Cataract surgery and artificial iris implantation in patient with oculocutaneous albinism: a case report.

We present a case report detailing the successful phacoemulsification surgery with artificial iris implantation for two individuals with oculocutaneous albinism. These women suffered from cataracts, resulting in reduced visual acuity and heightened photophobia due to iris pigmentary epithelium deficiency. The patients underwent phacoemulsification along with prosthetic artificial iris implantation into the posterior chamber. This intervention resulted in improved visual acuity, reduced photophobia and glare, and an overall enhanced quality of life. Our report highlights two cases of successful phacoemulsification and artificial iris implantation in patients with oculocutaneous albinism and cataracts, leading to improved visual acuity, reduced photophobia, and enhanced quality of life. Notably, there are no prior records in South American literature of cataract surgery combined with artificial iris implantation for oculocutaneous albinism patients up to the time of this publication.

[Method of surgical treatment of patients with oculocutaneous albinism using artificial iris]. / Sposob khirurgicheskogo lecheniya patsientov s glazokozhnoi formoi al'binizma s primeneniem iskusstvennoi raduzhki.

Oculocutaneous albinism is a group of autosomal recessive disorders that directly affect the hypopigmentation of the skin, hair, and eyes. According to the World Health Organization, albinism occurs in 1 out of 20 000 people in Europe and North America. Congenital changes in the organ of vision in albinism lead to a significant decrease in the visual functions of patients, worsening their quality of life. PURPOSE: To study the results of surgical treatment with implantation of an artificial iris as a diaphragm element in patients with albinism. MATERIAL AND METHODS: This article was based on the examination and treatment of 2 patients suffering from oculocutaneous albinism, which is characterized by hypopigmentation of the iris and retinal pigment epithelium of the retina, foveolar hypoplasia, low visual acuity, nystagmus, strabismus and excruciating photophobia. The patients were implanted an artificial iris in both eyes with fixation in the capsular bag in the first patient, and in the ciliary sulcus in the second. RESULTS: In both patients, complaints of photophobia and optical phenomena have ceased. Visual acuity increased to 0.2 and remained without regression throughout the entire period of observation. Intraocular pressure and hydrodynamic parameters also remained stable throughout the observation period. The artificial irises were observed by ultrabiomicroscopy to remain in a stable position both when it was implanted in the capsule bag, and when it was seamlessly fixed in the ciliary sulcus. CONCLUSION: The surgical interventions relieved patients from glare, at the same time improving visual acuity without correction, as well as achieving a pronounced cosmetic effect and visual rehabilitation. Artificial irises have proven to be a safe and effective way to treat patients with oculocutaneous albinism.

Combined cataract surgery and aniridia ring implantation in oculocutaneous albinism.

PURPOSE: To present results of cataract surgery combined with aniridia ring implantation in patients with oculocutaneous albinism (OCA) in terms of vision, refraction, eye movements, and subjective photophobia and glare. SETTING: Anterior segment services of Noor Eye Hospital, Tehran, Iran. DESIGN: Consecutive clinical case series study. METHODS: Enrolled patients underwent phacoemulsification and posterior chamber intraocular lens (PC IOL) implantation and had 2 Morcher aniridia rings (types 50 F or 50 E) inserted intracapsularly. Preoperative and postoperative examinations included tests for visual acuity, manifest refraction, measurement of ocular alignment, and electronystagmography. After surgery, patients also completed a questionnaire to subjectively assess glare reduction. RESULTS: Twelve eyes of 6 OCA patients were included. Minimum follow-up was 6 months. None of the patients had any intraoperative or postoperative complications. In addition to improved uncorrected and corrected distance visual acuity and significant reduction of refractive error, all patients had a marked reduction of glare and photophobia after surgery. CONCLUSION: Iris ring implantation during cataract surgery was an effective procedure for increasing vision and reducing glare disability in patients with OCA. In light of the results and patients' expressed satisfaction, a study should be performed to assess the outcomes of refractive lens exchange along with implantation of a PC IOL and prosthetic iris device in OCA patients seeking refractive correction.

An interesting case of bilateral bifid insertion of superior rectus muscle as an intra-operative finding in a patient with oculocutaneous albinism.

We report a case of bilateral bifid insertion of superior rectus muscles, in a patient with oculocutaneous albinism as an incidental intraoperative finding during eye muscle surgery. The muscle was successfully operated on and the patient did well postoperatively. To our knowledge, this is the first report of this anomaly.

Implantation of phakic intraocular lens in 3 patients with oculocutaneous albinism.

UNLABELLED: We present 3 cases with oculocutaneous albinism in which a high refractive error was optically corrected by implantation of an Artisan iris-fixated phakic intraocular lens, with a follow-up of 8 to 14 years. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Presence of fusion in albinism after strabismus surgery augmented with botulinum toxin (type a) injection.

It is commonly accepted that albino patients with strabismus rarely achieve binocularity and depth perception after strabismus surgery. The presence of retino-geniculo-cortical misrouting, a hallmark of the visual system in albinism, does not necessarily cause total loss of binocular vision, however, not even in albino patients with strabismus. Recently some degrees of stereopsis were reported in albinism patients with minimal clinical nystagmus, if any, in the absence of strabismus. It is possible that patients with albinism and strabismus have binocular visual potential which appears after strabismus correction and provides appropriate postoperative alignment in the long term. Here we present two cases of clinically diagnosed oculocutaneous albinism, an 18-year-old girl and a 16-year-old boy, both with exotropia ≥40 prism diopter, who gained acceptable alignment and fusion after surgical correction of their strabismus as demonstrated on Bagolini testing. In cases of albinism accompanied by visual pathway abnormalities and strabismus, binocular visual potential is not impossible, and some levels can be expected. Thus, these patients, like other cases of strabismus, may benefit from treatment of strabismus at an earlier age to achieve appropriate alignment, cosmetic satisfaction, and a possibly increased chance of fusion.

