An update on ophthalmological perspectives in oculodermal melanocytosis (Nevus of Ota).

PURPOSE: To provide a review of the literature on oculodermal melanocytosis (ODM) with a focus on the diagnostic and therapeutic implications of multimodal imaging techniques in the management of ophthalmic complications. METHODS: The authors carried out a literature search on PubMed, Medline, and Scopus of English language articles published on ODM through August 2021. This review presents traditional and novel diagnostic methods in the diagnosis and follow-up of patients with particular emphasis on addressing the role of imaging in the management of the ophthalmic complications of the condition towards improving current practice patterns. RESULTS: ODM is a rare, prevalently unilateral, congenital condition that presents with brown or blue/gray flat asymptomatic lesions of the skin, mucosae, episclera/sclera, and uvea localized within the territory of distribution of the ophthalmic and mandibular branches of the trigeminal nerve. Glaucoma and predisposition to uveal melanoma are the main ophthalmic complications. Diagnosis and management are through comprehensive opthalmological examination and traditional imaging methods such as ultrasonography and fluorescein/indocyanine green angiography as pigmentation of the fundus can conceal subtle retinal and choroidal alterations. Anterior segment optical coherence tomography and ultrasound biomicroscopy are used to evaluate the anterior segment and the ciliary body in the presence of glaucoma or melanoma of the anterior uveal tract. Fundus autofluorescence and retinal pigment epithelium (RPE) alterations are of aid in the differential diagnosis between choroidal nevi and melanoma. Enhanced depth imaging spectral domain optical coherence tomography offers outstanding in vivo evaluation of the dimensions and details of tumors or nevi and surrounding choroidal tissues and small choroidal melanomas may show distortions of the retinal and sub-retinal profile, presence of intra and sub-retinal fluid, abnormalities of the RPE, and compression of the choriocapillaris. CONCLUSIONS: Novel multimodal imaging techniques are significant in the diagnosis and management of the ophthalmic complications of ODM. Fundus autofluorescence and enhanced depth spectral domain optical coherence tomography have adjunctive value in the detection of early-stage melanoma and differential diagnosis between nevi and melanoma. Awareness of current and emerging imaging techniques can propagate improved standardized definition and assessment of the complications of ODM.

[Treatment of nevus of Ota and Ito and epidermal nevus syndrome]. / Behandlung des Naevus Ota und Ito und des Pigmentnävussyndroms.

Nevus of Ota, nevus of Ito and nevus of Hori are special melanocytic nevi that have a slate-brown or blue/grey coloring. They are pigmented disorders characterized by its heterotopic melanocytic dermal location and by blue/brown unilateral and sometimes bilateral facial patch in case of nevus of Ota, and in the supraclavicular, scapular, and deltoid region in case of nevus of Ito. It is more common in patients with Asian and dark-skinned ethnic backgrounds. Histologically, elongated, dendritic melanocytes are seen scattered mainly throughout the upper third of reticular dermis. An acquired variant is called Hori's nevus with more bilateral facial distribution, similar to melasma. Dermal melanocytosis can also occur elsewhere on the body, including inside the mouth. Despite its benign nature, patients frequently seek therapy because of its facial involvement. QS lasers are used effectively to treat these lesions. The number of treatment sessions correlates with clinical improvement. Post laser hypo- and hyperpigmentation are common side effects mainly affecting patients with darker skin.

[Ocular melanocytosis and delayed psycho-intellectual development--other association?]. / Melanocitoza oculara congenitala si întârziere în dezvolarea psihointelectuala--o alta asociere?

Congenital ocular melanocytosis (COM) is an extremely rare melanocytic hyperplasia (0.038% in Caucasian population); it affects episclera and sclera. The conjunctival epithelium is not affected, as opposed to conjunctival melanosis. The pigmentation is grayish-blue and its consistency with the underlying tissues as the conjunctiva moves (deep pigmentation) is an essential diagnostic clue. COM may be isolated or associated with facial pigmentation, in the area innervated by the trigeminal nerve--known as oculodermal melanocytosis or nevus of Ota. COM may associate the following findings: iris hypercromia, iris mammillations, fundus hypercromia, uveal melanoma (most frequent choroidal melanoma) and glaucoma (10%). Melanocytes have been found in the brain, orbit, bucal mucosa in COM.

Nevus of Ota - an intraoral presentation: a case report.

BACKGROUND: Nevus of Ota or "oculodermal melanocytosis" is a rare congenital hamartoma of dermal melanocytes causing a blue-gray hyperpigmentation of the eye and surrounding structures. The condition, originally described by Ota and Tanino in 1939, mainly affects the ophthalmic and maxillary divisions of the trigeminal nerve. We describe the first reported case of unilateral oculodermal melanocytosis in a Caucasian woman with oral buccal mucosal involvement. Oral involvement of nevus of Ota is very rare. CASE PRESENTATION: A 48-year-old Caucasian woman was referred by the dermatology division to the oral medicine department at the University of Liverpool School of Dentistry with new-onset oral pigmentation to the left buccal mucosa. The patient had a previous diagnosis of oculodermal nevus. CONCLUSION: An incisional biopsy of the left buccal mucosa was completed. The report stated that histological and immunohistochemical features were in keeping with a blue nevus, but within the context of the preexisting occulodermal pigmentation, a diagnosis of oculodermal melanocytosis, also known as "nevus of Ota," was made. The patient will be kept under review in the oral medicine department because the progression of the lesion on the left buccal mucosa requires active monitoring owing to the potential for malignant change. The patient also requires regular review in the dermatology and ophthalmology divisions.

Mongolian spots with involvement of mandibular area.

A 1-year-old boy had grayish pigmentation on the left side of his face over the area supplied by the mandibular branch of the trigeminal nerve. Upon further examination, the lesion was also found on the left side of the neck, shoulder, upper arm, right lower back and buttock. The pigmentation was uniform in intensity. This case report demonstrates that Mongolian spots can occur on the face in the area supplied by the mandibular branch of the trigeminal nerve. These spots should not be misdiagnosed as nevus of Ota.

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.

Oculodermal melanocytosis--Nevus of Ota (with palatal pigmentation).

Nevus of Ota is a benign dermal melanocytosis, that involves facial skin as a macular discoloration, appearing as blotchy blue to blue gray. It involves the upper portion of the face, especially over the periorbital area. It also involves ocular structures such as episclera, sclera, conjunctiva, cornea, retina, uveal tract and also nasopharynx, auricular mucosa, tymphanic membrane and dura. Glaucoma and malignant melanoma are the two known ocular complications in patients with Nevus of Ota. 12-18% of these patients exhibits palatal pigmentation. Here we are reporting two cases of Oculodermal melanocytosis, one of which exhibited palatal pigmentation.

Microsurgical treatment of nevus of Ota.

A new micropeeling technique for treating nevus of Ota has been performed on 32 patients over a 3-year period. This technique was conducted by including unique implementations: instrumentally by using special one-by-one-toothed microforceps, and technically by incorporating an accurate system of incising and removing evenly spaced skin sections under a microscope. As a result, precise removal at the desired skin depth is obtained, with healing generally superior to that yielded by conventional techniques.