Importance ocularoculodermal oculodermal melanocytosis is a congenital periocular pigmentary condition that can lead to the development of uveal melanoma, estimated at 1 in 400 affected patients. Patients with the naevus of ota, ocular and oculodermal melanocytosis need to have their condition recognised and potential sequelae fully explained. April 2015 wills eye resident case series diagnosis. Some cases of dermal melanocytosis do not fit into any of these morphologic categories, however. It is characterized by melanocytic nevi in both the skin and the brain. Congenital ocular and oculodermal melanocytosis affect only about 0. An unusual presentation of congenital dermal melanocytosis fitting the rare diagnosis of dermal melanocyte hamartoma. Developed at philadelphias worldrenowned wills eye hospital, the color atlas and synopsis of clinical ophthalmology series covers the most clinically relevant aspects of ophthalmology in a highly visual, easytouse format.
Dermal melanocytosis rarely occurs in unusual regions or covers extensive areas of the body1. An unusual case of congenital dermal melanocytosis article pdf available in annals of dermatology 224. Slate grey nevus congenital dermal melanocytosis, mongolian spot is a benign, flat, congenital birthmark, with wavy borders and an irregular shape. Ocular or oculodermal melanocytosis is a condition characterized by hyperpigmentation of the episclera, uvea and skin, and is more common. Ocular melanocytosis is the strongest predisposing factor for uveal. Standardized echography in a case of congenital oculodermal.
A study to determine the prevalence rate of ocular melanocytosis. Ocular melanocytosis associated with intracranial melanoma article pdf available in british journal of ophthalmology 8011. Ocular and oculodermal melanocytosis associated with uveal. Pediatric ophthalmology 2nd edition pdf free download pdf. Ocular melanocytosis om constitutes 6% of all patients diagnosed within the spectrum of oculodermal melanocytosis odm, a congenital melanocytic hyperpigmentation of the mucosa. Ocular oculodermal melanocytosis is a congenital condition characterised by dermal pigmentation along the first and second divisions of the trigeminal nerve such as the ocular coats and periocular skin. While it may be regarded as a sporadic, congenital disease, only in about half. Ocular melanosis om is a congenital disease of the eye which affects about 1 in every 5000 people and is a risk factor for uveal melanoma. Ocular melanosis om is a congenital disease of the eye which affects about 1 in every 5000. Atypical types of dermal melanocytosis reported in the literature include dermal melanocyte hamartoma and congenital segmental dermal melanocytosis2,3. Uveal malignant melanoma associated with ocular and oculodermal melanocytosis. Pediatric ophthalmology 2nd edition pdf free download general overview. Dermal melanocytosis rarely occurs in unusual regions or covers extensive areas of the body 1. Intraocular pigmentation is also increased, which contributes to a higher incidence of glaucoma and increases the risk of malignant melanoma.
Yag lasers have been used on three cases of ectopic mongolian spots and one case of persistent mongolian spot, with efficacious results after one treatment session and with minimal adverse events. A congenital eye disorder featuring diffuse hyperplasia of melanocytes causing darkening of the iris of the affected eye especially at the pupil margin, brownish discolouration of the episclera, darkening of the fundus, and an increased risk of developing melanomas and glaucoma. Ocular melanocytosis melanosis oculi is a unilateral, congenital, pigmentary lesion that is a form of a blue nevus. Congenital ocular melanocytosis is an abnormality characterized by diffuse brown melanocytic hyperplasia in the sclera and uvea. Glaucoma at young age is an unusual presentation of congenital ocular melanocytosis. This condition has been labeled with several names including melanosis oculi, nevus of ota, and oculocutaneous melanosis. Neurocutaneous melanosis genetic and rare diseases. Asymptorr, tic neurocutaneous melanocytosis in patients with large congenital melanocytic nevi. Ocular melanosis is present at birth but clinical signs usually do not appear until the dog is older. Nov 06, 2016 neurocutaneous melanosis ncm is a rare, noninherited condition of the central nervous system. Ocular melanocytosis american academy of ophthalmology.
Ocular melanocytosis and secondary glaucoma in a child. Twothirds of people with ncm have giant congenital melanocytic nevi, and the remaining onethird have numerous lesions but no giant lesions. The incidence of malignant degeneration in a hyperpigmented eye is. Melanocytosis article about melanocytosis by the free. Association of ocular and oculodermal melanocytosis with the rate of uveal melanoma metastasis. Ocular melanosis in dogs symptoms, causes, diagnosis. Nevus of ota is a blue dubious discuss hyperpigmentation that occurs on the face, most often appearing on the white of the eye. Mar 22, 2019 intense pulsed light is also an effective method for the treatment of aberrant mongolian spots.
