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Chromatic visual phenomenon caused by a subluxed intraocular lens.

A 74-year-old man developed an unusual chromatic aberration as a complication of cataract extraction surgery with posterior chamber intraocular lens implantation. The patient could see the location and color of one of the haptics of the dislocated lens. His symptoms resolved with miotic therapy.

Aged↗

Tapetochoroidal degeneration combined with cataract and lentodonesis.

Presumably the combination of regional choroidal atrophy, cataract and lentodonesis is a new genetic entity. The hereditary transmission seems to be autosomal-recessive. As the first visual impairment, hemeralopia is noted during the second decade of life. In these early stages patches of choroidal atrophy are limited to the intermediate and perimacular zone. ERGs are not known from these early stages. Later on, no scotopic and only subnormal photopic reactions are registered. The patients can do their work relatively well until the end of the sixth decade, when a cataract develops and the choroidal atrophy gradually extends to the posterior pole. Together with the cataract, a lentodonesis is developing. The lentodonesis is caused by degeneration and elongation of the zonular fibres. Therefore, in order to escape the danger of a lens-subluxation or a lens-luxation into the anterior chamber, an intracapsular extraction should be done at an early stage of lentodonesis. In spite of this, the visual prognosis is poor, since the areas of choroidal atrophy are gradually enlarging. In these late stages the macula seems to be involved in all cases.

Atrophy↗

Contact lens correction of patients with Marfan syndrome.

BACKGROUND: Marfan syndrome, a genetic connective tissue disorder, manifests many problems: high myopia, astigmatism, crystalline lens subluxation, and cataracts. No published studies have described the contact lens corrections that can be used with Marfan syndrome patients. This report describes the variety of contact lens modalities used for a subpopulation of patients with Marfan syndrome. METHODS: The clinical records of eight patients with Marfan syndrome (16 eyes) from two hospital-based contact lens practices were retrospectively reviewed. Seven different contact lens modalities were used for this subpopulation. RESULTS: Mean unaided visual acuities were 20/296, while mean best-corrected optimal spectacle refraction visual acuities were 20/33. Contact lenses improved the mean visual acuity to 20/27. Patients wore the contact lenses for an average of 12 hours per day. Complications of contact lens wear occurred in approximately 70% of eyes and included neovascularization, giant papillary conjunctivitis, 3 and 9 o'clock staining, and central superficial punctate staining. Additionally, ocular non-contact lens-related complications occurred in 50% of patients. CONCLUSIONS: A variety of contact lens designs may be used during the care of patients with Marfan syndrome. However, patients may experience an increased rate of complications associated with contact lens wear or unrelated to lens wear, and thus they deserve more intensive follow-up care than non-Marfan syndrome patients.

Adolescent↗

Neodymium:YAG laser iridotomy as a possible contribution to lens dislocation.

We report on a patient with angle-closure glaucoma in the right eye that was apparently triggered by lens subluxation and tilting forward after trauma to the eye. Further inferior dislocation of the lens was detected after neodymium:yttrium aluminum garnet (Nd:YAG) laser iridotomy was performed superotemporally. We suspect that the Nd:YAG laser iridotomy, which was performed peripherally, might have induced further zonular rupture and lens dislocation due to a shock-wave effect, especially when the lens was already tilted forward. We suggest that the use of the Nd:YAG laser in cases in which subluxation of the lens is suspected should be considered as potentially hazardous.

Aged↗

Anterior megalophthalmos.

We describe a 36-year-old female who suffered from presenile cataract (nuclear sclerosis and posterior subcapsular opacity, with more-severe disease in the right eye than in the left) and report the use of some ophthalmologic examinations to evaluate the anatomic structures and visual functions of both eyes. Slit-lamp biomicroscopy showed increasing horizontal and vertical corneal diameters (14.0/13.5 mm) and iridodonesis. The pupils could not be fully dilated, and the lenses exhibited cataractous changes. The postlimbal depths were 1.772 mm (OD) and 1.690 mm (OS) (normal value, < 0.20 mm). The results of gonioscopy, specular microscopy, Goldmann visual field, and intraocular pressure evaluation were all within normal limits. Because the patient had bilateral megalocornea in the absence of glaucoma, a very deep anterior chamber depth, secondary effects of iridodonesis, and changes to cataractous lenses, anterior megalophthalmos was diagnosed. This disease is rare. Secondary effects such as iridodonesis, miosis, atrophy of the iris stroma lens subluxation, and occurrence of cataractous lens should be kept in mind.

