Naevus of Ota: clinical characteristics and proposal for a new ocular classification and grading system.

INTRODUCTION: Naevus of Ota is a congenital condition that may involve the skin, eyeball and even intracranial structures usually in the distribution of the ophthalmic and maxillary divisions of the trigeminal cranial nerve. The purpose of this study was to summarise our experience with the ocular clinical presentation, imaging, outcome, treatment of complications and to offer a new classification of patients with naevus of Ota. METHODS: We retrospectively reviewed the patients' medical records and the following parameters were retrieved and analysed: demographics, clinical presentation complications and treatment of complications. Imaging characteristics of patients with naevus of Ota were compared with images from the same period of time of 57 age-matched and gender-matched patients without naevus of Ota (control group). RESULTS: The series was composed of 40 patients (18 males, 22 females) whose mean age at diagnosis was 35.27 years (range 0.5-77 years). Thirty-three patients (82.5%) were type I naevus of Ota according to the Tanino classification, three patients (7.5%) were type II, one patient (2.5%) was type III and three patient (7.5%) were type IV (bilateral naevus of Ota). We further classified all cases in according to the ocular involvement extent. Three patients developed malignant transformation to choroidal melanoma and four patients developed glaucoma. CONCLUSIONS: In this study, a new clinical classification based on the involved ocular component and extent of the involvement (in quadrants) of the globe is suggested first. Further studies are needed to assess whether our clinical ocular classification can assist in identifying patients at risk for developing glaucoma and malignant transformation.

An extensive nevus of Ota with oro-buccal involvement: a case review and proposal of a new classification system.

Nevus of Ota (NO) also known as nevus fuscoceruleus ophthalmomaxillaris or oculodermal melanocytosis is a rare hamartoma. It is usually characterized by unilateral, patchy and speckled, blue/gray or brown macules on the forehead and periocular area. Here, we report a rare case of NO with oro-buccal involvement, which is an unusual condition. The patient was presented due to the rarity of oro-buccal involvement, extensive and remarkable expression of the lesions, and in order to propose a more comprehensive and neuroanatomy based classification system.

A retrospective study on laser treatment of nevus of Ota in Chinese children--a seven-year follow-up.

OBJECTIVE: To retrospectively study laser treatment of nevus of Ota in children. METHODS: Clinically analyzing characteristics, effects, side effects, and recurrence of a 104 cases of nevus of Ota in children under 12 years, including 32 boys and 72 girls. RESULTS: After seven treatments, cure rate of lesion color fading and area reducing were 79.81% and 75.96%, respectively. After 10 treatments, both of the two cure rates were 96.15%. Later the cure rate was constant with even more treatments. The younger the first treating age, the lesser the treatments are. The younger the age of onset, the higher the relapse after clearance. CONCLUSION: Nevus of Ota should be treated as early as possible to reach better efficacy with less treatments. The younger the onset age, the easier it recurs.

A new classification of nevus of Ota.

BACKGROUND: The nevus of Ota, is a common benign pigmentary dermatosis, mainly involve innervation area of first and second branch of trigeminal nerve. The classification of nevus of Ota was proposed by Tanino, based on 26 cases of nevus of Ota from 1937 to 1940. Studies about its classification are rarely seen in last 70 years, while it is still practical today. METHODS: Based on the clinical photographs, 1079 consecutive patients with nevus of Ota were verified and reclassified according to the innervation areas of the trigeminal nerve branches. RESULTS: In these 1079 cases, 866 patients were in line with Tanino's classification (80.26%), and 213 patients were not (19.74%). We put forward a new clinical classification (Peking Union Medical College Hospital classification, PUMCH classification) of nevus of Ota based on the innervation area of the trigeminal nerve branches, composed of 5 types and 14 subtypes. The 5 types were as follows: Type I - pigmentation maculeses involving the innervation area of one of the three trigeminal nerve branches, of which there were 424 cases (39.3%), comprising 6 subtypes; Type II - pigmentation macules involving the innervation area of two branches of the three trigeminal nerve branches, of which there were 221 cases (20.48%), comprising 4 subtypes; Type III - pigmentation macules involving the innervation area of all three trigeminal nerve branches, of which there were 361 cases (33.45%), comprising 2 subtypes; Type IV - bilateral type, in which the pigmentation macules involves the bilateral cheek, of which there were 63 cases (5.84%), comprising 2 subtypes; and Type V - complications occurred in the patient, of which there were 10 cases (0.93%). CONCLUSION: The new classification of nevus of Ota is based on the innervation area of the trigeminal nerve branches, and it covers all types of Tanino's classifications; on that basis, some new types and subtypes are brought in and cover almost every clinical condition.