Choroidal melanoma in a 14yearold patient with ocular. This condition is usually unilateral and classically involves the eyelids, sclera, uvea, orbit, and, less commonly, sites including the meninges, palate, and tympanic membrane. Bilateral ocular melanocytosis with malignant melanoma of the. Large plaques on a baby boy ocularoculodermal melanocytosis. Ocular melanosis in dogs is a congenital inherited condition which causes melanocytes to block the drainage of aqueous humor. Congenital choroidal melanocytoma in a child with ocular. Neurocutaneous melanocytosis is a rare syndrome defined by the proliferation of melanocytes in the central nervous system brain and spinal cord and the presence of a congenital melanocytic naevus 1,2. The naevus of ota swann 2010 clinical and experimental. Large plaques on a baby boy ocular oculodermal melanocytosis. Melanocytic glaucoma in a child associated with ocular. A retrospective descriptive case series study of 11 patients with similar appearing broadbased but entirely flat melanotic choroidal lesions was carried out. Association of ocular and oculodermal melanocytosis with the.
Twothirds of people with ncm have giant congenital melanocytic nevi, and the remaining one. The hypothesis that ocular melanocytosis is a precancerous condition that may lead to a choroidal melanoma should be seriously questioned for the following reasons. Oculodermal melanocytosis is defined as a congenital pigmentation of the periocular skin, episclera, uveal tract, and sometimes the orbit, meninges and hard palate. Other names, ocular melanocytosis or melanosis oculi. Atypical types of dermal melanocytosis reported in the literature include dermal melanocyte hamartoma and congenital segmental dermal melanocytosis 2, 3.
Importance ocular oculodermal oculodermal melanocytosis is a congenital periocular pigmentary condition that can lead to the development of uveal melanoma, estimated at 1 in 400 affected patients. A 30yearold woman with a giant congenital melanocytic nevus covering nearly the entire right thoracodorsal region and multiple disseminated melanocytic nevi presented with. The relationship of congenital ocular melanocytosis and neurofibromatosis to uveal. A few cases of unusual congenital dermal melanocytosis have been documented table 1. Aural, buccal, nasal and ocular mucosal involvement are common. Oct 01, 2000 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 crosssectional prevalence survey. Metastasis from uveal melanoma associated with congenital ocular melanocytosis. Pdf prevalence of ocular and oculodermal melanocytosis in. A crosssectional study was undertaken to determine the prevalence of ocular and oculodermal melanocytosis. A congenital pigmentary lesion, ocular melanocytosis melanosis oculi is characterized by unilateral patchy but extensive slategray or bluish discoloration of the sclera not conjunctiva. Management of primary acquired melanosis, nevus, and. In dogs is found almost exclusively in the cairn terrier, where until recently it was known as pigmentary glaucoma. Mar 21, 2006 the objective was to describe a distinct, limited form of congenital ocular melanocytosis that involves the choroid only.
It is possible that patients with bilateral ocular melanocytosis have a greater chance of developing of uveal malignant melanoma than do those with unilateral involvement. Congenital dermal melanocytosis mongolian spot treatment. The nevus is located in the deep episclera, sclera and uveal tract and can manifest clinically as iris heterochromia, patchy slategray or bluish discoloration of the sclera, and increased pigmentation of the ipsilateral fundus. Full text get a printable copy pdf file of the complete article 1. We report a young patient with a unilateral splinter disc hemorrhage, mild asymmetric optic nerve cupping, and increased intraocular pressures, suggestive of glaucoma, associated with bilateral ocular. All 11 lesions were homogeneously dark brown in color with at least one striated margin. Pdf an unusual case of congenital dermal melanocytosis. A nineyearold girl presented for evaluation of hyperpigmentation of her left eye panel a.
Neurocutaneous melanosis ncm is a rare, noninherited condition of the central nervous system. Oculodermal melanocytosis is a benign congenital anomalous pigmentation of deep facial skin and ocular tissues. The majority of cases are associated with a giant congenital melanocytic naevus and satellite lesions. Congenital ocular melanocytosis com is a congenital melanocytic hyperpigmentation of the ocular tissues and is associated with an increased risk of development of uveal melanoma.
