a case frosted branch angiitis · and bilateral acute retinal necrosis"7). sheathing is also...

British Journal of Ophthalmology, 1987, 71, 553-558

A case of frosted branch angiitisYOSHIHIRO WATANABE,' NORIO TAKEDA,2 AND EMIKO ADACHI-USAMI'

From the 'Department of Ophthalmology, School of Medicine, Chiba University, Chiba, Japan, and the'Department of Ophthalmology, Kashima Rosai Hospital, Ibaraki, Japan

SUMMARY We report a case of frosted branch angiitis in a 16-year-old-girl. She noted a sudden andsevere visual disturbance in both eyes, without other systemic symptoms. Diffuse retinal oedemaand unusual sheathing of retinal veins were characteristic in both fundi. Fluorescein angiographyshowed no occlusion of the sheathed retinal veins, but some paravenous extravasation of dye wasfound in the late phase. With high doses of systemic corticosteroids her fundi and visual acuityimproved greatly, though the vessels continued to show severe narrowing. At three months thepattern visually evoked cortical potentials were found to be normal, while flash electroretinogramswere absent. No systemic abnormality has been found to explain the aetiology of this condition.

In 1976 Ito et al. ' reported a case of a 6-year-old boywho suffered from a bilateral acute uveitis withsevere sheathing along all of the retinal vessels. Theynamed this condition 'frosted branch angiitis'. Sincethis publication five similar cases have been reportedin Japan, in Japanese.24 The disease occurs in childrenand has an acute onset with bilateral visual disturb-ance. It has been reported to respond well to steroidtherapy. We report here the case of a girl whoconformed quite well to the above description. TheCorrespondence to Yoshihiro Watanabe, MD, Department ofOphthalmology, School of Medicine, Chiba University, 1-8-1Inohana, Chiba 280, Japan.

clinical features of previous cases of frosted branchangiitis are summarised and compared with those ofsimilar retinal diseases.

Case report

A 16-year-old girl was first seen on 11 April 1985 with aone-day history of sudden redness and blurred visionin both eyes. Her visual acuity was counting fingers at50 cm in both eyes. Ciliary injection and a cellularreaction was seen in the anterior chamber. Theintraocular pressures were within normal limits. The

Fig. 1 Right eye, l April1985. Left, temporalperiphery. Severesheathing ofthe veins andsome blot haemorrhagesadjacent to veins. Right, posterior pole. Diffuse retinal oedema andseveresheathing ofthe veins areseen, but the optic disc isalmost normal.

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Yoshihiro Watanabe, Norio Takeda, and Emiko Adachi-Usami

Fig. 2 Change ofvisualfields.Top row, 11 April 1985. Secondrow, 17Aprill985. Third row,28April1985. Fourth row, 10 June1985.

vitreous was slightly hazy and the retina was oedema-tous, with scattered nerve fibre haemorrhages. Severesheathing along all the veins was found throughout theretina in both eyes except in areas surrounding theoptic discs; the sheathing was more severe peri-pherally (Fig. 1). Dilatation or tortuosity of the veinswas not seen. The optic discs were almost normal.A visual field test showed bilateral enlargement of

the blind spot and general deterioration of sensitivity(Fig. 2).Fundus fluorescein angiography demonstrated that

the retinal veins were patent, but extravasation andstaining of veins were obvious in the late stages (Fig.3). Because the patient complained of dizziness andmalaise after the injection of fluorescein, fundusangiography was not repeated.

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A case offrosted branch angiitis

Fig. 3 Right eye, 12 April1985. Fluorescein angiogram.Left, early phase. No abnormalities arefound. Right,photomontage, latephase. Vessels round the macula stainwithfluorescein, and extensive dye leakagefrom veins is seenin the periphery.