Nevus of Ota: clinical-ophthalmological findings / Nevo de Ota: achados clínicos e oftalmológicos

OBJECTIVE: To analyze the clinical and ophthalmological findings of patients with nevus of Ota. METHODS: Retrospective analysis of patients' charts with nevus of Ota. We registered the demographic data, location of the nevus and date of appearance, family history of similar spots, biomicroscopic, gonioscopic, tonometric, ophthalmoscopic and perimetric findings. RESULTS: We included 14 patients, six (43.0 percent) men and eight (57.0 percent) women, with a mean age of 21.7±17.5 years. Ten (71 percent) were mulatto, three (21.4 percent) white and one (7.1 percent) black. Twelve (85.7 percent) patients presented the spots at birth and two in puberty. Nine patients presented conjunctival and episcleral pigmentation in the right eye and five in the left eye. According to Tanino's classification, five (35.7 percent) nevi were class 1, eight (57.1 percent) class 2 and one (7.1 percent) class 3. Heterochromia iridis was found in eight (57.1 percent) patients. Anisocoria was present in three (21.4 percent) patients. Five (35.7 percent) patients presented a suspected glaucomatous cup disc ratio (≥0.7); six (42.9 percent) presented a cup disc ratio ≤ 0.5 and three (21.4 percent), no cup disc. We found two curious and remarkable findings: a nevus of Ota on the palate of one patient and other on the optic disc associated with a pigmentary mottling of the fundus in another patient. The pigmentary mottling of the fundus was also seen in four more eyes. CONCLUSIONS: The nevus of Ota was frequently present at birth, in mulattos, and classified as Tanino's class 1 and 2. Heterochromia iridis was a common finding. Anisocoria was present in a small percentage of eyes. No patient developed glaucoma or malignancy.

OBJETIVO: Analisar os achados clínico-oftalmológicos de portadores de nevo de Ota. MÉTODOS: Análise retrospectiva dos prontuários de pacientes com nevo de Ota. Foram registrados dados demográficos, localização do nevo e época do seu aparecimento, história familiar de manchas similares, olho acometido, achados ectoscópicos (classificação de Tanino), biomicroscópicos, fundoscópicos e campimétricos. RESULTADOS: Foram incluídos 14 pacientes, seis (47,0 por cento) homens e oito (53,0 por cento) mulheres com média de idade de 21,7±17,5 anos. Dez (71 por cento) pacientes eram feodérmicos, três (21,4 por cento) leucodérmicos e um (7,1 por cento) melanodérmico. Doze (85,7 por cento) pacientes apresentaram manchas ao nascimento e dois relataram seu surgimento após o nascimento. Três pacientes relataram manchas compatíveis com nevo de Ota em consanguíneos. A melanose conjuntivo-episcleral foi reconhecida no olho direito em cinco pacientes e em nove no olho esquerdo. Pela classificação de Tanino, cinco (35,7 por cento) dos nevos eram da classe 1, oito (57,1 por cento) da classe 2 e um (7,1 por cento) da classe 3. Presença de heterocromia da íris em oito (57,2 por cento) pacientes. Anisocoria ocorreu em três pacientes. Disco óptico suspeito de glaucoma (relação escavação/disco ≥ 0,7) foi observado em cinco pacientes (35.7 por cento); seis apresentaram escavação fisiológica e três não apresentavam escavação. Dois achados curiosos e raros: um nevo de Ota no palato de um paciente e outro no disco óptico de outro paciente associado com pigmentação difusa no polo posterior retiniano. Essa pigmentação foi também vista em mais quatro olhos. CONCLUSÃO: O nevo de Ota foi mais comumente presente ao nascimento, em feodérmicos e nas classes 1 e 2 de Tanino. Heterocromia iriana achado comum. Anisocoria foi diagnosticada num percentual pequeno de pacientes. Nenhum paciente desenvolveu glaucoma nem tumor maligno.

