlled study was performed to examine the effect of a localized and sustained delivery of 5-fluorouracil (5-FU) on the success of glaucoma filtration surgery in 18 rabbits. A bioerodible polyanhydride composed of bis (p-carboxyphenoxy) hexane (PCPH) and sebacic acid (SA) was used as the drug carrier. The polymer and 5-FU (10% by weight) were molded into 4-mm long cylinders by a 15-gauge needle. These implants, with and without the therapeutic agent, were placed at the site of filtration surgery intraoperatively. The results showed that intraocular pressures (IOPs) were lower in the experimental eyes during the second postoperative week, but eventually both experimental and control eyes returned to preoperative levels. Filtration blebs lasted longer in experimental eyes and bleb failure occurred before IOP failure in both experimental and control eyes. Implant disappearance occurred after IOP and bleb failure. Experimental eyes had more postoperative complications than control eyes. Eventually, the filtration surgery failed in both the experimental and control rabbit eyes.

Aniridia, congenital glaucoma, and hydrocephalus in a male infant with ring chromosome 6.

A premature infant with unilateral aniridia and congenital ectropion uveae, contralateral Rieger anomaly, bilateral congenital glaucoma, and hydrocephalus was found to have ring chromosome 6. The findings are consistent with multiple manifestations of a neural crest-derived maldevelopment of the anterior segment and central nervous system. Comparison with the 14 previously reported cases of ring chromosome 6 illustrates the phenotypic variability of this syndrome.

Coexistence of Prader-Willi syndrome, congenital ectropion uveae with glaucoma, and factor XI deficiency.

A patient with Prader-Willi syndrome and unilateral congenital ectropion uveae with glaucoma was found to have factor XI deficiency and reduced levels of serum luteinizing hormone, follicle-stimulating hormone, and testosterone. Administration of gonadorelin (LH-RH) increased serum levels of luteinizing hormone and follicle-stimulating hormone, while clomiphene citrate had no effect, suggesting a primary hypothalamic defect. Patients with congenital ectropion uveae should be followed up for the development of both glaucoma and neural crest disorders.

Ocular parasitic infection in Thailand.

Parasitic infestation is a major health problem in tropical countries. Increasing tourism and the influx of refugees from Southeast Asia demand a greater awareness of ocular parasitology. In Thailand, cysticercosis, gnathostomiasis, and angiostrongyliasis are the three commonest ocular parasitic diseases. No antiparasitic drugs are yet available to treat ocular involvement, and therapeutic success depends upon early and complete surgical removal. Because the eye is the only site at which direct visualization and surgical removal are possible, ocular examination is crucial to diagnosis and treatment.

The use of PhEA 34c in trabeculectomy.

PhEA 34c, a 1.9% solution of sodium hyaluronate (Healon = 1.0%) was injected intracamerally and between the scleral and conjunctival flaps after trabeculectomy in 15 eyes, while randomized controls received balanced salt solution (BSS). Anterior chamber depths in both groups in the early postoperative period were significantly different from baseline measurements, but were not different between the two groups at any interval. PhEA 34c did not prevent postoperative flat or shallow anterior chambers. There was no significant difference in the rate of success between the two groups for the duration of the study, but five patients in the PhEA 34c group developed loculated blebs.