Ophthalmic Surgery, Lasers and Imaging Retina

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Case Report 

Regression of Macular Cavernous Hemangioma with Systemic Infliximab

Ricardo M. Japiassú, MD; Oswaldo F. Moura Brasil, MD; Eduardo C. de Souza, MD

Abstract

Cavernous hemangioma of the retina is an unusual vascular hamartoma that has been reported to occur either sporadically or as a dominantly inherited trait. A case of a large and sporadic macular cavernous hemangioma that was successfully treated with systemic infliximab as a first line of treatment is presented. The patient was referred with decreased vision of the right eye for 6 months. Fundus examination disclosed a reddish elevated lesion in the posterior pole that obscured the macula and encroached on the fovea. It consisted of grapelike aneurysms. The patient underwent treatment with infliximab intravenously. Two weeks after the second infusion of infliximab, visual acuity improved to 20/80 with a marked involution of the tumor in the right eye. Although the data are limited, we think that a new perspective for treatment of retinal vascular tumors may appear in the future considering antibodies to tumor necrosis factor (TNF)-alpha.

Abstract

Cavernous hemangioma of the retina is an unusual vascular hamartoma that has been reported to occur either sporadically or as a dominantly inherited trait. A case of a large and sporadic macular cavernous hemangioma that was successfully treated with systemic infliximab as a first line of treatment is presented. The patient was referred with decreased vision of the right eye for 6 months. Fundus examination disclosed a reddish elevated lesion in the posterior pole that obscured the macula and encroached on the fovea. It consisted of grapelike aneurysms. The patient underwent treatment with infliximab intravenously. Two weeks after the second infusion of infliximab, visual acuity improved to 20/80 with a marked involution of the tumor in the right eye. Although the data are limited, we think that a new perspective for treatment of retinal vascular tumors may appear in the future considering antibodies to tumor necrosis factor (TNF)-alpha.

Regression of Macular Cavernous Hemangioma with Systemic Infliximab

From Pedro Ernesto University Hospital (RMJ), State University of Rio de Janeiro, Rio de Janeiro; and Vision Institute (OFMB, ECDS), Federal University of São Paulo, São Paulo, Brazil.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Ricardo Miguel Japiassú, MD, Rua Desembargador Isidro 121 APTO. 202-B – Tijuca, Rio de Janeiro – RJ, Brazil, CEP 20521-160.

Accepted: May 04, 2009
Posted Online: March 09, 2010

Case Report

A 63-year-old man was referred for decreased vision of the right eye for 6 months. He had no history of underlying diseases. Best-corrected visual acuity was 20/400 in the right eye and 20/20 in the left eye. Results of biomicroscopy and tonometry were unremarkable in both eyes. Fundus examination of the right eye revealed a reddish elevated lesion in the posterior pole that obscured the macula and encroached on the fovea. It consisted of numerous, saccular, grapelike aneurysms filled with blood. Fluorescein angiography showed delayed filling of the lesion with intravascular plasma–erythrocyte separation, typical of a retinal cavernous hemangioma. There was evidence of local leakage from the tumor in the late frames. Optical coherence tomography (OCT) showed retinal elevation caused by the tumoral tissue (Fig. 1). Optically clear cystic spaces and high reflective signs were seen arising from the inner part of the retina. The outer retina was thickened suggesting fluid accumulation from the lesion.

Colour Fundus Photograph, Fluorescein Angiography and OCT of the Right Eye Before Treatment. The Abnormal Vessels Show Fluorescence Capping and Leakage in the Macula. OCT Confirmed Retinal Elevation Caused by the Tumoral Tissue. Visual Acuity was 20/400.

Figure 1. Colour Fundus Photograph, Fluorescein Angiography and OCT of the Right Eye Before Treatment. The Abnormal Vessels Show Fluorescence Capping and Leakage in the Macula. OCT Confirmed Retinal Elevation Caused by the Tumoral Tissue. Visual Acuity was 20/400.

Photodynamic therapy was not possible in this case and the patient denied the idea of intraocular injections offered as first lines of treatment. Considering our previous clinical experience with only one patient treated with systemic infliximab (anti-tumor necrosis factor) for a retinal vascular lesion,5 this type of treatment was accepted by the patient. As an open-label program for spondyloarthropathy, an induction schema with infliximab (5 mg/kg at baseline, Week 2, week 6, and every 8 weeks for 12 months) was started. Two weeks after the second infusion of infliximab, corresponding to the first month after starting the treatment, visual acuity improved to 20/80 with a marked involution of the tumor in the right eye (Fig. 2). The patient has been followed for 6 months. Visual acuity was 20/60 in his right eye at his most recent exam. Ophthalmoscopic examination disclosed an inactive lesion in that eye.

