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Exudative retinal detachment and primary pulmonary hypertension
a r c h s o c e s p o f t a l m o l . 2 0 1 3;8 8(5):189–192
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Exudative retinal detachment and primary pulmonary hypertension夽 J.L. Sánchez-Sevila ∗ , M. Martínez-Rubio, I. López-Meca, J. Belmonte-Martínez Servicio de Oftalmología, Hospital General Universitario de Alicante, Alicante, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Case report: A 60-year-old woman who was seen in the emergency department due to sudden
Received 8 April 2011
loss of vision in left eye. The fundoscopy study showed exudative retinal detachment. The
Accepted 8 April 2012
patient referred to dyspnea and peripheral edema of one-year duration during the anamne-
Available online 29 June 2013
sis. The systematic study revealed the existence of pulmonary hypertension, and she was given diuretic treatment (furosemide). After 48 h the detachment was resolved. Subsequent
Keywords:
studies identified a primary pulmonary hypertension.
Pulmonary hypertension
Discussion: Exudative retinal detachment can be the first clinical sign of a serious disease
Exudative retinal detachment
like primary pulmonary hypertension. The ophthalmologist can be the first to detect this
Primary pulmonary hypertension
disease. © 2011 Sociedad Española de Oftalmología. Published by Elsevier España, S.L. All rights
Dyspnea
reserved.
Furosemide
Desprendimiento de retina exudativo e hipertensión pulmonar primaria r e s u m e n Palabras clave:
˜ Caso clínico: Mujer de 60 anos que acudió de urgencia por pérdida súbita de visión en ojo
Hipertensión pulmonar
izquierdo. El estudio fundoscópico mostró desprendimiento de retina exudativo. En la anam-
Desprendimiento de retina
˜ de evolución y edemas periféricos. El estudio sistémico reveló nesis refería disnea de un ano
exudativo
la existencia de hipertensión pulmonar recibiendo tratamiento diurético (furosemida). El
Hipertensión pulmonar primaria
desprendimiento se resolvió a las 48 h. Estudios posteriores determinaron una hipertensión
Disnea
pulmonar primaria.
Furosemida
Discusión: El desprendimiento de retina exudativo puede ser el primer signo clínico de una enfermedad grave como la hipertensión pulmonar primaria. El oftalmólogo puede ser el primero en detectar esta enfermedad. © 2011 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L. Todos los derechos reservados.
夽
Please cite this article as: Sánchez-Sevila JL, et al. Desprendimiento de retina exudativo e hipertensión pulmonar primaria. Arch Soc Esp Oftalmol. 2013;88:189–92. ∗ Corresponding author. E-mail address: [email protected] (J.L. Sánchez-Sevila). 2173-5794/$ – see front matter © 2011 Sociedad Española de Oftalmología. Published by Elsevier España, S.L. All rights reserved.
190
a r c h s o c e s p o f t a l m o l . 2 0 1 3;8 8(5):189–192
Introduction Exudative retinal detachment can be due to different ocular and extraocular causes. Primary pulmonary hypertension is a disease of unknown etiology that can exhibit a diversity of ophthalmological expressions as the result of diminished venous flow caused by increased systemic venous pressure.
Case report Female, 60, who visited the Emergency Service due to blurred vision in the left eye with a few hours of evolution and without the relevant symptoms. She did not refer arterial hypertension history or diabetes mellitus. The ophthalmic assessment gave a visual acuity of 1 in the right eye and of 0.1 in the left eye which did not improve with correction. Under biomicroscopic exploration, the anterior pole was observed to be normal, the same as the right eye ocular fundus. However, the left eye exhibited retina detachment in the macular area and temporal retinal area (Fig. 1) compatible with serous detachment. After 24–36 h of the blurred vision onset, fluorescein angiography and optic coherence tomography (OCT, Stratus Carl Zeiss) were taken. The angiography showed contrast aggregation in the perimacular area of the left eye which was more evident in late phases (Fig. 2). The OCT in the right eye was normal, while the left eye exhibited neurosensory detachment at the macular level with subfoveal liquid and intraretinal edema (Fig. 3). After performing a new anamnesis, the patient referred dyspnea when carrying out medium efforts as well as lower limb edema under one year of evolution. Accordingly, the cardiology and pneumology services were consulted. A systemic study revealed the existence of pulmonary hypertension, whereupon treatment was established with furosemide (approximately 48 h after the onset of diminished vision). The retina detachment resolved entirely 48 h after beginning the diuretic treatment (Figs. 4 and 5). After completing the study, it was concluded that the condition was primary pulmonary hypertension and vasodilating therapy was established. Three months after the episode, the
Fig. 1 – Exudative retina detachment in the left eye.
