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Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 340-342

Retinal cavernous haemangioma in an infant with aggressive posterior retinopathy of prematurity

Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission27-Jun-2020
Date of Acceptance30-Oct-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Deeksha Katoch
Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Sector 12, Chandigarh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2116_20

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A 2-month-old premature infant (37 weeks post-menstrual age) was referred for persistent pre-retinal bleed following laser treatment for retinopathy of prematurity. Detailed examination revealed a reddish purple, raised, lobulated lesion at the junction of the vascularised retina and laser scars. Typical clinical findings along with fluorescein angiography helped establish the diagnosis of retinal cavernous haemangioma. This report describes the youngest reported case of retinal cavernous haemangioma in a premature child.

Keywords: Aggressive posterior retinopathy of prematurity, angiography, APROP, prematurity, RetCam, retinal cavernous haemangioma, ROP

How to cite this article:
Singh SR, Arora A, Jain S, Dogra M, Dogra MR, Katoch D. Retinal cavernous haemangioma in an infant with aggressive posterior retinopathy of prematurity. Indian J Ophthalmol Case Rep 2021;1:340-2

How to cite this URL:
Singh SR, Arora A, Jain S, Dogra M, Dogra MR, Katoch D. Retinal cavernous haemangioma in an infant with aggressive posterior retinopathy of prematurity. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 29];1:340-2. Available from: https://www.ijoreports.in/text.asp?2021/1/2/340/312359

Retinal cavernous haemangioma (RCH) is a rare, benign vascular tumour, first described as 'angiomatosis retinae' in the year 1934.[1] It was not until 1971, when Gass described it as a separate clinical entity.[2] RCH is asymptomatic and incidentally detected in almost 50% of the cases.[3] Patients with RCH mostly present in the second to fourth decade of life with the median age of presentation being 21 years. The youngest reported case of RCH, which was diagnosed at the time of presentation is at 4 months of age.[3] We present a case of RCH in a 2-month-old premature infant, treated for aggressive posterior retinopathy of prematurity (APROP) and referred with a diagnosis of inadequate disease regression post-treatment.

  Case Report Top

A preterm infant born at a gestational age of 29 weeks, with a birth weight of 1500 grams was screened elsewhere for Retinopathy of Prematurity (ROP). The infant had a history of undergoing laser photocoagulation with frequency-doubled Nd: YAG laser for a diagnosis of Aggressive Posterior ROP at a postmenstrual age (PMA) of 33 weeks.[4] Four weeks after the intervention the infant was referred to our hospital for second opinion. The referral notes documented persistence of a 'large haemorrhage' in the left eye despite laser photocoagulation with some residual plus disease.

On examination of the infant at a PMA of 37 weeks, the pupils dilated fully in both eyes with no tunica vasculosa lentis. Posterior segment of the right eye had regressed ROP with laser scars in all quadrants beyond zone 1 [Figure 1]a. The left eye revealed a lobulated mass lesion, deep red to reddish black in colour, along the infero-temporal arcade with a size of approximately 5-6 disc diameters [Figure 1]b – white arrow]. Confluent laser scars were present peripheral to the lesion along with residual vascular tortuosity. ROP appeared regressed without evidence of any haemorrhage [Figure 1]b. An ultrasound b-scan passing through the lesion demonstrated a conical mass with heterogenous core and no back shadowing [Figure 1]c. Given the characteristic clinical picture resembling a cluster of grapes and no back shadowing on ultrasound, a provisional diagnosis of RCH was made.
Figure 1: Retinal cavernous haemangioma at presentation in a 2-month-old premature infant. (a) Right eye fundus showing regressed retinopathy of prematurity (b) Left eye fundus image showing deep reddish purple, raised and lobulated lesion along the inferotemporal arcade (white arrow) in the background of regressed retinopathy of prematurity. (c) Ultrasound b-scan showing a conical, hyperechoic mass lesion with no back-shadowing. (d) Fluorescein angiography image in the early phase showing hypo-fluorescence in the lesion. (e) Mid-phase angiogram showing beginning of dye filling in the lesion from the superior part. (f) late phase of the angiogram demonstrating hyper-fluorescence with pooling of dye in the upper half of each lobule demonstrating the classic ‘fluorescein cap’ in a retinal cavernous haemangioma