Outcomes of prosthetic iris implantation in patients with albinism.

PURPOSE: To assess the safety and efficacy of implantation of prosthetic iris devices in patients with albinism. SETTING: Private practice, Cincinnati, Ohio, USA. METHODS: This retrospective noncomparative case series comprised 13 eyes of 8 patients with diminished pigment epithelium of the iris secondary to albinism. All eyes had anterior segment surgery with intraocular lens (IOL) and prosthetic iris device implantation. Eleven eyes received an endocapsular-type iris reconstruction implant (Morcher aniridia interdigitating rings), and 2 eyes (1 patient) received a combined iris-IOL device (Ophtec model 311). The visual acuity, subjective glare reduction, postoperative anatomical outcomes, and intraoperative or postoperative complications were reviewed. RESULTS: All eyes achieved the desired anatomic result. The best corrected visual acuity improved in 8 of 13 eyes, remained stable in 3 eyes, and decreased in 2 eyes. Glare and photophobia improved subjectively in 6 of 8 patients, remained unchanged in 1 patient, and increased in 1 patient after implantation of an artificial iris diaphragm. There were no intraoperative or postoperative complications. CONCLUSIONS: Implantation of prosthetic iris devices appears to be useful in the management of patients with iris deficiency secondary to albinism. The procedure was safe and particularly effective in reducing glare and photophobia in most patients.

Black diaphragm aniridia intraocular lens for aniridia and albinism.

BACKGROUND: Our aim was to assess the safety and efficacy of primary and secondary implantation of a black diaphragm aniridia intraocular lens (IOL) in patients that lacked a complete iris diaphragm. METHODS: This was a retrospective non-comparative study of six eyes in five patients with iris defects. The causes of such defects included congenital aniridia, traumatic aniridia, and oculocutaneous albinism. Three eyes underwent primary implantations of a black diaphragm IOL, and three eyes were given secondary implantations. The visual acuity, subjective severity of glare, postoperative anatomical outcome and any intraoperative or postoperative complications were reviewed. RESULTS: The mean follow-up period was 20.6 months (range 3-29 months). All patients showed stable or improvement in best-corrected visual acuity postoperatively. Glare and photophobia had improved subjectively in all patients after implantation of the black diaphragm IOL. Intraoperative complication included one case of hyphaema and iris damage during insertion of the IOL. Postoperative complications included intraocular inflammation with choroidal detachment, secondary glaucoma, and persistent epithelial defect after surgery. None of the patients developed decentration of IOL after surgery. CONCLUSION: The black diaphragm aniridia IOL is useful in the management of the condition in patients with iris deficiency including oculocutaneous albinism. Intraoperative and postoperative complications are not uncommon, and patients should be monitored carefully in the perioperative period.

Effects of extraocular muscle surgery on 15 patients with oculo-cutaneous albinism (OCA) and infantile nystagmus syndrome (INS).

PURPOSE: The purpose of this report is to characterize the clinical and electrophysiological effects of extraocular muscle surgery in 15 patients with oculo-cutaneous albinism (OCA) and infantile nystagmus syndrome (INS). Our hypothesis is that surgery on the extraocular muscles of patients with OCA and INS changes their nystagmus and their visual function. DESIGN: Interventional, prospective, cohort, noncomparative case series. METHODS: All 15 patients had surgery on all four virgin horizontal recti; three for strabismus alone, three for nystagmus alone, five for an eccentric gaze null zone alone, and four for an eccentric gaze null zone plus strabismus. All patients have been followed for at least six months. All 15 patients had the subjective outcome measure of pre- and postoperative binocular best optically corrected acuity (BBOCA). Objective outcome measures included anomalous head posture (AHP) in nine patients, eye movement recording measures of expanded nystagmus acuity function (NAFX) in 10 patients, null zone position (NUZP) and null zone width (NUZW) in 10 patients, and foveation time (FOV) in nine patients. RESULTS: The results are summarized as follows; BBOCA increased 0.1 LogMar or greater in 14 of 15 patients. In those operated on for an AHP with or without associated strabismus the AHP improved significantly (P < .01 for all). The NAFX, NUZP, NUZW, and FOV measured from eye movement recordings showed persistent, significant increases in all patients (P < .01 for all). CONCLUSIONS: This report adds to the evidence that surgery on the extraocular muscles in patients with INS has independent neurologic and visual results.

Primary malignant melanoma of the lung in oculocutaneous albino patient.

Oculocutaneous albinism is a rare autosomal recessive disorder characterized by general depigmentation, nystagmus, photophobia, and decreased visual acuity. Malignant melanoma is extremely rare in patients with albinism. We present a 41-year-old albino male patient, who was admitted with a suspected bronchogenic carcinoma. He underwent a pulmonary resection and the diagnosis was primary malign melanoma of the lung. The patient died of tumor recurrence in the postoperative 46th month.

Posterior chamber intraocular lens implantation in a patient with oculocutaneous albinism.

A 56-year-old woman with tyrosinase-negative oculocutaneous albinism complained of gradually decreasing vision in the left eye. Her visual acuity was 20/300 in the right eye and hand motion in the left eye. She had pink skin and white hair and cilia. On examination, bilateral hypopigmented irides, an opaque lens in the left eye, a hypopigmented fundus in the right eye, and nystagmus were found. An extra-capsular cataract extraction with posterior chamber intraocular lens (IOL) implantation was performed. After retrobulbar anesthesia was administered, the nystagmus ceased temporarily. Postoperatively, visual acuity in the left eye improved to 20/200. We believe IOL implantation is useful in the treatment of cataracts in albino patients.