High-frequency ultrasonography findings in persistent hyperplastic primary vitreous (original) (raw)
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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.
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.
Significance of Ultrasonography in Evaluation of Vitreo-retinal Pathologies
Medical Archives, 2012
P urpose: To assess the diagnostic value of ultrasonography in the detection of vitreo-retinal pathologies. Material and Methods: This prospective study included all patients from retina department in Eye Clinic University Clinical Center Tuzla first time referred and examined by a single experienced vitreo-retinal surgeon in period from January 2011. to January 2012. Complete ophthalmic examination and ultrasonography-standardized echography were performed to all patients for detection and confirmation of vitreo-retinal pathology. Results: Study included 146 eyes from 122 patients. 58 (39,7%) eyes had poor posterior segment visualization, 21 (14,4%) eyes due to dense cataracts and 37 (25,3%) eyes due to different vitreous opacities. 88 (60,3%) eyes had good or partial posterior eye segment visualization, where 67 (45,9%) eyes had proliferative vitreo-retinopathy and 55 (37,7%) eyes had tractional retinal detachment. Most common causes for referral to vitreo-retinal surgeon were ocular complications of diabetes mellitus 94 (64,4%), ocular trauma 18 (12,3%), rhegmatogenous retinal detachment 16 (10,9%) and intraocular inflammation 9 (6,2%). Ultrasonography confirmed suspected diagnosis and operative management plan in 120 (82,2%) eyes, sublassified previously established diagnosis in 19 (13%) eyes and helped further operative planning and established completely new diagnosis and changed initial management plan in 7 (4,8%) eyes. The final clinical diagnosis confirmed the ultrasonography findings in 142 from 146 (97,3%) eyes. Conclusion: Ultrasonography is useful part of ophthalmic examination for detection, evaluation and follow up of different vitreo-retinal pathologies.
Unilateral persistent hyperplastic primary vitreous: Course and outcome
Journal of American Association for Pediatric Ophthalmology and Strabismus, 2002
Purpose: Improved surgical techniques enable more favorable results in the management of persistent hyperplastic primary vitreous (PHPV). The purpose of our study was to evaluate the outcome of PHPV eyes managed conservatively or after surgery (with or without intraocular lens implantation). Methods: A total of 89 children (37 boys, 52 girls) with PHPV in one eye (mean follow-up of 6.3 Ϯ 5.7 years) were included. The children were subgrouped according to treatment modality. Twenty-eight (31.5%) children were managed conservatively (nonoperated). Cataract extraction combined with vitrectomy and removal of embryonic remnants was carried out in 61 eyes (68.5%). Intraocular lenses were implanted in 30 of the operated eyes (pseudophakic) and 31 eyes remained without lens (aphakic). Results: Final evaluated visual acuity in the entire group was 6/15 or better in 12.6% (11 of 87) of the eyes. A total of 11.5% (10 of 87) had a visual acuity of 6/21 to 6/60, 46.0% (40 of 87) obtained 6/90 to light perception and 26 of 87 (29.9%) had no light perception in the involved eye. The rate of no light perception was significantly lower in patients with pseudophakia (10.0%) compared to those with aphakia (43.3%) or nonoperated (37.0%) eyes (P ϭ .009). Intraocular pressure was adequately assessed repeatedly in 72 eyes. High intraocular pressure and glaucomatous changes were observed in 7 of 31 (22.6%) patients with aphakia, 2 of 24 (8.3%) patients with pseudophakia and in 2 of 17 (11.8%) nonoperated eyes (P ϭ .34). Poor cosmetic outcome was seen in 12 of 31 (38.7%) children with aphakic eyes and 5 of 30 (16.7%) children with pseudophakic eyes (P ϭ .08). Prosthesis or cosmetic shells were needed for 8 of 31 patients with aphakia, for none of the patients with pseudophakia and for 2 of 28 of the nonoperated children (P ϭ .003). Conclusion: PHPV eyes have a potential for developing useful vision with favorable cosmetic outcome after surgery. Intraocular lens implantation may be a favorable and beneficial option for the management of children with unilateral PHPV. (J AAPOS 2002;6:92-9) Journal of AAPOS FIG 1. Age distribution at surgery of children with unilateral PHPV. The mean age for lensectomy was 0.9 Ϯ 1.5 years in the group with aphakia and 1.4 Ϯ 2.2 years in children with intraocular lens implantation (P ϭ .28).
Prenatal ultrasonographic diagnosis of persistent hyperplastic primary vitreous
Ultrasound in Obstetrics and Gynecology, 2008
Persistent hyperplastic primary vitreous (PHPV) is a rare developmental malformation of the eye characterized by the presence of a vascular membrane located behind the lens. We report, for the first time in the literature, the identification on ultrasound examination of bilateral cataract and thickened hyaloid artery-lens junction, leading to a diagnosis of bilateral PHPV, in a fetus at 23 weeks' gestation. Histological examination at postmortem confirmed the prenatal diagnosis of bilateral PHPV and cataract. A thorough ultrasound examination of the fetal eye in cases with cataract is recommended.
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.
Surgical results of persistent hyperplastic primary vitreous
Ophthalmology, 1999
To evaluate the surgical success of patients with persistent hyperplastic primary vitreous (PHPV) and to identify preoperative indicators of visual outcome. Design: Noncomparative case series. Methods: The diagnosis of PHPV was made in 35 eyes of 27 patients from 1982 to 1994. In each case, anterior and/or posterior PHPV findings, preoperative testing, surgical procedures, and visual outcomes were documented. Twenty-nine of 35 eyes were managed surgically. Follow-up ranged from 2 months to 12 years. Results: Of the 35 eyes, 2 (5.7%) had strictly anterior PHPV, 8 (22.9%) had strictly posterior PHPV, and 25 (71.4%) had components of both anterior and posterior disease. Initial lensectomy and vitrectomy was performed in 24 eyes (68.6%). Surgery was withheld in four eyes secondary to severity of disease with an unrecordable visual-evoked potential (VEP). Reoperation rate was 32.3% for membrane reproliferation, glaucoma, vitreous hemorrhage, retinal detachment, or strabismus. Best-corrected final visual acuity ranged from 20/60 to no light perception. Six eyes (17%) maintained Snellen visual acuity despite posterior PHPV with some degree of retinal dysplasia. Conclusions: Surgical treatment of PHPV can result in functional visual outcome despite posterior segment involvement. The degree of ocular malformation, however, will ultimately limit the amount of visual improvement.
Survey of Ophthalmology, 1978
This is a clinicopathologic study of 62 cases of persistent hyperplastic primary vitreous (PHPV). The cases were divided into two main groups. Group 1 consisted of 55 unilateral cases not associated with any systemic abnormalities, including 36 eyes (58%) which were considered "pure cases" (Group 1A) and 19 (31%) which disclosed other ocular abnormalities in addition to PHPV (Group 1B). Group 2 consisted of 7 (11%) bilateral cases of PHPV accompanied by other ocular and systemic malformations. The most common presenting clinical signs are leukocoria, microphthalmia and cataract. The main histopathologic features of this condition are outlined, including those responsible for the disastrous results to the eye (retinal detachment, glaucoma, phthisis bulbi). Several clinical entities, usually mistaken for or associated with PHPV, such as retinoblastoma, congenital cataract, retinal dysplasia, trisomy 13 syndrome, and falciform retinal folds are discussed briefly.