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Jerry A. Shields - One of the best experts on this subject based on the ideXlab platform.
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Choroidal Melanocytosis Evaluation with Enhanced Depth Imaging Optical Coherence Tomography
Ophthalmology, 2013Co-Authors: Marco Pellegrini, Carol L. Shields, Sruthi Arepalli, Jerry A. ShieldsAbstract:Objective To assess eyes with unilateral choroidal Melanocytosis using enhanced depth imaging (EDI) optical coherence tomography (OCT). Design Retrospective, observational case series. Participants Fifteen patients with unilateral choroidal Melanocytosis. Methods Each patient was evaluated with EDI-OCT, and comparison was made of involved versus uninvolved foveal retinal and choroidal layers. Main Outcome Measures Enhanced depth imaging OCT features of choroidal Melanocytosis. Results The mean patient age was 28 years (median, 23 years; range, 5–76 years). There were 9 male and 6 female patients. Best-corrected visual acuity ranged from 20/20 to 20/30. All patients had subfoveal involvement with choroidal Melanocytosis. Associated features included dermal (n = 6), scleral (n = 9), iris (n = 3), and palate (n = 1) Melanocytosis. Spectral domain EDI-OCT revealed normal inner retina (n = 15) and normal outer retina (n = 14). The only retinal abnormality was a defect in both the myoid zone and the ellipsoid junction (n = 1). The affected choroid showed smooth anterior contour (n = 15) and thinned or compressed choriocapillaris (n = 2), thinned (n = 3) or thickened (n = 3) medium vessels, and thinned large vessels (n = 2). The subfoveal choroid was a mean 23% thicker in the involved eye, with a mean thickness of 326.4 μm (median, 326 μm; range, 120–459 μm) compared with 264.4 μm (median, 260 μm; range, 94–462 μm) in the uninvolved eye ( P = 0.15). The choroidal perivascular interstitial tissue was 51% thicker in the study eye, enwrapping and silhouetting the medium and large vessels ( P = 0.01). The ratio between the perivascular stromal tissue and the subfoveal choroidal thickness was 66% in the study eye and 54% in the normal eye ( P = 0.0001). Deep partial (n = 5) or complete (n = 2) tissue shadowing was noted. Conclusions Enhanced depth imaging OCT revealed that choroidal Melanocytosis shows increased subfoveal choroidal thickness with an apparent increase in the choroidal perivascular stromal tissue and minimal effect on the overlying retina.
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Metastasis from Uveal Melanoma Associated with Congenital Ocular Melanocytosis: A Matched Study
Ophthalmology, 2013Co-Authors: Arman Mashayekhi, Carol L. Shields, Swathi Kaliki, Brianna Walker, Chantel Park, Neelema Sinha, Felina Zolotarev Kremer, Jerry A. ShieldsAbstract:Purpose To determine the rate of metastasis resulting from uveal melanoma associated with congenital ocular Melanocytosis (COM) and to compare it with the rate of metastasis resulting from uveal melanoma not associated with COM. Design Matched retrospective study. Participants Fifty-seven patients with uveal melanoma associated with ocular Melanocytosis (Melanocytosis group). Each patient in the Melanocytosis group was matched with 2 patients with uveal melanoma not associated with ocular Melanocytosis (no Melanocytosis group) for age, gender, location of anterior tumor margin, location of tumor epicenter, tumor basal diameter, and tumor thickness. Methods Review of medical records. Main Outcome Measures Metastasis resulting from uveal melanoma. Results The mean basal diameter and thickness of tumors in the Melanocytosis group were 10 and 4.9 mm, respectively, and most were located between the macula and equator (70%). The Melanocytosis and no Melanocytosis groups were similar with regard to all patient, ocular, and tumor features studied. Nineteen (33%) of 57 patients in the Melanocytosis group and 18 (16%) of 114 patients in the no Melanocytosis group demonstrated systemic metastasis during mean follow-up periods of 77 months (range, 1–402) and 64 months (range, 1–252), respectively ( P = 0.013). The Kaplan-Meier estimates for systemic metastasis in the Melanocytosis group at 5 and 15 years were 27% and 59%, respectively, compared with 15% and 33%, respectively, in the no Melanocytosis group (hazard ratio, 1.99; 95% confidence interval, 1.16–3.41). Conclusions In this matched study, patients with uveal melanoma associated with COM were twice as likely to have systemic metastasis compared with patients with uveal melanoma not associated with COM. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.
