Prenatal ultrasonographic diagnosis of persistent hyperplastic primary vitreous (original) (raw)
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Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine, 2016
Persistent hyperplastic primary vitreous is a spectrum of congenital ocular abnormalities characterized by leukocoria, microphthalmia, cataracts, extensive intravitreal hemorrhage, persistence of the hyaloid artery, glaucoma, and retinal detachment. It might be isolated or associated with congenital syndromes such as trisomy 13, Walker-Warburg syndrome, and Norrie disease. We present 2 cases of persistent hyperplastic primary vitreous diagnosed by prenatal sonography in the early third trimester. Bilateral hyperechoic lenses and retinal nonattachment were detected in the sonographic examination of the first case, whereas irregular echogenic bands between the lenses and posterior walls of the eyes were prominent in the second case. In both of the cases, ocular findings were accompanied by intracranial findings, including severe hydrocephalus, an abnormal gyral pattern, and cerebellar hypoplasia, suggesting the diagnosis of Walker-Warburg syndrome. We also present a review of the lite...
Case Report Unusual findings in persistent hyperplastic primary vitreous: two cases
Persistent hyperplastic primary vitreous is a result of failure of regression of the embryonic hyaloid artery and presents commonly as leucocoria in the first few weeks of life. It leads to abnormal lenticular development and secondary changes in the retina and orbit. We discuss two cases of persistent hyperplastic primary vitreous with unusual findings. The first case is an example of unilateral involvement, and on high frequency ultrasound, the commonly associated retrolental mass was absent. The hyaloid artery was well visualized on colour and spectral Doppler. The second case had bilateral retrolenticular masses. The hyaloid artery could be demonstrated in one side on colour Doppler. Thickening of the choroido-retinal layer was noted in both sides. Retinal detachment or vitreous haemorrhages, which are commonly associated findings with persistent hyperplastic primary vitreous, were not seen in either case.
Bilateral persistent hyperplastic primary vitreous
Indian Journal of Ophthalmology, 2009
Persistent hyperplastic primary vitreous (PHPV) is an uncommon condition, presenting clinically as leukokoria (white pupillary reß ex), micro-ophthalmia, and cataract. Bilateral PHPV is rare. Most important diff erential diagnosis is retinoblastoma, which can be diff erentiated by imaging features. Case Report A 3-month-old male infant presented with bilateral leukokoria. Birth history of the child was unremarkable. On initial examination, the size of the cornea looked smaller. The child was referred for ocular ultrasound examination, with a clinical suspicion of retinoblastoma. An ultrasound study was performed with a high-frequency transducer operating at 10 MHz (L5, GE Medical Systems, USA). Gray scale evaluation revealed an echogenic band in the posterior segment of both globes extending from the posterior surface of the lens capsule to the optic disc. The axial length of the globes was 16 mm, which was suggestive of micro-ophthalmia. Color doppler examination revealed the presence of blood flow in the band [Fig. 1A]. Spectral analysis of this blood vessel showed arterial waveforms [Fig. 1B]. Hyperechoic foci were seen in both the posterior segments adjacent to the artery, suggestive of associated vitreous hemorrhage [Fig. 1B]. No calciÞ cation was seen.
BMJ case reports, 2015
Persistent hyperplastic primary vitreous (PHPV) is an ocular developmental disorder resulting from incomplete apoptosis of the embryonic hyaloid vasculature. Unilateral PHPV is traditionally associated with a poor prognosis because of the challenges associated with managing progressive anisometropic amblyopia. We report a child with unilateral PHPV who underwent cataract extraction, primary posterior capsulotomy with anterior vitrectomy and intraocular lens implantation followed by combined trabeculectomy/trabeculotomy within the first 8 weeks of life. Intensive optometric and orthoptic input was required for many years to manage the increasing anisometropic amblyopia with final visual acuity of 20/40 unaided in the affected eye and without evidence of glaucomatous optic neuropathy. This case illustrates the excellent visual outcome possible in a child with complex, unilateral PHPV using an intensive management approach comprising: early surgical intervention for congenital cataract...
International Ophthalmology Clinics, 2008
Persistent hyperplastic primary vitreous (PHPV) has been called by various names, such as persistent tunica vasculosa lentis, persistent posterior fetal fibrovascular sheath of the lens, congenital retinal septum, and ablation falciformis congential. 1 It was first reported by Jules Cloquet in 1818 in Mémoire sur la membrane pupillae, et sur la formation du petit cercle artériel de l'iris. 2 In 1949, Reese defined this entity histopathologically. In 1955, Reese coined the term persistent hyperplastic primary vitreous (PHPV) 1,3,4 a congenital anomaly in which the embryonic hyaloid vasculature fails to regress normally. 4,5 In 1997, Goldberg 1 renamed this entity as persistent fetal vasculature (PFV) 3 as to encompass the complete spectrum of the disease. PHPV has been classified into 3 types 3,6-8 : Anterior: presence of retrolental opacity, elongated ciliary processes, or cataract. Posterior: presence of one or more of the following features: elevated vitreous membrane or stalk from the optic nerve, a retinal fold or retinal dysplasia, retinal detachment, or optic nerve hypoplasia. Combined (both anterior and posterior) depending on which ocular structures are involved.
The development of the fetal eye:In utero ultrasonographic measurements of the vitreous and lens
Prenatal Diagnosis, 1995
Our objective was to establish nomograms for fetal eye measurements from 12 weeks' gestation by using transvaginal and transabdominal high-resolution ultrasound techniques. A prospective cross-sectional study was performed on 450 normal singleton pregnancies between 12 and 37 weeks' gestation. Vitreous and lens circumferences were measured by transvaginal ultrasonography until 17 weeks, and by abdominal ultrasound between 18 and 37 weeks' gestation. Regression analyses were used to create nomograms, and several transformations were done to obtain linearity. Eye measurements of 12 fetuses at risk for ocular disturbances were plotted on the constructed nomograms. Linear relationships were fitted between vitreous (?=0.79) and lens (?=0-88) circumferences and gestational age. In addition, there was a sigdicant correlation between these measurements and the biparietal diameter. Data of the fetuses at risk showed that disturbances in ocular growth were associated mainly with abnormal cerebral development. These normative data may be helpful in the prenatal diagnosis of suspected congenital syndromes that include, among their manifestations, ocular growth disturbances such as microphthalmos and anophthalmos.
Pseudo-hyaloidal Stalk in Anterior Persistent Fetal Vasculature: A Report of Two Cases
Turkish Journal of Ophthalmology, 2021
Persistent fetal vasculature (PFV) syndrome is characterized by abnormal regression of the fetal hyaloid system and may occur in various forms. In this report, two atypical cases associated with posterior capsular defect and ectopic lens material located along Cloquet's canal are discussed. Ultrasonography of these patients presenting with bilateral total cataracts revealed a hyaloidal stalk extending from the optic nerve head to the retrolental area. During lensectomy, it was observed that lens particles were moving anteriorly from the central mid-vitreous to the aspiration port and that the posterior capsule was developmentally defective. There was no pathological vascular remnant, rather the lens material partially filled Cloquet's canal through the opening in the posterior capsule and created a pseudo-stalk appearance on the preoperative ultrasonography. We aim to discuss possible mechanisms underlying these cases, which may help to improve our understanding of the PFV spectrum.