Report of rare palatal expression of Nevus of Ota with amendment of Tanino's classification.

Nevus of Ota, a benign melanocytic pigmentary disorder with rare malignant transformation potential, affects 0.014-0.034% of the Asian and Black population and has a strong predilection for females. It occurs in the area innervated by the first and second division of the trigeminal nerve. Oral manifestation is rarely noted with only 14 cases reported till date. This report documents a case of Nevus of Ota with the infrequently noted oral involvement, in an Indian lady. Since oral manifestation is not acknowledged in Tanino's classification, the authors propose a modification to the same.

Nevus of Ota: a series of 15 cases.

BACKGROUND: The nevus of Ota is a dermal nevus characterized by bluish pigmentation in the distribution of the first and the second division of the trigeminal nerve. AIM: Our aim was to study the cutaneous and extracutaneous manifestations of the nevus of Ota. METHODS: A total of 15 cases were included in our study. A detailed history, clinical examination along with direct ophthalmoscopy and otoscopy were done for all the cases. RESULTS: Most of the patients (60%) had lesions at birth and the majority (86.7 %) were females. Five (33.3%) patients belonged to Tanino class II. Combined dermal and ocular involvement was observed in 60% of the cases. CONCLUSION: Tanino class II was the most common type observed in our studies. A few rarer associations such as nevus of Ito and hemangioma were also noted in our patients.

Pigmentary disorders in India.

This article addresses pigmentary disorders relevant to India. Many of these disorders are easily spotted because of the Indians' darker complexion. The authors examine hypopigmentary and hyperpigmentary disorders, defining the main characteristics of each and their relevance to the people of India, including social as well as physical ramifications. The authors propose the treatments available to Indians exhibiting these skin disorders.

Pigmentary disorders in the South East.

In this article, new information is introduced regarding vitiligo and melasma based on clinical studies of Korean patients and specific pigmentary disorders that occur in Asians. These disorders can be psychologically distressing because of their visible nature. They are especially resistant to various kinds of conventional treatments and tend to have a chronic progression that makes patients doubt the results and the prognosis.

Nevus of Ota: a new classification based on the response to laser treatment.

BACKGROUND AND OBJECTIVE: For 60 years, Tanino's classification has been used to classify the extent of nevus of Ota. However, such classification not only fails to address variants such as phacomatosis pigmentovascularis but also cannot be used to predict the therapeutic outcome. Our objective is to retrospectively study our series of laser-treated patients with the aim of re-classifying nevus of Ota, so that such important issues can be taken into account. STUDY DESIGN/MATERIALS AND METHODS: One hundred nineteen patients that had received Q-switched laser treatment were recruited into the study. They were recalled for interview and examination for evidence of coexisting birthmarks and extracutaneous involvement. Two observers assessed the pre- and posttreatment clinical photographs for evidence of periorbital under-response (panda's sign), defined as the degree of periorbital laser clearing significantly less than clearing in the other area. RESULTS: A total of 47.8% of the patients with periorbital pigmentation were considered by the observers to have significant periorbital under-response (panda's sign). Additionally, 10.1% had other birthmarks, and extracutaneous involvement was seen in 31.4% of the patients. CONCLUSION: Periorbital under-response is commonly seen in patients with periorbital pigmentation. Taking this and other factors into consideration, we have proposed a new classification for nevus of Ota that allows for the prediction of the clinical outcome of laser treatment.

