The most important factor in the prognosis of patients with persistent hyperplastic primary vitreous is the extent of the membrane. Persistent hyperplastic primary vitreous in transgenic mice. Persistent hyperplastic primary vitreous phpv causes considerable visual morbidity in affected eyes. The most important differential diagnosis is unilateral retinoblastoma, which occurs in the same age group and has. Pathologically it is a condition in which the embryonic hyaloid artery fail to regress. Persistent hyperplastic primary vitreous sciencedirect. Pdf unilateral persistent hyperplastic primary vitreous. The anterior and posterior hyaloid vascular systems regress independently. Persistent hyperplastic primary vitreous canine animal. Persistent hyperplastic primary vitreous phpv is a congenital anomaly that usually debuts during the first weeks after birth. American journal of ophthalmology 40 3, 317331 pubmed. Pdf persistent hyperplastic primary vitreous phpv is a pathologic entity resulting from the abnormal persistence of the fetal fibrovascular. It is divided into anterior and posterior types and is characterized by the presence of a vascular membrane located behind the lens. Extended report outcomes in persistent hyperplastic.
If left untreated, most eyes usually develop spontaneous hemorrhage and secondary. Most patients present clinically with unilateral decreased vision, strabismus or leukocoria. It occurs more commonly in children and has a clear male predominance. It is the second most common cause of acquired cataract. One congenital ocular disease which could be misdiagnosed as rd is persistent hyperplastic primary vitreous phpv, which itself can be associated with rd. Unilateral persistent hyperplastic primary vitreous. It typically presents unilaterally, without associated. Persistent hyperplastic primary vitreous phpv is a rare benign developmental disorder of the globe in which the embryonic hyaloid artery fails to regress normally, resulting in abnormal.
The effect of image quality on the reliability of oct angiography measurements in patients with diabetes. To evaluate the longterm visual outcome of eyes with hyperplastic persistent pupillary membrane. Emphasis is placed on early operative intervention in order to preserve the globe, as well as. Persistent fetal vasculature pfv, also known as persistent hyperplastic primary vitreous phpv is a constellation of ophthalmic findings typically consisting of microphthalmia, cataract figs.
Persistent hyperplastic primary vitreous findings in an. Diseases of the vitreous are uncommon but, as in the case of persistent hyperplastic vitreous phpv described here, can have severe vision consequences. Highfrequency ultrasonography findings in persistent hyperplastic primary vitreous. Ophthalmic doppler in persistent hyperplastic primary. These vessels represented early developmental arrest and may have been associated with abnormal development of the macula and optic nerve. Persistent hyperplastic primary vitreous phpv is a common congenital developmental anomaly of the eye that results following failure of the embryological. Three cases of persistent hyperplastic primary vitreous phpv showing a ruptured lens capsule and a phacoanaphylactoid reaction are presented. Persistent hyperplastic primary vitreous radiology case. Typically, there is a retrolental fibrovascular mass 3 caused by persistence of the primary vitreous that normally should regress 4. What causes recurrent bilateral persistent hyperplastic.
Persistent hyperplastic primary vitreous dr balaji anvekar. Vh is composed of cells and protein exudation and results in decrease visual acuity. Mri scanning features of retinal detachment and associated causes can assist in differentiating both entities effectively, allowing the radiologist to reach the proper conclusions with. To report outcomes in cases of persistent hyperplastic primary vitreous phpv and examine factors predictive of visual acuity in patients selected for surgery.
The hazy vitreous blocks light passing to the back of the eye. Embryologically the primary vitreous forms around 7th week of gestational life and starts regressing at around 2021 weeks of gestational life and is completely regressed at the time of birth. Formation of persistent hyperplastic primary vitreous in. Persistent hyperplastic vitreous definition of persistent. Discussion persistent hyperplastic primary vitreous was first described by reese in 1955 in his jackson memorial lecture. The vitreous is the jelly like material that fills the large central cavity of the eye. Phpv pathology usually occurs unilaterally, but a rare bilateral condition has also been described goldberg, 1997. Persistent hyperplastic primary vitreous phpv must be considered in the differential diagnosis of leukocoria. During development, this fetal vasculature nourishes the developing lens and vitreous. It happens due to a failure in the regression of foetal hyaloid vasculature and primary vitreous, which remains inside the hyaloid canal in the vitreous chamber leading to fibrosis and several abnormalities 1. Phacoanaphylactoid reaction in persistent hyperplastic primary. Posterior vitreous detachment a posterior vitreous detachment pvd is a rather dramatic event in the normal aging process of the human eye, usually occurring during the sixth and seventh decade of life.
