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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 32  |  Issue : 1  |  Page : 76-79

Sonologic similarity in the differential diagnosis of pediatric neovascular glaucoma


1 Jothi Eye Care Center, Puducherry, India
2 Department of Ophthalmology, JIPMER, Puducherry, India
3 Department of Ophthalmology, Fellow in Vitreoretina Services, LVPEI, Hyderabad, Telangana, India

Date of Submission17-Feb-2020
Date of Acceptance25-Feb-2020
Date of Web Publication17-Apr-2020

Correspondence Address:
Dr. Rajalakshmi Selvaraj
Jothi Eye Care Center, Puducherry - 605 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_17_20

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  Abstract 

B-mode ultrasonography is widely used in ophthalmology to assess the retina and vitreous when there is a media opacity or very small pupil size, which prevents direct examination of fundus. Unlike other specialties which request radiologists to perform ultrasonography as part of their investigation, we ophthalmologists are able to perform B-scan by our side of the department as and when required. Here, we report a pediatric case in which B-scan happened to be done by the radiologists; further details enhanced with Doppler uptake resulted in a diagnostic dilemma to both ophthalmologists and radiologists. A 10-year-old male child presented with severe pain and redness in the left eye with repeated vomiting. Ocular examination revealed neovascular glaucoma in the left eye with an absent perception of light. Ultrasound B-scan performed by a radiologist showed a linear hyperechoic structure extending from the posterior aspect of the lens to the optic disc with Doppler uptake, which they suggested as a possibility of persistent hyperplastic primary vitreous (PHPV). However, evaluation of previous treatment records revealed an earlier diagnosis of circumscribed choroidal hemangioma with bullous retinal detachment. Evaluation of pediatric neovascular glaucoma thought about as PHPV yielded a surprising diagnosis of choroidal hemangioma with retinal detachment. Hence, the latter needs to be kept in mind, although the radiological findings mislead to diagnose PHPV.

Keywords: Choroidal hemangioma, linear hyperechoic structure, neovascular glaucoma, persistent hyperplastic primary vitreous


How to cite this article:
Selvaraj R, Kasturi N, Sahoo NK. Sonologic similarity in the differential diagnosis of pediatric neovascular glaucoma. Kerala J Ophthalmol 2020;32:76-9

How to cite this URL:
Selvaraj R, Kasturi N, Sahoo NK. Sonologic similarity in the differential diagnosis of pediatric neovascular glaucoma. Kerala J Ophthalmol [serial online] 2020 [cited 2020 Aug 12];32:76-9. Available from: http://www.kjophthal.com/text.asp?2020/32/1/76/282650




  Introduction Top


B-scan machines made for ophthalmic purpose do not enable mode for Doppler uptake. It happened so that a pediatric patient with neovascular glaucoma and complicated cataract with media haze had to be scanned by radiologists. They could demonstrate a linear hyperechoic structure with Doppler uptake and gave a confusing opinion of PHPV. However, it turned out to be retinal detachment with choroidal hemangioma. Choroidal hemangioma of the retina is a benign hamartoma. It presents in two forms – either a well-circumscribed choroidal hemangioma (CCH) or a diffuse choroidal hemangioma.


  Case Report Top


A 10-year-old boy from Tamil Nadu presented to the ocular emergency service with repeated episodes of vomiting, pain, and redness in the left eye for 2 days. On examination, the visual acuity in the right eye was 6/6 with a normal fundus. Visual acuity in the left eye was an absent perception of light. Anterior-segment examination of the left eye showed circumcorneal congestion, diffuse corneal edema with microbullae, shallow anterior chamber, florid neovascularization of the iris, ectropion uveae, and superiorly subluxated cataractous lens with no view of the fundus [Figure 1], [Figure 2], [Figure 3]. Intraocular pressure was 56 mmHg with Goldmann applanation tonometry. The corneal diameters were normal in both eyes. Ultrasound showed a linear hyperechoic structure extending from the posterior aspect of the lens to the optic disc with Doppler uptake [Figure 4], suggestive of a probable diagnosis of persistent hyperplastic primary vitreous (PHPV) with exudative retinal detachment and vitreous hemorrhage.
Figure 1: Clinical photograph showing circumcorneal congestion, corneal edema, absence of microphthalmos

