• Users Online: 2367
  • Print this page
  • Email this page

 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 286-288

A case of oculodermal melanocytosis with secondary angle closure glaucoma

1 VST Glaucoma Center, L V Prasad Eye Institute, Banjara Hills, Hyderabad, Telangana, India
2 Ophthalmic Pathology Laboratory, LV Prasad Eye Institute, Hyderabad, Telangana, India
3 The Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad, Telangana, India

Date of Submission14-Apr-2020
Date of Acceptance27-Oct-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Sirisha Senthil
L.V Prasad Eye Institute, Kallam Anji Reddy Campus, L V Prasad Marg, Road No: 2, Banjara Hills, Hyderabad - 500 034 , Telangana
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_938_20

Rights and Permissions

Oculodermal melanocytosis (ODM) is a rare condition with glaucoma being the most common complication in these eyes. Open angle glaucoma is the commonest variant and presence of angle closure in these eyes should raise a suspicion of ciliary body or uveal tumor, which needs to be evaluated before any surgical intervention is planned. We report a case of unilateral ODM with secondary angle closure glaucoma. Angle closure in an eye with ODM is extremely rare, ultrasound B-scan showed thickened choroid, and ultrasound biomicroscopy showed 360° ciliary body hypertrophy. Incisional biopsy & fine needle aspiration cytology of the ciliary body tissue was performed, and histopathological features were suggestive of a benign hypertrophy ruling out ciliary body melanoma. Subsequently, a glaucoma drainage device surgery was performed in the right eye. To the best of our knowledge, benign ciliary body hypertrophy causing intractable angle closure glaucoma in a case of ODM has not been reported.

Keywords: Angle closure, glaucoma, oculodermal melanocytosis

How to cite this article:
Kolipaka GP, Dikshit S, Mishra D K, Kaliki S, Senthil S. A case of oculodermal melanocytosis with secondary angle closure glaucoma. Indian J Ophthalmol Case Rep 2021;1:286-8

How to cite this URL:
Kolipaka GP, Dikshit S, Mishra D K, Kaliki S, Senthil S. A case of oculodermal melanocytosis with secondary angle closure glaucoma. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 19];1:286-8. Available from: https://www.ijoreports.in/text.asp?2021/1/2/286/312418

Oculodermal melanocytosis (ODM) is a congenital pigmentary abnormality consisting of excess melanocytes within the periocular skin, sclera, uvea, orbit, meninges, palate, or tympanic membrane. The common associations of ODM include secondary glaucoma and uveal tumors.[1],[2],[3],[4],[5] Secondary open angle glaucoma due to blockage of filtration angle by melanocytosis or a developmental angle anomaly are proposed mechanisms associated with ODM.[1],[3],[4] However, other rare presentations like angle closure can occur and should raise a suspicion of ciliary body tumor. We report a case of ODM with secondary angle closure glaucoma due to hypertrophy of ciliary body, highlighting the importance of imaging and biopsy to rule out a malignant tumor in such a presentation.

  Case Report Top

A 24-year-old, male was referred to us for uncontrolled intraocular pressure (IOP). He had right eye ocular melanosis, with past history of high IOP with angle closure glaucoma, for which he underwent trabeculectomy with mitomycin-C elsewhere; 6 months later, phacoemulsification with posterior chamber intraocular lens implantation was performed.

At presentation, his best-corrected visual acuity was 20/32, N24 in the right eye and 20/20, N6 in the left eye. There was cutaneous pigmentation involving the ophthalmic and maxillary division of the trigeminal nerve on the right half of his face; the eyelids, the sclera, iris, and the right side of the hard palate were also densely pigmented.

The right eye showed superior flat bleb, dilated episcleral veins with extensive slate gray scleral pigmentation [Figure 1]a, microcystic corneal edema, peripheral anterior synechiae with shallow peripheral anterior chamber, hyperpigmented iris with interspersed areas of stromal atrophy, mid-dilated fixed pupil, pseudophakos with posterior capsular opacification, and the left eye was normal. The IOP in the right eye was 24 mmHg and in the left eye was normal.
Figure 1: (a) Slit lamp photograph of the right eye showing dilated episcleral veins with episcleral pigmentation, corneal edema, shallow anterior chamber, hyperpigmented iris, and posterior chamber intraocular lens; (b) The ultrasound B Scan of the right eye showing thickened choroid (red arrow) and ultrasound biomicroscopy picture of the right eye showing thickened, hypertrophic ciliary body (red star) and iris with closed angles (yellow arrow) (c) and normal ciliary body, iris and open angle in the left eye (d)

Click here to view

The gonioscopy of the right eye showed synechial angle closure in all four quadrants, while the left eye angles were open. The posterior segment evaluation in the right eye showed a cup-to-disc-ratio (CDR) of 0.7 (through a hazy view), and the left eye showed 0.2 CDR . There were no iris new vessels and no other posterior segment pathology in the right eye. Humphrey visual field analysis showed generalized depression in the right eye and the left eye was normal.