Adult↗

Pars plana vitrectomy, lensectomy, or extraction in transscleral intraocular lens fixation for the management of dislocated lenses in a family with Marfan's syndrome.

BACKGROUND AND OBJECTIVES: Congenital lens subluxation may be a difficult therapeutic problem. Surgical treatment options include iris manipulation or lens decision, aspiration, intracapsular or extracapsular extraction, and lensectomy through the pars plana. It is not established which kind of aphakic correction is the most appropriate in these cases. PATIENTS AND METHODS: A father and his two sons with Marfan's syndrome were operatively treated for lens dislocation in both eyes. Pars plana vitrectomy was done in all eyes. Dislocated lenses were removed by lensectomy in three eyes and with an intracapsular method in three eyes. The outside-in scleral fixation technique was used for primary posterior chamber intraocular lens (PC IOL) implantation in all cases. RESULTS: All eyes achieved good visual acuity (20/20-20/25). Time of observation ranged between 8 and 20 months. There were no intraoperative or post-operative complications. CONCLUSION: Pars plana vitrectomy and primary scleral-fixated IOL implantation is a safe procedure and gives good visual rehabilitation in adult patients with Marfan's syndrome.

Adult↗

[Post-traumatic glaucoma].

This paper presents theoretical aspects concerning the physiopathological mechanisms involved in producing the glaucoma following trauma, especially the lens related glaucoma, correct identification of the ethiopathogeny of the intraocular pressure elevation in this situation is essential for the appropriate management. These aspects are illustrated by two cases of recent glaucoma following trauma lens related (lens subluxation) treated by medication and surgery. The surgical procedure consisted of intracapsular extraction of the lens, anterior vitrectomy, iridectomy and implantation of sulcus sutured posterior chamber lens. We present the technique, the difficulties met intra- and postoperative, evolution and prognosis.

Eye Injuries↗

[The problem of amblyopia in subluxated lenses (author's transl)].

A re-evaluation of the incidence and development of amblyopia was carried out in 27 patients with a history of lens subluxation at pre-school age. Due to insufficiently accurate histories and the heterogeneous composition of the material it is impossible to make any valid statement regarding the critical phase of development of deprivation amblyopia. However, it appears that "form-deprivation" amblyopia may regress spontaneously up to early school age following hygienization of the optic media and if optimal spectacles or contact lenses are worn. In individual cases it has been shown that unilateral amblyopias can and should be treated (pleoptics) following hygienization of the optic media. Therefore, early surgical treatment should be considered in such cases, above all in view of the fact that complications are rare.

Adolescent↗

Genetic spontaneous late subluxation of the lens. A study of two families.

Patients with genetic spontaneous late subluxation of the lens may present signs and symptoms other than lens displacement due to secondary anatomic alteration of the anterior segment. Two families are described, one of them with affected individuals in five successive generations. The vast majority of the patients sought consultation because of progressive myopia or intermittent acute intraocular hypertensive crisis which obscured the basic condition. Upon recognition of the disease, the affected patients underwent an uneventful intracapsular extraction. Histopathologic examination of the removed lenses suggested a dysplastic development of lens zonules.

Adult↗

Phacoemulsification of the subluxated or atopic lens.

In cases of an ectopic lens (e.g. Marfan Syndrome) with elongated zonular fibers, the lens can be partially emulsified. This can be done in such a way that it leaves behind a diaphragm consisting of zonular fibers and posterior capsule which is strong enough to support a posterior chamber lens. In cases of traumatically subluxated lenses intercapsular phacoemulsification can be done. The zonular dialysis can be fixed by suturing the capsule directly into the sulcus. The PCL can then be implanted either into the capsular bag or into the sulcus. Suture fixation of a posterior capsule is also possible as a reconstructive measure in previously extracapsular operated eyes.

Adult↗

Corneal endothelium in Marfan syndrome. A clinical and specular microscopic study.

Marfan syndrome is classified as a heritable disorder of connective tissue, and it has been assumed to be an inborn error of protein metabolism particularly in collagen or elastin. We examined 41 Marfan syndrome patients and found in the corneal endothelium guttata-formation, considerable cell pleomorphism, non-reflecting endothelial black spots and in some cases abnormal looking endothelial cells. The relation of pathological endothelium to lens subluxation, axial length and other corneal findings is discussed.

Adolescent↗

Feline lens disorders.

