Pigment Proliferation Research Articles

OCT of Outer Retinal Hyperreflectivity, Neovascularization, and Pigment in Macular Telangiectasia Type 2

To investigate whether outer retina hyperreflectivity (ORHR) and outer retina neovascularization (ORNV) are visible in eyes with macular telangiectasia type 2 (MacTel 2) before pigment proliferation is visible. Retrospective study in a cohort of patients from the MacTel Project. Thirty-nine MacTel 2 eyes without pigment on color fundus photography (CFP) at presentation were studied over a mean of 36.6 months (range, 13.8-50.4 months; standard deviation, ±10 months). All patients routinely underwent Snellen best-corrected visual acuity (VA) measurement, CFP, spectral-domain OCT, and OCT angiography (OCTA). Pigment in the macula on CFP, best-corrected VA, presence of ORHR, presence of ORNV, presence and surface of ellipsoid zone (EZ) loss, and occurrence of ORHR, ORNV, and pigment. At presentation, 13 eyes showed no EZ loss, and no ORHR nor change was observed during the follow-up. In the 19 eyes with only EZ loss at baseline, ORHR appeared in 6 eyes, and one of them also showed visible pigment on CFP. Among the 7 eyes with ORHR and ORNV at baseline, pigment proliferation became visible in 4 eyes. Overall, at the end of follow-up, 14 eyes showed ORNV and 13 showed ORHR, of which 5 showed pigment on CFP. In all cases, ORHR on spectral-domain OCT corresponded to ORNV on OCTA. Hyperreflectivity and neovascularization developed within an EZ loss area or at its edge. At the end of follow-up, although the EZ loss area was larger in eyes with ORHR, pigment, or both than in eyes without any pigment migration (0.33 mm2 vs. 0.94 mm2; P= 0.01), VA was similar in both groups (0.32 logarithm of the minimum angle of resolution [logMAR] vs. 0.35 logMAR; Snellen equivalent, 20/40 vs. 20/50; P= 0.64). Spectral-domain OCT and OCTA showed that in MacTel 2 eyes, ORHR was associated with ORNV on OCTA and most often did not correspond to pigmentation on CFP. When ORNV developed, hyperreflectivity appeared along the abnormal capillary tracts after a mean follow-up of 36 months. In most cases, the presence of ORHR was not associated with rapid vision loss.

Artificial Skin INTEGRA Use in the Treatment of Large Melanocytic Nevi

Melanocytic nevi are congenital, benign, pigmented proliferations. They have high risk of malignancy, most frequently transform to malignant skin melanoma and 70% it occurs in the first life decade. The risk of malignant transformation increases proportionately to their size. The best way to avoid those risks is early and radical excision. In 1996 on the market appeared the Integra™ artificial skin, has made it possible to improve the methods for the elimination of large congenital nevi and significantly reduce the risk of malignant melanoma, at an early age.
 2011–2019, in Children’s Hospital, Affiliate of Vilnius University Hospital Santaros Clinics, the Integra™ was used to treat congenital melanocytic nevi in four children. Implantation of the skin substitute Integra™ has been successful for all the patients, the prognosis was good.
 The treatment of congenital melanocytic nevi is a necessity to avoid the risk of developing malignant melanoma. The rarity of this condition has led to the fact that so far there is no universal approach for all the cases. Integra™ artificial skin is an excellent choice for the treatment of congenital melanocytic nevi, especially for paediatric patients.

