Segmental Iris Research Articles

Exchange of aniridia IOLs for customized iris IOL implants

HintergrundEs können 3 Gruppen an Irisprothesen zur chirurgischen Versorgung von Irisdefekten unterschieden werden: (1) segmentale Irisimplantate, (2) kombinierte Irisblenden-Intraokularlinsen (IOL) und (3) reine Irisimplantate. Die meisten Irisrekonstruktionen gehen zusätzlich mit einer Aphakiekorrektur durch sekundäre Linsenimplantation einher. Auch wenn primäre Ziele die Herstellung einer Pupille sowie die Besserung des Blendungsempfindens, der Kontrastsensitivität und der Sehschärfe sind, spielt das ästhetische Ergebnis eine nicht zu vernachlässigende Rolle.Ziel der ArbeitDargestellt werden funktionelle und ästhetische Ergebnisse nach Austausch von Aniridie-IOL-Implantaten gegen eine individuell angefertigte künstliche Iris in Kombination mit einer IOL.Material und MethodenIn dieser retrospektiven Studie mit 7 Augen von 7 Patienten wurde eine Irisblenden-IOL (Morcher GmbH, Stuttgart) aus medizinischen Gründen (Subluxation) gegen eine individuell hergestellte künstliche Iris aus Silikon (ArtificialIris, HumanOptics, Erlangen) in Kombination mit einer angenähten IOL ausgetauscht. Die Nachbeobachtungszeit betrug mindestens 3 Monate. Bestkorrigierter Fernvisus (BCVA), Endothelzellzahl (ECC), Komplikationen, Blendungsempfinden, das ästhetische Ergebnis und die Patientenzufriedenheit wurden evaluiert.ErgebnisseBCVA und ECC zeigten keine statistisch signifikante Änderung zwischen prä- und postoperativ (p > 0,05). Es zeigte sich eine Dezentrierung des Iris-IOL-Implantats von 0,27 ± 0,19 mm 3 Monate postoperativ. Auf einer visuellen Analogskala (VAS) von 1 bis 10 (1 = gar nicht bis 10 = extrem zufrieden) wurde die Zufriedenheit mit dem Gesamtergebnis mit 8,6 ± 2,5 bewertet. Das subjektive Blendungsempfinden besserte sich auf 5,6 ± 3,5 und die subjektive ästhetische Beeinträchtigung auf 2,4 ± 2,0 auf der VAS (1 = gar nicht bis 10 = extrem stark). Die postoperativen Komplikationen umfassten eine vorübergehende intraokulare Hypotonie in zwei, einen Druckanstieg in zwei, eine Netzhautablösung und eine transiente Glaskörperblutung jeweils in einem Auge. Sechs von sieben Patienten würden den Eingriff wiederholen.SchlussfolgerungIm Vergleich zu einem starren Aniridie-IOL-Implantat bietet der Austausch gegen eine individuell angefertigte künstliche Iris in Kombination mit einer IOL neben einem guten funktionellen gleichzeitig auch ein ästhetisch ansprechendes Ergebnis.Video onlineDie Online-Version dieses Beitrags (10.1007/s00347-021-01447-9) enthält ein Video.

Axenfeld-Rieger Syndrome

A typical case of Axenfeld-Rieger syndrome (ARS) with clinical features of bilateral posterior embryotoxon, correctopia, pseudopolycoria and segmental iris hypoplasia is presented. Gonioscopy revealed anteriorly displaced Schwalbe's line and bands of iris extending across the iridocorneal angle to the trabecular meshwork. Fundus was within normal limits in both the eyes. Systemic examination revealed hypodontia, microdontia, prominent lower lip and redundant peri-umbilical skin. There was no associated cardiovascular or other associated systemic association. Axenfeld-Rieger syndrome is a multi-system anomaly requiring a multidisciplinary approach to management. The patient needs to be regularly followed-up for development of glaucoma