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Figure 2: Slit photograph showing superiorly subluxated cataractous lens with shallow anterior chamber

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Figure 3: Magnified picture showing neovascularization of iris with ectropion uvea

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Figure 4: Ultrasound B-scan image showing a uniformly reflective membranous structure with Doppler uptake extending from the posterior surface of the lens to the optic disc

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On inquiring further, the child's mother gave a history of gross diminution of vision in the left eye for the past 2 years for which he consulted elsewhere and was explained nil intervention, poor visual prognosis, and follow-up. Previous hospital records revealed a diagnosis of CCH with bullous retinal detachment in the left eye. The child was managed with systemic and topical antiglaucoma medications and inferior quadrant cyclocryotherapy to relieve pain.


  Discussion Top


Pediatric neovascular glaucoma can have multiple differentials. The risk factors for neovascular glaucoma include retinal ischemia, irradiation, tumors, chronic uveitis, and severe ocular inflammation. Accordingly, to prioritize, the first thing to be ruled out in a child with neovascular glaucoma would be retinoblastoma. However, the scan did not reveal evidence of retinal mass or calcifications. With very few reasons that could be enlisted for secondary unilateral glaucoma in the pediatric population, the next thing which comes to mind is Sturge– Weber syndrome More Details. However, the child did not have port-wine stain or blood in Schlemm canal, indicative of raised episcleral venous pressure. Thus, with an idea of the possibility of chronic retinal detachment leading to neovascular component, B-scan was done. Interestingly, due to temporary nonfunctioning of ophthalmic B-scan, ultrasonography was done by radiologists. They identified a liner hyperechoic structure across the vitreous, and the presence of Doppler uptake made them think persistent hyperplastic primary vitreous as the most probable radiological diagnosis. As the child did not have microphthalmos or long ciliary processes, we had to rethink on the radiologists' opinion, only when the mother showed previous examination records that revealed hemangioma of the retina. However, neither it could not be made out now due to cataract, nor was it picked up in B scan.

Ultrasonography has a wide application in ophthalmology. Two types of devices, A-scan and B-scan, are used diagnostically. A-scan ultrasound refers to a one-dimensional amplitude modulation scan, while B-scan ultrasound refers to a two-dimensional, cross-section brightness scan. It is typically used to evaluate the posterior segment and orbital pathology, particularly when the ocular media are cloudy and a direct view is not possible. The ultrasonogram uses piezoelectric lead-zirconate-titanate crystals which generate ultrasonic (inaudible, >20 kHz) sound waves from electricity. The ultrasonic wave goes and gets reflected from any echodense object. The piezoelectric crystal picks up the reflected sound and converts it to electric signals which give the image of the echogram. The echodensity of the object determines the amount of reflected sound wave received by the probe and hence the intensity of whiteness in B-scan and the amplitude in the A-scan.

CCH of the retina typically appears as a round or oval, orange-red elevated mass posterior to the equator. Lesions are usually solitary and unilateral, vascular tumors; however, prominent intrinsic tumor vessels, or “feeder vessels,” are not present on clinical examination.[1] Ultrasonographic findings of CCH reveal a smooth-contoured, dome-shaped choroidal mass which on A-scan demonstrates a high initial spike that corresponds to the anterior tumor surface and high internal reflectivity between 50% and 100% due to multiple vascular channels throughout these tumors.[2] Pigmentary changes, disorganization, and proliferation of the overlying retinal pigment epithelium, a choroidal neovascular membrane, fibrous metaplasia, cystoid macular edema, retinoschisis, and ossification, epiretinal membrane, subretinal hemorrhage and exudates, total retinal detachment, and neovascularization of the iris and angle have been reported.[2]