Fig. 2 – Left eye fluorescein angiography with aggregation of perimacular contrast in late phases.
Fig. 3 – Left eye optic coherence tomography with neurosensory detachment at the macular level and intraretinal edema.
a r c h s o c e s p o f t a l m o l . 2 0 1 3;8 8(5):189–192
191
Fig. 4 – Complete resolution of the exudative detachment in the left eye 48 h after beginning treatment.
Fig. 5 – Left eye optic coherence tomography 48 h after beginning treatment showing the resolution of the detachment at the macular level and of the intraretinal edema.
visual acuity in the left eye is of 0.9 and the patient remains stable, in follow-up by pneumology.
Discussion Primary pulmonary hypertension is a disease of unknown etiology characterized by an increase of pulmonary pressure above 25 mmHg at rest and of 30 mmHg while exercising due to vasoconstriction of the pulmonary vascular Tree.1,2 Predisposing genetic factors associated to familial cases with dominant autosomic inheritance has been identified.3–5 In what concerns pathogeny, histological and functional alterations of endothelial cells of pulmonary blood vessels have been identified.1 Pulmonary hypertension gives rise to right side cardiac insufficiency, increase of systemic, orbitary and episcleral venous pressure with outgoing venous flow reduction, producing ocular complications.3
Said ocular expressions include dilatation of the conjunctival and episcleral veins,2 central retina vein occlusion, foveal effusion with choroidal detachment, intraocular pressure increase (open angle secondary glaucoma) and exudative retinal detachment.2,3 In addition, cases with subacute myopia and exophthalmos have been described.5 Exudative detachment arises due to the inability of the retinal pigment epithelium to pump the excess liquid which accumulates in the subretinal space due to increased hydrostatic pressure in the choriocapillary.3 Therapeutic options are based on vasodilating therapy, support therapy and pulmonary transplant as the last resource.1 Diuretics have a beneficial effect on patients with pulmonary hypertension, particularly in those with right ventricle failure and systemic venous congestion as in the patient of this report. The significance of this case resides in the role of the ophthalmologist to suspect major systemic diseases on the basis of ocular signs.
192
a r c h s o c e s p o f t a l m o l . 2 0 1 3;8 8(5):189–192
Conflict of interests No conflict of interest has been declared by the authors.
references
1. Crespo Martínez C, Morales León V, Alonso Ramos H, Alonso Ortiz B, Molero Gómez R. Hipertensión pulmonar primaria y su tratamiento. Farm Hosp. 2004;28:48–55. 2. Offret H, Labetoulle M, Offret O. Manifestations oculaires de l’hypertension primitive de l’artère pulmonaire. J Fr Ophtalmol. 2009;32:56e1–7e.
3. Senthil S, Kaur B, Jalali S, Garudadri C. Secondary open-angle glaucoma and central retinal vein occlusion in a patient with primary pulmonary hypertension. Ophtalmic Surg Lasers Imaging. 2009;40:50–3. 4. Saran BR, Brucker AJ, Bandello F, Verougstraete C. Familial primary pulmonary hypertension and associated ocular findings. Retina. 2001;21:34–9. 5. Paire V, Weber M, Salama B, Agla EK, Closs-Prophète F, Banchereau M. Hypertension artèrielle pulmonaire primitive familial rèvélée par l’association bilaterale chémosis, myopisation et exophtalmie. J Fr Ophtalmol. 2006;29:e27.
