Nevus of Ota with midface tumors and a vanishing orbital mass.

Nevus of Ota, also known as oculodermal melanocytosis, is a benign melanocytic lesion that develops along the distribution of the V1 and V2 branches of the trigeminal nerve. Prior reports have described the typical imaging and clinical features of nevus of Ota. We present a rare case of a 31 year-old female with midface tumors and presumed hemorrhage into an orbital lesion in the setting of nevus of Ota resulting in acute loss of vision.

In vivo reflectance confocal microscopy for evaluating common facial hyperpigmentation.

BACKGROUND: In clinical settings, atypical facial hyperpigmentation such as nevus of Ota, acquired bilateral nevus of Ota-like macules (ABNOM), melasma, and café-au-lait spots are often missed and misdiagnosed. Summarizing in vivo reflectance confocal microscopy (RCM) features of the hyperpigmentation is helpful in the diagnosis of ambiguous lesions. METHODS: We recruited 196 patients referred for unequivocal facial hyperpigmentation, including 55 patients with nevus of Ota, 45 patients with ABNOM, 62 patients with melasma, and 34 patients with café-au-lait spots. The RCM images were evaluated at the epidermis, the dermis-epidermis junction (DEJ), and the upper papillary dermis from both hyperpigmented patches and normal skin. RESULTS: In the superficial and middle dermis, 41 of 55 patients (74.5%) with nevus of Ota were characterized by a cord-like or lumpy structure between the collagen fibers. And there was no melanin deposition detected in the dermis in 14 of 55 (25.5%) patients. In ABNOM, 37 of 45 (82.2%) patients were characterized by a cord-like or lumpy structure in the superficial dermis and 8 of 45 patients (17.8%) was no melanin deposition detected in the dermis. The epidermis was no difference between nevus of Ota, ABNOM, and the normal skin. Melasma was detected increased cobblestone pattern in the epidermis of all patients, branching architecture in 21 of 62 patients (33.9%), and focally aggregated round to triangular cells in the upper dermis of 18 of 62 patients (29.0%). In all patients with afé-au-lait spots, increased cobblestone pattern in the epidermis and regular and increased density of ringed pattern in the DEJ were visualized. CONCLUSIONS: Our findings indicate that RCM may be useful in the auxiliary diagnosis of nevus of Ota, ABNOM, melasma, and café-au-lait spots.

Ultrasound biomicroscopy of the ciliary body in ocular/oculodermal melanocytosis.

PURPOSE: To describe the ultrasound biomicroscopy (UBM) findings of the ciliary body in patients with ocular/oculodermal melanocytosis. DESIGN: Retrospective observational case series study. METHODS: A retrospective chart and imaging database review was conducted for patients with unilateral ocular/oculodermal melanocytosis who underwent UBM examination at the Ocular Oncology Clinic of Princess Margaret Hospital. Radial images of the ciliary body at the 3-, 6-, 9-, and 12-o'clock positions were obtained in both eyes. UBM characteristics included ciliary body thickness and reflectivity. The eye with ocular/oculodermal melanocytosis was compared with the contralateral unaffected eye as a control. Statistical significance was analyzed with Student t test. RESULTS: Twelve patients were included. All patients showed unilateral diffuse pigmentation involving episclera and anterior chamber angle. The iris showed diffuse pigmentation in 10 cases and sectorial in 2. Mean ciliary body thickness of the affected eyes was 0.581 ± 0.058 mm (range 0.489-0.744) compared with 0.475 ± 0.048 mm (range 0.406-0.622) in the contralateral eye, which was found to be a statistically significant difference (P < .001). The affected ciliary body showed hyperreflectivity when compared with the unaffected eye. All affected eyes were graded as medium to high reflectivity compared with the unaffected eyes that showed a medium to medium/low reflectivity. CONCLUSION: Ciliary body involvement in ocular/oculodermal melanocytosis presents as increased thickness and higher ultrasound reflectivity on UBM when compared with the unaffected eye. UBM is helpful in imaging clinically undetectable areas of melanocytosis involving the ciliary body.