Rieger’s Anomaly without Posterior Embryotoxon: A Rare Presentation

Rieger’s anomaly is a rare congenital ocular defect with autosomal dominant inheritance, characterised by dysgenesis of the anterior segment. Ocular features of typical Reiger’s anomaly include a prominent anteriorly displaced Schwalbe's line (posterior embryotoxon), iris stromal hypoplasia, corectopia, and glaucoma. An atypical presentation of Rieger’s anomaly is described in the current case report. A 26-year-old female presented with diminution of vision in right eye and mild photophobia in both eyes, since childhood. On examination, it was recognised as an atypical isolated case of Rieger’s anomaly with several classical features including segmental full thickness iris defect and ectropion uveae. This was associated with total cataract in right eye and persistent pupillary membrane in left eye, without posterior embryotoxon and glaucoma. There were no other associated ocular or systemic anomalies. Patient was operated for cataract surgery in right eye under guarded visual prognosis. The postoperative visual acuity was 3/60, signifying pre-existing amblyopia. The patient was kept on close follow-up for the development of glaucoma. This is a rare case of Anterior Segment Dysgenesis (ASD) manifesting as atypical Rieger’s anomaly without posterior embryotoxon and any systemic association, signifying the fact that posterior embryotoxon is not an essential diagnostic criterion.

Clinical Manifestations and Characteristics of In Vivo Confocal Microscopy in Varicella Zoster Virus-Related Corneal Endotheliitis

ABSTRACTObjective: To explore the clinical characteristics and in vivo confocal microscopic (IVCM) findings of varicella zoster virus (VZV)-related corneal endotheliitis.Methods: Retrospectively reviewed 20 eyes with corneal edema which were diagnosed by real-time polymerase chain reaction.Results: one had VZV infection. Three had epithelial lesions. Six had mydriasis. Four had loss of iris pigment. Keratic precipitates (KPs) were mixed. Subbasal nerves had disappeared in 12 eyes. Langerhans cells were observed in seven eyes. The deviations in endothelial cell layers consisted of guttate (n = 1), enlarged intercellular gaps (n = 11), infiltration of inflammatory cells (n = 8), loss of defined cell boundaries (n = 1) and anomalous nucleus (n = 9). The shape of KPs in IVCM included type I (n = 6), type III (n = 3) and type IV (n = 4).Conclusion: VZV-related corneal endotheliitis is remarkably difficult to detect clinically. Most cases have no typical skin lesions. The typical clinical feature is that of segmental iris atrophy and mixed KPs.

Segmental ischaemic infarction of the iris after autologous fat injection into the lower eyelid tissue: a case report

BackgroundAutologous fat injection is getting popular in cosmetic procedures, however, still has a risk of fat embolism. Herein, we report the first case of segmental ischaemic infarction of the iris, which occurred after autologous fat injection into the lower eyelid.Case presentationA 28-year-old Korean woman complained of the discolouration of the iris after the fat injection. Slit-lamp biomicroscopy revealed segmental depigmented atrophic iris with sectoral sphincter palsy.ConclusionsWe found that iris atrophy could be caused by autologous fat transplantation. The plastic surgeons should pay more attention to possibility of fat embolism-induced ocular complications in the procedure of fat injection.