Oral pigmentation in physiologic conditions, post-inflammatory affections and systemic diseases

Melanocytes are found throughout the oral mucosa but usually go unnoticed because of their relatively low level of pigment production. When focally or generally active in pigment production or proliferation they may be responsible for several affections in the oral mucosae ranging from physiologic pigmentation, systemic diseases to malignant neoplasms. The diagnosis of oral pigmentations (OP) is usually challenging for the physician, but a careful examination of the oral cavity may reveal the first manifestation of underlying systemic diseases. Therefore, a full medical history (including drug assumption and smoking) together with a general dermatological examination are mandatory and represent the first approach to OPs. When the diagnosis cannot be reached clinically with enough certainty, a biopsy for histological examination is needed, also in order to exclude possible life threatening conditions such as melanoma. Dermoscopy is another reliable diagnostic tool to make a differential diagnosis between melanocytic lesions and other conditions and then to manage the follow-up of patients. Few papers on the subject have been published in the dermatological literature and the oral cavity is often poorly investigated during routine dermatological examinations. We therefore decided to perform a review of benign OPs, classifying them into diffuse (physiological/racial pigmentations, smoker's melanosis, drug-induced hyperpigmentation, post-inflammatory hyperpigmentation, black hairy tongue, OPs associated to systemic diseases) and localized (amalgam tattoo, melanocytic nevi, melanoacanthoma, melanosis) lesions.

Characteristics of fundus image in macular telangiectasia type 2

Objective To observe the fundus image characteristics of macular telangiectasia type 2 (MacTel type 2) patients. Methods A total of 8 patients (16 eyes) diagnosed of MacTel type 2 were included in this study. There were 4 males and 4 females, age ranged from 44 to 69 years old with a median age of (59.88±7.85) years. All patients received examination of best-corrected visual acuity (BCVA), slit lamp microscope, indirect ophthalmoscopy, fundus color photography, fundus autofluorescence (AF), fundus fluorescein angiography (FFA), spectral domain optical coherence tomography (OCT) and macular pigment optical density (MPOD). Four eyes of 2 patients received OCT angiography examination at the same time. Classification was made according to the Gass and Blodi′s criteria. The follow-up time was from 1 to 19 months with the average time of (11.00±8.91) months. The clinical characteristics were observed and analyzed. Results The BCVA was 0.07 - 0.8. There were 1 eye in stage 1, 1 eye in stage 2, 6 eyes in stage 3, 8 eyes in stage 4. The disease showed a bilateral appearance with a low progression. Fundus features included loss of retinal transparency (14 eyes, 87.5%), blunted retinal venule (15 eyes, 93.75%), pseudo-lamellar hole (5 eyes, 31.25%), pigment proliferation (9 eyes, 56.25%). FFA findings were telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. Spectral domain OCT features included depletion of the retinal inner, outer structures, cavity (7 eyes, 43.75%), and atrophy of the neurosensory retina (9 eyes, 56.25%). On AF, reduced foveal masking due to loss of macular pigment can be observed. The loss of macular pigment could also be seen on MPOD. OCTA showed the increased intervascular spaces, broken regular network of foveal avascular zone (FAZ), right-angled vessel dipping, dilatations, traction of superficial and deep capillary layers in both the superficial and deep layers, especially in the deep layer. Only one eye of one patient proceeded from stage 4 to stage 5. Conclusions Characteristic features of MacTel type 2 included cavities and outer retina atrophy on OCT; loss of MP on MPOD; broken regular network of FAZ, morphological and structural abnormalities of superficial and deep layers of perifoveal capillary network on OCTA. Key words: Retinal Diseases/diagnosis; Macula lutea/injuries; Disease attributes

The noninvasive retro-mode imaging of confocal scanning laser ophthalmoscopy in myopic maculopathy: a prospective observational study.

To investigate the morphological features of myopic maculopathy with a new and noninvasive retro-mode imaging (RMI) technique using a confocal scanning laser ophthalmoscope. A total of 42 patients (69 eyes) with myopic maculopathy were included. RMI combined with fundus photography, fundus fluorescein angiography, and optical coherence tomography together were used to observe and evaluate the morphological features of disease. Four in 4 eyes (100%) with macular retinoschisis were found with a characteristic pattern by RMI (firework pattern centrally with surrounding fingerprint pattern). Twenty-four in 24 eyes (100%) with pigment proliferation were found by RMI as dark plain patches, and 23 in 24 eyes with hemorrhage (95.8%) were found by RMI as gray bump. Atrophy of different degrees (12 in 14 eyes, 85.7%) was found by RMI as an area of pseudo-3D choroidal vessels or a fuzzy shadow but both without a clear boundary. Choroidal neovascularization (12 in 16 eyes, 75%) was identified laboriously by RMI as a vague raised region. Lacquer cracks were difficult to figure out in RMI. Retinoschisis, pigment proliferation, hemorrhage, and atrophy secondary to myopic maculopathy have characteristic morphologic features in RMI; however, choroidal neovascularization and lacquer crack are not easily distinguishable in RMI.

