BILATERAL OPTIC NEUROPATHY :AN UNUSUAL URAEMIC MANIFESTATION

OF END-STAGE RENAL DISEASE :a case report and littérature review

Abstract :

Background : uraemia Optic neuropathy(UON) is a very rare

complication of renal failure with a few cases in nephrology’s literature.It
is considered as an interdisciplinary emergency as the early diagnosis of
it and the immediate institution of treatment can potentially reserve the
visual impairment and prevent long-term consequences.We report our
first case of uraemic ischemic optic neuropathy in a man with end-stage
renal disease.
Key words : Visual loss. optic neuropathy .End-stage renal disease. uraemia.

Introduction :

Case report:

A 48-year-old man with end-stage renal disease(ESRD) of secondary

focal segmental glomerulosclerosis was on maintenance HD from a
month.One a day,he was presented with acute onset of painless blurring
of two eyes vision above all on left eye,with no other neurological
symptoms.On admission,Blood pressure and pulse rate were
respectively

110/65 mm Hg and 75 beats/min. The physical examination especially

neurological did not show any abnormal findings. The Ophthalmic
examination revealed visual acuity of 4/10 on the right and 5/10 on the
left.Besides,the Extraocular movements were intact.whereas,a Relative
afferent papillary defect was noted at the right eye.

The rest of the examination was unremarkable unless a bilateral optic

disc oedema and a pale left optic disc revealed on the fundoscopy.

In addition, there was no mass neither vascular lesion on Computed

tomography scan and magnetic resonance imaging of the head.

biological investigation revealed haemoglobin of 69 g/L,serum creatinine

of 1312 μmol/L and urea of 44 mmol/L.the immunological tests were
negative and there was no vitamin deficiency.But,he was noticed to
have intra- dialytic chronic systemic hypotension.
In summary,the diagnosis of anterior ischemic optic neuropathy (AION)
secondary to uraemia was retained based on the clinical course, the
fundoscopic examination and the absence of cardiovascular and
collagen disease .

We promptly optimized haemodialysis followed by prescribing aspirin and

2 weeks of corticosteroid treatment which was interrepted gradually over
1 month.Two units of packed red blood cells were given to the patient to
maximize oxygen delivery.His vision improved remarkably to 6/10 in the
right eye,but there were no improvement on the left one because of
complete atrophy of the optic nerve .
Discussion :

We presented the first case of a new-hemodialysed patient who

presented a bilateral UON due to intra-dialytic hypotension worsen by
anemia.He responded partially to steroids ,aspirin and hemodialysis. The
diagnosis of UON is held in front of the signs of sudden painless visual
loss ,an optic disc oedema and a visual field defect .The involvment can
be unilateral or bilateral.

Optic neuropathy in uraemia is rare (10,11).Only a few cases have

been reported in nephrology littérature from 1988. knox et al(1) was the
first who stated the cases of six patients who presented the progressive
loss of vision commonly in one or both eyes in a few days.The common
characteristic was severe renal disease with uraemia,anemia and
elevated blood pressure(in three cases). For all patients ,there was not a
long-term dialysis treatment.

in four patients a significant improvment of their vision was reached

following to undergoing treatment with hemodialysis and steroids or
both.Concerning the fifth patient of Knox(case5,table),the nerve heads
was not elevated and the improvment of his vision was partial after
peritoneal dialysis. Since knox et al,few cases was reported in the
littérature(table).

The pathogenesis of UON is poorly understood. Some authors

postulated that direct toxicity to the optic nerve by dialyzable metabolites
would cause a visual impairment(7,8).That’s why ,it’s frequently observed
with subjects with undiagnosed renal disease or at the beginning of
dialysis as our patient.

Moreover,it was suggested that this complication may involve

interference with the posterior celiary artery blood supply to the
preliminar optic nerve(13).This might be brought about by decreased
blood delivery,increased resistance to blood supply or low blood oxygen
carrying supply ().That’s why,Anemia,atherosclerosis and hypertension
were factors precipating this complication(8) which are commonly found
in patients under long-term dialysis.

On the other hand ,the main precipitating factor in most dialyzed

patients who experience UON is intra-dialytic hypotension(9) which is a
frequent complication on dialysis(14,18).According to Ghaffar,it concerns
9% of dialysis patients(18).

But the treatment of UON remains unclear. The effectivness of steroids

is controversial. ,there was a significant and rapid degression of disc
oedema but there was no evidence that a shorter duration of disc edema
is associated with improved visual outcome.

Treatment of UON in dialysis is still unclear.

