Imaging Findings of Congenital Glaucoma in Opitz Syndrome

Choudhary, Abhishek; Jha, Bhaskar

doi:10.3174/ajnr.a1020

Cited by 3 publications

(2 citation statements)

References 6 publications

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“…In addition to a corneal melt, nontraumatic causes of anterior chamber enlargement include a uveal melanoma arising from the ciliary body or the iris, 11 an ocular metastasis, 12 and congenital glaucoma. 13 In summary, the uncommon occurrence of a corneal melt has a unique CT appearance that should allow its imaging diagnosis. …”

Section: Discussionmentioning

confidence: 99%

The CT Appearance of a Corneal Melt: Report of 2 Cases

Bluestone¹,

Ackert²,

Som³

2011

AJNR Am J Neuroradiol

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SUMMARY:When the cornea of the eye dissolves, most commonly secondary to infection or trauma, the ventral wall of the anterior chamber of the eye is no longer supported and thus bulges forward, enlarging the anterior chamber volume. This is referred to as a corneal melt, and it has a unique CT appearance that has not been previously described.A corneal melt is a complete breakdown of the corneal structure, which can be initiated by a variety of conditions most commonly related to infections, prior surgery, systemic or localized autoimmune diseases, or trauma. When a corneal melt occurs, it results in an enlarged anterior chamber of the eye and it has a unique CT morphology, which does not appear to have been previously described, to our knowledge. The purpose of this article is to report 2 cases of corneal melt: 1 related to herpes simplex infection and 1 related to trauma after cataract surgery and a corneal transplant. Case Reports Case 1The patient was a 51-year-old man with no significant medical history. His ocular history was significant for herpetic keratitis treated at an outside institution. The patient reportedly had a neurotrophic ulcer secondary to herpetic eye disease, which he said resolved 4 months earlier. Approximately 3 months before his present admission, he noted progressively worsening vision associated with ocular pain. At the time of presentation, he had no light perception in the right eye and was 20/40 in the left eye. He was noted to have a tension of 76 in the affected eye. The anterior segment of the eye was disorganized with opacification, and no corneal layers could be identified. There was no view to the posterior pole. On B-scan sonography, the retina was flat without any significant vitritis. The lens was noted to be thickened and slightly displaced anteriorly. A CT scan of the orbits showed a grossly enlarged anterior chamber in an otherwise unremarkable eye (Fig 1). Five days later, the patient had an enucleation of the right eye. Case 2The patient was a 90-year-old woman with a medical history significant for migraines and gait imbalance, and an ocular history significant for bilateral cataract extractions 20 years ago and corneal transplantation in the left eye 9 years ago, with a limbal relaxing incision performed 1 year later for a residual refractive error. On presentation to the emergency department for a fall, she had decreased vision in her left eye, and an examination revealed multiple facial lacerations. Her ophthalmic examination showed a flat anterior chamber with prolapsed uveal tissue. Her CT of the head and orbits showed no evidence of acute orbital fracture. However, there was an enlarged anterior chamber in her left eye (Fig 2). After appropriate antibiotic and tetanus vaccine prophylaxis, she was taken to the operating room where it was found that her corneal transplant wound had dehisced and the intraocular lens placed at the time of cataract surgery had extruded from the eye and was found in the inferior fornix. DiscussionThe corneal epithelium is important ...

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Section: Discussionmentioning

confidence: 99%

The CT Appearance of a Corneal Melt: Report of 2 Cases

Bluestone¹,

Ackert²,

Som³

2011

AJNR Am J Neuroradiol

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“…Buphthalmos has a prevalence of 1 : 30000 births [ 54 ], and in 80% of cases is bilateral [ 53 – 55 ]. The most common cause of buphthalmos is congenital glaucoma which may be isolated or syndromic and occurs at birth or within the first 3 years [ 11 , 56 ]. The enlargement of the globe coexists with an increased diameter of the cornea (>12 mm) and rupture of Descemet's membrane (Haab's striae) [ 54 ].…”

Section: Macrophthalmiamentioning

confidence: 99%

Congenital Malformations of the Eye: A Pictorial Review and Clinico-Radiological Correlations

Guarnera,

Valente,

Pasquini

et al. 2024

Journal of Ophthalmology

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Congenital malformations of the eye represent a wide and heterogeneous spectrum of abnormalities that may be part of a complex syndrome or be isolated. Ocular malformation severity depends on the timing of the causative event during eye formation, ranging from the complete absence of the eye if injury occurs during the first weeks of gestation, to subtle abnormalities if the cause occurs later on. Knowledge of ocular malformations is crucial to performing a tailored imaging protocol and correctly reporting imaging findings. Together with the ophthalmologic evaluation, imaging may help frame ocular malformations and identify underlying genetic conditions. The purpose of this pictorial review is to describe the imaging features of the main ocular malformations and the related ophthalmologic findings in order to provide a clinico-radiological overview of these abnormalities to the clinical radiologist. Sight is a crucial sense for children to explore the world and relate with their parents from birth. Vision impairment or even blindness secondary to ocular malformations deeply affects children’s growth and quality of life.

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