Nonchoroidal intraorbital malignant melanoma arising from naevus of Ota.

A case report of nonchoroidal intraorbital malignant melanoma arising from naevus of Ota, in a Caucasian female.

Iris melanoma arising in iris nevus in oculo(dermal) melanocytosis.

A 50-year-old white man with oculo(dermal) melanocytosis and longstanding iris nevus was found to have growth of the iris mass. Excision and histopathologic examination revealed a mixed cell type malignant melanoma. Benign nevus cells were present at the periphery of the tumor surrounding the entire melanoma. White patients with oculo(dermal) melanocytosis have a predisposition to uveal melanoma, which is usually choroidal in origin. Literature review showed only three confirmed cases of iris melanoma in this setting. Two additional cases initially published as spindle A melanoma have been reclassified as iris nevi based on the modified Callender classification of uveal melanomas. It is recommended that patients with oculo(dermal) melanocytosis be followed for the occurrence of uveal melanoma.

Meningeal melanocytoma of the brain and oculodermal melanocytosis (nevus of Ota): case report and literature review.

BACKGROUND: Meningeal melanocytomas are rare benign pigmented tumors of meningeal melanocytes. Nevus of Ota is a melanocytic pigmentation of the sclera and the skin adjacent to the eye. We report a rare case of parietal convexity meningeal melanocytoma associated with ipsilateral mucocutaneous melanosis and review the literature. METHODS: A 17-year-old man presented with headache and blindness. There was left scalp and facial mucocutaneous melanosis. During surgery, speckled hyperpigmentation was seen in all layers of the scalp, bone, dura, and even ependyma. Pathologic examination of the tumor and scalp consisted of light microscopy and immunohistochemistry. In a literature review, the demographics (age, sex), clinical characteristics (site of lesion, duration of symptoms), therapeutic options (surgical excision, radiotherapy), and prognosis (recurrence, death, death related to the disease, and follow-up) were analyzed. For statistical analysis we used the independent sample t-test and chi(2) tests. RESULTS: Ninety-five cases (45 intracranial and 50 spinal) of meningeal melanocytoma have been reported. The median age of patients with intracranial lesions (40 years) was significantly lower than that of patients with spinal lesions (49 years) (p = 0.016). Of the 95 patients, 57.9% were female. In the nervous system, melanocytes are more common in the infratentorial and cervical areas, but more than half of the tumors have been located in the supratentorial region and the thoracic spine. In 46 months median follow-up, tumor recurrence rate and tumor related death rate were 26.3% and 10.5%, respectively. These were not statistically significant for different therapeutic modalities. There was a trend toward shorter survival for patients who underwent subtotal tumor resection without radiotherapy compared with other groups. CONCLUSION: The prognostic criteria, differential diagnosis, and the embryological aspects of meningeal melanocytoma are discussed with a review of the related literature.

A new neurocutaneous syndrome possibly related to Ota's nevus.

The case of a 42-year-old Sudanese man is reported who had developed hyperpigmentation of the left face and rapidly progressive right hemiparesis followed by myoclonus at the age of 30. Blue-grey hyperpigmentation was noted in the territory of the left ophthalmic, maxillary and mandibular trigeminal nerve branches, with alopecia of the right face and axilla. CT of the brain revealed left-sided paraventricular calcifications and MRI showed atrophy of the left cerebral hemisphere and mesencephalon, a calcified haemangioma in the left basal ganglia, an old infarct in the right central pons, and cerebellar atrophy. This case resembles Ota's syndrome; however, atypical distribution of the facial nevus, the epidermal location of hyperpigmentation, and the combination of vascular and degenerative cerebral malformations indicate that this is a new hitherto unreported neurocutaneous vascular syndrome.