SA population-based study of macular choroidal neovascularization using optical coherence tomography in Eastern China.

The aim of the present study was to investigate the pathomorphological and functional variations of choroidal neovascularization (CNV) in age-related macular degeneration (AMD) in a Chinese population using optical coherence tomography (OCT). This population-based study enrolled 59 patients (age, >45 years; eyes, 70) with early and intermediate-stage AMD from Youyi Road Community, Baoshan District, Shanghai, China. Comprehensive standardized ophthalmic examinations included visual acuity, anterior segment analysis using a slit lamp, dilated fundus evaluation by direct ophthalmoscopy, 90D handheld lens analysis, fundus photography, fundus fluorescein angiography (FFA) and fast optic disk scans using OCT. The macular CNV characteristic profiles in early and intermediate-stage AMD were determined by OCT. Data were obtained on the first visit and the follow-up period ranged between 6 and 24 months, where FFA and OCT outcomes of early and intermediate-stage AMD patients were analyzed. Three profiles of early and intermediate-stage AMD were created from the OCT and FFA results, each with a different prognosis. Firstly, drusens with unclear boundaries and evident pigment proliferation, as well as hypofluorescence around the drusens, was observed via FFA. A slight small arch field located in the retinal pigment epithelium (RPE)/choriocapillary layer (CCL) was shown on OCT scans, indicating exudative AMD. Secondly, RPE detachments of >1 pupillary distance, without CNV in the macular area, indicated geographic chorioretinitis atrophy. Finally, drusens with clear boundaries and few pigment proliferations and no certain surrounding fluorescence was observed via FFA, while a clear RPE/CCL band on the OCT scans indicated slow progress. The results of the present study demonstrated that combined OCT and FFA was the most efficient method for identifying CNV and diagnosing AMD. If the two techniques are not available concurrently, then OCT is a safer and more reliable technique to follow-up early and intermediate-stage AMD patients.

Intraocular pigmented proliferations in the context of cytologic evaluation

This article is written to give an overview of the various intraocular pigmented proliferations as it pertains to cytologic evaluation and interpretation. It reviews the various epithelial and melanocytic lesions, their location and the various clinical approaches the ophthalmologist uses to aspirate the lesion. It also reviews the current thinking in the differentiation between Class I and Class II melanomas and how molecular profiling may be necessary in its differentiation which could help stratify those patients whose tumors are refractory to treatment and may benefit from adjuvant chemotherapy and eye-sparing surgery.

CD34+ Pigmented Fibrous Proliferations: The Morphologic Overlap Between Pigmented Dermatofibromas and Bednar Tumors

Pigmented dermatofibrosarcoma protuberans (DFSP; Bednar tumor) constitutes 5%-10% of all cases of DFSP and shows morphologic features that overlap with melanocytic and fibrous proliferations. We report 2 unusual cases of pigmented fibrous proliferations that demonstrate features of dermatofibromas and DFSP. The first case is that of a 19-year-old man with a 3-year history of a slowly growing pigmented lesion on the right arm. On clinical exam, the lesion was a 7-mm firm pigmented papulonodular lesion. The second case is that of a 31-year-old woman with a 4- to 5-year history of a slowly enlarging, asymptomatic "dark area" on the right buttock. On clinical exam, the lesion was a 2-cm darkly pigmented flat nodule. Morphologically, both lesions are primarily dermal proliferations of spindled cells admixed with pigmented dendritic melanocytes. The lesional cells trap collagen fibers at the periphery and there is basal cell hyperpigmentation. Adnexal structures are effaced, but significant trapping of subcutaneous fat is not present. By immunohistochemistry, both lesions show focal CD34 positivity but are negative for Factor XIIIa and melanocytic markers. Although overlap between standard dermatofibromas and DFSP is well documented in the literature, pigmented fibrous lesions with features of both entities are not well described.