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Exudative retinal detachment and primary pulmonary hypertension夽 J.L. Sánchez-Sevila ∗ , M. Martínez-Rubio, I. López-Meca, J. Belmonte-Martínez Servicio de Oftalmología, Hospital General Universitario de Alicante, Alicante, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Case report: A 60-year-old woman who was seen in the emergency department due to sudden
Received 8 April 2011
loss of vision in left eye. The fundoscopy study showed exudative retinal detachment. The
Accepted 8 April 2012
patient referred to dyspnea and peripheral edema of one-year duration during the anamne-
Available online 29 June 2013
sis. The systematic study revealed the existence of pulmonary hypertension, and she was given diuretic treatment (furosemide). After 48 h the detachment was resolved. Subsequent
Keywords:
studies identified a primary pulmonary hypertension.
Pulmonary hypertension
Discussion: Exudative retinal detachment can be the first clinical sign of a serious disease
Exudative retinal detachment
like primary pulmonary hypertension. The ophthalmologist can be the first to detect this
Primary pulmonary hypertension
disease. © 2011 Sociedad Española de Oftalmología. Published by Elsevier España, S.L. All rights
Dyspnea
reserved.
Furosemide
Desprendimiento de retina exudativo e hipertensión pulmonar primaria r e s u m e n Palabras clave:
˜ Caso clínico: Mujer de 60 anos que acudió de urgencia por pérdida súbita de visión en ojo
Hipertensión pulmonar
izquierdo. El estudio fundoscópico mostró desprendimiento de retina exudativo. En la anam-
Desprendimiento de retina
˜ de evolución y edemas periféricos. El estudio sistémico reveló nesis refería disnea de un ano
exudativo
la existencia de hipertensión pulmonar recibiendo tratamiento diurético (furosemida). El
Hipertensión pulmonar primaria
desprendimiento se resolvió a las 48 h. Estudios posteriores determinaron una hipertensión
Disnea
pulmonar primaria.
Furosemida
Discusión: El desprendimiento de retina exudativo puede ser el primer signo clínico de una enfermedad grave como la hipertensión pulmonar primaria. El oftalmólogo puede ser el primero en detectar esta enfermedad. © 2011 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L. Todos los derechos reservados.
夽
Please cite this article as: Sánchez-Sevila JL, et al. Desprendimiento de retina exudativo e hipertensión pulmonar primaria. Arch Soc Esp Oftalmol. 2013;88:189–92. ∗ Corresponding author. E-mail address: [email protected] (J.L. Sánchez-Sevila). 2173-5794/$ – see front matter © 2011 Sociedad Española de Oftalmología. Published by Elsevier España, S.L. All rights reserved.
190
a r c h s o c e s p o f t a l m o l . 2 0 1 3;8 8(5):189–192
Introduction Exudative retinal detachment can be due to different ocular and extraocular causes. Primary pulmonary hypertension is a disease of unknown etiology that can exhibit a diversity of ophthalmological expressions as the result of diminished venous flow caused by increased systemic venous pressure.
Case report Female, 60, who visited the Emergency Service due to blurred vision in the left eye with a few hours of evolution and without the relevant symptoms. She did not refer arterial hypertension history or diabetes mellitus. The ophthalmic assessment gave a visual acuity of 1 in the right eye and of 0.1 in the left eye which did not improve with correction. Under biomicroscopic exploration, the anterior pole was observed to be normal, the same as the right eye ocular fundus. However, the left eye exhibited retina detachment in the macular area and temporal retinal area (Fig. 1) compatible with serous detachment. After 24–36 h of the blurred vision onset, fluorescein angiography and optic coherence tomography (OCT, Stratus Carl Zeiss) were taken. The angiography showed contrast aggregation in the perimacular area of the left eye which was more evident in late phases (Fig. 2). The OCT in the right eye was normal, while the left eye exhibited neurosensory detachment at the macular level with subfoveal liquid and intraretinal edema (Fig. 3). After performing a new anamnesis, the patient referred dyspnea when carrying out medium efforts as well as lower limb edema under one year of evolution. Accordingly, the cardiology and pneumology services were consulted. A systemic study revealed the existence of pulmonary hypertension, whereupon treatment was established with furosemide (approximately 48 h after the onset of diminished vision). The retina detachment resolved entirely 48 h after beginning the diuretic treatment (Figs. 4 and 5). After completing the study, it was concluded that the condition was primary pulmonary hypertension and vasodilating therapy was established. Three months after the episode, the
Fig. 1 – Exudative retina detachment in the left eye.
