Answer for CoW 15 Oct 2017
Persistent hyperplastic primary vitreous (PHPV)
Findings
Right eye is relatively smaller than left. Absence of lens in right eye. Anterior chamber- deeper. T2 Hypo intense, funnel shaped band like structure in the globe extending from posterior capsule and converging at the optic disc with foci of calcification in its posterior end. - giving “martini glass”
appearance
Discussion
Persistent hyperplastic primary vitreous (PHPV), also known as the persistent fetal vasculature, refers to a rare congenital developmental malformation of the eye. Clinically, this condition usually manifests as unilateral or bilateral leucocoria. Patients may also have poor vision, small eye (microphthalmia) and strabismus. It arises due to a failure of normal regression of the embryonic hyaloid vascular system. Persistent fetal vasculature in PHPV can lead to fibrosis, resulting in elongation of the ciliary processes, retinal detachment, and spontaneous cataracts.
IMAGING: CT- soft-tissue replacement (infiltration) of the vitreous body retrolental soft tissue along the Cloquet canal: fine linear structure extending from the head of the optic nerve to the posterior surface of the lens Microphthalmus MRI - The retrolenticular tissue characteristic of this condition has a triangular shape, like that of a ”martini glass” appearing as low T2 low signal against the normal high T2 signal of the globe.
Findings
Right eye is relatively smaller than left. Absence of lens in right eye. Anterior chamber- deeper. T2 Hypo intense, funnel shaped band like structure in the globe extending from posterior capsule and converging at the optic disc with foci of calcification in its posterior end. - giving “martini glass”
appearance
Discussion
Persistent hyperplastic primary vitreous (PHPV), also known as the persistent fetal vasculature, refers to a rare congenital developmental malformation of the eye. Clinically, this condition usually manifests as unilateral or bilateral leucocoria. Patients may also have poor vision, small eye (microphthalmia) and strabismus. It arises due to a failure of normal regression of the embryonic hyaloid vascular system. Persistent fetal vasculature in PHPV can lead to fibrosis, resulting in elongation of the ciliary processes, retinal detachment, and spontaneous cataracts.
IMAGING: CT- soft-tissue replacement (infiltration) of the vitreous body retrolental soft tissue along the Cloquet canal: fine linear structure extending from the head of the optic nerve to the posterior surface of the lens Microphthalmus MRI - The retrolenticular tissue characteristic of this condition has a triangular shape, like that of a ”martini glass” appearing as low T2 low signal against the normal high T2 signal of the globe.
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!