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unusual multifocal pigmented lesions of the uvea in a patient with ocular Melanocytosis
Retinal Cases & Brief Reports, 2013Co-Authors: David Reichstein, Jerry A. Shields, Uyen Tran, Carol L. ShieldsAbstract:PURPOSE To report findings of unusual multifocal pigmented lesions in a single patient with sector uveal Melanocytosis. METHODS Retrospective case report of an otherwise healthy 64-year-old man with unusual uveal pigmentation. Complete ocular examination, including slit-lamp biomicroscopy, ophthalmoscopy, optical coherence tomography, and ultrasound biomicroscopy, was performed. RESULTS Slit-lamp examination disclosed inferior sector pigmentation of the iris. Ophthalmoscopy showed multiple discrete, deeply pigmented, inferior choroidal lesions, and ultrasound biomicroscopy confirmed a small inferior ciliary body mass. The findings were consistent with sector uveal Melanocytosis, believed to be the same as a diffuse melanocytoma. CONCLUSION Melanocytoma is typically a unilateral, unifocal lesion that occurs on the optic disc but can be located anywhere in the uveal tract. This is a case of unusual pigmented uveal lesions, likely representing a variant of sector uveal Melanocytosis or multiple diffuse uveal melanocytomas.
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Sector (partial) oculo(dermal) Melanocytosis in 89 eyes.
Ophthalmology, 2011Co-Authors: Carol L. Shields, Arman Mashayekhi, Chantel Park, Neelema Sinha, Anam Qureshi, Felina Zolotarev, Jerry A. ShieldsAbstract:Purpose To describe sector (partial) involvement of the uvea with Melanocytosis. Design Noninterventional, retrospective case series. Participants A total of 89 eyes of 86 patients. Methods Review of medical records, color photographs, and ultrasound images. Main Outcome Measures Clinical features and relationship with uveal melanoma. Results Approximately all patients were Caucasian (n = 83, 97%), and sector Melanocytosis involved the right (n = 41, 46%) or left (n = 48, 54%) eye. The involved tissue included iris (n = 58, 65%), choroid (n = 48, 54%), and both iris and choroid (n = 17, 19%). The Melanocytosis affected a mean of 6 clock hours of iris and 5 clock hours of choroid. Related Melanocytosis involved the sclera (n = 39, 44%), eyelid (n = 4, 4%), temple (n = 4, 4%), scalp (n = 1, 1%), and palate (n = 1, 1%). Uveal melanoma was found at presentation in 7 patients (8%) and was multifocal in 2 of these patients. A comparison of eyes with versus without melanoma revealed clinically significant factors (odds ratio [OR] > 2) of male gender (71% vs. 43% [OR 3.36]); cutaneous/palate Melanocytosis (14% vs. 7% [OR 2.11]); scleral Melanocytosis heaviest in superior, temporal, or nasal quadrants (57% vs. 29% [OR 2.41, confidence interval, 2.24–3.92]); and any degree of choroidal Melanocytosis (86% vs. 70% [OR 2.63]), particularly diffuse choroidal Melanocytosis (29% vs. 16% [OR 3.85]). None of these factors reached statistical significance in this small cohort. Over a mean follow-up of 6 years, there was no metastatic event. Conclusions Ocular Melanocytosis can be sectoral (partial), affecting only a mean of 5 to 6 clock hours of the uvea and can manifest melanoma within the Melanocytosis region. There were no specific features of Melanocytosis statistically related to the presence of melanoma. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.
