AC TA 0 P H T H A L M O LO G I C A
69 (i99lj 805-809
CASE REPORT
Exudative retinal detachment in familial pulmonary hypertension lngimundur Gislason, Fridbert Jonasson and Einar Stefansson Department of Ophthalmology,University of Iceland, Landakotsspitali,Reykjavik,Iceland
Abstract. In a family with pulmonary hypertension, one woman developed bilateral exudative retinal detachments and her daughter developed a unilateral exudative retinal detachment during pregnancy. Treatment with Acetazolamideprovided quick recovery in the bilateral case, whereas the unilateral exudative detachment recovered without treatment after delivery. Exudative retinal detachments have not previously been associated with pulmonary hypertension. Key words: exudative retinal detachment - pulmonary hypertension - Acetazolamide - retinal pigment epithelium.
Exudative retinal detachment is known to occur in a variety of vascular disorders including malignant arterial hypertension, toxemia of pregnancy and disseminated intravascular coagulatopathy (Brismer & Schimmelpfennig 1989; Stropes & Luft 1977). It is also sometimes found in inflammatory eye diseases and intraocular tumours. In this paper we present two patients, a mother and her daughter with exudative retinal detachments and familial pulmonary hypertension. As far as we know this association has not been previously reported.
Case reports Case 1 A 28-year-old female, daughter of Case No. 2. In August 1988,when she had been pregnant for four months, the patient developed dyspnoea which
worsened until in October when it stabilized. She was fmst examined by an ophthalmologist in November 1988 who found dilated episcleral- and conjunctival veins on the left bulbus. Visual acuity was 616 in each eye and fundoscopy revealed no abnormalities. Both eyes were emmetropic. There was a three-year history of intermittent red left eye without any sign of inflammation or conjunctival discharge. There was no history of decreased visual acuity or painful eyes. There was no history of arterial hypertension, collagen vascular or rheumatoid diseases. An elective cesarean section was successfully performed in the beginning of December. The patient was then admitted to the Department of Cardiology for cardiac catherterization because of suspected pulmonary hypertension. The pressure in the pulmonary arteries was found to be moderately increased with a systolic pulmonary artery pressure of 50 mmHg and the patient was diagnosed as having primary pulmonary hypertension. Few days later the patient complained of decreased vision in the left eye and she was again examined by an ophthalmologist on December 20th, 1988. The vision was 6/6 in the right eye and 6/60 in the left eye. The episcleral and conjunctival veins in both eyes were greatly dilated (Fig. l),and in the left eye there was a central exudative retinal detachment (Fig. 2). There was a low choroidal detachment in the retinal periphery, most marked in the lower quadrants. The retinal vessels were normal. The fundus in the right eye was normal. 805
Fig. 1. (Gislason,Jonasson & Stefansson). Case 1. Anterior view of the right eye, showing dilated conjunctival and episcleral veins.
Fig. 2. (Gislason,Jonasson & Stefknsson).Case 1. Fundus photograph of the left eye, showing an exudative retinal detachment in the posterior fundus.
The findings in the eyes were at the time thought to be caused by thrombotic occlusion in the venous system and anticoagulation treatment was started, but had to be discontinued in January 1989 because of a heavy menstrual bleeding. The patient did not return for a follow-upexamination until November 1989. She then reported episodes of decreased vision in the left eye of approx. six month's duration, the last episode occurring in earlyJune 1989.The visual acuity was 615 in the right eye and 6/6 in the left eye. The episcleral and conjunctival veins in both eyes were dilated but not as marked as in December 1988. There
were no signs of retinal detachment but in the fundus of the left eye there was a slight irregularity of the pigmentation in the macula. The patient has two children and an ophthalmological examination of her nine-year-old daughter did not reveal any abnormalities.
Case 2 A 49-year-old female with essential pulmonary hypertension, the mother of Case 1, underwent cardiac catheterization in November of 1988 and felt that her vision was reduced for a few days following this procedure. However, her eyes were not
E
A
Fig 3. (Gislason,Jbnasson & Stefansson).Case 2. Fundus photograph (A) and fluorescein angiogram (B) on September 11, 1989. Note pale spots on the fundus photograph.
