Table 1.

Review of the reported ocular problems in Congenital Protein C deficiency

Report	Reported cases	Involved eye	Anterior segment involvement	Posterior segment involvement	Global involvement
Estelles A et al [1984] [6]	1	OU		Bilateral vitreous hemorrhage and intravitreal mass
Marciniak E et al. [1985] [7]	1	OU	Corneal opacity Case1: Corneal opacity, pupil not visible in one eye Case2: Bilateral cataract,	Case 1: Vitreous opacity in one eye Case 2: total retinal detachment with retinal new and old hemorrhages	Microphthalmia
Rappaport ES et al. [1987] [8]	1	OU		Hyperplastic vitreous bilaterally
Pulido JS [1987] [9]	1	OU	Prominent iris vessels, shallow anterior chamber, synechia, cataract, retrolental membrane	Bilateral vitreal hemorrhage, and funnel-shaped retinal detachment in both eyes
Auletta MJ and Headington JT [1988] [10]	1	OU	Raised intraocular pressure, flattened anterior chambers, iris atrophy, lens adhesions	Bilateral retinal detachment	Bilateral leukocoria
Hartman KR [1989] [11]	1	OU		Vitreal eye hemorrhages and	Intraparenchymal brain infarction
Hermsen VM et al [1990] [12]	1	OU	Vascularized lens, non-existent anterior chambers	Funnel-shaped retrolental mass, normal retina and optic nerve	Bilateral PHPV, microphthalmos
Soria JM et al. [1985] [13]	1			Persistence of primary vitreous	Microphthalmia, irregular globe
Cassels-Brown A et al. [1994] [14]	2	OU	Case 1: Posterior embryotoxon, shallow anterior chambers, ectropion uvea, posterior synechia Case 2: marked periorbital edema and hemorrhagic conjunctival chemosis	Case 1: subconjunctival hemorrhage, a right sided retinal arterial occlusion and bilateral florid retinal hemorrhages with swollen hemorrhagic optic discs secondary to retinal venous occlusions, vitreous hemorrhages retinal detachment in one eye Case 2: hazy media, minor bilateral vitreous hemorrhages, and minor right sided retinal hemorrhage	Case 1: Strabismus, nystagmus, leukocoria Case 2: early birth 25 wk., end up to death at 23 days of life because of some subarachnoid hemorrhage and with worsening lung function
Acheson JF et al. [1994] [15]	4	OD		Bilateral recurrent ischemic optic neuropathy	Association with Protein S deficiency, antithrombin III
Dreyfus M et al. [1995] [16]	9	OU			Unilateral or bilateral blindness 6/9 cases bilateral PHPV
Hattenbach LO et al. [1999] [5]	2	OU	Case 1. Shallow anterior chamber Case 2. Shallow anterior chamber, posterior synechia	Case 1. Left retrolental opacities, funnel retinal detachment, vitreous hemorrhage Case 2. Right vitreous hemorrhage, funnel retinal detachment	Case 1. Strabismus, microphthalmos Case 2. Microphthalmos
Ergenekon E et al. [2000] [17]	1	OS		Eye ultrasound revealed an10x7 mm hyperechogenic structure underneath. Retina of the left eye consistent with subretinal hemorrhage	Leukocoria
Churchill AJ et al. [2001] [18]	2	OU	Case2. Right leukocoria	Case 1. Bilateral central retinal vein occlusions, vitreous hemorrhage, and a right central retinal artery occlusion Case 2. Total retinal detachment and a left macular hemorrhage
Paysse EA [2002] [19]	1	OU	Flat anterior chamber	Bilateral retinal detachment, fibrotic hyaloid arteries
Sirachainan N et al [2003] [20]	2	OU			Preserving eye function, high myopia, cerebral palsy
Park UC et al. [2005] [21]	1	OU	Bilateral corneal opacity, microphthalmia, posterior synechia, pupillary membrane, shallow anterior chamber	Vitreoretinopathy suggesting, PHPV, intravitreal masses, funnel-shaped retinal detachment with bilateral retinal dysplasia	Bilateral leukocoria, no microphthalmos, blind OU
de Lemus-Varela ML [2005) [22]	1	OU		Vitreous hemorrhage
Douglas AG et al. [2010] [23]	1	OU	No red reflex OU	Vitreoretinal dysplasia, severe hazy media in one eye and OU PFV	Leukocoria OS, bilateral microphthalmia,, deranged VEP, poor vision, nystagmus
Wang BZ [2012] [24]	1	OU		Macular hypoplasia with blond retinal background	Doll’s eye movements, no fix and follow movement of the eyes, ponto-cerebellar hypoplasia, nystagmus, strabismus, Flat ERG
Desai S et.al. [2014] [25]	1	OU		Combined central retinal venous and arterial obstruction	Severe Type II protein C deficiency with factor V Leiden mutation, Glaucoma
Almarzouki HS et al [2016] [26]	2	OU	Peter’s anomaly
Baothman AA et al. [2017] [27]	5	OU	Peter’s anomaly, corneal opacity (3/5)	Blindness (4/5OU), ocular hemorrhage shortly after birth resulted in visual loss (1/5)
Present study	1	OU	Left eye shallow anterior chamber, cataract, leukoma, posterior synechia	Dysplastic retina and total retinal detachment in the other eye	Flat ERG, fluorescent spots

ERG Electroretinography, OD Right eye, OS Left eye, OU Both eyes, PHPV Persistent primary hyperplastic vitreous