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Year : 1991  |  Volume : 39  |  Issue : 3  |  Page : 122-124

The additive influence of nevus flammeus and the nevus of Ota on congenital glaucoma

Dr. Rajendra Prasad Centre for Ophthalmic Sciences All Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Ramanjit Sihota
Dr. Rajendra Prasad Centre for Ophthalmic Sciences All Institute of Medical Sciences, New Delhi-110 029
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Source of Support: None, Conflict of Interest: None

PMID: 1841885

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Nevus flammeus and nevus of Ota are commonly encountered dermatological conditions, but both individually, are only occasionally associated with glaucoma. The occurrence of isolated nevus flammeus along with nevus of Ota in the same patient is also extremely rare. We are presenting two unusual cases, both having a nevus of Ota, together with nevus flammeus and severe congenital glaucoma.

How to cite this article:
Sihota R, Kumar H, Sood N N. The additive influence of nevus flammeus and the nevus of Ota on congenital glaucoma. Indian J Ophthalmol 1991;39:122-4

How to cite this URL:
Sihota R, Kumar H, Sood N N. The additive influence of nevus flammeus and the nevus of Ota on congenital glaucoma. Indian J Ophthalmol [serial online] 1991 [cited 2022 Dec 7];39:122-4. Available from: https://www.ijo.in/text.asp?1991/39/3/122/24455

  Introduction Top

Nevus flammeus and nevus of Ota are dermatological conditions frequently seen by ophthalmologists. Oculodermal melanocytosis or nevus of Ota is not generally associated with glaucoma, neither is the variant known as ocular melanocytosis [1],[2] Nevus flammeus when associated with neurological abnor­malities such as meningeal angiomatosis, cerebral calcification, epilepsy, mental retardation or paresis constitutes the Sturge Weber syndrome and is fre­quently associated with glaucoma [3].However, there are only occasional case reports of an isolated nevus flammeus causing an ipsilateral glaucoma [4],[5]

The occurrence of an isolated nevus flammeus along with a nevus of Ota in the same patient is extremely rare [5],[6] but we encountered two such cases in which an isolated nevus flammeus was associated with ipsilateral ocular melanocytosis. Both patients had a recalcitrant glaucoma.

  Report of cases Top

Case 1

A 10-year old female (B.P) came to us with a history of watering OS since birth. The `black' of the eye was also noted to be enlarged at birth. The patient had undergone a goniotomy at the age of one month and a trabeculectomy at 6 months of age in the same eye. She was using Pilocarpine 2% drops t.i.d. in the affected eye since the last operation, with applanation pressures around 25 ± 3 mm. Hg. Timolol maleate 0.5% b.d was added about a year back, with little additional benefit. General physical condition of the child was found to be essentially normal, except 'for diffuse reddish patches on the skin over the left side of the face, left lids, neck, chest, arm and forearm, which were diagnosed as nevus flammeus [Figure - 1][Figure - 2]. The patches had irregular margins and varied in size from 5 x 5 mm, to much large confluent patches, which blanched on pressure. Ocular examination revealed a best cor­rected visual acuity of 6/6 O.D. and 6/36 O.S., the left eye being 7 dioptres myopic. The corneal diameters were 11.75 mm and 14.5 mm in the vertical meridians and 12.5 mm and 16 mm in the horizontal meridians, in the right and the left eyes respectively. Examination of the right eye showed that it was essentially normal. The left eye had an enlarged though otherwise normal cornea, a deep anterior chamber and a markedly hyperpigmented iris. The sclera had areas of blackish pigm-3niation, which on slit lamp examination were seen as cendritic melanocytes y closely bunched together iii various areas [Figure - 3]. The cup disc ratio was 0.8:1, the cup being deep with a total loss of the temporal neuroretinal rim. Applanation tonometry revealed an IOP of 17.0 mm O.D. and 40 mm O.S. The scleral rigidity was 0.033 and 0.034, and the C-value was 0.18 and 0.10 uI/min/mm. Hg in the right and left eyes respectively. On gonioscopy, an open angle with marked hyperpigmentation, 360, was noted in the left eye.

Radiological investigations, including a CT scan, failed to reveal any other angiomata either in the viscera or in the leptomeninges. The patient also did not have any history of seizures. A 360 o argon laser trabeculoplasty failed to control the IOP, and a repeat trabeculectomy in the inferior quadrant was performed. The patient was discharged with an applanation tension of 20 mm. Hg O.S.