806
B
A
Fig 4. (Cislason,Jonasson & Stefansson).Case 2. Fundus photograph (A) and fluorescein angiogram (B) on October 16, 1989. The exudative retinal elevation is seen, and there is diffuse leakage of fluorescein into the subretinal space (B).
examined at that time and vision recovered spontaneously. She was re-examined on September 11, 1989 when the visual acuity was 6/6 in the right eye and 6/7.5 in the left eye. Both eyes were emmetropic. The intraocular tension was 12 mmHg in the right eye and 22 mmHg in the left eye. Slitlamp examination of anterior segments revealed tortuous and dilated episcleral and conjunctival vessels, but was otherwise normal. Gonioscopy revealed blood in Schlemm’s canal on the left side, but not on the right side. Fundus examination revealed many pale yellowish flat spots at the level of the retinal pigment epithelium (Fig. 3). In October of 1989 her vision deteriorated rapidly in both eyes and on examination on October 16, she was found to have visual acuity 2/60 in both eyes and large central exudative retinal detachments in both eyes (Fig. 4).She also complained of ankle edema and headaches. There was no history of painful eyes. Treatment was initiated with Furosemide 80 mg p.0. and the following day Acetazolamide 250 mg p.0. q.i.d. and the exudative detachment rapidly decreased. On October 18 vision.had improved to 6/7.5 and 6/6 in the right and left eye, respectively, and the subretinal fluid had completely disappeared in the left eye while a very small amount remained in the right eye. She remained on Acetazolamide 125 mg p.0. b.i.d. and one week later the subretinal fluid had completely disappeared in both eyes. The yellowish spots remained in the fundus, now with the addition of hyperpigmented spots in the retinal pigment epithelium in both
eyes (Fig. 5). When the Acetazolamide treatment was stopped the retinal detachments recurred. Acetazolamide treatment was re-established and the detachments subsequently subsided. The episcleral and conjunctival vessels remained dilated and tortuous and the intraocular tension was 12 and 23 mmHg in the right and left eyes, respectively. The patient was last examined on March 30, 1990. She had then taken Acetazolamide 125 mg p.0. daily for several months and remained in stable condition regarding visual acuity and attachment of the retina in both eyes. Cardiac catheterization was performed in No-
Fig. 5. (Cislason,Jonasson & Stefansson).Case 2. Fundus photograph taken after recovery on October 23,1989. The subretinal fluid and the pale spots have disappeared.
807
vember of 1988 and revealed pulmonary hypertension with the pulmonary artery pressure 125155 mmHg whereas the aortic blood pressure was 951 65 mmHg. The PO, in the aorta was 47 mmHg and 30 mmHg in the pulmonary artery. She did not have right heart failure and the jugular venous pressure and the liver size were normal. There was no history of arterial hypertension, collagen vascular or rheumatoid diseases. The patient has three daughters and two sons. One daughter is Case 1 in this report. The oldest daughter is born 1957 and she had some clinical signs of pulmonary hypertension, but cardiac catheterization had not revealed any elevation of pulmonary artery pressure. Nothing abnormal was found when her eyes were examined. The two sons have been examined by the authors and do not have any signs or symptom of eye disease nor of pulmonary hypertension.
Discussion We report on a female and her daughter, both having familial pulmonary hypertension and bilateral exudative retinal detachment and temporary visual loss. The ophthalmic literature is very scant in describing the coexistence of exudative retinal detachments and disturbances in the venous circulation. Exudative detachments have, however, been associated with extreme venous congestion (DukeElder 1962). Exudative retinal detachment has been reported in toxemia of pregnancy and other forms of malignant arterial hypertension. Bilateral exudative retinal detachments have been described in a normal pregnancy. Re-attachment started promptly after delivery @rismar & Schimmelpfening 1989; Stropes & Luft 1977). Exudative retinal detachment is sometimes seen in various specfic choroidal diseases such as malignant melanomas and diseases affecting the retinal pigment epithelium. Dilated conjunctival and episcleral vessels may be found in the idiopathic uveal effusion syndrome (Gass & Jellow 1982) and also in secondary uveal effusion, for example in carotid-cavernous and dural-arteriovenous fistulas (Harbison et al. 1978).In our cases some pale yellowish, as well as hyperpigmented spots were seen at the level of the retinal pigment epithelium which may represent 808
leakage at the level of the retinal pigment epithelium or secondary changes caused by the detachment itself. We believed that the exudative retinal detachments might be related to increased systemic venous pressure and this was supported by the finding of dilated and tortuous episcleral and conjunctival vessels, blood in Schlemm’s canal and the ocular hypertension seen on the left side in Case 2. However, the patients did not show general s i p of increased venous pressure, with the possible exception of the ankle edema in Case 2, and slight elevation of systemicvenous pressure, such as are associated with right sided heart failure, has not previously been reported to cause exudative retinal detachments. The rapid recovery seen with treatment of Furosemide and in particular Acetazolamide in Case 2 is interesting. This may either be coincidental or work in two ways, on the one hand by decreasing the systemic fluid load or on the other hand by increasing the retinal pigment epithelial pumping and reabsorption of subretinal fluid. The last consideration is the more plausible in light of a recent prospective study that showed that patients with chronic macular edema responded to oral Acetazolamide with resolution of edema and improvement of visual acuity when there was evidence of retinal pigment epithelial dysfunction (Cox et al. 1988). In both our cases there was some irregularity of the retinal pigment epithelium in the macula, which we thought to be secondary to the repeated elevation of the sensory retina, but may rather represent evidence of debilitated retinal pigment epithelium. However, experimental research has shown that intravenous administration of Acetazolamidein high doses to rabbits does not alter the reabsorption rate of subretinal serum significantly (Kawano & Marmor 1988).