Adie’s Tonic Pupil

A 33-year-old woman with no past medical history presented to the emergency department with asymmetric pupils. At 7:30am while putting on makeup, she noted her pupils were equal in size. One hour later, she developed light sensitivity in her right eye, and soon after noticed her right pupil was significantly enlarged. She denied headache, facial or extremity weakness, dysarthria, or ataxia. On exam, her left pupil was reactive from 4 to 3 mm and her right pupil was sluggishly reactive and 8 mm (Figure). No abnormalities in her visual acuity, extraocular movement or fundoscopic exam were detected. Neurologic consultation was obtained, but the patient had an unremarkable brain computed tomography (CT)/CT-Angiography and magnetic resonance imaging. Figure. Photograph of 33-year-old female presenting with asymmetric pupils. A tonic pupil results from parasympathetic denervation at the level of the ciliary ganglion. It is characterized by a large, regular pupil with decreased response to light but preserved or enhanced constriction to accommodation, segmental iris constriction, vermiform movements of the pupillary border, and hypersensitivity to pharmacologic constricting agents.1–4 The diagnosis was established in consultation with ophthalmology and confirmed with rapid miotic response of the affected pupil to 0.125% pilocarpine drop.1,2 Most cases are idiopathic, occurring in women 20–40 years of age, and referred to as the Adie’s tonic pupil, though this disorder can be due to local disorders within the orbit, including tumor, inflammation, trauma, surgery, ischemia or infection. 3 Most patients do not require any treatment and can be reassured once the diagnosis is confirmed.

Irisprothetik

Aniridia or iris deficiencies can severely affect visual function in mostly younger patients and should therefore be corrected. Currently various technical options for the treatment of iris deficiencies are available. The present review considers the main causes of aniridia and the incidence and summarizes the negative effects on visual function. A brief historical overview of the development of prosthetic iris devices is given. Currently available prosthetic iris devices, mainly iris diaphragm lenses and segmental prosthetic iris devices, are presented along with the specific advantages and disadvantages. The management of patients with iris deficiencies is often challenging due to the great heterogeneity and complexity of the underlying ocular problems. Thus an individual approach considering the often difficult pathological situation is mandatory. However, some universally valid recommendations for the implantation of prosthetic iris devices can be given. The literature from 2000 onward is reviewed and all articles on implantation of prosthetic iris devices are evaluated. The results are discussed and also summarized in a table.

A Case of Waardenburg Syndrome Type 2 With Anisocoria

Purpose: To report a case of Type 2 Waardenburg syndrome with anisocoria. Case summary: A 53-year-old woman with congenital deafness visited the clinic complaining of photophobia and heterochromic iris in the left eye. Her best-corrected visual acuity was 1.0 in the right eye and 0.7 in the left eye. The patient’s left eye showed complete iris hypopigmentation, and the right eye showed segmental iris hypopigmentation. Fundus examination demonstrated generalized decrease in retinal pigment with focal hypopigmented lesions in both eyes. The pupil sizes were 3 mm in the right eye and 5 mm in the left eye. A pure tone audiogram showed profound bilateral sensorineural hearing loss. Conclusions: In the present case, mydriasis was found to exist in a completely hypopigmented iris. Therefore, the neural crest from which the pupillary constrictor muscle originates may play a role in the pathogenesis of Waardenburg syndrome. J Korean Ophthalmol Soc 2010;51(10):1423-1426

Arachnoid Cyst Causing Third Cranial Nerve Palsy Manifesting as Isolated Internal Ophthalmoplegia and Iris Cholinergic Supersensitivity

An 8-month-old boy presented with anisocoria, a sluggishly reactive right pupil, and cholinergic supersensitivity as the only signs of what proved months later to be compressive third cranial nerve palsy due to an arachnoid cyst. Tonic constriction and dilation, segmental iris sphincter palsy, aberrant regeneration phenomena, ductional deficits, and ptosis were absent. The initial diagnosis was postganglionic internal ophthalmoplegia attributed to a viral ciliary ganglionopathy. Nineteen months later, he had developed an incomitant exodeviation and a supraduction deficit. Brain MRI revealed a mass consistent with an arachnoid cyst compressing the third cranial nerve in the right interpeduncular cistern. Resection of the cyst led to a persistent complete third cranial nerve palsy. This is the second reported case of prolonged internal ophthalmoplegia in a young child as a manifestation of a compressive third cranial nerve palsy. Our patient serves as a reminder that isolated internal ophthalmoplegia with cholinergic supersensitivity is compatible with a preganglionic compressive third nerve lesion, particularly in a young child.