Histopathology of the scleral tissue revealed dendritic uveal melanocytes in abundance, with surrounding normal tissue.

Case 2

A 2-year old female (PR) was brought to us, because her parents noticed an enlargement and discoloration of the black of the right eye. The patient had no symptoms related to the central nervous system and the family history was noncontributory. The general physical examination was essentially normal, but for dermatological manifestations. The child's face had a bilateral reddish macular pigmentation [Figure - 4]. There were a few small, 2 x 5 mm, isolated patches, while the rest was a confluent patch with irregular margins, blanching on pressure. The abdomen and chest were diffusely covered with similar lesions. Ocular ex­amination revealed a visual acuity of 6/18 in both the eyes. The corneal diameters were 14.0 mm and 11.0 mm in the vertical meridian and 14.5 mm and 12.00 mm in the horizqntal meridian in the right and the left eyes respectively.

The sclera of the right eye showed a slate grey pigmentation in patches which was resolved under the high magnification of a slit lamp to be dendritic melanocytes similar to one seen in Case 1. Gonios­copy of the right eye revealed high insertion of the iris root, a heavily pigmented trabecular meshwork with evidence of abnormal tissue glistening in the angle. The iris was totally hyperpigmented and so was the fundus in the right eye. The cup:disc ratio was 0.7:1 in the right and 0.4:1 in the left eye. The applanation IOP was 30 mm. Hg O.D. and 17 mm O.S. A detailed neurological check-up, including CT scans failed to reveal any other abnormality. The histology of the sclera revealed branching uveal melanocytes. The post-trabeculectomy tensions were within normal limits.

  Discussion Top

The presence of ocular melanocytosis and nevus flammeus in the same patient is extremely unusual. Individually these conditions are not generally as­sociated with glaucoma, but both our cases had a combination of these congenital anomalies and had a severe glaucoma. In Case 2, the right eye had both a nevus flammeus and ocular melanosis with obvious, advanced glaucoma, while the left eye was only affected by nevus flammeus and had a cup disc ratio of 0.4:1, without angle anomalies or raised intraocular pressure. This brings home to us the fact that, the presence of two otherwise mild predisposing factors for glaucoma when occurring together, can give rise to a higher incidence of severe glaucoma.

Nevus flammeus, refers to a pink or deep burgundy, macular vascular malformation that is often distributed along the course of the peripheral nerve, or is systematised. These may be a cutaneous manifes­tation of other syndromes, in which the viscera as well as the skin are involved, like the Sturge Weber and Klippel-Trenaunay Weber syndromes. A review of ocular findings in nevus flammeus showed that, of the 26 patients in which the nevus. flammeus showed that, of the 26 patients in which the nevus involved the first and/or second divisions of the trigeminal nerve, 4 patients had glaucoma and another 8 were glaucoma suspects. One patient with glaucoma had CNS involvement and would fit more appropriately into a Sturge Weber syndrome. Thus only 11.5% of isolated nevus flammeus cases had glaucoma. This glaucoma had been thought to be due to a faulty development of the angle or due to increased episcleral venous pressure [4].

The nevus of Ota is a distinctive, congenital, benign, pigmented lesion, which usually occurs unilaterally, affecting particularly the skin of the periorbital region, in the area supplied by the 1st and 2nd, and rarely 3rd divisions of the trigeminal nerve, sometimes the pharynx and ear and frequently, in addition the conjunctiva, sclera, iris, choroid and optic disc[8]. The dermal component of the nevus of Ota, also called oculodermal melanocytosis was thought to be es­sential to the diagnosis of this lesion. However, it is now felt that ocular melanosis (ocular pigmentation without dermal involvement) and nevus of Ota are clinically and histop 2 athologically identical, differing only in their extent . The occurrence of glaucoma is rare with the nevus of Ota and when it does occur, it is usually associated with inflammation or Tumour [1][5][9][10]. It has been suggested that a melanocytic infiltration of the outflow channels could be the cause of glaucoma in such cases[11]sub, although the heavy pigmentation is present in the tissues of the trabecular meshwork and not free in the inter­trabecular spaces[9]. In a gonioscopic review of 6 cases of nevus of Ota, five were found to be having heavy trabecular pigmentation, though none had glaucoma[12]. Thus it seems that heavy pigmentation alone could not compromise the outflow enough to cause a glaucoma.

