Year : 1973 | Volume
: 21 | Issue : 4 | Page : 190--194
Bilateral retinopathy, papilloedema and retinal detachment in a case of phaeochromocytoma
H Saiduzzafar, K Nath, MW Ansari
Aligarh Muslim University Institute of Ophthalmology and Jawaharlal Nehru Medical College, Aligarh, India
Aligarh Muslim University Institute of Ophthalmology and Jawaharlal Nehru Medical College, Aligarh
|How to cite this article:|
Saiduzzafar H, Nath K, Ansari M W. Bilateral retinopathy, papilloedema and retinal detachment in a case of phaeochromocytoma.Indian J Ophthalmol 1973;21:190-194
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Saiduzzafar H, Nath K, Ansari M W. Bilateral retinopathy, papilloedema and retinal detachment in a case of phaeochromocytoma. Indian J Ophthalmol [serial online] 1973 [cited 2020 Jul 4 ];21:190-194
Available from: http://www.ijo.in/text.asp?1973/21/4/190/34628
BALLANTYNE AND MICHAELSON  have mentioned about a boy aged 14 years, in whom malignant hypertension gave rise to severe retinopathy, papilloedema and retinal detachment in both eyes. Three similar cases were reported by MIRATYNSKA AND KRAWICZ.  Such a sequence of events although frequently described in textbooks, has seldom been reported by way of detailed case-reports in the literature, and even SHELBOURNE  in his book has given it only a passing reference. Recently we studied a case of Phaeochromocytoma, leading to malignant hypertension, retinopathy, papilloedema and bilateral massive exudative peripheral retinal detachment. This clinical picture associated with phaeochromocytoma does not appear to have hitherto been published, therefore this case is being reported.
A Muslim girl, 17 years of age and unmarried, was carried into the Gandhi Eye Hospital, Aligarh, on 14-5-1966, in a very anxious state, and with the complaint of complete blackout of vision for 3 days. The chain of events had commenced a month prior to her admission, when she started getting occasional headaches in the occipital region. This gradually increased in intensity, frequency and duration, spreading all over the head and temples until 8 days earlier-when it became constant and very severe. She was having nausea throughout this period, and vomiting had started 16 days earlier and occurred 4-5 times a day. As a result she had hardly digested any food during the past week, was very constipated, emaciated, weak and pale. She had not slept at all for the previous 4 days. Fresh blood was detected in her urine nine days earlier and had lasted for three days; thereafter the urine passed was scanty, concentrated and black in colour. Drooping of the right side of the face was observed 6 days earlier, but it lasted only 2 days and was attributed to exposure to cold.
During the previous 15 days she was being treated for abdominal ailments on account of the nausea, vomiting and constipation, until 3 days earlier when she developed the sudden black-out in both the eyes, which forced the parents to seek advice at the Gandhi Eye Hospital.
Family history was non-contributory. Her parents, 4 brothers and 1 sister are all alive and healthy. The patient is the youngest issue.
Menstrual history: Periods started at the age of 14 and were normal until 4 months ago, when the interval between periods was reduced from 28 to 20 days, and their duration from 6 to 3 days.
The patient looked very anxious, pale, weak and emaciated. Her skin and tongue were dry. She was unable to move about by herself and had to be carried, and talked incoherently. She constantly stretched her fingers, limbs and feet. Her body was rather cold.
The apex-beat was thumping in the 5th left intercostal space, a little medial to the nipple line.
Both heart sounds were very loud, with little gap in between. The diastolic pause was reduced making the ventricular filling difficult.
Systolic blood-pressure fluctuated above 260, while diastolic was 190 mm Hg. Pulse was 180/min. with hardly any volume.
Respiratory rate was 32/min. Otherwise the respiratory system was normal.
The abdomen was soft, but tenderness was found in the left lumbar region.
Her eyelids were retracted, and the eyes were freely moving aimlessly in all directions, without any particular fixation. Pupils were dilated. The right pupil responded sluggishly to direct light, but not consensually. The left pupil had no direct reaction, but faint consensual reaction was present. Perception of light was confined to a small central area in the right eye, but was completely absent in the left eye. Other cranial nerves were normal. Externally the eyes presented no other abnormality. A greyish pupilary reflex was seen, specially in the right eye.