References Brismar G & SchimmelpfennigW (1989):Bilateralewdative retinal detachment in pregnancy. Acta Ophthalmol (Coped) 67: 699-702. Cox S N, Hay E & Bird A C (1988): Treatment of chronic macular edema with Acetazolamide. Arch Ophthalmol 106 1190-1195. Duke-Elder S (1962):System of Ophthalmology.Vol X, p 775. Henry Kampton, London.
Gass J D M & Jellow S (1982): Serous detachment of the choroid, ciliary body, and retina (uveal effusion syndrome). Ophthalmology 89: 1018-1032. Harbison J W, Guerry D & Wiesinger H (1978):Dural arteriovenous fistula and spontaneous choroidal detachment: new cause of an old disease. Br J Ophthalmol62: 483-490. Kawano S & Marmor M F (1988):Metabolic influences on the absorption of serous subretinal fluid. Invest Ophthalmol Vis Sci 29: 1255-1257.
Stropes L L & Luft F C (1977):Hypertensive crisis with bilateral bullous retinal detachments. JAMA 238: 19481949. Received on February 15th, 1991. Author's address:
Ingimundur Gislason, MD, Department of Ophthalmology, University of Iceland, Landakotsspitali, IS-101 Reykjavik, Iceland.
809
69 (i99lj 805-809
CASE REPORT
Exudative retinal detachment in familial pulmonary hypertension lngimundur Gislason, Fridbert Jonasson and Einar Stefansson Department of Ophthalmology,University of Iceland, Landakotsspitali,Reykjavik,Iceland
Abstract. In a family with pulmonary hypertension, one woman developed bilateral exudative retinal detachments and her daughter developed a unilateral exudative retinal detachment during pregnancy. Treatment with Acetazolamideprovided quick recovery in the bilateral case, whereas the unilateral exudative detachment recovered without treatment after delivery. Exudative retinal detachments have not previously been associated with pulmonary hypertension. Key words: exudative retinal detachment - pulmonary hypertension - Acetazolamide - retinal pigment epithelium.
Exudative retinal detachment is known to occur in a variety of vascular disorders including malignant arterial hypertension, toxemia of pregnancy and disseminated intravascular coagulatopathy (Brismer & Schimmelpfennig 1989; Stropes & Luft 1977). It is also sometimes found in inflammatory eye diseases and intraocular tumours. In this paper we present two patients, a mother and her daughter with exudative retinal detachments and familial pulmonary hypertension. As far as we know this association has not been previously reported.
Case reports Case 1 A 28-year-old female, daughter of Case No. 2. In August 1988,when she had been pregnant for four months, the patient developed dyspnoea which
worsened until in October when it stabilized. She was fmst examined by an ophthalmologist in November 1988 who found dilated episcleral- and conjunctival veins on the left bulbus. Visual acuity was 616 in each eye and fundoscopy revealed no abnormalities. Both eyes were emmetropic. There was a three-year history of intermittent red left eye without any sign of inflammation or conjunctival discharge. There was no history of decreased visual acuity or painful eyes. There was no history of arterial hypertension, collagen vascular or rheumatoid diseases. An elective cesarean section was successfully performed in the beginning of December. The patient was then admitted to the Department of Cardiology for cardiac catherterization because of suspected pulmonary hypertension. The pressure in the pulmonary arteries was found to be moderately increased with a systolic pulmonary artery pressure of 50 mmHg and the patient was diagnosed as having primary pulmonary hypertension. Few days later the patient complained of decreased vision in the left eye and she was again examined by an ophthalmologist on December 20th, 1988. The vision was 6/6 in the right eye and 6/60 in the left eye. The episcleral and conjunctival veins in both eyes were greatly dilated (Fig. l),and in the left eye there was a central exudative retinal detachment (Fig. 2). There was a low choroidal detachment in the retinal periphery, most marked in the lower quadrants. The retinal vessels were normal. The fundus in the right eye was normal. 805
Fig. 1. (Gislason,Jonasson & Stefansson). Case 1. Anterior view of the right eye, showing dilated conjunctival and episcleral veins.