2 Weeks After the Second Infusion of Infliximab, Visual Acuity Improved to 20/80 with a Marked Involution of the Tumor in the Right Eye.

Figure 2. 2 Weeks After the Second Infusion of Infliximab, Visual Acuity Improved to 20/80 with a Marked Involution of the Tumor in the Right Eye.

Discussion

This case presented an isolated macular cavernous hemangioma in the right eye, confirmed by fluorescein angiography. We initially suspected other similar abnormal retinal blood vessels disorders such as Coats disease (idiopathic macular telangiectasia Type-1) or Leber’s miliary aneurysms, but these conditions are usually accompanied by exudation and lack fluorescence capping.

Besides exudation and lack of fluorescence capping, a retinal capillary hemangioma (von Hippel tumour) was also ruled out in this case due to the absence of typical feeder vessels.4 Several systemic abnormalities can occur in association with retinal cavernous hemangioma, including cavernous hemangioma of the central nervous system2 and several types of cutaneous hemangiomas.2,3,4 No evidence of cutaneous or central nervous system abnormalities was found in our patient. Although there was no family history of ocular or vision abnormalities, we were unable to examine family members.

Similar to previous reports,1,2,3 fluorescein angiography in our case demonstrated relatively slow flow through the tumor with early hypofluorescence, which was followed by slow filling of the aneurysms and a classic erythrocyte–plasma interface. Some extravasation of dye was observed in the macula in the late phases of the exam. Although the mechanism of visual loss in this case was unclear, we suspect that the tumor may have caused macular edema sufficient to have impaired visual function. We discussed with the patient the most appropriated modalities of treatment for his condition, such as photodynamic therapy, transpupillary thermal therapy and intraocular antiangiogenics. He refused to receive injections inside the eye, and bevacizumab was the only modality of treatment available at the time. According to our positive experience with only one case with a vasoproliferative retinal lesion,5 we decided to try the same systemic type of treatment with this patient. Systemic infliximab rapidly improved the central vision and enabled a dramatic reduction in the macular cavernous hemangioma in this case. Such evidence was readily observed at the slit lamp (Volk 78) but not entirely angiographically. The medication appeared to have closed down many of the leaking grape-like lesions from the tumor. The patient was followed for 6 months and his visual acuity was 20/60 at his most recent exam. Ophthalmoscopic examination disclosed an inactive lesion in that eye. The beneficial effect of infliximab in this patient may reflect the antiangiogenic properties of this drug and could also be attributed to its inhibitory properties for vascular endothelial growth factor actions.6 These findings suggest that therapeutic blockade of TNF, for some retinal vascular diseases, merits further investigation.

References

  1. Gass JDM. Macular dysfunction caused by retinal vascular diseases. In: Gass JDM, ed. StereoscopicAtlas of Macular Diseases, Diagnosis and Treatment. St Louis, Mo: Mosby–Year Book; 1997: 437–599.
  2. Gass JDM. Cavernous hemangioma of the retina: a neuro-oculo-cutaneous syndrome. Am J Ophthalmol. 1971;71:799–814.
  3. Lewis RA, Cohen MH, Wise GN. Cavernous hemangioma of the retina and optic disc. Br J Ophthalmol. 1975;59:422–424. doi:10.1136/bjo.59.8.422 [CrossRef]
  4. Soheila Naftchi and Morten la Cour. A case of central visual loss in a child due to macular cavernous haemangioma of the retina. Acta Ophthalmol. Scand. 2002:80: 550–552.
  5. Japiassú RM, Brasil OF, Cunha AL, de Souza EC. Regression of vasoproliferative tumor with systemic infliximab. Ophthalmic Surg Lasers Imaging. 2008Jul–Aug;39(4):348–9. doi:10.3928/15428877-20080701-09 [CrossRef]
  6. Sfikakis P.P., Kollias G., TNF biology in experimental and clinical arthritis, Curr Opin Rheumatol. 2003:15: 380–386. doi:10.1097/00002281-200307000-00003 [CrossRef]
Authors

From Pedro Ernesto University Hospital (RMJ), State University of Rio de Janeiro, Rio de Janeiro; and Vision Institute (OFMB, ECDS), Federal University of São Paulo, São Paulo, Brazil.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Ricardo Miguel Japiassú, MD, Rua Desembargador Isidro 121 APTO. 202-B – Tijuca, Rio de Janeiro – RJ, Brazil, CEP 20521-160.

10.3928/15428877-20100215-34

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