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phacomatosis pigmentovascularis of cesioflammea type in 7 patients combination of ocular pigmentation Melanocytosis or melanosis and nevus flammeus with risk for melanoma
Archives of Ophthalmology, 2011Co-Authors: Carol L. Shields, Brad E Kligman, Mayerling Suriano, Juan C Iturralde, Margaret V Shields, Vanessa Viloria, Jerry A. ShieldsAbstract:Objective To describe the features of phacomatosis pigmentovascularis (cesioflammea type). Design Noninterventional retrospective case series composed of 7 patients. Results Nevus flammeus combined with ipsilateral ocular Melanocytosis or melanosis was seen in all 7 patients. Additional contralateral nevus flammeus was observed in 3 patients. Nevus flammeus (unilateral in 4 patients and bilateral in 3 patients) was distributed in trigeminal nerves V1 (n = 3), V2 (n = 7), and V3 (n = 5). Related findings included diffuse choroidal hemangioma (n = 1) and glaucoma (n = 1), with no patients having brain hemangioma or seizures. Ocular pigmentary abnormalities (unilateral in all 7 patients) included congenital ocular Melanocytosis (n = 6) and conjunctival acquired melanosis (n = 1). Pigmentation was sectorial (partial) in 5 patients and complete in 2 patients. Melanocytosis involved the periocular skin in 1 patient, sclera in 2 patients, iris in 2 patients, and choroid in 4 patients. In 3 of 6 patients, Melanocytosis was visible in the choroid only on dilated fundus evaluation. Related tumors included choroidal melanoma (n = 3), optic disc melanocytoma (n = 1), and conjunctival melanoma in situ (primary acquired melanosis) (n = 1). Melanoma metastasis developed in 1 patient. Conclusions Phacomatosis pigmentovascularis shows features of nevus flammeus and more serious ocular pigmentary abnormalities (uveoscleral Melanocytosis and conjunctival melanosis). Melanocytosis may be detected only by dilated ocular fundus examination, as found in 3 of 6 patients. Furthermore, choroidal melanoma can develop from Melanocytosis, as noted in 3 of our 6 patients (50%). All patients with nevus flammeus should be examined for phacomatosis pigmentovascularis by an ophthalmologist because ocular Melanocytosis and uveal melanoma may remain hidden within the eye.
Carol L. Shields - One of the best experts on this subject based on the ideXlab platform.
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Choroidal Melanocytosis Evaluation with Enhanced Depth Imaging Optical Coherence Tomography
Ophthalmology, 2013Co-Authors: Marco Pellegrini, Carol L. Shields, Sruthi Arepalli, Jerry A. ShieldsAbstract:Objective To assess eyes with unilateral choroidal Melanocytosis using enhanced depth imaging (EDI) optical coherence tomography (OCT). Design Retrospective, observational case series. Participants Fifteen patients with unilateral choroidal Melanocytosis. Methods Each patient was evaluated with EDI-OCT, and comparison was made of involved versus uninvolved foveal retinal and choroidal layers. Main Outcome Measures Enhanced depth imaging OCT features of choroidal Melanocytosis. Results The mean patient age was 28 years (median, 23 years; range, 5–76 years). There were 9 male and 6 female patients. Best-corrected visual acuity ranged from 20/20 to 20/30. All patients had subfoveal involvement with choroidal Melanocytosis. Associated features included dermal (n = 6), scleral (n = 9), iris (n = 3), and palate (n = 1) Melanocytosis. Spectral domain EDI-OCT revealed normal inner retina (n = 15) and normal outer retina (n = 14). The only retinal abnormality was a defect in both the myoid zone and the ellipsoid junction (n = 1). The affected choroid showed smooth anterior contour (n = 15) and thinned or compressed choriocapillaris (n = 2), thinned (n = 3) or thickened (n = 3) medium vessels, and thinned large vessels (n = 2). The subfoveal choroid was a mean 23% thicker in the involved eye, with a mean thickness of 326.4 μm (median, 326 μm; range, 120–459 μm) compared with 264.4 μm (median, 260 μm; range, 94–462 μm) in the uninvolved eye ( P = 0.15). The choroidal perivascular interstitial tissue was 51% thicker in the study eye, enwrapping and silhouetting the medium and large vessels ( P = 0.01). The ratio between the perivascular stromal tissue and the subfoveal choroidal thickness was 66% in the study eye and 54% in the normal eye ( P = 0.0001). Deep partial (n = 5) or complete (n = 2) tissue shadowing was noted. Conclusions Enhanced depth imaging OCT revealed that choroidal Melanocytosis shows increased subfoveal choroidal thickness with an apparent increase in the choroidal perivascular stromal tissue and minimal effect on the overlying retina.
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Association of ocular and oculodermal Melanocytosis with the rate of uveal melanoma metastasis: analysis of 7872 consecutive eyes.