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The parents were counselled about the need for examination under anaesthesia combined with handheld contact-based fluorescein angiogram (FA) for confirming the diagnosis. Under general anaesthesia, sodium fluorescein 20% was injected at a dose of 7.7 mg/kg followed by an immediate saline flush and images were captured using RetCam3 (Natus Medical Incorporated, Pleasanton, CA, USA) in video mode using the filter provided along with.[5] The lesion remained hypofluorescent in the early and mid-phases of the angiogram [Figure 1]d and [Figure 1]e. In the late phase the individual lobulations of the lesion showed hyperfluorescence with dye pooling. One half of some of the lobules however remained hypofluorescent [Figure 1]f appearing as the 'fluorescein cap' sign on FA described in RCH. The “cap” has been attributed to stagnation of blood leading to a plasma-erythrocyte interface.[6] It is important to bear the fact in mind that the typical “caps” are seen in patients with angiograms done in sitting position and our patient was a small infant with angiography done in supine position. Typically the lobules fill very slowly and in the late phase only. FA also confirmed a regressed APROP. A contrast-enhanced magnetic resonance imaging of brain was ordered to rule out central nervous system (CNS) involvement, the findings of which were unremarkable. Fundus and systemic examination of the parents and siblings did not reveal any abnormality. Given the benign nature of the lesion, the parents were advised observation at this point of time. One-year following this, the RCH had shrunk in size with presence of fibro-glial tissue over it [Figure 2].
Figure 2: One-year follow up image showing shrinkage of the retinal cavernous haemangioma with presence of fibro-glial tissue over the lesion (white arrow)

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  Discussion Top

RCH is a congenital retinal vascular anomaly, which is likely present since birth.[3] However, there have been no reports of RCH in premature children diagnosed during infancy. Shields et al.[7] reported a term baby with RCH, who presented with hyphema at birth. Although, it was not until 22 years of age when the definitive diagnosis of RCH was made. In our case, the presence of APROP with extensive haemorrhages at presentation could have masked the RCH. However, after regression of ROP following laser treatment, this lesion stood out. The reddish-black hue of the lesion masqueraded as a pre-retinal haemorrhage and made the primary ophthalmologist to infer incomplete regression of ROP. This could have led to over-treatment in a relatively benign pathology. The elevated nature of the lesion with the typical hue and lobulations helped differentiate it from a pre-retinal haemorrhage. Other retinal lesions that can sometime confuse ophthalmologists due to similar appearance to an RCH are retinal capillary haemangioma and telangiectasia of Coats' disease. FA features helped distinguish them from RCH. The lack of a distinct feeder/draining vessel and hypoflorescence in the early phase of the FA helped distinguish it from a retinal capillary haemangioma. Telangiectasia of Coats' disease was ruled out due absence of exudation and absence of the bulb-like telangiectasias and leakage on FA.

Prognosis in cases with RCH is generally favourable. Majority have been reported to remain stable with occasional vitreous haemorrhage being the only significant complication.[3] In our case, the RCH lesion shrunk in size over the course of one year with development of fibro-glial tissue over it. This has been shown to be an important association in eyes which have recurrent vitreous haemorrhage. The fibrous tissue contracts over time and can lead to small breaks in the frail wall of the RCH.[3],[8] It would be prudent to counsel the parents for a regular follow-up in such a case for early detection of any amblyogenic vitreous haemorrhage as smaller children might not report the same themselves.

  Conclusion Top

In conclusion, this case to the best of our knowledge, represents the youngest diagnosed case of RCH in literature and the first case of RCH along with ROP. This is a reminder to all of us who examine and treat ROP that other retinal pathology can be present in addition to ROP. We need to be aware of the incidental associations to diagnose and treat them appropriately.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Niccol W, Niccol W, Moore RF. A case of angiomatosis retinae. Br J Ophthalmol 1934;18:454-7.  Back to cited text no. 1
Gass JD. Cavernous hemangioma of the retina. A neuro-oculo-cutaneous syndrome. Am J Ophthalmol 1971;71:799-814.  Back to cited text no. 2
Wang W, Chen L. Cavernous hemangioma of the retina: A comprehensive review of the literature (1934-2015). Retina 2017;37:611-21.  Back to cited text no. 3
Singh SR, Katoch D, Handa S, Kaur S, Moharana B, Dogra M, et al. Safety and efficacy of 532 nm frequencydoubled NdYAG green laser photocoagulation for treatment of retinopathy of prematurity. Indian J Ophthalmol 2019;67:860-5.  Back to cited text no. 4
[PUBMED]  [Full text]  
Temkar S, Azad SV, Chawla R, Damodaran S, Garg G, Regani H, et al. Ultra-widefield fundus fluorescein angiography in pediatric retinal vascular diseases. Indian J Ophthalmol 2019;67:788-94.  Back to cited text no. 5
[PUBMED]  [Full text]  
Kumar M, Reddy N, Konana VK, Kanakamedla A, Ruia S, Gudimetla J. Fluorescein cap: Fluorescein angiographic feature of retinal cavernous hemangioma. Indian J Ophthalmol 2018;66:1473-4.  Back to cited text no. 6
[PUBMED]  [Full text]  
Shields JA, Eagle RJ, Ewing MQ, Lally SE, Shields C. Retinal cavernous hemangioma: Fifty-two years of clinical follow-up with clinicopathologic correlation. Retina 2014;34:1253-7.  Back to cited text no. 7
Pringle E, Chen S, Rubinstein A, Patel CK, Downes S. Optical coherence tomography in retinal cavernous haemangioma may explain the mechanism of vitreous haemorrhage. Eye (Lond) 2009;23:1242-3.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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