Answer for BIR CoW 22 Apr 2018
Coat's disease
Findings
Right eye is T1, T2 hyperintense- possibly due to proteinacious exudate ,with enhancing soft tissue thickening in posterior globe with restricted diffusion possibly detached retina. Features suggestive of Coats disease right eye.
Discussion
Introduction: Coats’ disease is an idiopathic, ophthalmic condition characterized by retinal telangiectasis, intraretinal and subretinal exudation, which can lead to retinal detachment. It is mostly unilateral, progressive and affects mainly males during childhood, although adult cases have also been described. Affected eyes can develop outer retinal thickening and total or partial exudative retinal detachment. The subretinal fluid is viscous and laden with lipid crystals. Retinal capillary dropout is seen distal to the telangiectasias and can lead to neovascularization of the disc with increasing severity of ischemia. The pathology is present in the retinal vasculature, which acquires numerous aneurysms that rarely bleed. Signs and symptoms: Patients can present with a range of signs, with the most common being decreased visual acuity, strabismus and leukocoria . Other signs that can be present in patients with Coats’ disease are pain, heterochromia of the iris and nystagmus Radiographic features: Ultrasound: Ultrasound is an excellent modality for the assessment of the globe, however is hampered in advanced cases by limited specificity and difficulty in distinguishing Coats disease from non-calcified retinoblastoma. The typical features of Coats’ disease on ultrasound were subretinal opacities due to cholesterolosis present from the exudates, as well as retinal detachment which is able to be visualized as a linear echo CT :Findings depend on the stage of disease. Early on, examination may be normal. Enhancement may be seen at the margins of the exudate and may have a V-shaped pattern similar to retinal detachment . In advanced cases the affected globe is hyperdense due to proteinaceous exudates and the vitreous space may be obliterated due to extensive retinal detachment. Calcification is uncommon but has been reported. The affected eye in Coats disease is usually significantly smaller than the non-affected eye in older children, and is thought to represent an impairment of growth rather than the reduction in volume of a previously normal globe MRI:MRI is extremely useful in the diagnosis of advanced Coats’ disease, but may have lesser utility during the initial stages; MRI is superior to CT, in ruling out retinoblastoma as the difference between subretinal exudation and a solid mass is clearer on MRI . Specifically, the exudate in Coats’ disease is hyperintense on both T 1 -weighted and T 2 -weighted MRI images, whereas in retinoblastoma,T 1 -weighted image will show a hyperintense mass, but T 2 -weighted image shows a hypointense mass T1: high signal due to proteinaceous nature of the exudates T2: high signal due to proteinaceous nature of the exudates T1 C+ (Gd) enhancement of the detached retina may be visible rarely an 'enhancing mass' may appear to be present in advanced cases Treatment Treatment remains controversial, and options include laser photocoagulation, cryotherapy and surgical repair of retinal detachment. In general, patients who present at an earlier age will fare worse than older patients . Differential diagnosis persistent hyperplastic primary vitreous (PHPV) retinopathy of prematurity non-calcifying retinoblastoma this is especially worrisome in younger patients CT and MRI with contrast should demonstrate a mass the globe is normal or increased in size .95% of retinoblastomas are calcified. References Ophthalmologica 2012;227:175–182 Samim Ghorbanian a Adil Jaulim a Irini P. Chatziralli b Radiopaedia
Findings
Right eye is T1, T2 hyperintense- possibly due to proteinacious exudate ,with enhancing soft tissue thickening in posterior globe with restricted diffusion possibly detached retina. Features suggestive of Coats disease right eye.
Discussion
Introduction: Coats’ disease is an idiopathic, ophthalmic condition characterized by retinal telangiectasis, intraretinal and subretinal exudation, which can lead to retinal detachment. It is mostly unilateral, progressive and affects mainly males during childhood, although adult cases have also been described. Affected eyes can develop outer retinal thickening and total or partial exudative retinal detachment. The subretinal fluid is viscous and laden with lipid crystals. Retinal capillary dropout is seen distal to the telangiectasias and can lead to neovascularization of the disc with increasing severity of ischemia. The pathology is present in the retinal vasculature, which acquires numerous aneurysms that rarely bleed. Signs and symptoms: Patients can present with a range of signs, with the most common being decreased visual acuity, strabismus and leukocoria . Other signs that can be present in patients with Coats’ disease are pain, heterochromia of the iris and nystagmus Radiographic features: Ultrasound: Ultrasound is an excellent modality for the assessment of the globe, however is hampered in advanced cases by limited specificity and difficulty in distinguishing Coats disease from non-calcified retinoblastoma. The typical features of Coats’ disease on ultrasound were subretinal opacities due to cholesterolosis present from the exudates, as well as retinal detachment which is able to be visualized as a linear echo CT :Findings depend on the stage of disease. Early on, examination may be normal. Enhancement may be seen at the margins of the exudate and may have a V-shaped pattern similar to retinal detachment . In advanced cases the affected globe is hyperdense due to proteinaceous exudates and the vitreous space may be obliterated due to extensive retinal detachment. Calcification is uncommon but has been reported. The affected eye in Coats disease is usually significantly smaller than the non-affected eye in older children, and is thought to represent an impairment of growth rather than the reduction in volume of a previously normal globe MRI:MRI is extremely useful in the diagnosis of advanced Coats’ disease, but may have lesser utility during the initial stages; MRI is superior to CT, in ruling out retinoblastoma as the difference between subretinal exudation and a solid mass is clearer on MRI . Specifically, the exudate in Coats’ disease is hyperintense on both T 1 -weighted and T 2 -weighted MRI images, whereas in retinoblastoma,T 1 -weighted image will show a hyperintense mass, but T 2 -weighted image shows a hypointense mass T1: high signal due to proteinaceous nature of the exudates T2: high signal due to proteinaceous nature of the exudates T1 C+ (Gd) enhancement of the detached retina may be visible rarely an 'enhancing mass' may appear to be present in advanced cases Treatment Treatment remains controversial, and options include laser photocoagulation, cryotherapy and surgical repair of retinal detachment. In general, patients who present at an earlier age will fare worse than older patients . Differential diagnosis persistent hyperplastic primary vitreous (PHPV) retinopathy of prematurity non-calcifying retinoblastoma this is especially worrisome in younger patients CT and MRI with contrast should demonstrate a mass the globe is normal or increased in size .95% of retinoblastomas are calcified. References Ophthalmologica 2012;227:175–182 Samim Ghorbanian a Adil Jaulim a Irini P. Chatziralli b Radiopaedia
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!