JAMA ophthalmology, 2013Co-Authors: Carol L. Shields, Swathi Kaliki, Michael Livesey, Brianna Walker, Robert Garoon, Marissa Bucci, Eric Feinstein, Aline Pesch, Cristina Gonzalez, Sara E. LallyAbstract:Importance Ocular/oculodermal (oculo[dermal]) Melanocytosis is a congenital periocular pigmentary condition that can lead to the development of uveal melanoma, estimated at 1 in 400 affected patients. In this study, patients with Melanocytosis who developed uveal melanoma were found to have double the risk for metastasis compared with those without Melanocytosis. Objective To determine the relationship of oculo(dermal) Melanocytosis to the prognosis of patients with uveal melanoma. Design, Setting, and Patients Retrospective chart review of 7872 patients with uveal melanoma treated at the Ocular Oncology Service, Wills Eye Institute, from August 25, 1970, through August 27, 2008. Exposures Enucleation, plaque radiotherapy, local resection, or thermotherapy. Main Outcomes and Measures Metastasis and death. Results Of 7872 patients with uveal melanoma, oculo(dermal) Melanocytosis was present in 230 (3%). The Melanocytosis involved the sclera (92%), iris (17%), choroid (12%), eyelid (8%), and temporal fossa (1%). Eyes with melanoma and oculo(dermal) Melanocytosis had a relative risk for metastasis 1.6 times greater compared with those with no Melanocytosis ( P P P = .02), and 1.9 times higher with scleral Melanocytosis ( P P P = .001) and the presence of subretinal fluid ( P = .05), and the only factor predictive of death was increased tumor thickness ( P = .009). Conclusions and Relevance Patients with uveal melanoma associated with oculo(dermal) Melanocytosis have double the risk for metastasis compared with those with no Melanocytosis. All patients with oculo(dermal) Melanocytosis should undergo ophthalmic examination and imaging on a twice-yearly basis because this could help with the early detection of melanoma.
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Metastasis from Uveal Melanoma Associated with Congenital Ocular Melanocytosis: A Matched Study
Ophthalmology, 2013Co-Authors: Arman Mashayekhi, Carol L. Shields, Swathi Kaliki, Brianna Walker, Chantel Park, Neelema Sinha, Felina Zolotarev Kremer, Jerry A. ShieldsAbstract:Purpose To determine the rate of metastasis resulting from uveal melanoma associated with congenital ocular Melanocytosis (COM) and to compare it with the rate of metastasis resulting from uveal melanoma not associated with COM. Design Matched retrospective study. Participants Fifty-seven patients with uveal melanoma associated with ocular Melanocytosis (Melanocytosis group). Each patient in the Melanocytosis group was matched with 2 patients with uveal melanoma not associated with ocular Melanocytosis (no Melanocytosis group) for age, gender, location of anterior tumor margin, location of tumor epicenter, tumor basal diameter, and tumor thickness. Methods Review of medical records. Main Outcome Measures Metastasis resulting from uveal melanoma. Results The mean basal diameter and thickness of tumors in the Melanocytosis group were 10 and 4.9 mm, respectively, and most were located between the macula and equator (70%). The Melanocytosis and no Melanocytosis groups were similar with regard to all patient, ocular, and tumor features studied. Nineteen (33%) of 57 patients in the Melanocytosis group and 18 (16%) of 114 patients in the no Melanocytosis group demonstrated systemic metastasis during mean follow-up periods of 77 months (range, 1–402) and 64 months (range, 1–252), respectively ( P = 0.013). The Kaplan-Meier estimates for systemic metastasis in the Melanocytosis group at 5 and 15 years were 27% and 59%, respectively, compared with 15% and 33%, respectively, in the no Melanocytosis group (hazard ratio, 1.99; 95% confidence interval, 1.16–3.41). Conclusions In this matched study, patients with uveal melanoma associated with COM were twice as likely to have systemic metastasis compared with patients with uveal melanoma not associated with COM. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.