Melanocytoma of the choroid: 29-year follow-up.

In Brief Melanocytoma is a benign pigmented tumor that may be misdiagnosed as a malignant choroidal melanoma. Melanocytomas may have unusual changes such as spontaneous necrosis of retina and choroid with pigment proliferation.

Symptomatic choroidal neovascularization in blacks.

To acquire descriptive clinical information regarding choroidal neovascularization (CNV) in black Americans. Retrospective review of 1308 fluorescein angiograms obtained during a 4-year interval. Color photographs and clinical records of all black patients with angiographically apparent CNV were subsequently reviewed. Retina service of an inner-city county hospital in Atlanta, Ga, serving a predominantly black population. Thirty blacks with CNV (36 of 59 eyes) were identified, 26 (87%) of whom were female. Active, exudative neovascularization was present in at least one eye of 21 patients (70%). Patients were assigned to one of four diagnostic groups for analysis. Group 1 was made up of 13 patients (43%) with age-related macular degeneration with CNV. Women outnumbered men 5.5:1. Choroidal neovascularization was peripapillary in seven (54%) of these 13 patients. Group 2 was made up of six patients (20%) with idiopathic CNV, which was peripapillary in all eyes. Group 3 consisted of three women (10%) with idiopathic polypoidal choroidal vasculopathy. Group 4 was composed of eight patients (27%) with secondary CNV. The CNV was peripapillary in three (33%) of nine eyes, and women outnumbered men 7:1. The spectrum of neovascular maculopathy in blacks in the current study differed from that typically seen in whites, both clinically and demographically. Clinically, CNV was most commonly juxtapapillary (13 [68%] of 19 patients) and unilateral (12 [92%] of 13 patients) among the age-related macular degeneration and idiopathic groups, while six (20%) of 30 patients (all older than 50 years) had CNV in the absence of drusen or other known predisposing conditions. Disciform-stage CNV in both groups was associated with a greater degree of pigment proliferation than that typically noted in whites. Demographically, female predominance (87% overall) was dramatic compared with prior studies.

Degenerative retinopathy in captive Atlantic menhaden, Brevoortia tyrannus (L.)

Abstract. Blindness developed in Atlantic menhaden, Brevoortia tyrannus (L.), within 4 weeks of captivity in an indoor open culture system. Ophthalmoscopic changes indicative of retinal degeneration were substantiated by the absence of retinal electrical activity as measured by conventional electroretinography. Results of histological evaluation suggest that photo‐receptor degeneration, occurring initially in the rods of the central retina, was the primary abnormality. With chronicity, all retinal layers degenerated, and marked pigment proliferation developed. Other pathological changes included uveal vascular engorgement, vitreal and anterior chamber haemorrhage, and cataracts. With the exception of dermal hyperpigmentation, no systemic abnormalities were consistently observed. Phototoxicity is the suspected cause of this degenerative retinopathy.

Complications of Rigid Anterior Chamber Implants

Despite accurate lens sizing, 100 eyes with rigid anterior chamber implants showed a high incidence of lens-related trauma postoperatively: pigment dispersion (pseudoguttata), angle recession, peripheral anterior synechiae, iris erosion/atrophy, iris holes from stretching, angle fibrosis, haptic tip erosion into sclera and iris, neovascularization of peripheral iris and angle, lens rotation through iridectomy and into ciliary body, lens tilting with corneal endothelial touch, high refractive cylinder with long intraocular lenses, pigment proliferation onto iris surface, partial slippage of the intraocular lens into vitreous following intracapsular procedure, and pupillary-block glaucoma. Vision results were good. Glaucoma and cystoid macular edema rates were low. Bullous keratopathy and retinal detachment didn't occur. High endothelial cell loss was associated with those lenses that “propellered” and those that tilted and touched endothelium. The adverse findings may be static or may represent a continuing process of tissue damage.