No systemic abnormality was found. Red and whitecell counts, haemoglobin, plasma proteins, urea, andelectrolytes were normal. Chest x-rays were alsonormal. There was no serological evidence of syphilis,toxoplasmosis, or rheumatoid arthritis. A skin test fortuberculosis was negative. Serum complement levelsfor herpes simplex virus, herpes zoster virus, andcytomegalovirus were within normal limits.

CLINICAL COURSEThe patient was admitted to hospital immediately,

20mg

and treatment was started with topical cycloplegicsand 0-1% betamethasone, and with systemic beta-methasone (3 mg/day). This treatment was continuedfor three consecutive days, but there was no improve-ment, and the visual acuity fell to counting fingers at 30cm in both eyes. On the fourth day the patient wastherefore switched to 20 mg/day of dexamethasone(Fig. 4). The retinal oedema, sheathing along theveins, and cellular floaters in the anterior chamberimproved by the next day. The sheathing almostdisappeared on the 10th day, but sheathed veins andadjacent retinal haemorrhages remained in the peri-pheral areas, and hard exudates similar to the macularstar figure were seen in both posterior poles. At thisstage irregularities in calibre of the veins and nar-rowing of the arteries appeared (Fig. 5).

After 20 days of treatment the cells in the anteriorchamber had almost disappeared and the visual acuity

0 10 20 30 40

Days after admission

50 60

3

._

'aen(_

Fig. 4 Steroid therapy and timecourse ofvisual acuity.

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Fig. 5 Right eye, 22 April1985. Left, temporalperiphery. Diffuse hard exudates and haemorrhages along the veins. Arteriesare very narrow. Right, posteriorpole. Hard exudates like a macularstarfigureareseen.

had slightly improved (Fig. 4). By the 40th day heracuity had improved to 6/12 in both eyes, butattenuation of retinal veins continued to be seen. Shewas discharged on the 41st day. Dexamethasone wasreduced gradually and completed on the 45th day. Thetotal dosage of dexamethasone was 141-5 mg.Three months after the patient's initial presentation

faint hard exudates in the macular areas and abnormalreflexes around the maculae were still present. In themid and far periphery the vessels were so narrow thatthey were difficult to recognise, and even in theposterior pole the arteries were severely attenuated.Some blot haemorrhages were present in the midperiphery. Coalescence ofsmall, sharply demarcated,atrophic lesions was seen in the far peripheral areas,especially in the temporal periphery (Fig. 6). Hervisual acuity improved to 6/9 five months after onset.

CHANGES OF VISUAL FIELDS

The bilateral central scotoma that appeared six daysafter initial presentation disappeared in about twoweeks. However, generalised constriction of visualfields progressed. Her visual field then enlargedslowly, but the improvement almost stopped near theend ofthe second month after initial presentation. Thepatient was left with a general constriction and slightdeterioration of central sensitivity. Visual fieldconstriction was more severe on the nasal side, whichcorresponded well with the atrophic appearance ofthetemporal peripheral retina.

ELECTROPHYSIOLOGICAL AND PSYCHOPHYSICAL TESTING

Almost no electroretinogram (ERG) response couldbe elicited on the fifth day after the patient's initialpresentation. Only a slight recovery of the response

Fig. 6 Right eye, 22 July 1985. Left, temporalperiphery. Sharply demarcated atrophic lesions and narrowing of vessels likethreads. Right, posterior pole. Hard exudates have almost disappeared. Narrowing of vessels, particularly arteries, is seen.

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A case offrosted branch angiitis

was found on the 54th day, and no further improve-ment was observed even on about the 100th day. Onthe other hand pattern visually evoked corticalpotentials (pattern VECPs) using check size of 18-9minutes, contrast of80%, reversing at a rate of 3 or 10per second, performed three months after onset werenormal both in peak latencies and in amplitudes (Fig.7). Colour vision of both eyes was normal with theIshihara test plates and Farnsworth panel D-15, butthe left eye showed a mild red-green deficiency withstandard pseudoisochromatic plates.