Fig. 2. (Gislason,Jonasson & Stefknsson).Case 1. Fundus photograph of the left eye, showing an exudative retinal detachment in the posterior fundus.
The findings in the eyes were at the time thought to be caused by thrombotic occlusion in the venous system and anticoagulation treatment was started, but had to be discontinued in January 1989 because of a heavy menstrual bleeding. The patient did not return for a follow-upexamination until November 1989. She then reported episodes of decreased vision in the left eye of approx. six month's duration, the last episode occurring in earlyJune 1989.The visual acuity was 615 in the right eye and 6/6 in the left eye. The episcleral and conjunctival veins in both eyes were dilated but not as marked as in December 1988. There
were no signs of retinal detachment but in the fundus of the left eye there was a slight irregularity of the pigmentation in the macula. The patient has two children and an ophthalmological examination of her nine-year-old daughter did not reveal any abnormalities.
Case 2 A 49-year-old female with essential pulmonary hypertension, the mother of Case 1, underwent cardiac catheterization in November of 1988 and felt that her vision was reduced for a few days following this procedure. However, her eyes were not
E
A
Fig 3. (Gislason,Jbnasson & Stefansson).Case 2. Fundus photograph (A) and fluorescein angiogram (B) on September 11, 1989. Note pale spots on the fundus photograph.
806
B
A
Fig 4. (Cislason,Jonasson & Stefansson).Case 2. Fundus photograph (A) and fluorescein angiogram (B) on October 16, 1989. The exudative retinal elevation is seen, and there is diffuse leakage of fluorescein into the subretinal space (B).
examined at that time and vision recovered spontaneously. She was re-examined on September 11, 1989 when the visual acuity was 6/6 in the right eye and 6/7.5 in the left eye. Both eyes were emmetropic. The intraocular tension was 12 mmHg in the right eye and 22 mmHg in the left eye. Slitlamp examination of anterior segments revealed tortuous and dilated episcleral and conjunctival vessels, but was otherwise normal. Gonioscopy revealed blood in Schlemm’s canal on the left side, but not on the right side. Fundus examination revealed many pale yellowish flat spots at the level of the retinal pigment epithelium (Fig. 3). In October of 1989 her vision deteriorated rapidly in both eyes and on examination on October 16, she was found to have visual acuity 2/60 in both eyes and large central exudative retinal detachments in both eyes (Fig. 4).She also complained of ankle edema and headaches. There was no history of painful eyes. Treatment was initiated with Furosemide 80 mg p.0. and the following day Acetazolamide 250 mg p.0. q.i.d. and the exudative detachment rapidly decreased. On October 18 vision.had improved to 6/7.5 and 6/6 in the right and left eye, respectively, and the subretinal fluid had completely disappeared in the left eye while a very small amount remained in the right eye. She remained on Acetazolamide 125 mg p.0. b.i.d. and one week later the subretinal fluid had completely disappeared in both eyes. The yellowish spots remained in the fundus, now with the addition of hyperpigmented spots in the retinal pigment epithelium in both
eyes (Fig. 5). When the Acetazolamide treatment was stopped the retinal detachments recurred. Acetazolamide treatment was re-established and the detachments subsequently subsided. The episcleral and conjunctival vessels remained dilated and tortuous and the intraocular tension was 12 and 23 mmHg in the right and left eyes, respectively. The patient was last examined on March 30, 1990. She had then taken Acetazolamide 125 mg p.0. daily for several months and remained in stable condition regarding visual acuity and attachment of the retina in both eyes. Cardiac catheterization was performed in No-
Fig. 5. (Cislason,Jonasson & Stefansson).Case 2. Fundus photograph taken after recovery on October 23,1989. The subretinal fluid and the pale spots have disappeared.