Conolly reported visual improvment in hypotensive UON following

vigorous pressure rise. It ‘s known that Hyperbaric oxygen therapy
elevates the dissolved oxygen content of blood, thereby providing
increased tissue oxygen and potentially reducing damage in injured
axons(19).

The evaluation of the effect of hyperbaric oxygen in 20 nonarteritic AION

patients between 3 and 20 days of onset of visual loss was done. It was
noticed That there was no recovery in visual acuity or visual field even in
the subgroup treated within 9 days of onset of symptoms(20) .

That’s why, avoiding hypotensive events is very important in preventing

potentially blinding condition within patients undergoing chronic dialysis.

Table :characteristics of reported cases of optic neuropathy in uraemia.

Ca Autho Case Gend Eye Underlyin BUN( s- Hb( Bloo Dial cortico asp revers Diag
se r N er/age affe g mg/dl) creat( g/dl) d ysis steroid irin ibility nosis
N (y) cte diagnosis mg/dl) pres
d sure
1 Knox 1988 F/30 rig Chronic 210 NF 119 Ye 50mg N Yes UON
et ht pyelonep
al(1) hritis NF /64 s /d o
2 Knox 1988 F/19 bot Henoch- 120 9 ,2 6,9 170 No 60mg N Yes UON
et h schonlein
al(1) purpura /15 /d o
0
3 Knox 1988 F/53 Bo Multiple 240 34 NF 170 Ye No N Mar UON
et th myeloma
al(1) /10 s( o gina
0 PD l
)
4 Knox 1988 F/46 Bo Non 84  8,6 6,1 200 Ye No N No UON
et th specific
al(1) glomerulo /16 s o
nephritis 0
5 Knox 1988 M/60 Bo Renal N NF NF NF NF Ye No N Yes UON
et th calculi
al(1) F s o
(P
D)
6 Knox 1988 M/16 Bo igA 119 15 6,5 192 Ye No N Yes UON
et th nephropat
al(1) hy /13 s( o
2 PD
the
n
HD
)
7 Kian 2011 M/17 Undetermi 34 1312 7 , Ye 2wee no Yes
et ned UON
al(2) nephropat 7 s ks
hy
8 Winke 2001 M/60 Light- 219 46,8 6,6 100 Ye 2wee N No UON
lmaye chain
r et disease /60 s ks o
al(3)
9 LM 1989 M/61 Bo Nephroscl 45 12,4 13, 140 ye No N Yes
Hame th erosis
d et 6 /80 s o
al(4)
10 LM 1989 M/40 1 Glomerul 92 16 Se 188 ye Yes N No UON
Hame onephritis
d et ver /12 s o
al(4) e 0
an
ae
mi
a
11 LM 1989 F/41 1 Reflux 81 15,6 Sev 200 Ye 6mo No Yes UON
Hame nephropat ere
d et hy ana /13 s nths
al(4) emi 0
a
12 Korze 1998 F/41 1 Renal 180 7,2 13, 140 Ye yes No Yes UON
ts et agenesis
al(5) 5 /95 s
13 2013 M/55 Diabetic 70/ Ye yes No
nephritis
40 s
14 2013 M/48
15 Ji min 2016 M/40 1 17 102, 16,8 11, 109 Ye yes No Yes UON
lee et ye
1 8 /52 s
 al((6) ars
16 Korze 2004 M /7 1 Diabetic ≤10 Yes Yes No
ts et nephritis
al(9) 7 0
16 Seo et 2011 M/22 Bot Chronic 53,6 6 150 Yes Yes No Yes UON
al(10) h glomerulo
nephritis /90
17 Haide 1993 M/53 1 Polycystic 25 1075 9 80/ Yes No No - UON
r et kidneys
al(12) 40
18 Haide 1993 F/42 1 Indetermi 3y 35 990 6,6 No Yes No No No UON
r et ned ear
al(12) nephropat s hy
hy pot
en
sio
n
18 Haide 1993 F/77 bo Indetermi 35 650 7 Yes Yes No No UON
r et ned
al(12) th nephropat
hy
18 Haide 1993 M/32 1 Diabetic 28 1200 9 No Yes No No No UON
r et nephropat
al(12) hy
Kare
n et
al(15)
Jain 2017 F/45 Bot Indeterm 3y 78 6,2 9 ,5 110 Yes Yes No No UON
et h ined ea /80
al(17) nephrop
athy rs

Conclusion:

We illustrated with this case a rare complication of uremia,an ischemic

optic neuropathy which can lead rapidly to vision loss.An increased
vigilance should be given to such complications to every hemodialysis
patient.

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