Discussion

The main features of frosted branch angiitis asoriginally reported by Ito et al. can be summarised asfollows: (1) bilateral acute visual disturbance in ahealthy child. (2)Thewhole retina is swollen and there

Pattern VECPs

3rev/s

OD

Os

1 Orev s

2,uV

50msFig. 7 Pattern VECPs recorded three months after onset.Thepeak latency ofP100 component oftransient responses(Top) is normal and the amplitude is not reduced both intransient andsteady state responses (bottom). (Stimulusfield8 x 10 degrees, check size 18 9 minutes, contrast ratio 80%,128pattern reversals.)

is severe sheathing of the retinal vessels, so that theylook like frosted branches of a tree, especially at theperiphery. (3) Fundus fluorescein angiography showsalmost a normal pattern in the first stage, but leakageof the dye from vessels is seen in later stages; inparticular, the sheathed vessels show no sign ofocclusion or stasis. (4) Visual fields show a markedconcentric constriction during the acute stages of thedisease but improve with the reduction of the swellingand inflammation. (5) ERGs are markedly abnormal.(6) No systemic abnormality is found. (7) High dosesof systemic corticosteroids are effective in treating thedisorder, and the visual acuity recovers to almost anormal level. (8) The disease has not recurred.The features of the other cases of frosted branch

angiitis previously reported are summarised inTable 1.

In all cases both eyes were affected, and the visualacuity fell rapidly within a week to less than 6/30.The fundus findings in these cases were similar and

can be summarised as follows: (1) The optic discs werealmost always normal, though slight oedema orhyperaemia was seen in some cases. (2) In the acutestage the whole retina was oedematous and opacified.Intense sheathing of retinal vessels, especially veins,was seen throughout the fundi, but was severer in theperipheral areas, showing the appearance of frostedbranches of a tree. These inflammatory changesgradually became normal by absorption of retinaloedema and improvement in the sheathing. (3)Geographic atrophic lesions remained at the peri-phery in most cases. Hard exudates were often seen inthe maculae or elsewhere during the absorption of theretinal oedema.

In the present case intense narrowing of vesselsremained after subsidence ofinflammation in additionto the above mentioned peripheral atrophic lesions.As the retinae were atrophied by the severe inflam-mation, it is doubtful whether the vascular narrowingwill return to normal.From the above summary of reported cases,

including ours, the pathological condition of frostedbranch angiitis can be defined as follows. It is an acutepanuveitis with severe vasculitis that occurs in thewhole retina. Sakanishi et al. 2 suggested that theatrophic lesions that remained at the periphery are aconsequence ofpoorblood perfusion ofthe peripheralretina. The peripheral areas are therefore moresusceptible to damage. The present patient had brieflya central scotoma similar to optic neuritis, but thecause is likely to be macular oedema rather than directdamage to the optic nerve. This explanation issupported by the exudates that appeared later in themacular area. Diminished ERGs recorded threemonths after onset suggest that substantial damageoccurred to the retina as a whole. The normal pattern

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Table 1 Cases offrosted branch angiitis

Authors Year Age, sex Worst VA Final VA Worst VF Final VF

Right Left Right Left

Ito etal.' 1976 6 M 6/120 6/60 6/6 6/5 Concentricdefect NormalSakanishietal. 2 1984 9 F CF 6/120 6/5 6/5 Notdescribed

8 M 6/60 6/30 6/9 6/6 Not describedYamaneetal. 1985 9 F CF CF 6/5 6/5 NotdescribedHiguchi etal. 1985 9 F LP LP 6/12 6/7 Not describedWatanabe etal. 1986 16 F CF CF 6/9 6/9 Concentric defect Generalised

Central scotoma constriction

VA = visual acuity. VF = visual field. CF = counting fingers. LP - light perception.

VECPsfound at the same time, however, indicate thatthe visual pathway from macula to occipital lobereturned to a normal condition.No aetiological abnormality has yet been identified.