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unusual multifocal pigmented lesions of the uvea in a patient with ocular Melanocytosis
Retinal Cases & Brief Reports, 2013Co-Authors: David Reichstein, Jerry A. Shields, Uyen Tran, Carol L. ShieldsAbstract:PURPOSE To report findings of unusual multifocal pigmented lesions in a single patient with sector uveal Melanocytosis. METHODS Retrospective case report of an otherwise healthy 64-year-old man with unusual uveal pigmentation. Complete ocular examination, including slit-lamp biomicroscopy, ophthalmoscopy, optical coherence tomography, and ultrasound biomicroscopy, was performed. RESULTS Slit-lamp examination disclosed inferior sector pigmentation of the iris. Ophthalmoscopy showed multiple discrete, deeply pigmented, inferior choroidal lesions, and ultrasound biomicroscopy confirmed a small inferior ciliary body mass. The findings were consistent with sector uveal Melanocytosis, believed to be the same as a diffuse melanocytoma. CONCLUSION Melanocytoma is typically a unilateral, unifocal lesion that occurs on the optic disc but can be located anywhere in the uveal tract. This is a case of unusual pigmented uveal lesions, likely representing a variant of sector uveal Melanocytosis or multiple diffuse uveal melanocytomas.
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Sector (partial) oculo(dermal) Melanocytosis in 89 eyes.
Ophthalmology, 2011Co-Authors: Carol L. Shields, Arman Mashayekhi, Chantel Park, Neelema Sinha, Anam Qureshi, Felina Zolotarev, Jerry A. ShieldsAbstract:Purpose To describe sector (partial) involvement of the uvea with Melanocytosis. Design Noninterventional, retrospective case series. Participants A total of 89 eyes of 86 patients. Methods Review of medical records, color photographs, and ultrasound images. Main Outcome Measures Clinical features and relationship with uveal melanoma. Results Approximately all patients were Caucasian (n = 83, 97%), and sector Melanocytosis involved the right (n = 41, 46%) or left (n = 48, 54%) eye. The involved tissue included iris (n = 58, 65%), choroid (n = 48, 54%), and both iris and choroid (n = 17, 19%). The Melanocytosis affected a mean of 6 clock hours of iris and 5 clock hours of choroid. Related Melanocytosis involved the sclera (n = 39, 44%), eyelid (n = 4, 4%), temple (n = 4, 4%), scalp (n = 1, 1%), and palate (n = 1, 1%). Uveal melanoma was found at presentation in 7 patients (8%) and was multifocal in 2 of these patients. A comparison of eyes with versus without melanoma revealed clinically significant factors (odds ratio [OR] > 2) of male gender (71% vs. 43% [OR 3.36]); cutaneous/palate Melanocytosis (14% vs. 7% [OR 2.11]); scleral Melanocytosis heaviest in superior, temporal, or nasal quadrants (57% vs. 29% [OR 2.41, confidence interval, 2.24–3.92]); and any degree of choroidal Melanocytosis (86% vs. 70% [OR 2.63]), particularly diffuse choroidal Melanocytosis (29% vs. 16% [OR 3.85]). None of these factors reached statistical significance in this small cohort. Over a mean follow-up of 6 years, there was no metastatic event. Conclusions Ocular Melanocytosis can be sectoral (partial), affecting only a mean of 5 to 6 clock hours of the uvea and can manifest melanoma within the Melanocytosis region. There were no specific features of Melanocytosis statistically related to the presence of melanoma. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.
Thomas Krausz - One of the best experts on this subject based on the ideXlab platform.
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pagetoid Melanocytosis when is it significant
Pathology, 2004Co-Authors: Vesna Petronicrosic, Christopher R Shea, Thomas KrauszAbstract:Summary Pagetoid Melanocytosis refers to the presence of solitary and small groups of melanocytes in the superficial layers of the epidermis. Although it is generally considered to be a diagnostic hallmark of melanoma, it may also be seen in certain melanocytic naevi. Attempts to formulate reliable histological criteria for distinction between benign and malignant pagetoid Melanocytosis have been only partly successful. Extensive and diffuse pagetoid spread of melanocytes, especially when it extends laterally beyond the underlying junctional component, combined with marked cytological atypia, favours melanoma (Fig. 1). In naevi, pagetoid spread tends to be more limited and there is no marked cytological atypia. Since these criteria are applicable in only a subset of cases, the correct diagnosis of melanocytic lesions must be based not only on the features of pagetoid Melanocytosis but also on all histological and clinical findings. The pathogenesis of pagetoid Melanocytosis is, at best, hypothetical and presumes an active infiltrative process in the setting of malignancy. However, novel data support the contention of a passive drift of melanocytes carried upwards by proliferating keratinocytes under particular circumstances, such as trauma or UV exposure. In this review, we evaluate the histological features of pagetoid Melanocytosis in the light of current experimental data, reflecting on the pathogenesis of this intriguing phenomenon.