807
vember of 1988 and revealed pulmonary hypertension with the pulmonary artery pressure 125155 mmHg whereas the aortic blood pressure was 951 65 mmHg. The PO, in the aorta was 47 mmHg and 30 mmHg in the pulmonary artery. She did not have right heart failure and the jugular venous pressure and the liver size were normal. There was no history of arterial hypertension, collagen vascular or rheumatoid diseases. The patient has three daughters and two sons. One daughter is Case 1 in this report. The oldest daughter is born 1957 and she had some clinical signs of pulmonary hypertension, but cardiac catheterization had not revealed any elevation of pulmonary artery pressure. Nothing abnormal was found when her eyes were examined. The two sons have been examined by the authors and do not have any signs or symptom of eye disease nor of pulmonary hypertension.
Discussion We report on a female and her daughter, both having familial pulmonary hypertension and bilateral exudative retinal detachment and temporary visual loss. The ophthalmic literature is very scant in describing the coexistence of exudative retinal detachments and disturbances in the venous circulation. Exudative detachments have, however, been associated with extreme venous congestion (DukeElder 1962). Exudative retinal detachment has been reported in toxemia of pregnancy and other forms of malignant arterial hypertension. Bilateral exudative retinal detachments have been described in a normal pregnancy. Re-attachment started promptly after delivery @rismar & Schimmelpfening 1989; Stropes & Luft 1977). Exudative retinal detachment is sometimes seen in various specfic choroidal diseases such as malignant melanomas and diseases affecting the retinal pigment epithelium. Dilated conjunctival and episcleral vessels may be found in the idiopathic uveal effusion syndrome (Gass & Jellow 1982) and also in secondary uveal effusion, for example in carotid-cavernous and dural-arteriovenous fistulas (Harbison et al. 1978).In our cases some pale yellowish, as well as hyperpigmented spots were seen at the level of the retinal pigment epithelium which may represent 808
leakage at the level of the retinal pigment epithelium or secondary changes caused by the detachment itself. We believed that the exudative retinal detachments might be related to increased systemic venous pressure and this was supported by the finding of dilated and tortuous episcleral and conjunctival vessels, blood in Schlemm’s canal and the ocular hypertension seen on the left side in Case 2. However, the patients did not show general s i p of increased venous pressure, with the possible exception of the ankle edema in Case 2, and slight elevation of systemicvenous pressure, such as are associated with right sided heart failure, has not previously been reported to cause exudative retinal detachments. The rapid recovery seen with treatment of Furosemide and in particular Acetazolamide in Case 2 is interesting. This may either be coincidental or work in two ways, on the one hand by decreasing the systemic fluid load or on the other hand by increasing the retinal pigment epithelial pumping and reabsorption of subretinal fluid. The last consideration is the more plausible in light of a recent prospective study that showed that patients with chronic macular edema responded to oral Acetazolamide with resolution of edema and improvement of visual acuity when there was evidence of retinal pigment epithelial dysfunction (Cox et al. 1988). In both our cases there was some irregularity of the retinal pigment epithelium in the macula, which we thought to be secondary to the repeated elevation of the sensory retina, but may rather represent evidence of debilitated retinal pigment epithelium. However, experimental research has shown that intravenous administration of Acetazolamidein high doses to rabbits does not alter the reabsorption rate of subretinal serum significantly (Kawano & Marmor 1988).
References Brismar G & SchimmelpfennigW (1989):Bilateralewdative retinal detachment in pregnancy. Acta Ophthalmol (Coped) 67: 699-702. Cox S N, Hay E & Bird A C (1988): Treatment of chronic macular edema with Acetazolamide. Arch Ophthalmol 106 1190-1195. Duke-Elder S (1962):System of Ophthalmology.Vol X, p 775. Henry Kampton, London.
Gass J D M & Jellow S (1982): Serous detachment of the choroid, ciliary body, and retina (uveal effusion syndrome). Ophthalmology 89: 1018-1032. Harbison J W, Guerry D & Wiesinger H (1978):Dural arteriovenous fistula and spontaneous choroidal detachment: new cause of an old disease. Br J Ophthalmol62: 483-490. Kawano S & Marmor M F (1988):Metabolic influences on the absorption of serous subretinal fluid. Invest Ophthalmol Vis Sci 29: 1255-1257.
Stropes L L & Luft F C (1977):Hypertensive crisis with bilateral bullous retinal detachments. JAMA 238: 19481949. Received on February 15th, 1991. Author's address:
Ingimundur Gislason, MD, Department of Ophthalmology, University of Iceland, Landakotsspitali, IS-101 Reykjavik, Iceland.
809