The lens is the second most powerful structure for refraction in the eye, and is important in the fine-focusing ability in individual species. Specific disorders of the feline lens are frequent in clinical practice and include: congenital anomalies, nuclear sclerosis, cataracts, lens subluxations and luxations, intraocular sarcomas, and anterior lens displacement associated with aqueous misdirection glaucoma. Clinical signs and current therapeutic options are discussed for the general practitioner. A close working relationship with the regional veterinary ophthalmologist is advised.

Animals↗

Ocular injuries caused by elastic cords.

Five patients with ocular trauma due to elastic cords with attached metal or plastic hooks were evaluated over a 13-month period. Injuries included hyphema, angle recession, lens subluxation, choroidal rupture, vitreous hemorrhage, retinal detachment, and scleral rupture. Two patients suffered penetrating ocular injuries, and three patients had final visual acuities of 20/200 or less. This series emphasizes the potential severity of ocular injury due to these commonly used devices and reiterates a modification in design that might decrease the likelihood of accidental trauma from elastic cords.

Adult↗

Elastic cord-induced ocular injuries.

PURPOSE: To evaluate the incidence and severity of ocular injuries caused by elastic cords. PATIENTS AND METHOD: A retrospective review of patients' records from the Emergency Department was conducted to identify cases of elastic cord-induced ocular injuries. Over a 67-month period starting in October 1996, 28 patients (24 males and 4 females, mean age 38.6 years) with elastic cord-associated ocular trauma were identified. Patient demographics, mechanism of injury, presenting and final visual acuity, ocular injuries, medical and surgical intervention and length of follow-up were noted. RESULTS: The most common anterior and posterior segment injuries were hyphaema/microhyphaema (71.4%) and commotio retinae (60.7%), respectively. The most severe injuries sustained were corneal or scleral lacerations (7.1%), formation of retinal tears (10.7%) or retinal detachment (3.6%), lens subluxation (7.1%) and traumatic cataract (7.1%). Eight patients (28.6%) were admitted for surgical or medical treatment. Mean visual acuity was 20/40 on presentation and 20/25 at the end of the follow-up period. CONCLUSIONS: Elastic cords may cause a wide range of ocular traumas of varying severity. Appropriate design modifications that would prevent the spontaneous release of their hooks are likely to make their use significantly safer.

Adult↗

Uncontrolled glaucoma secondary to an arteriovenous malformation in a Weill-Marchesani patient.

BACKGROUND: Weill-Marchesani syndrome is a rare condition with ocular and systemic manifestations. Common ocular signs are microspherophakia, ectopia lentis, and secondary glaucoma. The glaucoma is usually treatable through medical or surgical management. The most-common arteriovenous malformation (AVM) with ocular effects is the carotid-cavernous sinus fistula. It is very uncommon to observe ocular complications from an AVM in the area of the straight sinus and vein of Galen. AVMs in any location are unusual with Weill-Marchesani syndrome. CASE REPORT: We present a case of severe asymmetric glaucoma in a Weill-Marchesani patient with an AVM in the area of the straight sinus and vein of Galen. The patient demonstrates the known clinical signs of Weill-Marchesani syndrome: short stature, microspherophakia, lens subluxation, and secondary glaucoma. An AVM is diagnosed with corresponding monocular proptosis and asymmetric glaucoma. The patient's serious systemic health problems and the location of the AVM make treatment difficult. CONCLUSION: This case documents the very unusual occurrence of unresponsive glaucoma secondary to an AVM not located in the cavernous sinus. The Weill-Marchesani syndrome may be partially responsible for the glaucoma. Patients with Weill-Marchesani syndrome require early diagnosis in order to treat serious ocular and life-threatening systemic complications.

Adult↗

Effects of alpha chymotrypsin on the canine eye.

Injection of 150, 500, or 750 U of alpha chymotrypsin into the posterior chamber of clinically normal Beagles resulted in changes in intraocular pressure and iridocyclitis. In the 6 eyes treated with 500 or 750 U, lens subluxation occurred in 4 eyes, retinal detachment in 1 eye, retinal degeneration in 1 eye, and optic nerve degeneration in 6 eyes. Scanning electron microscopic changes included dose-related zonulolysis and zonulary remnants within the aqueous humor outflow pathways. Ultrastructural changes by transmission microscopy of the optic nerve degeneration revealed loss of axoplasm and organelles and disruption of the myelin sheaths. Retinal and optic nerve changes may represent the direct toxic effects of alpha chymotrypsin and/or the indirect effects of increased intraocular pressure.

Animals↗