Vesna Petronicrosic - One of the best experts on this subject based on the ideXlab platform.
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pagetoid Melanocytosis when is it significant
Pathology, 2004Co-Authors: Vesna Petronicrosic, Christopher R Shea, Thomas KrauszAbstract:Summary Pagetoid Melanocytosis refers to the presence of solitary and small groups of melanocytes in the superficial layers of the epidermis. Although it is generally considered to be a diagnostic hallmark of melanoma, it may also be seen in certain melanocytic naevi. Attempts to formulate reliable histological criteria for distinction between benign and malignant pagetoid Melanocytosis have been only partly successful. Extensive and diffuse pagetoid spread of melanocytes, especially when it extends laterally beyond the underlying junctional component, combined with marked cytological atypia, favours melanoma (Fig. 1). In naevi, pagetoid spread tends to be more limited and there is no marked cytological atypia. Since these criteria are applicable in only a subset of cases, the correct diagnosis of melanocytic lesions must be based not only on the features of pagetoid Melanocytosis but also on all histological and clinical findings. The pathogenesis of pagetoid Melanocytosis is, at best, hypothetical and presumes an active infiltrative process in the setting of malignancy. However, novel data support the contention of a passive drift of melanocytes carried upwards by proliferating keratinocytes under particular circumstances, such as trauma or UV exposure. In this review, we evaluate the histological features of pagetoid Melanocytosis in the light of current experimental data, reflecting on the pathogenesis of this intriguing phenomenon.
Reginald S Sauve - One of the best experts on this subject based on the ideXlab platform.
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scleral Melanocytosis and oculodermal Melanocytosis nevus of ota in chinese children
The Journal of Pediatrics, 2000Co-Authors: Alexander K C Leung, Matthew C K Choi, Reginald S SauveAbstract:Abstract Two thousand nine hundred fourteen Chinese children (1510 males and 1404 females) were examined for the presence of scleral Melanocytosis (SM) and oculodermal Melanocytosis in a cross-sectional prevalence survey. SM was found in 4.9% of boys and 4.1% of girls under the age of 1 year. The peak prevalence was at 6 years of age, when 44.6% of boys and 46.6% of girls were affected. At 18 years of age, only 11.1% of boys and 13.2% of girls had SM. The overall prevalence, regardless of age, was 27.6% in boys and 27.1% in girls. The condition was bilateral in 78% of cases. The medial superior quadrant was the most frequently affected site, and the lateral inferior quadrant was the least frequently affected site. Oculodermal Melanocytosis occurred only in one patient; the pigmentation affected the left side of the face and the ipsilateral sclera. (J Pediatr 2000;137:581-4)
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Scleral Melanocytosis and oculodermal Melanocytosis (nevus of Ota) in Chinese children
The Journal of pediatrics, 2000Co-Authors: Alexander K C Leung, Matthew C K Choi, C. Pion Kao, Helen Y.h. Cho, May P.m. Siu, Reginald S SauveAbstract:Two thousand nine hundred fourteen Chinese children (1510 males and 1404 females) were examined for the presence of scleral Melanocytosis (SM) and oculodermal Melanocytosis in a cross-sectional prevalence survey. SM was found in 4.9% of boys and 4.1% of girls under the age of 1 year. The peak prevalence was at 6 years of age, when 44.6% of boys and 46.6% of girls were affected. At 18 years of age, only 11.1% of boys and 13.2% of girls had SM. The overall prevalence, regardless of age, was 27.6% in boys and 27. 1% in girls. The condition was bilateral in 78% of cases. The medial superior quadrant was the most frequently affected site, and the lateral inferior quadrant was the least frequently affected site. Oculodermal Melanocytosis occurred only in one patient; the pigmentation affected the left side of the face and the ipsilateral sclera.