Continuous Wave Argon Laser Iridectomy in Angle-Closure Glaucoma

By using a continuous wave argon laser in a multiburn fashion, successful iridectomies were achieved in 42 out of 45 phakic eyes with either acute or chronic angle-closure glaucoma. Five of the successful procedures required multiple sittings on different days or abandonment of the primary treatment site in favor of an alternate site, or both, to attain patency. Within the immediate postoperative period, ten eyes needed retreatment to eliminate moderate pigment proliferation at the perforation site. The procedure was performed on an outpatient basis under topical anesthesia, usually required less than one-half hour for completion, and resulted in only minimal complications. Therefore, continuous wave argon laser iridectomy appears to present a viable alternative to surgical iridectomy for the definitive treatment of angle-closure glaucoma, although long-term evaluation of this modality of therapy is presently unavailable.

Retrocorneal Pigment Proliferation after Cataract Extraction

Retrocorneal pigment proliferation after cataract extraction is not rare. Downgrowths proliferate from fragments of iris pigment or peripheral iris in the corneal wound edges and occur slowly. Vision is affected if the central cornea is involved. Preventive measures include minimizing iris trauma and irrigating pigment fragments from the cornea and incision during surgery. In the study presented, patches of dislodged iris pigment epithelium were observed in 22 eyes. Lace-like downgrowths occurred in 18 eyes of 14 patients.

Fluorescein angiography of degenerative lesions of the peripheral fundus and rhegmatogenous retinal detachment.

Since the number of cases for each of the conditions in our study was small, only preliminary conclusions can be made; in order to establish the fluorescein pattern of each of these peripheral retinal lesions, additional studies will be required. The following summarizes our preliminary observations. 1. Areas of retinal white-with-pressure or without and peripheral retinal cystoid degeneration did not reveal remarkable fluorescein angiographic findings. One exception was in an area of pigment epithelial disturbance characterized by atrophy and proliferation, which showed a "window and masking" effect of choroidal fluorescence. 2. Fluorescein angiography of areas of the fundus with lattice retinal degeneration showed little or no findings in early or mild cases. In severe or advanced cases, the affected retina revealed poor or absent perfusion caused by vascular occlusion. The retinal and choroidal circulation was devoid of fluorescein leakage. Advanced lesions displayed choroidal hypofluorescence in areas of pigment proliferation and hyperfluorescence in areas of pigment atrophy. 3. In advanced cases, occlusive vascular changes over areas of acquired retinoschisis were observed. There was intraretinal leakage of the dye from deep capillaries and pooling of the dye in cystic cavities near the margin of the retinoschisis. 4. There was no perfusion of the choroid and retina in the area of the hole and in the retina surrounding it. This finding suggests choroidal and retinal ischemia in the pathogenesis of a retinal hole. 5. Fluorescein angiography of retinal tears revealed fluorescein leakage along the edge of the tear and absent perfusion of the retinal flap. The retinal and choroidal circulation-around the tear was otherwise unremarkable. The choroidal fluorescein underlying the retinal flap was not visible, perhaps because it was masked by the retinal flap. 6. Our fluorescein angiographic findings in cases of rhegmatogenous retinal detachment confirmed those of others [7, 8, 10]. Transit of fluorescein through the retinal circulation was sluggish. The retinal capillaries were dilated. 7. In cases of rhegmatogenous retinal detachment which had become reattached surgically, areas treated with diathermy or cryoapplications showed absent or diminished choroidal and retinal perfusion. Leakage of the fluorescein from capillaries in the optic disc and retina in the posterior pole was sometimes persistent several months postoperatively.

