Table 1.

Previous case reports and case series of optic nerve head granuloma due to sarcoidosis.

Study	Age in years, gender, and racial origin (where stated)	Unilateral or bilateral granulomas	Previous diagnosis of sarcoidosis	Initial visual acuity	Additional ocular features apart from the granuloma	Extra-ocular clinical features of sarcoidosis	Treatment	Length of follow-up	Final visual acuity
Goldberg and Newell, 19446	24, male, African American	Unilateral	No	20/50	New vessels, subhyaloid, macular, and peripheral haemorrhages, and irregular constricted veins	Parotid gland enlargement, generalised and hilar lymphadenopathy	None	5 months	20/30
Laval, 19527	48, female, White Caucasian	Unilateral	No	PL	Anterior chamber cells, posterior synechiae, distended retinal blood vessels, retinal haemorrhages, retinal nerve fibre layer oedema, and retinal nodules	None	None	None - enucleated on presentation	PL
Brunste, 19588	35, male	Bilateral	No	6/6 OU	Pigmented precipitates on posterior corneal surfaces, white excrescences of the irises, and moderate venous congestion OU	Hilar lymphadenopathy	Corticotropin	1 year	6/18 OD, “normal” OS
Kojima, 19699	11, male	Unilateral	Yes	0.06	Macular star and venous thrombosis	Chest—type of involvement not stated	Oral steroids, and vitamins B1 and E	1 year	0.9
Ingestadand Stigmar, 19705	26, male	Unilateral	No	0.1	Venous congestion, macular oedema and periarterial “yellow spots” with later haemorrhages, exudates, small vessel sheathing, and posterior vitreous detachment	Lung infiltrates and hilar lymphadenopathy, then later parotid swelling and diabetes insipid us	Prednisolone	20 months	NPL
Laties and Scheie, 197010	25, female, African American	Unilateral	No	6/6	Small vessels on surface of granuloma, haemorrhages, and whitish deposits on retinal veins	Lung interstitial fibrosis and hilar lymphadenopathy	None	8 months	6/6
	37, male, African American	Unilateral	Yes	“Blind”	None	Epilepsy and hilar lymphadenopathy	Steroids	4 years	Not stated—patient died due to status epilepticus
Jampol et al., 197211	29, female, African American	Bilateral	Yes	20/20 OU	Peripapillary retinal folds, distended retinal veins OU and a single retinal haemorrhage OS	Left lung infiltrate, cervical and hilar lymphadenopathy	Prednisone	3 months	Not stated
Kelley and Green, 197312	31, male, African American	Unilateral	Yes	PL	New vessels and peripapillary choroiditis, with later uveitis, synechiae, cataract, and glaucoma	Headaches, left lateral rectus palsy, enlarged parotid glands, left-sided neural deafness, cervical and hilar lymphadenopathy	Steroids	15 years	NPL and enucleated
Turner et al., 197513	29, female, White Caucasian	Bilateral	Yes	6/12 OD 6/9 OS	OD: Dilated capilaries, engorged retinal veins, candle-wax spots; cotton wool spots, haemorrhages, peripheral venous sheathing, and macular oedema OS: Dilated capillaries, engorged retinal veins, candle-wax spots, two small discrete retinal lesions, and nerve fibre bundle defects	Papular erythema of the legs, fever, polyarthralgia, facial palsy, knee effusions, hypercalciuria, and hilar lymphadenopathy	Prednisone then dexamethasone	9 months	Not stated
Burns, 197614	22, female, African American	Unilateral	Yes	20/200	Localized exudative apparent retinal detachment and sub-retinal haemorrhages	Amenorrhoea, polydipsia, polyuria, polyphagia, skin nodules, supraclavicular and hilar lymphadenopathy	Prednisone	4, months	20/15
Gass and Olson, 197615	41, male	Bilateral	Yes	20/25 OD NPL OS	OD: Vitreous cells, retinal exudates and haemorrhages OS: Keratic precipitates, aqueous ray, posterior synechiae, and cataract	Epilepsy, ataxia, nystagmus, personality change, right-sided facial weakness, skin nodules, and an abnormal chest radiograph	Triamcinolone	20 months	20/80 OD NPL OS—patient died due to status epilepticus following an assault
Lustgarten et al., 198316	32, female, African American	Unilateral	No	20/30 worsening to NPL	Initial swelling and papillophlebitis, then CRVO, increased granuloma size, and vitreous opacities	None	Prednisone	13 months	NPL
Beardsley et al., 198417	23, male	Unilateral	No	20/200 worsening to 20/400	Vitreous opacities, peripapillary retinal detachment, perivenous exudates, and cotton-wool spots	Hypercalciuria and hilar lymphadenopathy	Prednisone	9 months	20/20
	16, male	Unilateral	No	HM worsening to NPL	Vitreous opacities, peripapillary serous retinal detachment, and perivascular exudates, with later severe anterior uveitis and secondary glaucoma	Hilar lymphadenopathy	Periocular triamcinolone and prednisone	9 months	NPL and enucleated
