We report a case of bilateral persistent hyperplastic primary vitreous (PHPV) in a 5-month-old infant who presented with bilateral leukokoria. The child was referred for ocular ultrasound with a clinical suspicion of retinoblastoma. Grey-scale evaluation revealed an echogenic band in the posterior segment of the left globe extending from the posterior surface of the lens capsule to the optic disc. Doppler examination revealed the presence of blood flow in the band. Ultrasound assessment of the contralateral globe showed an elevated mass of echogenic tissue in the posterior segment, in contact with the optic disc. Most cases of PHPV are sporadic and unilateral. Bilateral PHPV is rare. In a study by Pollard of 83 cases, only two patients (2.4%) had bilateral PHPV. The imaging features in this case point toward the diagnosis of bilateral PHPV. We suggest that this entity, although rare, should be considered in the differential diagnosis while evaluating bilateral leukokoria.
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http://dx.doi.org/10.1111/j.1440-1673.2005.01402.x | DOI Listing |
Cureus
April 2024
Ophthalmology, Hopital des Specialités, Rabat, MAR.
J Int Med Res
January 2024
Department of Ophthalmology, The Second Hospital of Lanzhou University, Lanzhou, China.
Persistent hyperplastic primary vitreous (PHPV) is a rare congenital developmental ocular disorder caused by incomplete regression of the embryonic hyaloid vasculature; bilateral presentations are even rarer. We report a 6-year-old child with bilateral PHPV who visited our hospital for strabismus, without exhibiting leukocoria, microphthalmia, and systemic diseases. These unique characteristics distinguish our case from other cases of PHPV.
View Article and Find Full Text PDFJ Appl Physiol (1985)
August 2022
Department of Cardiovascular Sciences, Cerebral Haemodynamics in Ageing and Stroke Medicine (CHiASM) Research Group, University of Leicester, Leicester, United Kingdom.
Cerebral blood flow (CBF) can be altered by a change in partial pressure of arterial CO (Pco), being reduced during hyperventilation (HPV). Critical closing pressure (CrCP) and resistance area product (RAP) are parameters that can be studied to understand this change, but their dynamic response has not been investigated during paced HPV (PHPV). Seventy-five participants had recordings at rest and during PHPV.
View Article and Find Full Text PDFArch Soc Esp Oftalmol (Engl Ed)
January 2022
Hospital universitario San Vicente Fundación, Medellín, Colombia.
The most cases of persistence hyperplastic primary vitreous (PHPV) are unilateral and sporadic, however, bilateral presentation could be present in a small number of patients, in whom other genetic diseases must be ruled out. We describe a case of a 2 months child with bilateral persistence hyperplastic primary vitreous confirmed by ultrasound. In addition, with neurodevelopmental defects, microcephaly, facial dimorphism, axial hypotonia, and without brain abnormalities on MRI, in whom a de novo mutation of the CTNNB1 gene was found during the genetic study, which explains the findings.
View Article and Find Full Text PDFCureus
February 2021
Medicine, Nishtar Medical University, Multan, PAK.
Bilateral persistent hyperplastic primary vitreous (PHPV) is a rare ocular disorder. Its clinical manifestations include bilateral corneal haziness, microphthalmia, and cataract. It is the second most common cause of leukocoria after retinoblastoma.
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