Ophthalmology mcq 11 persistent hyperplastic primary vitreous. Persistent hyperplastic primary vitreous in association. Petra meier, peter wiedemann, in retina fifth edition, 20. Get a printable copy pdf file of the complete article 15m, or click on a page. Hyperplastic polyp definition of hyperplastic polyp by.
Prenatal ultrasonographic diagnosis of persistent hyperplastic primary vitreous e. Persistent hyperplastic primary vitreous is a rare benign developmental disorder of the globe in which the embryonic hyaloid artery fails to regress normally, resulting in abnormal lenticular development and secondary changes of the retina and globe. Persistent hyperplastic primary vitreous request pdf. Persistent hyperplastic primary vitreous versus retinal. Highfrequency ultrasonography findings in persistent.
Persistent hyperplastic primary vitreous phpv, also known as persistent hyperplastic tunica vasculosa lentis phtvl or phpvphtvl, is a condition of the vitreous in which the ophthalmic embryologic vascular structures do not regress normally. What is persistent hyperplastic primary vitreous phpv. Previous surgical series have documented variable visual and anatomical outcomes even with highly selected cases. Persistent hyperplastic primary vitreous with glaucoma presenting in infancy. International journal of retina and vitreous articles. Persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a congenital anomaly of the eye that results from failure of the embryological, primary vitreous, and the hyaloid vasculature to regress. Case report prepapillary vascular loop associated with persistent hyperplastic primary vitreous shinjimakino,yukoohkubo,andhironobutampo department of ophthalmology, jichi medical university, shimotsuke, yakushiji, tochigi, japan correspondence should be addressed to shinji makino.
Persistent hyperplastic primary vitreous in an adult. Pdf while the great majority of patients with persistent hyperplastic primary vitreous never obtain useful vision, it is encouraging that 18. Grading scale of vitreous haze in patients with uveitis using. The clinical presentation and ultrasound features are most consistent of bilateral persistent hyperplastic primary vitreous, more conspicuous on t. Diagnosis, treatment and results article pdf available in transactions of the american ophthalmological society 95. We discuss nine cases of persistent hyperplastic primary vitreous seen recently, including mode of presentation, management and postsurgical followup. Children with phpv are born with a hazy, scarred vitreous. It contains the hyaloid vessels and fibrillar ectodermal tissues, then gradually disappears in the fifth to sixth month of gestation and is replaced by the secondary vitreous, which is the final vitreous. In the normal situation the primary vitreous forms around the 7 th week of gestation life and starts involuting around 20 th week and nearly always disappears at the time of birth.
Persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a congenital anomaly in which the normal regression of the primary vitreous and hyaloid vasculature does not occur. Persistent fetal vasculature, previously known as persistent hyperplastic primary vitreous, is a spectrum of disease and can present with no clinical effects or with severe morbidity. Families should be told that if their child has only an anterior presentation, then surgical and amblyopia therapies offer a good chance for useful vision. Persistent hyperplastic primary vitreous hereditary.
Failed regression of the primary vitreous has severe consequences, the most prominent resulting in the eye disease persistent hyperplastic primary vitreous phpv, more recently named as persistent fetal vasculature pfv. Persistent hyperplastic primary vitreous article in nippon ganka gakkai zasshi 10510. Persistent hyperplastic primary vitreous phpv springerlink. Posterior vitreous detachment maine family eye care. Phpv is unilateral in more than 80% of cases, and bilateral in about 20%. The posterior chamber of the eye contains the retina the tissue that responds to light and is filled with a transparent jellylike substance called the vitreous humor. Posterior hyperplastic primary vitreous american journal of. Computed tomography in the evaluation of patients with persistent hyperplastic primary vitreous phpv. Persistent hyperplastic primary vitreous phpv is a rare congenital developmental malformation of the eye that commonly manifests in a fullterm infant with leukocoria and microphthalmos 1,2. Persistent hyperplastic primary vitreous phpv is a vitreoretinal malformation characterized by defects in the primary vitreous regression1.