The Argyll Robertson Pupil

The Argyll Robertson (AR) pupil has been defined as a pupil that is small and constricts poorly to direct light but briskly when a target within reading distance is viewed ("light-near dissociation"). Most descriptions of the AR pupil do not mention segmental iris sphincter constriction, or slow, sustained constriction with a near vision effort. Such features are considered typical of the light-near dissociation of Adie syndrome and of neuropathic tonic pupils, where damage to the ciliary ganglion or ciliary nerves is believed to be the mechanism. Because the AR pupil lacks these features, it has been attributed to a dorsal midbrain lesion that interrupts the pupillary light reflex pathway but spares the more ventral pupillary near reflex pathway. However, lesions in this region have not been reliably demonstrated in syphilis. Resolving the issue about the location of the syphilitic lesion that produces the AR pupil will depend on careful examination of patients with techniques designed to disclose segmental palsy of the iris. If segmental iris sphincter palsy is found and the light-near dissociation has tonic features, one must conclude that the mechanism of the pupil disorder is a ciliary (peripheral) rather than a midbrain (central) denervation. Until better evidence settles the localization of the AR pupil, it is appropriate to screen patients with bilateral tonic pupils for syphilis.

Effect of Angle Closure Glaucoma and Surgical Intervention on the Corneal Endothelium

To assess the effect of acute angle closure glaucoma (AACG) and related surgical intervention on the corneal endothelium, specular microscopy was performed following surgery in a series of 69 patients: 27 for AACG, 9 for incipient angle closure, 17 for chronic angle closure glaucoma (CACG), and 16 for chronic open angle glaucoma (COAG). Peripheral iridectomy for incipient angle closure glaucoma caused no significant effect on the corneal endothelium, but, following peripheral iridectomy or drainage operation for AACG, significant lowering of the endothelial cell count was present in the affected eye (p less than 0.05) with 1,000 cells/mm2 in 7 cases. This was related to the presence of segmental iris atrophy (p less than 0.01). Peripheral iridectomy for CACG or incipient angle closure glaucoma was not accompanied by a significant effect, but drainage operation for CACG or COAG was associated with a significant fall in count (p less than 0.01). Thus, both AACG and drainage operation significantly affect the corneal endothelium, and this should be assessed before undertaking further surgery.

Anterior Segment Ischemia and Sector Iris Atrophy: After Strabismus Surgery In A Patient With Chronic Lymphocytic Leukemia

A 69-year-old woman with chronic lymphocytic leukemia developed segmental iris atrophy and iridocyclitis after routine surgery for exotropia. Both the clinical picture and fluorescein angiogram indicated anterior segment ischemia. It is postulated that this was related to hyperviscosity of the blood caused by a high white blood cell count (114,000/cu mm). The possibility of anterior segment ischemia should be kept in mind when contemplating strabismus or retinal detachment surgery in the presence of hematologic disorders likely to increase blood viscosity. In these cases a minimal amount of surgery should be done with proper supportive therapy. Strabismus surgery should be done in stages allowing for hemodynamic compensation between procedures.

Ocular Manifestations in Sickle Cell Disease

Ocular findings in sickle cell disease are manifestations of sluggish blood flow, intravascular sickling, and occlusion of vessels. The effects on ocular tissues include sludged flow, dilatation, and segmentalization of conjunctival vessels; segmental ischemic iris atrophy with occluded vessels and neovascularization; vitreous hemorrhages that become organized by fibrovascular proliferative membranes arising from retinal vessels; marked tortuosity of retinal vessels, occlusion of retinal vessels leading to ischemic infarcts, retinal hemorrhages, chalk-white vessels, retinal atrophy, splitting of the retina, and retinal holes; neovascularization with the abnormal arteriovenous connections causing proliferation of new vessels that form sea-fan lesions; iridescent intraretinal deposits and rhegmatogenous and traction retinal detachment; and chorioretinal infarcts and heavily pigmented scars.