Extended report outcomes in persistent hyperplastic primary. During development, this fetal vasculature nourishes the developing lens and vitreous. Phpv is a developmental abnormality of the eye which occurs in the puppy. The imaging features of persistent hyperplastic primary vitreous phpv affecting the posterior eye are well known. Three cases of persistent hyperplastic primary vitreous phpv showing a ruptured lens capsule and a phacoanaphylactoid reaction are presented. Vh is composed of cells and protein exudation and results in decrease visual acuity. Phpv is unilateral in more than 80% of cases, and bilateral in about 20%. We are moving in the same direction and will complete all the test as you suggested. 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. Persistent hyperplastic primary vitreous radiology case. Persistent hyperplastic primary vitreous hereditary. Persistent hyperplastic primary vitreous of the eye.
Reese a b 1955 persistent hyperplastic primary vitreous. Posterior hyperplastic primary vitreous american journal of. Persistent hyperplastic primary vitreous versus retinal. What causes recurrent bilateral persistent hyperplastic. It typically presents unilaterally, without associated. Visual outcomes following lensectomy and vitrectomy for. Persistent hyperplastic primary vitreous phpv is a vitreoretinal malformation characterized by defects in the primary vitreous regression1.
Variation in persistent hyperplastic primary vitreous. 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. The study of persistent primary vitreous has been mentioned in research publications which can be found using our bioinformatics tool below. Persistent hyperplastic primary vitreous phpv is a congenital anomaly that usually debuts during the first weeks after birth. 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. 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. Extended report outcomes in persistent hyperplastic. Coats disease and persistent hyperplastic primary vitreous. It is divided into anterior and posterior types and is characterized by the presence of a vascular membrane located behind the lens. The clinical presentation and ultrasound features are most consistent of bilateral persistent hyperplastic primary vitreous, more conspicuous on t.
Persistent hyperplastic primary vitreous phpv, a potentially blinding childhood disease, is one of the most common congenital malformation syndromes of the eye. Children with phpv are born with a hazy, scarred vitreous. We present the clinical and imaging findings of this unusual entity and discuss the therapeutic options available for its management. Diseases of the vitreous are uncommon but, as in the case of persistent hyperplastic vitreous phpv. Persistent hyperplastic primary vitreous dr balaji anvekar. It typically presents unilaterally, without associated systemic findings in normal fullterm infants. 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. Persistent hyperplastic primary vitreous phpv causes considerable visual morbidity in affected eyes. Persistent hyperplastic primary vitreous with glaucoma presenting in infancy. 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. Persistent hyperplastic primary vitreous was encountered in 32 children over the past 10 years. Outcome of hyperplastic persistent pupillary membrane. Most important differential diagnosis is retinoblastoma, which can be differentiated by imaging features.
Persistent hyperplastic primary vitreous phpv, a developmental cause of leukocoria, is due to incomplete regression of the fetal blood supply to the eye. 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. Get a printable copy pdf file of the complete article 15m, or click on a page. 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. Pathologically it is a condition in which the embryonic hyaloid artery fail to regress. Persistent hyperplastic primary vitreous in association. View full size version of persistent hyperplastic primary vitreous. It occurs more commonly in children and has a clear male predominance. Persistent hyperplastic primary vitreous phpv springerlink. The space at the back of the eye, behind the lens, is normally filled with a clear jelly. Dec 08, 2014 patients with uveitis may present with vitreous haze vh resulting in obscuration of fundus details. While the great majority of patients with persistent hyperplastic primary vitreous never obtain useful vision, it is encouraging that 18.
Disease bioinformatics research of persistent primary vitreous has been linked to fracture, atrial fibrillation, fractures, compression, cardiac fibrillation, pain. The vitreous is the jelly like material that fills the large central cavity of the eye. 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. Neogeninloss in neural crest cells results in persistent. One congenital ocular disease which could be misdiagnosed as rd is persistent hyperplastic primary vitreous phpv, which itself can be associated with rd. 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 wikipedia. The most important factor in the prognosis of patients with persistent hyperplastic primary vitreous is the extent of the membrane. Abstract persistent hyperplastic primary vitreous phpv, also known as persistent fetal vasculature, is a rare congenital developmental. Posterior vitreous detachment maine family eye care. Rae joy the doberman and the staffordshire bull terrier are considered to be breeds that may be vulnerable to this condition. Patients may also have poor vision, small eye microphthalmia and strabismus.
