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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 3  |  Page : 752-753

A rare case of giant conjunctival nevus in an 11-month-old child


Department of Cornea and Refractive Surgery, Medical Research Foundation, Sankara Nethralaya, Chennai, Tamil Nadu, India

Date of Submission08-Mar-2022
Date of Acceptance12-Apr-2022
Date of Web Publication16-Jul-2022

Correspondence Address:
Dr. Rama Rajagopal
Medical Research Foundation, Sankara Nethralaya, 41/18, College Road, Chennai - 600 006, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_625_22

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  Abstract 


Giant conjunctival nevi are considered congenital in origin, with the youngest case reported at 12 years. We describe an 11-month-old baby presenting since birth with a giant conjunctival nevus in the right eye involving inferior 9 o'clock hours of bulbar conjunctiva. Intralesional cysts were noted both on clinical examination and anterior segment optical coherence tomography (ASOCT). Gonioscopy revealed increased trabecular pigmentation. Parents were advised periodic review. Our case highlights that this can occur at birth and emphasizes the need for careful documentation and periodic review considering the rare risk of malignant transformation in these eyes.

Keywords: ASOCT, cystic spaces, giant conjunctival nevus, trabecular pigmentation


How to cite this article:
Rajagopal R, Nathani KR, Patil TS, Jayavel K, Balaji JJ. A rare case of giant conjunctival nevus in an 11-month-old child. Indian J Ophthalmol Case Rep 2022;2:752-3

How to cite this URL:
Rajagopal R, Nathani KR, Patil TS, Jayavel K, Balaji JJ. A rare case of giant conjunctival nevus in an 11-month-old child. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 13];2:752-3. Available from: https://www.ijoreports.in/text.asp?2022/2/3/752/351207



Conjunctival nevi are the most common benign melanocytic tumor of the ocular surface with a mean basal diameter of 3.5 mm.[1] Large nevi with a basal diameter of more than 10 mm in diameter called giant conjunctival nevi (GCN) are rare and account for 5% of conjunctival nevi.[1] Unlike conjunctival nevi, which are localized and reasonably well circumscribed, these are diffuse, can have variable pigmentation and indistinct margins. Although it is considered a congenital abnormality, the youngest case report in literature so far is of a 12-year-old.[2] We herein report a case of an 11-month-old child who presented with history of lesion since birth and clinical, anterior segment optical coherence tomography (ASOCT) features suggestive of GCN. This may be the youngest case reported in literature so far. Besides, there was increased trabecular meshwork pigmentation in the affected eye, thus far not documented in literature, highlighting the importance of a routine gonioscopy and intraocular pressure evaluation in these eyes.


  Case Report Top


An 11-month-old baby was brought by parents for a pigmented lesion in the right eye since birth. Examination under general anesthesia revealed a diffusely pigmented lesion involving contiguous areas of nasal, inferior, and temporal conjunctiva of about 9 o'clock hours between 1 and 10 o'clock, and a maximum vertical extent of 4.5 mm nasally, 4.5 mm inferiorly and 6 mm temporally from the limbus. Margins were indistinct in some areas temporally. Besides cystic spaces and feeder vessels, there were areas of variable pigmentation temporally [Figure 1]. Fornix, caruncle, and upper tarsal conjunctiva were free. Rest of the eye, other eye, and systemic examinations were normal.
Figure 1: Clinical photograph showing diffuse pigmentation involving nasal, inferior and temporal conjunctiva

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Gonioscopy revealed 2+ pigmentation of trabecular meshwork. ASOCT performed using Bioptigen (Bioptigen, Inc., Morrisville, NC, USA) confirmed the presence of multiple intralesional cysts [Figure 2] and back shadowing in areas with dense pigmentation. Parents were advised to review periodically.
Figure 2: (a) ASOCT image of GCN between 1 and 10 o'clock position; (b and c) Nasal, inferior, and temporal ASOCT image. Large red arrow indicates scan position. Small white arrow indicates presence of multiple cystic spaces