Asociación de síndromes de Sturge-Weber-Dimitri y Klippel-Trénaunay con nevo de Ota bilateral: una variante de la facomatosis pigmentovascular / An association of Stuge-Weber-Dimitri and Klippel-Trenaunay syndromes with bilateral Ota's naevus: a variant of pigmentovascular phakomatosis

Presentamos un caso de asociación de los síndromes de Sturge-Weber-Dimitri y Klippel-Trénaunay con nevo de Ota bilateral. Además de destacar la infrecuencia de dicha asociación y encuadrarla dentro de las facomatosis pigmentovasculares, se pondera la ubicación nosológica de los nevus flammeus como malformación vascular y se hace una revisión de la clasificación de nevo de Ota

Asociación de síndromes de Sturge-Weber-Dimitri y Klippel-Trénaunay con nevo de Ota bilateral: una variante de la facomatosis pigmentovascular / An association of Stuge-Weber-Dimitri and Klippel-Trenaunay syndromes with bilateral Otas naevus: a variant of pigmentovascular phakomatosis

Presentamos un caso de asociación de los síndromes de Sturge-Weber-Dimitri y Klippel-Trénaunay con nevo de Ota bilateral. Además de destacar la infrecuencia de dicha asociación y encuadrarla dentro de las facomatosis pigmentovasculares, se pondera la ubicación nosológica de los nevus flammeus como malformación vascular y se hace una revisión de la clasificación de nevo de Ota (AU)

[Nevus of Ota associated with chronic glaucoma]. / Naevus de Ota associé à un glaucome chronique.

Oculodermal melanocytosis (nevus of Ota) is an abnormally large accumulation of pigment in ocular tissues as well as the skin in the distribution of the trigeminal nerve. Elevated intraocular pressure is a serious complication of this disease, as well as transformation to malignant melanoma. We report a case of nevus of Ota, documented clinically and histopathologically, associated with severe chronic glaucoma.

Nevo de Ota bilateral / Bilateral Nevus of Ota

Se estudia un paciente de sexo femenino, de 29 años de edad, con nevo de Ota bilateral, sin complicaciones al examen oftalmológico y leve hipoacusia de conducción derecha. Radiografía, tomografía y electroencefalograma normal. Se pasa revista a la clasificación y se lo considera de tipo difuso con pigmento en dermis papilar y reticular. En el cuadro N§1 se puntualizan las diferencias clínicas con el melasma, nevo zigomático y el nevo de Ota-"like". Se enfatiza en la necesidad de educar al paciente para su examen periódico y controlar la aparición de complicaciones, recordando que puede sufrir una transformación maligna, principalmente en piel, coroides, iris, órbita y cerebro

Nevo de Ota bilateral / Bilateral Nevus of Ota

Se estudia un paciente de sexo femenino, de 29 años de edad, con nevo de Ota bilateral, sin complicaciones al examen oftalmológico y leve hipoacusia de conducción derecha. Radiografía, tomografía y electroencefalograma normal. Se pasa revista a la clasificación y se lo considera de tipo difuso con pigmento en dermis papilar y reticular. En el cuadro Nº1 se puntualizan las diferencias clínicas con el melasma, nevo zigomático y el nevo de Ota-"like". Se enfatiza en la necesidad de educar al paciente para su examen periódico y controlar la aparición de complicaciones, recordando que puede sufrir una transformación maligna, principalmente en piel, coroides, iris, órbita y cerebro (AU)

A new classification of Ota's nevus based on histopathological features.

According to the distribution of the dermal melanocytes, Ota's nevus was classified into 5 types: superficial (type S), superficial dominant (type SD), diffuse (type Di), deep dominant (type DD), and deep (type De). The histological types were, as expected, relatively well-correlated with the color of the nevus: the most brownish lesions represented type S or type SD, while the most bluish lesions showed types Di, DD or De. Furthermore these types were strongly correlated with the location of the nevus: types S and SD were more frequent on cheeks, whereas types Di, DD and De more frequently occurred on eyelid, temple and forehead. When combining both colorations and locations of the nevus, our classification appeared much more significant. Our new histological classification of Ota's nevus may be very useful in making a therapeutic prognosis of the disease.