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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 885

Congenital cystic eyeball with intracranial anomalies: A rare entity

1 Dr. Rajendra Prasad Centre for Ophthalmic Sciences; All India Institute of Medical Sciences, New Delhi, India
2 Northern Railway Central Hospital, New Delhi, India

Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Sahil Agrawal
Oculoplastic, Paediatric Ophthalmology and Oncology Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_161_21

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How to cite this article:
Das D, Bajaj MS, Gupta S, Agrawal S. Congenital cystic eyeball with intracranial anomalies: A rare entity. Indian J Ophthalmol Case Rep 2021;1:885

How to cite this URL:
Das D, Bajaj MS, Gupta S, Agrawal S. Congenital cystic eyeball with intracranial anomalies: A rare entity. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 21];1:885. Available from: https://www.ijoreports.in/text.asp?2021/1/4/885/327717

A 15-day-old female with left orbital mass since birth, showed upper eyelid fullness with a palpable cyst underneath. It was translucent, non-tender, non-reducible and non-pulsatile [Figure 1]a1 and [Figure 1]a2. Imaging revealed dysgenesis of corpus callosum with dorsal inter-hemispheric cyst communicating with the third ventricle. There was no evidence of the left globe, extraocular muscles, or optic nerve [Figure 1]b, [Figure 1]c, [Figure 1]d. A diagnosis of the left congenital cystic eyeball (CCE) was made. It is the rarest congenital cystic orbital lesion caused by invagination arrest of the primary optic vesicle between 2 to 7 mm stages.[1] The orbit contains a cyst with proliferating glial tissue and may be associated with intracranial abnormalities and other non-ocular malformations.[2] Excisional biopsy confirms tissue diagnosis, though in this case a clincio-radiological diagnosis was made.
Figure 1: (a1) Clinical Pictures Of 3 Months Old Infant with Large Left Orbital Cyst Distending the Upper Eyelid, and (a2) Showing Everted Eyelid, with Cystic Lesion. (b) Axial T1MRI of Left Orbit Shows a Cyst (White Thin Arrow), Well Defined Homogeneous, Multilocular, Extending to The Orbital Apex with No Ocular Structures Seen. (c) Axial T1MRI of Brain Showing Corpus Callosum Dysgenesis with a Dorsal Inter-Hemispheric Cyst Communicating with the Third Ventricle (White Dotted Arrow). (d) Axial T1MRI Showing Intermediate Anteriorly Predominant Subcortical Frontal Heterotropia (White Thick Arrow)

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Conflicts of interest

There are no conflicts of interest.

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.

  References Top

Mann I. Development Abnormalities of the Eye. Philadelphia: JB Lippincott Co.; 1957. p. 66-9.  Back to cited text no. 1
Pasquale LR, Romayananda N, Kubacki J, Johnson MH, Chan GH. Congenital cystic eye with multiple ocular and intracranial anomalies. Arch Ophthalmol 1991;109:985-7.  Back to cited text no. 2


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