Persistent hyperplastic primary vitreous concept id. Here we present a case of bilateral persistent hyperplastic primary vitreous phpv which is a rare entity, in a 2. Grading scale of vitreous haze in patients with uveitis using. Persistent hyperplastic primary vitreous phpv is a unilateral disorder in a microphthalmic eye, seen in fullterm infants. The hazy vitreous blocks light passing to the back of the eye.
International journal of retina and vitreous articles. A rare case of binocular persistent hyperplastic primary. Aug 30, 2006 coats disease is a nonhereditary ocular disease, with no systemic manifestation, first described by coats in 1908. 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. Clinicalhistopathological correlation in a case of coats. Ophthalmic doppler in persistent hyperplastic primary.
Hyperplastic polyp definition of hyperplastic polyp by. Which one of the following statements, concerning persistent hyperplastic primary vitreous phpv is true. Computed tomography in the evaluation of patients with persistent hyperplastic primary vitreous phpv. The most important differential diagnosis is unilateral retinoblastoma, which occurs in the same age group and has. 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. View the article pdf and any associated supplements and figures for a period of 48 hours.
Highfrequency ultrasonography findings in persistent. Vascular anastomoses between the iris and persistent. Persistent hyperplastic primary vitreous phpv must be considered in the differential diagnosis of leukocoria. 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. Phacoanaphylactoid reaction in persistent hyperplastic primary. It is the second most common cause of acquired cataract. Treatment of persistent hyperplastic primary vitreous. 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.
Phpv pathology usually occurs unilaterally, but a rare bilateral condition has also been described goldberg, 1997. This study aimed to determine the relationship between image quality and measurement repeatability of optical coherence tomography angiography octa parameters in patients with nonproliferative diabetic reti. 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. Persistent fetal vasculature pfv, also known as persistent hyperplastic primary vitreous phpv is a constellation of ophthalmic findings typically consisting of microphthalmia, cataract figs.
Discussion persistent hyperplastic primary vitreous was first described by reese in 1955 in his jackson memorial lecture. Find out information about primary persistent hyperplastic vitreous. Phpv persistent hyperplastic primary vitreous courtesy of animal info publications author. 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. Persistent hyperplastic primary vitreous with glaucoma. Clinically, this condition usually manifests as unilateral or bilateral leucocoria. The anterior and posterior hyaloid vascular systems regress independently. Pdf ophthalmic doppler in persistent hyperplastic primary. Persistent hyperplastic primary vitreous involving the. The degree of vh is an important indicator of disease activity, severity and response to therapy in patients with uveitis.
Diagnosis, treatment and results article pdf available in transactions of the american ophthalmological society 95. Persistent hypertrophic primary vitreous radiology. Persistent hyperplastic primary vitreous an overview. This condition arises from failure of the hyaloid vasculature to undergo normal programmed involution. 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. 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. To report outcomes in cases of persistent hyperplastic primary vitreous phpv and examine factors predictive of visual acuity in patients selected for surgery. Typically, there is a retrolental fibrovascular mass 3 caused by persistence of the primary vitreous that normally should regress 4. Primary persistent hyperplastic vitreous article about. Persistent fetal vasculature in phpv can lead to fibrosis, resulting in elongation of the ciliary processes, retinal detachment, and spontaneous cataracts. Persistent hyperplastic primary vitreous sciencedirect.
American journal of ophthalmology 40 3, 317331 pubmed. 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. Persistent hyperplastic primary vitreous phpv is an uncommon condition, presenting clinically as leukokoria white pupillary reflex, microophthalmia, and cataract. Persistent hyperplastic vitreous definition of persistent. The successful surgical excision of persistent primary hyperplastic vitreous in two patients is described. Persistent hyperplastic primary vitreous radiology.