Fig. 2 – Left eye fluorescein angiography with aggregation of perimacular contrast in late phases.
Fig. 3 – Left eye optic coherence tomography with neurosensory detachment at the macular level and intraretinal edema.
a r c h s o c e s p o f t a l m o l . 2 0 1 3;8 8(5):189–192
191
Fig. 4 – Complete resolution of the exudative detachment in the left eye 48 h after beginning treatment.
Fig. 5 – Left eye optic coherence tomography 48 h after beginning treatment showing the resolution of the detachment at the macular level and of the intraretinal edema.
visual acuity in the left eye is of 0.9 and the patient remains stable, in follow-up by pneumology.
Discussion Primary pulmonary hypertension is a disease of unknown etiology characterized by an increase of pulmonary pressure above 25 mmHg at rest and of 30 mmHg while exercising due to vasoconstriction of the pulmonary vascular Tree.1,2 Predisposing genetic factors associated to familial cases with dominant autosomic inheritance has been identified.3–5 In what concerns pathogeny, histological and functional alterations of endothelial cells of pulmonary blood vessels have been identified.1 Pulmonary hypertension gives rise to right side cardiac insufficiency, increase of systemic, orbitary and episcleral venous pressure with outgoing venous flow reduction, producing ocular complications.3
Said ocular expressions include dilatation of the conjunctival and episcleral veins,2 central retina vein occlusion, foveal effusion with choroidal detachment, intraocular pressure increase (open angle secondary glaucoma) and exudative retinal detachment.2,3 In addition, cases with subacute myopia and exophthalmos have been described.5 Exudative detachment arises due to the inability of the retinal pigment epithelium to pump the excess liquid which accumulates in the subretinal space due to increased hydrostatic pressure in the choriocapillary.3 Therapeutic options are based on vasodilating therapy, support therapy and pulmonary transplant as the last resource.1 Diuretics have a beneficial effect on patients with pulmonary hypertension, particularly in those with right ventricle failure and systemic venous congestion as in the patient of this report. The significance of this case resides in the role of the ophthalmologist to suspect major systemic diseases on the basis of ocular signs.
192
a r c h s o c e s p o f t a l m o l . 2 0 1 3;8 8(5):189–192
Conflict of interests No conflict of interest has been declared by the authors.
references
1. Crespo Martínez C, Morales León V, Alonso Ramos H, Alonso Ortiz B, Molero Gómez R. Hipertensión pulmonar primaria y su tratamiento. Farm Hosp. 2004;28:48–55. 2. Offret H, Labetoulle M, Offret O. Manifestations oculaires de l’hypertension primitive de l’artère pulmonaire. J Fr Ophtalmol. 2009;32:56e1–7e.
3. Senthil S, Kaur B, Jalali S, Garudadri C. Secondary open-angle glaucoma and central retinal vein occlusion in a patient with primary pulmonary hypertension. Ophtalmic Surg Lasers Imaging. 2009;40:50–3. 4. Saran BR, Brucker AJ, Bandello F, Verougstraete C. Familial primary pulmonary hypertension and associated ocular findings. Retina. 2001;21:34–9. 5. Paire V, Weber M, Salama B, Agla EK, Closs-Prophète F, Banchereau M. Hypertension artèrielle pulmonaire primitive familial rèvélée par l’association bilaterale chémosis, myopisation et exophtalmie. J Fr Ophtalmol. 2006;29:e27.