Congenital dermal melanocytosis mongolian spot clinical. Giant congenital melanocytic nevi and neurocutaneous melanosis. In this study, patients with melanocytosis who developed uveal melanoma were found to have double the risk for metastasis compared with those. A congenital pigmentary lesion, ocular melanocytosis melanosis oculi is characterized by unilateral patchy but extensive slategray or bluish discoloration of. We reported a case of malignant melanoma and acquired dermal melanocytosis that appeared on congenital nevus spilus. It also occurs on the forehead, nose, cheek, periorbital region, and temple. Episcleral melanoma as a complication of ocular melanocytosis. We report a young patient with a unilateral splinter disc hemorrhage, mild asymmetric optic nerve cupping, and increased intraocular pressures, suggestive of glaucoma, associated with bilateral ocular melanocytosis. Scleral melanocytosis and oculodermal melanocytosis nevus of. The peak prevalence was at 6 years of age, when 44.
The topic congenital dermal melanocytosis you are seeking is a synonym, or alternative name, or is closely related to the medical condition mongolian spot. Asymptomatic neurocutaneous melanocytosis in patients with large congenital melanocytic nevi. Mar 22, 2019 an unusual presentation of congenital dermal melanocytosis fitting the rare diagnosis of dermal melanocyte hamartoma. Melanocytosis definition of melanocytosis by medical dictionary. T1 episcleral melanoma as a complication of ocular melanocytosis. Prevalence of ocular and oculodermal melanocytosis in spanish. Association of ocular and oculodermal melanocytosis with. Thirteen thousand one hundred fifty patients were examined for ocular and oculodermal melanocytosis. Pdf ocular melanocytosis associated with intracranial. Metastasis from uveal melanoma associated with congenital.
In this study, patients with melanocytosis who developed uveal melanoma were found to have double the risk for metastasis compared with those without melanocytosis. Malignant melanoma and acquired dermal melanocytosis on. Ocular melanocytosis, although rare in white races, is associated with somehow increased risk of uveal malignant melanoma. Association of ocular and oculodermal melanocytosis with the rate. Case report choroidal melanoma in ocular melanocytosis. In 1883, it was described and named after mongolians by erwin balz, a german anthropologist based in japan, who erroneously believed it to be most prevalent among his mongolian patients. The presence of increased numbers of melanocytes in these affected tissues may provide. Ocular melanocytosis om, a congenital condition first described by the japanese dermatologist ota in 1939, 1 is characterised by hyperpigmentation of the episclera and uvea 2, 3 due to an increased number of fusiform dendritic melanocytes. Ocular melanocytosis and secondary glaucoma in a child glaucoma at young age is an unusual presentation of congenital ocular melanocytosis. In darkskinned races, the disease entity is comparatively more common but its association for developing uveal melanomas is extremely rare. More recently, novel picosecond 755nm alexandrite and 1064nm nd.
A major risk factor that contributes to neurocutaneous melanocytosis formation is the presence of congenital melanocytic nevi, which is a large pigmented birthmark in the head and neck region it is important to note that having a risk factor does not mean that one will get the condition. The authors present a unique case of bilateral isolated choroidal melanocytosis in an annular pattern that, to our knowledge, has not previously been described. A mongolian spot is a benign condition where there is increased pigmentation of skin affecting the lower back and buttocks. In the same year, burkhart and gohara 2 described an 18monthold male with diffuse, bilateral grayblue pigmentation on his buttocks that extended in a. The major medical concern with giant congenital melanocytic nevi cmn is high risk of developing cutaneous melanoma, leptomeningeal melanoma, and neurocutaneous melanocytosis. Risk factors include fair skin, lightcoloured eyes, congenital ocular melanocytosis, ocular melanocytoma and the bap1tumour predisposition syndrome. We report a case of congenital ocular melanocytosis, later on complicated by. Ocular melanocytosis definition of ocular melanocytosis by. Intense pulsed light is also an effective method for the treatment of aberrant mongolian spots. Congenital episcleral melanocytosis can occur as a solitary lesion but, in a clinical setting, it is more often seen as a component of ocular melanocytosis or oculodermal melanocytosis nevus of ota 1,2,3,4,5,6,7,8. Nov 05, 2010 a few cases of unusual congenital dermal melanocytosis have been documented table 1. Ocular melanocytosis om, a congenital condition first described by the japanese dermatologist ota in 1939, is characterised by. The incidence of malignant degeneration in a hyperpigmented eye is unknown and overreported.