An ultrasound B-scan (USG) and ultrasound biomicroscopy (UBM) were performed for the right eye to rule out ciliary body ring melanoma. Gross thickening of the choroid (red arrow) was noted on B scan [Figure 1]b, the UBM showed 360° ciliary body (red star) and iris hypertrophy with closed angles (yellow arrow) [Figure 1]c, and the left eye USG and UBM [Figure 1]d were within normal limits. The patient was evaluated at the ocular oncology clinic and incisional biopsy through trabeculectomy site & fine needle aspiration cytology (FNAC) from ciliary body was advised and performed. FNAC from the ciliary body showed few pigmented cells against the background of proteinaceous fluid. Atypical cells or evidence of melanoma were absent.

Gross pathological examination revealed tiny fragments of pigmented tissue measuring 2 mm × 1 mm to 3 mm × 2 mm. Microscopic examination shows hyperpigmented tissue with few spindle cells [Figure 2]. The tissue was bleached to remove melanin pigments; section from the bleached tissue showed few spindle cells against the fibro-collagenous tissue. There was no necrosis or mitotic figures ruling out malignant melanoma.
Figure 2: Histopathology section from the ciliary body biopsy showing few spindle cells against the fibrocollagenous tissue. Necrosis and mitotic figures are absent

Click here to view

Subsequently, Ahmed glaucoma valve implantation in the infero-temporal quadrant was performed. A small-sized implant (FP8) was used in view of severe conjunctival adhesions and limited conjunctival dissection that was possible. The extensive scarring was possibly related to the disease process. Note the slate gray to black sclera on to which the implant and the tube were sutured, the tube entry was made with a 24 guage needle track (3–4 mm) and in view of 360° angle closure; the tube was placed in the ciliary sulcus. The tube was covered with a scleral patch graft and conjunctiva sutured with 8-0 vicryl suture [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d. Postoperatively, the IOP was controlled with a well-placed sulcus tube [Figure 4]. Two topical antiglaucoma medications were started in view of hypertensive phase at 2 weeks and the vision and IOP were maintained at 15 mm Hg until 6 months.
Figure 3: (a) Intraoperative pictures showing the inferior conjunctiva with underlying scleral pigmentation (yellow star), (b) note the slate gray to black scleral appearance after the conjunctival dissection (arrow), (c) implant and tube sutured to the sclera (arrow), and (d) less pigmented appearance after application of the scleral patch graft to conver the tube and conjunctival closure (yellow star)

Click here to view
Figure 4: Postoperative photograph of the right eye showing a sulcus placed AGV tube (yellow arrow)

Click here to view

  Discussion Top

Ota first described ODM in 1939. The term Nevus of Ota is used only if dermal involvement is present, and the condition is termed melanosis oculi if only eye is involved. ODM is associated with glaucoma in 10.3% of cases warranting a regular follow-up examination for glaucoma in these cases.[1] It is also associated with ocular melanoma (1 in 400), hence necessitating periodic fundus examination in these cases.[2],[5] USG and UBM may be necessary when there is no fundus view or if a ciliary body melanoma is suspected.2 A study on oculodermal melanocytosis in 194 patients revealed glaucoma in 20 patients. Of the 20 patients, 15 developed ocular hypertension or open-angle glaucoma (70%), 3 patients had congenital or developmental glaucoma (15%), and 3 others had primary angle-closure glaucoma (15%);[1] these three patients had bilateral angle-closure glaucoma despite unilateral ocular melanocytosis indication a primary angle closure. The commonest cause of elevated IOP in these eyes is secondary open angle glaucoma occurring due to blockage of filtration angle by melanocytes followed by developmental angle anomaly leading to elevated IOP.

Unilateral angle closure (secondary) in these eyes is extremely rare and should rise a suspicion of ciliary body or uveal tumor. In our case, with similar suspicion, evaluation with imaging and biopsy were performed and ciliary body mass was ruled out. The cause of secondary angle closure glaucoma in our case was probably hypertrophy of the ciliary body causing direct angle closure. The cause of uveal hypertrophy in our case could be related to dense ocular melanocytosis mimicking a diffuse uveal melanoma. The glaucoma in these eyes may be refractory to medical treatment, and there are fewer surgical options to manage them in view of severe ocular melanosis. Benign ciliary body hypertrophy versus a malignant tumor may be difficult to clinically differentiate and would require a biopsy and histopathological evaluation before a definitive surgical intervention is planned.

  Conclusion Top

To the best of our knowledge, ciliary body hypertrophy causing intractable angle closure glaucoma in a case of ODM has not been reported previously.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Teekhasaenee C, Ritch R, Rutnin U, Leelawongs N. Ocular findings in oculodermal melanocytosis. Arch Ophthalmol 1990;108:1114-20.  Back to cited text no. 1
Shields CL, Kaliki S, Livesey M, Walker B, Garoon R, Bucci M, et al. Association of ocular and oculodermal melanocytosis with the rate of uveal melanoma metastasis. JAMA Ophthalmol 2013;131:993-1003.  Back to cited text no. 2
Khawly JA, Imami N, Shields MB. Glaucoma associated with the nevus of Ota. Arch Ophthalmol 1995;113:1208-9.  Back to cited text no. 3
Liu JC, Ball SF. Nevus of Ota with glaucoma: Report of three cases. Ann Ophthalmol 1991;23:286-9.  Back to cited text no. 4
Bordon AF, Wray ML, Belfort R, McLean IW, Burnier M. Choroidal malignant melanoma in association with oculodermal melanocytosis in a black patient. Br J Ophthalmol 1995;79:191-2.  Back to cited text no. 5


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


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded30    
    Comments [Add]    

Recommend this journal