However, systemic steroid was reported to beeffective. Although the natural course of frostedbranch angiitis is unknown, anti-inflammatory drugsare essential to minimise retinal inflammatorydamage.The differential diagnosis includes four diseases

with sheathing of retinal vessels:(1) Acute retinal necrosis' (Kirisawa type uveitis

and bilateral acute retinal necrosis"7). Sheathingis also seen, but is mainly restricted to arteries.Fluorescein angiography shows occlusion of arteriesand capillary beds. This disease may occur mono-cularly, and in bilateral cases the day of onset in eacheye is usually different. In addition children are rarelyaffected. The prognosis is extremely poor in contrastto that of frosted branch angiitis.

(2) Peripheral uveitis.' This disease has beenconsidered to be not a single entity but a syndromefrom various causes. Though sheathing of veins maybe seen in peripheral areas, subjective signs arecommonly mild.

(3) Retinitis caused by cytomegalovirus or herpessimplex virus.9 12 These viral infections tend to affectpatients who are immunosuppressed. Pathologicalchanges of the fundus are not so diffuse as in frostedbranch angiitis. Titres against these viruses havealways been negative in frosted branch angiitis.

(4) Eales' disease.' Although vessels may besheathed in peripheral areas, fluorescein angiographyshows obliteration of retinal capillaries and vessels.

Central visual acuity is retained unless vitreoushaemorrhages cover the posterior pole.

Frosted branch angiitis seems to be a newly recog-nised disease, and further studies will be necessary tounderstand its causation.References

1 Ito Y, Nakano M, Kyu N. Takeuchi M. Frosted-branch angiitis ina child. Jpn J Clin Ophthalmol 1976; 30: 797-803.

2 Sakanishi Y, Kanagami S, Ohara K. Frosted retinal angitis inchildren. Jpn J Clin Ophthalmol 1984; 38: 803-7.

3 Yamane S. Nishiuchi T, Nakagawa Y, Iwase T, Hama M. A caseof frosted branch angitis of the retina. Folia Ophthalmol Jpn1985; 36: 1822-5.

4 Higuchi K, Maeda K, Uji T, Yokoyama M. A case of acuteinfantile uveitis with frosted branch angiitis. Jpn Rev ClinOphthalmol 1985; 79: 2260-4.

5 Hayasaka S, Asano T, Yabata K, Ide A. Acute retinal necrosis.Br J Ophthalmol 1983; 67: 455-60.

6 Young NJA, Bird AC. Bilateral acute retinal necrosis.Br J Ophthalmol 1978; 62: 581-9t).

7 Willerson D Jr, Aaberg TM, Reeser FH. Necrotizing vaso-occlusive retinitis. Am J Ophthalmol 1977; 84: 209-19.

8 Koshibu A, Ohkuma H, Uyama M. Analysis of clinical picturesof peripheral uveitis. Nippon Ganka Gakkai Zasshi 1978; 82:363-9.

9 Chumbley LC, Robertson DM, Smith TF, Campbell RJ. Adultcytomegalovirus inclusion retino-uveitis. Am J Ophthalmol1975; 80: 807-16.

10 Brown RM, Mendis U. Retinal arteritis complicating herpeszoster ophthalmicus. Br J Ophthalmol 1973; 57: 344-6.

11 Pavan-Langston D, Brockhurst RJ. Herpes simplex panuveitis.Arch Ophthalmol 1969; 81: 783-7.

12 Diddle KR, Schanzlin DJ, Mausolf FA. Necrotizing retinitiscaused by opportunistic virus infection in a patient withHodgkin's disease. Am J Ophthalmol 1979; 88: 668-73.

13 Asdourian DK. Peripheral retinal neovascularization -differential diagnosis. In: Peyman GA, Sanders DR, GoldbergMF, eds. Principles andpractice ofophthalmology. Philadelphia:Saunders, 198t); 2: 1277-98.

Acceptedfor publication 15 August 1986.

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a case frosted branch angiitis · and bilateral acute retinal necrosis"7). sheathing is also...

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