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  Discussion Top


GCN is a rare melanocytic clinical condition and can present at birth as in our patient or later.[1] It can involve cornea, limbus, bulbar conjunctiva, fornix, tarsus, semilunar fold and caruncle.[1] They are similar in presentation to conjunctival nevi as both have cystic spaces and variable pigmentation, but differ in their large size and diffuse nature. Both can be usually observed as the risk of malignant transformation is less than 1% in conjunctival nevi and 4% in GCN.[1] In children, change in both pigmentation and size have been associated with hormonal fluctuations[2] and hence can be observed with emphasis on periodic review. However, even in this age group, considering the rare but definite risk of melanoma arising from GCN,[1] it may be prudent to interpret unexplained change in size or pigmentation with caution and when in doubt consider surgical excision. In adults, excision may be needed in cases with sudden increase in size and/or pigmentation providing a definite histopathological confirmation. Unlike localized nevi wherein complete excision is possible, in GCN it may be difficult due to extensive involvement. Limbal stem cell deficiency in lesions proximal to limbus and conjunctival scarring following excision are definite concerns. Surface reconstruction with amniotic membrane grafting can help in these cases.[2],[3] In some patients, cryotherapy has been combined with excision.[2] Recurrence is rare.[1]

Among the pigmented lesions, conjunctival melanoma and primary acquired melanosis (PAM) may be considered in the differential diagnosis, but each has its distinct clinical and ASOCT features. In GCN, usually the onset is at a younger age with pigmentation at epithelial or stromal level. However, it has been described at an older age as well.[4] Shields et al.[1] have described a series of 32 cases with GCN with a mean age of 34 years. Subtle changes can occur with hormonal fluctuation especially in the growing age and is usually stable thereafter.[5] In general, cystic spaces indicate chronicity and are quite often seen in conjunctival nevi on clinical examination. Shields et al. noticed this in 78% of cases on ASOCT.[1] PAM occurs in middle age, is flat and the pigmentation is usually epithelial. Cystic spaces are not seen. Melanoma is usually thicker with an age of onset usually in slightly older patients. Pigmentation in melanoma is mostly within the stroma.[5] Cystic spaces are usually absent and can rarely be seen in melanomas arising from nevi.[6] In older age, increase in thickness/enlargement in size and/or change in pigmentation is usually suspicious of a malignant change and needs excision with histopathological analysis of the specimen. In ASOCT, intrinsic vascularity and back shadowing in areas of dense pigmentation can be features common to both conjunctival nevi and melanoma.[2]


  Conclusion Top


Giant conjunctival nevus represents a rare subgroup of primary melanocytic lesions of the conjunctiva. Young children with GCN with no ocular or systemic risk factors can be observed with emphasis on periodic evaluation. This should include clinical and photographic documentation of height and size, presence of cystic spaces, both clinically and on ASOCT, estimation of intraocular pressure, gonioscopy and a dilated fundus examination. Even in this age group, suspicious lesions may need early surgical intervention and histopathological confirmation. In adults, any change should be interpreted with caution and is preferably excised. Complete excision in GCN can be difficult and may need surface reconstruction. Increased trabecular pigmentation could be a feature in these eyes. Periodic follow-up is essential, considering the rare but definite risk of malignant transformation.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Shields CL, Regillo AC, Mellen PL, Kaliki S, Lally SE, Shields JA. Giant conjunctival nevus: Clinical features and natural course in 32 cases. JAMA Ophthalmol 2013;131:857-63.  Back to cited text no. 1
    
2.
Tóth-Molnár E, Vizvári E, Skribek Á, Vörös A. Giant conjunctival nevus in a 12-year-old child. Case Rep Ophthalmol Med 2017;2017:8414352. doi: 10.1155/2017/8414352.  Back to cited text no. 2
    
3.
Tomita M, Goto H, Muramatsu R, Usui M. Treatment of large conjunctival nevus by resection and reconstruction using amniotic membrane. Graefes Arch Clin Exp Ophthalmol 2006;244:761-4.  Back to cited text no. 3
    
4.
Mori H, Takahashi K. Case of a giant conjunctival melanocytic nevus. Ocul Oncol Pathol 2021;7:97-102.  Back to cited text no. 4
    
5.
Shields CL, Fasiuddin AF, Mashayekhi A, Shields JA. Conjunctival nevi: Clinical features and natural course in 410 consecutive patients. Arch Ophthalmol 2004;122:167-75.  Back to cited text no. 5
    
6.
Esposito E, Zoroquiain P, Mastromonaco C, Morales MC, Belfort Neto R, Burnier M Jr. Epithelial inclusion cyst in conjunctival melanoma. Int J Surg Pathol 2016;24:562-67.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]



 

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