Ophthalmoscopic examination revealed five bluish-white globular ballooned-out areas of detached retina in the right eye, and four in the left eye. They were focussed with + 6D to + 10D [Figure 1]. Their surfaces were wavy and showed prominent veins, threadlike arteries (scarcely visible), scattered excessively whitish areas of exudates and multiple haemorrhages of various sizes. In the centre, a hazy outline of the optic discs could be made out. The discs were oedematous, swollen (+ 3D to + 4D), hyperaemic with a few superficial haemorrhages. The veins were dark, dilated and tortuous, describing the typical S-shaped curve crossing the disc-margin. The retina surrounding the disc and in between the detached areas showed oedema, haemorrhages, soft exudates, dilated and tortuous veins, with spastic thread-like arteries, completely obliterated in places. The A-V ratio was 1 : 1.5.
Urine examination on 16-5-1966 showed specific gravity - 1025 albumin 4+ sugar 2% large numbers of R.B.C., pus cells, and epithelial casts. This picture steadily improved, till it was normal after about 4 months of treatment.
Stool revealed no abnormality. [Table 1]
Platelet count, bleeding and coagulation time were normal.
Blood urea was initially 37.5 mgm/100 c.c. of blood nitrogen, but eventually came down to 17.9 mgm.
Sugar tolerance curve showed a steady rise, and was at 214 mgm/ 100 c.c. of blood even after 3 hours.
Intravenous pyelography was inconclusive.
Urine culture (catheter specimen) was sterile.
Regitine test for phaeochromocytoma was positive, and showed a fall of 44/70 mm. Hg.
Treatment and Progress
The patient was given anti-hypertensive, anti-diabetic drugs and general haematinics in consultation with the physicians.
After 24 hours her blood-pressure came down to 190/150 (from 260/ 190), and after 3 days more it was reduced to 140/100, at which stage the retinal detachment also got partially settled, and the pulse came down to 88/min. (from 180/min.). After one week 75% of the detached retina had settled down, and the pulse was 78/min.
After 10 days the detachment completely settled, and the papilloedema disappeared, but the optic disc started getting pale and showed progressive optic strophy. - With the subsidence of the papilloedema, pale confluent hard exudates were revealed in the papillomacular areas of both fundi [Figure 2].
During the next 4 months, the other retinal changes, viz., haemorrhages, exudates, retinal oedema and dilatation of veins showed slow regression. However, the pale hard exudates in the papillo-macular area were slow to regress. The arteries remained narrow and showed sheathing at most of the places and complete obliteration at some places. Gradually the veins also became narrow.
Initially, (when first seen) she had perception of light in the central field of the right eye only, and this too disappeared during the next ten days following admission when postpapilloedemic optic atrophy started. The left eye which had no vision at all on admission, started getting perception of light after a month, and during the next three months she could count fingers at one metre with the left eye.
The left lumbar region of the patient was tender on admission, and subsequently a mass could be palpated in this region, which appeared to be a renal mass. Regitine test (vide supra) confirmed the diagnosis of Phaeochromocytoma, and therefore the patient was transferred to surgical wards.
This case of phaeochromocytoma caused malignant hypertension, hypertensive retinopathy, papilloedema and bilateral bullous retinal detachment.
Although most text-books mention that malignant hypertension causes bilateral retinal detachment, yet the authors could actually find only four such cases reported in the literature. , However, we feel that such cases are not very rare, because one of us (K.N.) has seen at least 3 other similar cases though not of course associated with phaeochromocytoma.
On the other hand, there are several reports of hypertensive retinopathy following phaeochromocytoma, but none was so severe as to cause bilateral bullous retinal detachment.
Whether a phaeochromocytoma causes a specific retinopathy is still an open question. All the retinal changes assigned to phaeochromocytoma could possibly occur secondarily to the malignant hypertension. The presence of large pale, confluent, hard exudates in the papillo-macular area is significant, though not conclusive. BRUCE  who reviewed 108 cases of phaeochromocytoma, found that the fundus had been described in only 38 cases, and these did not show any specific retinopathy. 3 of his own cases were also without any specific changes in the retina.
More recently, THOMAS, ROOKE AND KUAB  reviewed 100 cases of phaeochromocytoma and found visual abnormalities in 4% of cases; and GEORGAKAKIS  found papilloedema
and hypertensive retinopathy among cases of chromaffin tumours.
It is possible that in ecclampsia the hypertension, papilloedema, retinopathy and retinal detachment occur due to increased secretion of adrenaline and noradrenaline, as occurs in phaeochromocytoma.
A case of Phaeochromocytoma causing malignant hypertension, retinopathy, papilloedema, and bilateral bullous detachment of the retina in a young girl is presented. This is the first case report of a phaeochromocytoma giving rise to bilateral retinal detachment, and the fifth report of a case of malignant hypertension associated with bilateral retinopathy, papilloedema and retinal detachment.
The question of a specific retinopathy due to phaeochromocytoma is still an open one.
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