Stades f c 1980 persistent hyperplastic tunica vasculosa lentis and persistent hyperplastic primary vitreous phtvlphpv in 90 closely related doberman pinschers. Persistent hyperplastic primary vitreous phpv emconsulte. We present the clinical and imaging findings of this unusual entity and discuss the therapeutic options available for its management. Persistent hyperplastic primary vitreous was encountered in 32 children over the past 10 years. Bilateral persistent hyperplastic primary vitreous europe. We recently encountered a patient with the anterior variant of phpv who had mr imaging of the orbits. Outcomes in persistent hyperplastic primary vitreous. Phpv persistent hyperplastic primary vitreous courtesy of animal info publications author. Infants, eyes and orbits persistent hyperplastic primary vitreous phpv is a pathologic entity resulting from abnormal hypertrophy of the fetal fibrovascular primitive stroma hyaloid system of the eye 1.
Surgical excision of persistent hyperplastic primary vitreous. Persistent hyperplastic primary vitreous phpv is a unilateral disorder in a microphthalmic eye, seen in fullterm infants. Persistent hyperplastic primary vitreous phpv is also referred to as persistent fetal vasculature pfv or persistent fetal vasculature syndrome pfvs, omim 618 autosomal dominant phpv or omim 6111 autosomal recessive phpv. Rae joy the doberman and the staffordshire bull terrier are considered to be breeds that may be vulnerable to this condition. View full size version of persistent hyperplastic primary vitreous.
The successful surgical excision of persistent primary hyperplastic vitreous in two patients is described. Diseases of the vitreous are uncommon but, as in the case of persistent hyperplastic vitreous phpv. Treatment of persistent hyperplastic primary vitreous. View the article pdf and any associated supplements and figures for a period of 48 hours. Here we present a case of bilateral persistent hyperplastic primary vitreous phpv which is a rare entity, in a 2. Phpv is a developmental abnormality of the eye which occurs in the puppy. Persistent hyperplastic primary vitreous springerlink. Persistent hyperplastic primary vitreous an overview. The study of persistent primary vitreous has been mentioned in research publications which can be found using our bioinformatics tool below. Dec 01, 2009 persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a rare congenital developmental malformation of the eye, caused by the failure of regression of the primary vitreous. Case report prepapillary vascular loop associated with. Persistent hyperplastic primary vitreous phpv, a potentially blinding childhood disease, is one of the most common congenital malformation syndromes of the eye. Persistent hyperplastic primary vitreous with glaucoma.
Often it is only one of a group of ocular anomalies which appear to result from a posterior form of persistent or hy perplastic primary vitreous. Coats disease and persistent hyperplastic primary vitreous. Persistent hyperplastic primary vitreous phpv is a congenital developmental anomaly of the eye caused by the failure of regression of primary vitreous with the abnormal persistence of hyaloid vasculature. Phpv rarely is bilateral in patients with norries disease, warburg syndrome, or patients with retinal dysplasia.
Persistent hyperplastic primary vitreous concept id. The primary vitreous lies between the lens and the retina. We report the mri features of phpv of the dorsal type to facilitate differential diagnosis from other causes of leukocoria, namely retinoblastoma, which may have major therapeutic consequences. Persistent hyperplastic primary vitreous phpv is thought to be a congenital anomaly in which the normal regression of the primary vitreous body and hyaloid. This condition arises from failure of the hyaloid vasculature to undergo normal programmed involution.
Persistent hyperplastic primary vitreous phpv, a developmental cause of leukocoria, is due to incomplete regression of the fetal blood supply to the eye. Persistent hyperplastic primary vitreous of the eye. Persistent hyperplastic primary vitreous radiology. Primary persistent hyperplastic vitreous article about.