	24, female	Unilateral	No	20/20	Flare and cells in the anterior chamber, exudates extending along the retinal blood vessels with overlying vitreous debris	Malar skin eruption	Prednisone and isoniazid	18 months	20/15
	33, male	Bilateral	No	20/25 OU worsening to 20/40 OU	None	Hepatosplenomegaly, diffuse interstitial pulmonary markings, hilar and right paratracheal lymphadenopathy	Prednisone	4 months	20/40 OU
Karma and Mustonen, 198518	42, female	Unilateral	Yes	1.0 worsening to 0.1	Initial nodular uveitis then iris granulomas, dilated leaking capillaries, nodes on the lower temporal vein and along scar margins of healed granulomas in the peripheral retina. Later vitreous opacification	Skin purple plaques, joint symptoms, liver dysfunction, and hilar lymphadenopathy	Prednisone	3 years	0.1
	35, female	Unilateral	Yes	1.0 worsening to CF	Initial lacrimal gland enlargement, corneal keratic precipitates, peripapillary iris atrophy, vitreous cells and snowball-like opacities, candle-wax exudates, and chorioretinitic lesions in the lower periphery. Later serous retinal detachment	Epilepsy, lupus pernio, submandibular gland enlargement, lung infiltration, and hilar lymphadenopathy	Prednisone	2 years	CF
Katz et al., 199119	56, female	Unilateral	No	20/50	Fullness of the peripapillary retina	Middle cranial fossa gadolinium-enhancing mass on MRI	Corticosteroids	Not stated	Not stated
Sivakumar and Chee, 199820	41, male, South Asian	Unilateral	No	6/18	RAPD, keratic precipitates, anterior chamber cells, flare, retrolental cells, vitritis, snowballs, and focal retinal vasculitis with perivascular sheathing	Hilar lymphadenopathy	IVMP followed by prednisolone	2 years	6/6
Farr et al., 200021	31, female, African American	Unilateral	No	20/25 worsening to 20/400	Conjunctival nodules and haemorrhages peripheral to the granuloma	Hilar lymphadenopathy	Prednisone	18 months	20/25
Asensio Sanchez et al., 200322	59, male	Unilateral	No	0.6	Macular folds	67Gallium uptake in the lungs and lacrimal glands	Prednisone	1 year	Not stated
Frohman et al., 200323	50, female	Unilateral	No	NPL	Periphlebitis and macular exudate	Not stated	Not stated	Not stated	Not stated
	30, female	Bilateral	No	20/20 OU	Busacca nodules OU	Not stated	Not stated	Not stated	Not stated
	32, female	Unilateral	No	20/70	Lacrimal gland enlargement, ptosis, uveitis, vitreous snowballs, and optic disc shunt vessels	Not stated	Not stated	Not stated	Not stated
Ismail et al., 200524	40, male, Malay	Unilateral	No	6/24	Mild anterior chamber reaction, moderate vitritis with a few snowballs and vitreous strands, tortuous retinal vessels with sheathing of the superior branch of the retinal vein, and multiple yellow-white choroidal lesions in the peripheral retina	67Gallium uptake in he right lacrimal gland with “Panda sign”	Topical dexamethasone and prednisolone	Not stated	6/6
Al-Jamal and Kivelä, 200825	27, male	Unilateral	No	0.33 worsening to CF	Vitreous opacities and an exudative retinal detachment	Lung infiltrates and hilar lymphadenopathy	IVMP followed by prednisolone	1 year	0.1
Jafferji and Biswas, 200826	49, female	Unilateral	No	6/24	Hard exudates, optic nerve head vascular tortuosity with haemorrhages, and pars plana exudates	None	IVMP followed by prednisolone and methotrexate	24 months	6/5
Koczman et al., 20084	24, male, African American	Unilateral	No	20/25	Keratoconjunctivitis sicca and panuveitis	Testicular involvement and hilar lymphadenopathy	IV corticosteroids then prednisone and methotrexate	Not stated	20/15
Moschos and Guex-Crosier, 200827	19, male, Black	Bilateral	No	1.0 OD 0.8 OS	OD: No other changes OS: Corneal keratic precipitates, iris nodule, optic disc leakage, and multifocal choroidal lesions	Hilar lymphadenopathy	Not stated	Not stated	Not stated
Goldberg et al., 201428	41, female	Unilateral	Yes	CF	Infiltrative lesion on the choroid with thickening of peripapillary retina and attenuation of the photoreceptor ellipsoid zone on OCT	Not stated	Not stated	Not stated	Not stated
	38, male	Unilateral	No	20/50	Vitritis and cotton wool spots with retinal oedema on OCT	Not stated	IVMP followed by prednisone	6 weeks	20/20
Present case	57, female, White Caucasian	Unilateral	Yes	6/24	Vitreous cells, intra-retinal oedema, retinal inflammatory infiltrate, and sub-retinal fluid	Vertigo and hilar lymphadenopathy	IVMP followed by prednisolone and methotrexate	14 months	6/9−2

Note. CF = counting fingers; CRVO = central retinal vein occlusion; HM = perception of hand movements; IV = intravenous; IVMP = Intravenous methylprednisolone; MRI = magnetic resonance imaging; NPL = no perception of light; OCT = optical coherence tomography; PL = perception of light; RAPD = relative afferent pupillary defect.