Recessive Familial Spastic Paraplegia with Retinal Degeneration

A family is described in which spastic paraplegia and retinal degeneration were observed in 5 out of 11 sibs in one generation. All affected members of this family had the onset of bilateral ocular symptoms during the fourth to fifth decade. Visual impairment was slowly progressive and ranged from 20/25 to 20/100.There was a macular and perimacular speckling with small, irregular, discrete spots and central pigment proliferation, at the level of the pigment epithelium. Fluorescein angiography revealed patches of proliferating retinal pigment epithelium blocking transmission of fluorescein, surrounded by a larger area of disruption of the retinal pigment epithelium giving a “window-effect” choroidal pattern. The right macula showed a nonspecific, nonfluorescent, reticular pattern.The most likely explanation of the ocular findings was a retinal degeneration involving the retinal pigment epithelium and/or the chorio-capillaries. Extensive laboratory work was negative.

Reduction of energy in photocoagulation with the Zeiss-Oberkochen Xenon-Are Apparatus. II. Application of short-pulse, small-spot technique to humans.

A short-pulse, small-spot xenon arc coagulation technique, worked out on rabbits as described in a preceding paper, has been applied to humans with good results. The lesions can be made to resemble in most respects to those generated by weak ruby laser pulses, insofar as the choroid and the nerve fiber layer are not damaged, resorption of edema is rapid and pigment proliferation abundant. The technique consists in superposing the cathode hot-spot of the xenon arc by its mirror image, using small field stops and electronically controlled exposure times of typically 0.1 sec. The apparatus used was the photocoagulator of Zeiss-Oberkochen equipped with the optical attachement which allows of working with the Goldmann contact glass. Detailed description of some cases treated illustrates this method.

Reduction of energy in photocoagulation with the Zeiss-Oberkochen Xenon-Are apparatus. I. Development of a short-pulse, small-spot technique on rabbit eyes.

Using our optical attachment to the photocoagulator of Zeiss-Oberkochen, enabling to work with the Goldmann 3 mirror contact glass, an effort has been made to reduce both pulse energy and damage to the choroid and nerve fiber layer. This has been found to be possible by electronic control of exposure time in combination with measurement of radiation dose. Small, not overdosed lesions, which in most respects resemble those generated by low-power pulsed lasers, can be obtained by superposing the cathode hot spot of the xenon arc with its mirror image, using small field stops and an exposure time of, typically, 0.1 sec. No modification of the electrical equipment of the coagulator is necessary. The lesions produced in this way on rabbits are characterized by a relatively rapid resorption of edema and abundant pigment proliferation. The choroid and nerve fiber layer are not affected.

A FUNDUS DYSTROPHY WITH UNUSUAL FEATURES (Late onset and dominant inheritance of a central retinal lesion showing oedema, haemorrhage and exudates developing into generalised choroidal atrophy with massive pigment proliferation)

A FUNDUS DYSTROPHY WITH UNUSUAL FEATURES (Late onset and dominant inheritance of a central retinal lesion showing oedema, haemorrhage and exudates developing into generalised choroidal atrophy with massive pigment proliferation)

ATYPICAL (UPWARD) COLOBOMA OF THE CHOROID

I have had the opportunity to observe the following case : H. H. M., a woman, aged 27, came to the outpatient department of Mercedes Hospital on Jan. 18, 1930, complaining that for the last few months she had noticed that the sight of her left eye was poor. On examination the fundus of this eye was normal. That of the right eye showed an upward coloboma of the choroid. This (fig. 1) extended from the upper margin of the disk to the periphery of the fundus above, forming a sector somewhat over 30 degrees ; it was pearl white, with pigment proliferation along its edges, especially below, where this encroached a little over the upper margin of the disk; a branched retinal vessel was to be seen coursing along its temporal margin, and at the lower inner angle some choroidal vessels were visible. There were no other retinal vessels in the