PHPV is subclassified into three types: (1) most often an anterior PHPV (retrolental fibrovascular membrane, elongated ciliary processes, cataract, and microphthalmia); (2) posterior PHPV (vitreous membrane and stalk, retinal fold, traction retinal detachment, hypoplastic optic nerve and macula, and microphthalmia); (3) a combination of anterior and posterior. PHPV is unilateral in 90% of cases and consists of a whitish vascularized membranous structure behind the lens, associated with microphthalmos, shallow anterior chamber, and long ciliary process. Since the posterior capsule of the lens is often absent, the metabolism of the lens is disrupted, resulting in opacification, and swelling of the lens, which can precipitate secondary angle-closure glaucoma either by forward displacement of the lens and iris, or by causing a pupillary block. A retrolenticular membrane may contract causing anterior movement of the lens and iris, or an intralenticular hemorrhage may occur, with subsequent angle closure.[3] In addition, bleeding from the vessels can cause a vitreous hemorrhage and results in inflammatory or ghost cell glaucoma. The hemorrhage may organize and develop fibrous membrane, leading to tractional retinal detachment. Long-standing serous or tractional retinal detachments can lead to hypoxia of the retina, release of vascular endothelial growth factor (VEGF) to the aqueous, resulting in neovascularization of the angle and iris with the development of neovascular glaucoma.

If the old records stating choroidal hemangioma were not available, we would have arrived at the diagnosis of PHPV with retinal detachment and vitreous hemorrhage as the cause of neovascular glaucoma in this child. Management of the condition was with atropine, steroids, antiglaucoma medications, cryotherapy as per the general protocol of treating neovascular glaucoma no matter whatever be the differential diagnosis. However, if media were clear enough to make out the hemangioma, then other methods of treating the same would have come into play such as photodynamic therapy (PDT), plaque brachytherapy, external beam and proton beam radiation, stereotactic radiosurgery, transpupillary thermotherapy, laser photocoagulation, oral propranolol, and anti-VEGF injections. While PDT currently entails the best results,[4],[5] propranolol may induce vasoconstriction, decrease expression of angiogenic factors, and inhibit endothelial proliferation.[6]


  Conclusion Top


Had the B-scan for the child been done by an ophthalmologist, would have concluded as closed funnel retinal detachment for the evaluation of the cause. Incidentally and interestingly, the scan performed by the radiologist with Doppler uptake was opined as PHPV and hence this discussion. The imaging was quite intriguing for both the ophthalmologists and radiologists.

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.
Medina CA, Singh AD. Diagnosis and management of choroidal hemangiomas. Retin Physician 2013;10:32-7.  Back to cited text no. 1
    
2.
Karimi S, Nourinia R, Mashayekhi A. Circumscribed choroidal hemangioma. J Ophthalmic Vis Res 2015;10:320-8.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Regillo C, Holekamp N, Johnson M, Kaiser P, Schubert H, Spaide R, et al. Diseases of the vitreous. In: Basic and Clinical Science Course. American Academy of Ophthalmology: Retina and Vitreous. Sec. 12. Singapore: LEO; 2011. p. 307-8.  Back to cited text no. 3
    
4.
Tsipursky MS, Golchet PR, Jampol LM. Photodynamic therapy of choroidal hemangioma in Sturge-Weber syndrome, with a review of treatments for diffuse and circumscribed choroidal hemangiomas. Surv Ophthalmol 2011;56:68-85.  Back to cited text no. 4
    
5.
Poh KW, Wai YZ, Rahmat J, Shunmugam M, Alagaratnam J, Ramasamy S. Treatment of diffuse choroidal haemangioma using photodynamic therapy. Int J Ophthalmol 2017;10:488-90.  Back to cited text no. 5
    
6.
Sanz-Marco E, Gallego R, Diaz-Llopis M. Oral propranolol for circumscribed choroidal hemangioma. Case Rep Ophthalmol 2011;2:84-90.  Back to cited text no. 6
    


    Figures

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



 

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