Our patients had a nevus flammeus and a nevus of Ota with trabecular hyperpigmentation in the eye affected, but there were no associated hemangioma or prominent bulbar vessels. Case 2 also showed evidence of an anomalous development of the angle in the glaucomatous eye. The contralateral eye, though affected by the nevus flammeus had no ocular melanosis and no glaucoma. It is unusual enough to see two such cases, but both having a recalcitrant glaucoma in only the eye having the double nevus made us think of possible reasons for this phenomenon.

An anomalous vascular development early in embryonic life, at about the 11 mm stage, could result in congenital vascular anomalies of both the eyes and the face. This is also the time that the angle structures develop. It is known that abnor­malities in neural crest cell migration have a role in the pathogenesis of congenital glaucomas, with or without changes in the gonioscopic appearance of the anterior chamber angle. The neural crest cells also influence the migration of pigment cells, both ocular and non-ocular [13],[14]. The neural crest cells could possibly be further disturbed by an overlying vascular malformation, like a nevus flammeus. This would explain the presence of angle anomalies and the nevus of Ota in our cases of nevus flammeus. Similar anomalies of the angle leading to congenital galucoma have also been reported with another overlying vascular abnormality, cutis marmorata [15]. Our patients showed a nevus flammeus, ocular melanosis, trabecular hyperpigmentation and angle anomalies, in the eyes involved, emphasising that one anomaly alone may cause mild/no glaucoma but the presence of both anomalies, as in our patients, caused a recalcitrant glaucoma.

Thus the occurrence of a nevus of Ota with a nevus flammeus in the same patient, should alert the physician to the possibility of the existence of a severe outflow obstruction and glaucoma. Timely detection of this would enable adequate steps to prevent visual loss and other complications.

  References Top

Foulks GN, Shields MB. Glaucoma in oculodermal melanocytosis. Annals Ophthalmol 1977; 9:1299-1304.  Back to cited text no. 1
Palmer DD, Perry MO. Melanosis oculi - a variant of nevus of Ota. Arch Dermatol 1962; 85:740-42.  Back to cited text no. 2
Duke Elder S. System of Ophthalmology. Vol. 3, Part 2. London: Kimpton, 1964: 3(2) : 1120-26.  Back to cited text no. 3
Stevenson RF, Morin JD. Ocular findings in nevus flammeus. Canad J Ophthalmol 1975: 10:136-139.  Back to cited text no. 4
Rienke RT Maber K, Josselson A. Ota nevus, multiple hemangioma and Takayasu arteritis. Arch Dermatol 1974; 110:447-50.  Back to cited text no. 5
Moschella SL, Hurley HJ. Dermatology, Vol. 2, 2nd edition, Philadelphia, : W.B. Saunders Co., 1975; 2:1281-82.  Back to cited text no. 6
Fitzpatrik TB, Arthur Z Eisen, Wolff K, Freedberg IM, Austin KF Dermatology in General Medicine. 3rd edition, New York : McGraw Hill, 1987 : 1067-68.  Back to cited text no. 7
Duke Elder S. System of Ophthalmology. Vol. 3, Part 2, London Kimpton, 1964: 3(3) : 798-99.   Back to cited text no. 8
Fishman GRA, Anderson R. Nevus of Ota. Report of two cases - one with open angle glaucoma. Am J Ophthalmol 1962: 54 : 453-57.  Back to cited text no. 9
Front R. Reynolds AN, Zimmerman LE. Diffuse malignant melanoma of the iris in the nevus of Ota. Arch Ophthalmol 1967; 77 : 513-518.  Back to cited text no. 10
Shaffer RN, Weiss E. Congenital and pediatric glaucomas. St. Louis The CV Mosby Co., 1970, pp. 72-74.  Back to cited text no. 11
Sood NN, Nevus of Ota, a clinicopathological study. J Paed Ophthalmol 1969: 6:28-35.  Back to cited text no. 12
Kupfer C, Kupfer KM. Observations in the development of the anterior chamber angle with reference to the pathogenesis of congenital glaucomas. Am J Opthalmol 1979: 88:424-435.  Back to cited text no. 13
Sato SE, Herscheler J, Lynch PJ et al. Congenital glaucomma associated with cutis marmorata, telengiectatica congenita. Two case reports. J Paed Ophthalmol Stab 1988; 25:13-17.  Back to cited text no. 14
Beauchamp GR, Knepper PA. Role of the neural crest in anterior segment development of disease. J Paediatr Ophthalmol Strab 1984; 21: 209-214.  Back to cited text no. 15


  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]


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