Reese a b 1955 persistent hyperplastic primary vitreous. Coats disease primary retinal telangiectasis is an idiopathic disorder characterized by telangiectatic and aneurysmal retinal vessels with progressive deposition of intraretinal and subretinal exudates that leads to massive exudative retinal detachment. Pdf outcomes in persistent hyperplastic primary vitreous. Visual outcomes following lensectomy and vitrectomy for. Abstract persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a rare congenital developmental. Congenital, due to incomplete regression of fetal vasculature of vitreous humor, producing a retrolental mass usually unilateral associated with small eye, white reflex behind pupil leukokoria, fibrous tissue behind lens, often cataract. Most important differential diagnosis is retinoblastoma, which can be differentiated by imaging features.
Extended report outcomes in persistent hyperplastic primary. Persistent hypertrophic primary vitreous radiology. Persistent hyperplastic primary vitreous involving the. Phpv is a developmental abnormality of the eye which occurs in the puppy embryo at about 45 weeks of age after. Detection of these abnormal iris vessels suggested the presence of persistent hyperplastic primary vitreous and related syndromes even when the diagnosis was obscured by the presence of an opaque lens. Clinically, this condition usually manifests as unilateral or bilateral leucocoria.
Variation in persistent hyperplastic primary vitreous. Phpv is a congenital condition with persistent hyaloid vasculature and mesenchymal tissue from the embryonic primary vitreous in a microphthalmic eye. It typically presents unilaterally, without associated systemic findings in normal fullterm infants. The primary vitreous forms around the seventh week of life and begins involuting. The space at the back of the eye, behind the lens, is normally filled with a clear jelly. Persistent hyperplastic primary vitreous phpv, also known as the persistent fetal vasculature, refers to a rare congenital developmental malformation of the eye. Dec 08, 2014 patients with uveitis may present with vitreous haze vh resulting in obscuration of fundus details. Aug 30, 2006 coats disease is a nonhereditary ocular disease, with no systemic manifestation, first described by coats in 1908. George coats 9 made the first detailed description of this entity in 1908. Persistent hyperplastic primary vitreous wikipedia. In 1955 reese 1 characterized persistent hyperplastic primary vitreous phpv as a congenital condition that leads to enucleation in infancy or childhood because a retinoblastoma is suspected or because complications intrinsic to phpv, such as intraocular hemorrhage, secondary glaucoma, or corneal opacification, have occurred. Most cases are unilateral and sporadic, with no differences of incidence between males and females.
Disease bioinformatics research of persistent primary vitreous has been linked to fracture, atrial fibrillation, fractures, compression, cardiac fibrillation, pain. Bilateral persistent hyperplastic primary vitreous. The imaging features of persistent hyperplastic primary vitreous phpv affecting the posterior eye are well known. We are moving in the same direction and will complete all the test as you suggested. Persistent hyperplastic primary vitreous phpv definition. Persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature pfv, is a rare congenital developmental anomaly of the eye that results following failure of the embryological, primary vitreous and hyaloid vasculature to regress. Clinically, there is a white pupil, elongated ciliary processes, and often microphthalmia. Persistent hyperplastic primary vitreous phpv is an uncommon condition, presenting clinically as leukokoria white pupillary reflex, microophthalmia, and cataract. Mar 16, 2014 in the normal situation the primary vitreous forms around 7 th week of gestation life and starts involuting around 20 th week and nearly always disappears at the time of birth. Persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a rare congenital developmental malformation of the eye, caused by the failure of regression of the primary vitreous. Clinicalhistopathological correlation in a case of coats. This disorder is complicated by anterior and posterior chamber anomalies and is clinically characterized by leukocoria, shallow anterior chamber, cataracts, angleclosure glaucoma, elongated ciliary processes.
Retrospective case series of 55 eyes in 50 patients presenting between 1990 and 2001 at the childrens hospital, westmead. Persistent hyperplastic primary vitreous phpv, also termed persistent fetal vasculature, is a developmental malformation of the eye in which the primary vitreous fails to regress in utero, resulting in the presence of a retrolental fibrovascular membrane with persistence of the posterior portion of the tunica vasculosa lentis and hyaloid artery. Outcome of hyperplastic persistent pupillary membrane. While the great majority of patients with persistent hyperplastic primary vitreous never obtain useful vision, it is encouraging that 18.
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