Outcomes in persistent hyperplastic primary vitreous article pdf available in british journal of ophthalmology 897. The effect of image quality on the reliability of oct angiography measurements in patients with diabetes. 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. We recently encountered a patient with the anterior variant of phpv who had mr imaging of the orbits. Surgical excision of persistent hyperplastic primary vitreous. Pdf outcomes in persistent hyperplastic primary vitreous. Previous surgical series have documented variable visual and anatomical outcomes even with highly selected cases.
Case report prepapillary vascular loop associated with. Persistent hyperplastic primary vitreous findings in an. Bilateral persistent hyperplastic primary vitreous. 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.
Retrospective case series of 55 eyes in 50 patients presenting between 1990 and 2001 at the childrens hospital, westmead. In this variant of persistent hyperplastic primary vitreous, retrolental mass was absent and use of color doppler was diagnostic. Phpv is a congenital condition with persistent hyaloid vasculature and mesenchymal tissue from the embryonic primary vitreous in a microphthalmic eye. Unilateral persistent hyperplastic primary vitreous. Diseases of the vitreous are uncommon but, as in the case of persistent hyperplastic vitreous phpv described here, can have severe vision consequences. Persistent hyperplastic primary vitreous phpv is a common congenital developmental anomaly of the eye that results following failure of the embryological. Persistent hyperplastic primary vitreous request pdf. 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, 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. If left untreated, most eyes usually develop spontaneous hemorrhage and secondary.
Persistent hyperplastic primary vitreous canine animal. Highfrequency ultrasonography findings in persistent hyperplastic primary vitreous. Achiron the ultrasound unit, department of obstetrics and gynecology and the goldschleger eye institute, the chaim sheba medical center. Petra meier, peter wiedemann, in retina fifth edition, 20. 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 phpv, also known as the persistent fetal vasculature, refers to a rare congenital developmental malformation of the eye. 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. To evaluate the longterm visual outcome of eyes with hyperplastic persistent pupillary membrane. George coats 9 made the first detailed description of this entity in 1908. Emphasis is placed on early operative intervention in order to preserve the globe, as well as. Formation of persistent hyperplastic primary vitreous in. 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.
Most cases are unilateral and sporadic, with no differences of incidence between males and females. Pdf persistent hyperplastic primary vitreous phpv is a pathologic entity resulting from the abnormal persistence of the fetal fibrovascular. The primary vitreous lies between the lens and the retina. Persistent hyperplastic primary vitreous springerlink. 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. Phpv rarely is bilateral in patients with norries disease, warburg syndrome, or patients with retinal dysplasia. 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. Most patients present clinically with unilateral decreased vision, strabismus or leukocoria. What is persistent hyperplastic primary vitreous phpv. Persistent hyperplastic primary vitreous phpv emconsulte.
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. Persistent hyperplastic primary vitreous article in nippon ganka gakkai zasshi 10510. 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. We discuss nine cases of persistent hyperplastic primary vitreous seen recently, including mode of presentation, management and postsurgical followup. Persistent hyperplastic primary vitreous in an adult.
Persistent hyperplastic primary vitreous in transgenic mice. 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. Clinically, there is a white pupil, elongated ciliary processes, and often microphthalmia. 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. Ophthalmology mcq 11 persistent hyperplastic primary vitreous. Persistent hyperplastic primary vitreous phpv is thought to be a congenital anomaly in which the normal regression of the primary vitreous body and hyaloid. Outcomes in persistent hyperplastic primary vitreous. Prenatal ultrasonographic diagnosis of persistent hyperplastic primary vitreous e. Phpv is a developmental abnormality of the eye which occurs in the puppy embryo at about 45 weeks of age after. The primary vitreous forms around the seventh week of life and begins involuting. Bilateral persistent hyperplastic primary vitreous europe. Pdf unilateral persistent hyperplastic primary vitreous. These vessels represented early developmental arrest and may have been associated with abnormal development of the macula and optic nerve.
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